Semantic dementia, also called semantic variant primary progressive aphasia, is a type of frontotemporal dementia.
Others of frontotemporal dementia include frontotemporal dementia with behavioral variant and primary progressive nonfluid aphasia.
Left temporal atrophy (arrows) in a patient with semantic dementia
The pattern of disturbances in this form of dementia is different from that seen in more common dementias, such as Alzheimer’s disease, in which disturbances in recent memory, for example, are often an early symptom.
In contrast, in semantic dementia, recent daily memories and visuospatial function are relatively well preserved.
This means that people with this form of dementia are often able to recall recent events and can walk around their locality on their own without getting lost.
There are, however, significant changes in communication skills, personality, and behavior in semantic dementia.
The loss of semantic knowledge leads to difficulty finding words in conversation, including the names of people, places, and objects, as well as significant difficulty understanding the speech of others.
Semantic loss extends beyond language skills to affect nonverbal understanding of the world.
This leads to problems recognizing and understanding words, objects, faces, sounds, smells, touch and tastes, and causes challenges with activities of daily living.
Thus, sufferers may not be able to demonstrate how to use common items, such as a hammer or a comb.
Personality and behavioral changes are also common in this dementia, with behavioral characteristics having much in common with the behavioral variant of frontotemporal dementia in the later stages of the disease.
Mixed dementia is a disease in which abnormalities characteristic of more than one type of dementia occur simultaneously.
Doctors may also call mixed dementia « dementia – multifactorial. »
In the most common form of mixed dementia, the abnormal protein deposits associated with Alzheimer’s disease (amyloid proteins) coexist with blood vessel problems related to vascular dementia. Alzheimer’s brain changes often coexist with the Lewy body. In some cases, a person may have brain changes related to all three forms of dementia: Alzheimer’s disease, vascular dementia, and Lewy body dementia.
Researchers don’t know exactly how many older people currently diagnosed with a specific type of dementia actually have mixed dementia, but autopsies show the condition may be much more common than previously realized.
Autopsy studies play a key role in shedding light on mixed dementia because scientists cannot yet measure most dementia-related brain changes in living individuals.
In the most informative studies, researchers correlate any problem diagnosed during the participant’s lifetime with analysis of their brain after death.
In a study conducted in the United States, data from the first 141 volunteers showed that more than 50% of those whose brains met the criteria for Alzheimer’s disease also had at least one other type of dementia.
Symptoms of Mixed Dementia
Symptoms can vary depending on the types of brain changes involved and the brain regions affected.
In many cases, the symptoms may be similar or even indistinguishable from those of Alzheimer’s disease or another type of dementia. In other cases, a person’s symptoms may suggest the presence of more than one type of dementia.
Frontotemporal dementia is a form of dementia characterized by behavioral and language disorders associated with cognitive decline.
Clinicians distinguish between three forms of frontotemporal dementia based on the signs and symptoms that affect the individual:
Behavioral-variant frontotemporal dementia.
semantic dementia
Progressive non-fluent aphasia
Pick’s disease is also considered one of the forms of frontotemporal degeneration, manifested by changes in behavior: isolation, disinhibition, neglect of hygiene, distraction or continual repetition of the same gestures.
At first, frontotemporal dementia is mainly manifested by behavioral and language disorders that appear together or successively over time. They vary from patient to patient.
As it occurs remembered at a young age, it can be difficult to differentiate it from schizophrenia or an affective disorder.
Most often, behavioral problems predominate.
The majority of cases (about 70%) are sporadic (i.e. isolated cases with no hereditary form). There are therefore approximately 30% of familial cases of FTD which can be caused by at least four known genes (the tau and progranulin genes, and more rarely the VCP and CHMP2B genes).
Most often, the genetic anomaly seems to be transmitted autosomally (from generation to generation), which means that the patient has a risk in two of transmitting the disease to his children.
Prevalence of frontotemporal dementia
The prevalence (number of patients affected at a given time) of frontotemporal dementia varies between 3 and 15 cases per 100,000 people. It increases with age. Men seem to be more affected than women.
It is the 3rd cause of degenerative dementia. It represents 15 to 20% of the causes of dementia.
The age of onset is in the late 50s (range ages 20 to 75).
Life expectancy, from the moment of diagnosis, is on average about ten years.
Signs and Symptoms of Frontotemporal Dementia
1. Behavioral variant of frontotemporal dementia
This form affects about 60% of patients with one of the three forms of frontotemporal dementia.
The diagnostic criteria for this form are:
A. Progressive deterioration in behavior and/or cognition.
B. Three of the six behavioral/cognitive symptoms must be present. Rather, they must be persistent or recurring.
• Early disinhibition: inappropriate social behavior, loss of propriety or politeness and/or impulsive, thoughtless or even reckless acts. Disinhibition results in foul language, familiarity, or inappropriate attitudes (for example, they undress, behave badly at the table, urinate in public, etc.).
• Early apathy/inertia.
• Early loss of sympathy or empathy: diminished response to other people’s needs and feelings and/or social disinterest, impaired interpersonal relationships, emotional blunting.
• Early stereotyped or compulsive/obsessional behavior: simple repetitive movements, complex compulsive behaviors and/or verbal stereotypies: the patient repeats the same gestures, has fixed time habits, becomes fixated on certain objects or practices certain hobbies to excess such as the casino.
• Hyperorality and change in eating habits: changes in food tastes and/or gluttony, increased consumption of alcohol or cigarettes.
• Neuropsychological profile: executive function deficit and relative loss of episodic memory and visuo-spatial functions.
2. Semantic dementia
It concerns about 20% of cases and is mainly characterized by language disorders.
• Anomie-type disorders (difficulty finding words) despite preserved verbal fluency. For example, the person uses the word « bird » rather than « sparrow » or « thing, thing » rather than the appropriate word. Understanding is preserved.
• In more severe cases, the patient has problems identifying objects or animals. He can for example ask the following question: « What is a bird? » « . The person can, however, continue to manipulate numbers.
From an anatomical point of view, it is characterized by atrophy of the left temporal lobe.
3. Progressive non-fluent aphasia
It concerns about 20% of cases and is also characterized by language disorders. The person generally speaks slowly, has difficulty understanding the meaning of words, lacks verbal fluency, has great difficulty speaking on the telephone, expressing himself within a group or understanding complex sentences. Many of them develop Parkinson’s-like motor symptoms (for example, muscle rigidity in the arms and legs).
At the onset of the disease, memory impairment is minimal. Neuropsychological tests indicate that the information is well stored by the patient’s brain but that it has difficulty in being restored (for example, the patient does not retain such and such a word unless given a clue).
Fronto-temporal atrophy predominates in the left hemisphere, with a language disorder on expression, comprehension being relatively preserved.
Other Disorders
• Judgment and planning problems: patients cannot plan an activity.
• Swallowing disorders (severe stage of the disease).
Evaluation of frontotemporal dementia
The Frontal Dysfunction Scale assesses the severity of behavioral disturbances in patients with mild-stage dementia.
What brain imaging says
Unlike Alzheimer’s disease, which affects almost the entire brain, frontotemporal dementia preferentially affects the temporal lobe and the frontal lobe.
Neuroimaging techniques have made it possible to observe lesions of the frontal lobes (seat of reasoning, abstract ideas, planning, motor skills) and temporal (seat of the formation and retrieval of memories), and sometimes the lobes parietals.
In the late phase of the disease, there is significant neuronal loss in the cortex.
Magnetic resonance imaging (MRI) or CT scans are used to support a diagnosis of frontotemporal dementia.
Single photon emission computed tomography (SPECT) is an imaging technique that assesses the activity of the frontal and temporal lobes (in DFT, this activity is decreased).
These techniques do not make it possible to distinguish between the different forms of frontotemporal dementia. Detecting Pick’s bodies can only be done on post-mortem tissues (i.e. after autopsy).
Differential diagnosis
Frontotemporal dementia can be confused with many other neuropsychiatric diseases, especially early in the disease; eg depression, bipolar or obsessive-compulsive disorder), with a tumor of the frontal lobe, head trauma, certain infectious diseases, dementia associated with movement pathology or vascular dementia or chronic alcoholism.
Alzheimer’s disease is distinguished from frontotemporal dementia by early memory and orientation disorders marked from the onset of the disease.
Therapies
Medicines are prescribed in low doses at the beginning of treatment, before these are gradually increased according to possible side effects.
Antidepressants
Antidepressants are used to treat the following disorders:
• Anxiety and panic attack
• Obsessive Compulsive Disorder
• Aggressiveness
Selective serotonin reuptake inhibitors are the types of antidepressants of choice:
Antipsychotics are prescribed in low doses to reduce aggression, obsessive/compulsive disorder, irrational behavior with, however, undesirable side effects: weight gain, slowed thoughts, increased risk of heart disease. Atypical antipsychotics are the types of antipsychotics of choice: olanzepine (Zyprexa®), quetiapine (Seroquel® or Ketipinor®), risperidone (Risperdal®), ziprasidone (Geodon®), aripiprazole (Abilify®), paliperidone (Invega®). Memantine, a drug already used in moderate to severe forms of Alzheimer’s disease, would improve the memory function of patients with frontotemporal dementia.
Exercises
A training program combining aerobic (walking) and strength training is beneficial for the patient (for example the practice of cardiovascular exercises for 30 minutes 2 to 4 times a week, in the form of walking for example).
Clinical case
Mrs X, 79 years old, is being followed by a neurologist because she has mild cognitive deficits (mini mental state examination score of 26 out of 30). She was subsequently hospitalized for behavioral problems with periods of agitation and delusional disorders.
His entourage indicates that these behavioral changes appeared 4 years ago, with sometimes running away, compulsive shopping and a lack of social etiquette.
Treatment consisted of prescribing an antipsychotic (risperidone), a mood stabilizer (Valproate) and an antidepressant.
There is also a reduction in verbal fluency while episodic and visuo-spatial memories appear normal.
Neuroimaging reveals decreased activity in the frontal cortex and temporal lobe.
A diagnosis of frontotemporal dementia (degeneration) is made. His cognitive performance deteriorates with, in particular, a loss of language.
As a reminder, the symptoms of frontotemporal dementia are characterized:
In three quarters of cases by apathy, depression.
In a quarter of cases by mania, disinhibition (loss of social etiquette), hypersexuality and compulsive shopping.
Mild cognitive deficits at the onset of the disease (no memory loss).
Attention and executive function disorders.
Eating disorders (especially bulimia).
Treatment is limited to antidepressants and mood stabilizers.
Frontotemporal dementia linked to abnormal eating behavior
Patients with frontotemporal dementia have inappropriate eating habits.
While patients with the behavioral variant overeat (especially foods high in sugar) patients with semantic dementia (another form of frontotemporal dementia) exhibit very rigid eating behavior, sometimes focusing on a specific food .
Brain imaging has also suggested that these eating habits are controlled by complex neural networks rather than a particular brain structure.
Frontotemporal dementia (FTD) is the second leading cause of dementia occurring in people under the age of 65. There are several types of FTD: the behavioral variant which is manifested in particular by inappropriate behavior and an inability to plan, while semantic dementia is characterized by a loss of the meaning of words.
“It is possible that changing dietary habits may have a protective role in frontotemporal dementia. It is necessary to know if we can change these eating behaviors and if it will make a difference,” says the study’s lead author.
49 patients with three different types of dementia (19 with the behavioral variant, 15 with semantic dementia, and 15 with Alzheimer’s disease) participated in the study. Their eating habits were compared with those of 25 healthy controls.
All participants fasted overnight and then received a breakfast buffet. The researchers calculated the total amount and type of food eaten.
The results showed that all patients with the behavioral variant consumed more calories than the other participants, with an average of 1344 calories, compared to 710 calories for the Alzheimer group, 573 calories for the semantic dementia group and 603 calories for group control.
“Patients with the behavioral variant who ate the least even consumed more calories than those in the other groups who ate the most. »
In contrast, patients with semantic dementia showed rigid eating behavior, often refusing to eat the food offered.
The lead author (Dr. Ahmed) noted that sugar craving appears to be a particular feature of the behavioral variant of FTD.
It is possible that these patients have changes in their metabolism that could be the cause of the overeating.
The researchers also studied the participants’ brains (with the behavioral variant) using MRI to look for dysfunctions in specific areas of the brain. They found that overeating is associated with complex brain networks involved in reward, autonomic function, and vision, not specific brain structure.
Source: Rebekah M. Ahmed et al. Eating Disruption and Associated Eating Behavior Assessment of Neural Networks in Frontotemporal Dementia. JAMA Neurol. 2016; 73 (3): 282-290.
Patients with frontotemporal dementia have less emotion
An Australian study has shown for the first time why patients suffering from frontotemporal dementia have less emotion when faced with a memory. This explains why they feel no emotion when they remember a significant event (e.g. a wedding or a funeral). Professor Piguet, author of the study, says, “Until now, we knew that emotional memories were linked to the amygdala, a region of the brain involved in emotions. This study is the first to demonstrate the involvement of another structure, the orbitofrontal cortex, in this process. » Another researcher in the group, Fiona Kumfor, argues that these findings will help caregivers better understand why their sick loved ones may have difficulty interacting with them.
“Imagine attending your daughter’s wedding, or meeting your grandchild for the first time, but the event is as memorable as grocery shopping, says Dr. Kumfor. Source: The orbitofrontal cortex is involved in emotional enhancement of memory: evidence from the dementias. Brain, 2013.
Lewy body dementia is characterized by cognitive decline, visual and spatial disturbances, fluctuations in alertness and attention, hallucinations and motor disturbances.
Most cases of Lewy body dementia are sporadic. Some hereditary cases have been reported.
Historical
1912: the scientist Friederich Lewy observes for the first time ‘inclusions’ of about ten microns (one thousandth of a millimeter) in diameter, inside neurons. These inclusions, made up of deposits of proteins called alpha-synuclein, had previously been described in Parkinson’s disease. These inclusions are subsequently called Lewy bodies.
Lewy body (1) inside a neuron (2), stained pink with hematoxylin (a cytoplasm stain). The central part of the body of Lewy (1), of dense color, is surrounded by a halo, of pale color (3). The shape of Lewy bodies depends on their location. Thus, the Lewy bodies located in the cerebral cortex are devoid of halo, unlike those located in the brainstem.
1923: Friederich Lewy reports the existence of mental disorders in patients with Parkinson’s disease. He suggests the presence of ‘foreign bodies’ (later called Lewy bodies by Prof. Tretiakoff) inside neurons.
1961: Professor Okazaki reports the case of two patients with dementia associated with behavioral and motor disorders. The autopsy reveals the presence of Lewy bodies in the cerebral cortex.
1984: Pr Kosaka proposes the term Lewy body disease after collecting several cases of dementia patients. These patients presented with Lewy bodies in the brainstem and in certain regions of the temporal lobe related to the limbic system (anterior cingulate cortex, amygdala, and hippocampus).
1996: A group of researchers hold a conference to define diagnostic criteria for what is now called Lewy body dementia (abbreviated as LDB). LDB is therefore defined as a neurodegenerative dementia grouping the following terms: diffuse Lewy body disease, cortical Lewy body disease, Lewy body disease, senile dementia of the Lewy type, dementia with Lewy bodies, the variant with Lewy bodies of Alzheimer’s disease.
This working group revised the clinical and pathological diagnostic criteria and included new recommendations on how to better detect clinical symptoms.
Epidemiology
LDB accounts for 10% to 22% of post-autopsy dementia cases. By way of comparison, Alzheimer’s disease and vascular dementia account for 40-70% and 10-15% of dementia cases respectively. It is therefore the second leading cause of dementia.
The average age of onset is between 50 and 70 years, and affects more particularly men. A minority of MCI patients also have Parkinson’s disease. However, unlike patients with Parkinson’s, motor symptoms are usually mild and not accompanied by resting tremors. Most people with MCI have symptoms and lesions characteristic of Alzheimer’s disease.
Genetic factors
Most cases of LDB are sporadic, that is, affecting individuals irregularly. However, there are genetic mutations that can increase the risk of developing a sporadic form of the disease.
There are also family forms of the disease, explained by the mutation of genes (for example, the gene for alpha-synuclein).
Histological lesions of Lewy body dementia
Lewy bodies. These typical lesions contain proteins called alpha-synuclein and ubiquitin. These Lewy bodies are found inside neurons located in cortical (hippocampus, cingulate cortex, amygdala, basal nucleus of Meynert, hypothalamus) and subcortical (substance nigra, locus coeruleus, raphe nucleus, motor nucleus of vagus nerve) of the brain. The regions of the frontal cortex are less affected.
Lewy body (black arrow) inside a neuron. Post-mortem brain section from a patient with Lewy body dementia.
The presence of Lewy bodies in cortical regions seems to be related to the deterioration of intellectual functions.
Extensions of Lewy bodies are observed in the regions of the limbic system and the temporal lobe.
Lewy bodies and Alzheimer’s disease. A notable proportion of LDB patients present with lesions suggestive of Alzheimer’s disease: deposits of paired filaments in a helix (also called neurofibrillary tangles) and amyloid plaques. However, these filament deposits are less common in the brains of patients. According to the Braak scale, their number is between those of a healthy subject of the same age and that of an Alzheimer patient. On the other hand, the number of amyloid plaques is substantially identical in the two diseases, and appear more diffuse in LDB.
Lewy bodies and Parkinson’s disease. Lewy bodies are detected in the subcortical regions (substantia nigra and locus coeruleus) of brains of Parkinsonian patients.
Neuroimaging shows mild hippocampal atrophy in LDB compared to brains of healthy subjects. The activity of the primary visual cortex is reduced in patients with MCI.
Other types of lesions – called spongioses – appear in regions of the temporal lobe, particularly the tonsil.
Changes in neurotransmitters
The presence of these lesions in certain specific regions of the brain obviously has repercussions on the functioning of neurons.
Dementia with Lewy bodies is accompanied by a loss of neurons in the substantia nigra and the basal nucleus of Meynert, resulting in a decrease in the production of neurotransmitters called dopamine (DA) and acetylcholine (Ach), respectively.
Ach is the neurotransmitter of memory and attention, while DA is involved in motor control.
The death of neurons secreting Ach is probably responsible for cognitive disorders affecting in particular attention, concentration, visuo-spatial abilities, as well as visual hallucinations.
Motor disorders of the parkinsonian type are caused by a loss of neurons in the substantia nigra producing AD. Neuronal loss has also been described in the locus coeruleus, a region of the brain closely linked to the limbic system and synthesizing norepinephrine, an excitatory neurotransmitter. This neuronal death may be the cause of the behavioral disorders of paradoxical sleep observed in the disease.
Three main neural systems are therefore affected in LDB cholinergic (Ach), dopaminergic (DA) and noradrenergic (NA) systems.
The cholinergic system . Meynert’s basal nucleus contains neuron cell bodies (black circle) projecting to much of the cortex and the amygdala. These neurons synthesize acetylcholine, a neurotransmitter involved in memory storage and recall, motivation and attention. There is a significant loss of neurons from the basal nucleus of Meynert in DLB and Alzheimer’s disease (about two-thirds loss). The other acetylcholine-secreting nucleus is the septum (blue circle), whose neurons project into the hippocampus (blue arrow).
The dopaminergic system . The substantia nigra is made up of neurons (green circle) projecting into the striatum (a brain region involved in motor functions). These neurons synthesize dopamine and are particularly affected in DLB and Parkinson’s disease.
The noradrenergic system . The locus coeruleus (red circle), located in the brainstem, contains neurons projecting to the cortex, cerebellum and spinal cord (red arrows). These neurons synthesize norepinephrine, an excitatory neurotransmitter.
Clinical signs of Lewy body dementia
Clinical signs are characterized by both neurological, psychological and behavioral disorders, occurring insidiously and progressing over a period of several months to several years. The age of onset of LDB varies between 50 and 80 years. Men are slightly more affected than women.
According to the recommendations of the group of international experts, the essential criteria for the diagnosis of LDB are:
Progressive dementia, i.e. progressive cognitive decline severe enough to interfere with activities of daily living (domestic and social activities).
Predominance of visual and spatial disturbances (e.g. the patient no longer recognizes a route that he usually takes). Executive functions (reasoning, decision-making, abstract thinking, etc.) also decline. Visuospatial deficits are due to the presence of Lewy bodies in the visual associative cortex, located at the base of the temporal and occipital lobes. It should be noted that these lesions are not responsible for the slowing down of motor functions. The lack of perception is apparently at the origin of delusional disorders and misidentification (for example paramnesia, Capgras syndrome or the fact of not recognizing a relative). The patient also suffers from a significant attention deficit.
Memory problems (in particular verbal memory) which do not necessarily appear at the start of the disease, but which gradually worsen.
In addition to these main criteria, there are so-called secondary criteria. Two criteria are sufficient for the diagnosis of probable MCI, one criterion for the diagnosis of possible MCI).
Fluctuations in alertness and attention . These cognitive fluctuations are more marked and more frequent than in Alzheimer’s patients with a comparable cognitive deficit. They vary in duration and are similar to delusional disorders – they are sometimes called ‘pseudodelirium’. The characteristic signs are: loss of attention, incoherent language, hypersomnolence, impaired consciousness, staring into space. The patient suddenly returns to normal, confusing the family. The prevalence of fluctuations varies from 25 to 75%. Inexperienced physicians may have difficulty determining whether these fluctuations are secondary criteria for LDB.
A four-item questionnaire assesses the presence of these fluctuations with some precision. These items are:
the person is sleepy and has a lethargic behavior during the day, although he sleeps through the night,
she sleeps at least two hours during the day (before 7 p.m.),
she has a lost look for long moments and
sometimes disordered and confused thoughts.
This questionnaire detects with a high probability (positive predictive value* = 83%) the presence of DCL (and the absence of Alzheimer’s disease) if three of the four criteria are met.
Positive predictive value of a sign for a diagnosis: probability that the diagnosis is true if the sign is present.
Visual hallucinations recurrent and detailed, sometimes accompanied by auditory hallucinations. They are one of the cardinal signs of the disease, although they also appear in other forms of mental illness (Alzheimer’s disease, vascular dementia, hallucinatory psychosis). Hallucinations occur early in the disease and are persistent. They come in the form of innocuous three-dimensional images, representing people, animals or objects. Patients suffering from visual hallucinations early generally see their physical and mental condition worsen more quickly. A deficit of acetylcholine in the temporal lobe is probably responsible for these visual hallucinations. It should be noted that the associative visual cortex of the temporal lobe contains Lewy bodies.
Parkinsonian-like motor disorders. The vast majority of patients (up to 70%) show parkinsonian signs. These disorders are usually mild. They precede or follow the cognitive and behavioral disorders in a time interval not exceeding one year. If this is not the case, the doctor will make a differential diagnosis: other parkinsonian syndrome, dementia associated with Parkinson’s disease. Bradykinesia (slowness of body movements) is the main symptom. Gait disturbance, unsteady gait, limb stiffness and dystonia may occur as the disease progresses, or in response to treatment with antipsychotics (e.g. clozapine, risperidone) for soothe hallucinations. On the other hand, tremors at rest are rare.
Other criteria support the presence of a DLB:
Orthostatic arterial hypotension and postural instability, resulting in dizziness, fainting, transient loss of consciousness, with or without falls. Orthostatic hypotension with syncope is observed in a quarter of patients.
Hypersensitivity to antipsychotics (neuroleptics). Adverse effects suffered by patients with Lewy body dementia and treated with antipsychotics are frequent: sedation, rigidity, postural instability, falls and confusional state. The risk of mortality is doubled in patients treated with antipsychotics.
Oculomotor disorders.
Sleeping troubles. REM sleep (oneiric period of sleep) is characterized by muscle atony in healthy people. Patients with DLB develop REM sleep behavior disorders with violent shaking of the limbs during dream activity, excessive restlessness, persistent muscle tone, and absence of epileptiform electroencephalogram activity. The patient feels tired, but is usually unaware of this behavior. These disorders can be caused by cholinesterase inhibitors (eg Aricept, Exelon, Reminyl) prescribed for the treatment of cognitive disorders. Finally, a study has observed that these disorders can take years or even decades to dementia or Parkinson’s disease.
Depression. Depression and apathy are common and may be related to the severity of movement disorders.
Psychomotor restlessness.
Delusions, often elaborate.
Diagnostic criteria
Criteria
Essential criteria
Gradual cognitive decline, Persistent memory impairment, not necessarily early stage, Deficit in attention and visual/spatial ability.
Important signs of the disease
Cognitive fluctuations, Recurrent visual hallucinations, Parkinsonian-like motor symptoms.
Exclusion criteria
Presence of cerebrovascular disease (stroke, transient ischemic) validated by cerebral imaging, Parkinsonian symptoms occurring only in the severe stage of dementia.
These criteria make it possible to distinguish, with a small margin of error, a patient with MCI from a normal healthy subject (specificity* of the order of 80-100%). On the other hand, their sensitivity** is low and very variable (from 22 to 80%), suggesting that the prevalence of Lewy body dementia is underestimated.
It is estimated that an individual has an 80% risk of having MCI if they suffer from:
of progressive dementia
parkinsonian symptoms
visual hallucinations
The medical community emphasizes fluctuations in alertness and attention.
Differential diagnosis
The doctor will rule out the diagnosis of LDB and will suspect another pathology depending on whether the symptoms are dominated by motor disorders of the Parkinsonian type, a cognitive deficit with loss of autonomy, or psychological and behavioral disorders. The conditions close to LDB on which the doctor will pay particular attention are the following:
Alzheimer’s disease (AD). Disorders of episodic memory (recall of recent events) are earlier and more marked in AD than in DLB. Patients with Lewy body dementia, on the other hand, have a more impaired working memory, and more marked disturbances in attention, misidentification and delusional ideas. Visual hallucinations are observed later in AD. If the dementia appears more than a year before the onset of motor disorders, AD accompanied by a parkinsonian syndrome is preferred.
Vascular dementia. The cognitive disorders observed in vascular dementia depend on the location of the cerebral infarction, and are sometimes accompanied by an apathetic state and motor disorders. Cerebrovascular lesions are uncommon in LDB.
Parkinsonian type motor disorders. If the motor disorders precede the cognitive and behavioral disorders by more than a year, the presence of Parkinson’s disease or another form of parkinsonism is likely. Parkinson’s disease is likely if the patient suffers from tremors at rest and postural instability.
Other forms of parkinsonism can be considered such as:
Progressive supranuclear palsy, differing from LDB by a more marked gait disorder and the absence of delusional ideas. The patient also has oculomotor disorders.
Multiple system atrophy, which is very rarely accompanied by dementia. This parkinsonian syndrome is dominated by rigidity, akinesia (slow initiation of movements), and signs of dysautonomia (orthostatic hypotension, pupillary abnormalities, sphincter disorders).
Corticobasal degeneration: apraxic disorders are more pronounced than in Lewy body dementia. The motor signs (akinesia, rigidity) are very asymmetrical.
Creutzfeld-Jacob disease. Although the two diseases have common characteristics (abnormalities of the electroencephalogram, muscular contractions), the evolution of Creutzfeld-Jacob disease is generally fatal in less than a year. Analysis of the cerebrospinal fluid can help with the diagnosis, but only the autopsy can confirm it.
Schizophrenia. Also called hallucinatory psychosis, this is preferred if the hallucinations are both visual and auditory (for example the patient hears voices telling him to harm himself), and are accompanied by delusions without cognitive decline or disorder engine.
Metabolic dementia. Laboratory tests – blood tests, evaluation of thyroid function, blood levels of vitamin B12, brain imaging – will rule out or not vitamin B12 deficiency (often accompanied by psychosis), hypothyroidism (insufficient secretion of the thyroid resulting in psychomotor retardation, executive function disorders and psychosis). Cerebral imaging allows or not to eliminate the presence of lesions (lesions of the nuclei of the basal ganglia, of the thalamus and of the associative visual cortex) or cerebral anomalies (hydrocephalus, subdural hematoma) causing symptoms similar to those observed in Lewy body dementia. Imaging can also detect brain tumors affecting the lower temporal lobe and responsible for hallucinations and confusion. However, these tumors are rare.
Treatments of Lewy body dementia
Treatment of psychotic and behavioral disorders with antipsychotics is tricky because these patients are hypersensitive to this class of drugs.
This hypersensitivity aggravates the motor syndrome and induces states of acute agitation, leading to institutionalization.
Antipsychotics are the drug of choice, while limiting its dosage. Indeed, whether typical or atypical, they increase the risk:
death and stroke in older people with dementia. The risk factors are: age over 65, existence of sedation, malnutrition, dehydration, lung disease or concomitant treatment with benzodiazepines.
fall, this risk being already present in the Lewy body dementia.
They could also aggravate the cognitive deficit by promoting the appearance of lesions characteristic of Alzheimer’s disease (deposits of amyloid and neurofibrils).
Rivastigmine, a drug prescribed for cognitive disorders in Alzheimer’s disease, seems to reduce neuropsychiatric symptoms (eg delirium) in MCI and improve the quality of life of these patients.
Clinical case
An 80-year-old patient was hospitalized in a psychiatric unit for behavioral disorders (aggressiveness, confusional state) following treatment with an antipsychotic.
This person was previously diagnosed with dementia associated with a motor syndrome with:
cognitive disorders: temporal, gnostic and practical spatial disorders;
motor disorders: frozen face, voice and swallowing disorders, difficult walking.
He had been receiving rivastigmine and piribedil (a dopaminergic agent) for a year.
Despite the discontinuation of the antipsychotic treatment (replaced by an anxiolytic), the patient continued to exhibit agitation. Another anxiolytic was prescribed along with low-dose clozapine treatment. The gait disturbances were rapidly reduced, as well as his psychotic disturbances (hallucinations, agitation, aggressiveness).
Communication disorders in patients with dementia are increasingly present as the disease progresses, due to language and behavioral disorders. Factors favoring communication include the bringing into play of emotions, the application of body care and a good relationship between the patient and those around him.
Language is affected
Dementia is a disease of communication and, above all, of verbal communication. Indeed, the progression of the disease inevitably impoverishes the capacities of verbal exchange of the patient with his entourage. The real communication difficulties will mainly arise in the severe stage of his illness.
Verbal communication with the patient suffering from a cognitive function deficit is all the more difficult as the extent of the cerebral lesions – in particular the language areas – is marked. This deficit concerns:
comprehension disorders;
oral expression disorders dominated by a lack of words;
frequent paraphasias : words are distorted or substitute for others;
a simplification of the syntax;
in the most severe cases, the language becomes jargon with numerous neologisms.
Communication aggravating factors
Whether it is a family or medical environment, several factors can aggravate the exchange:
lack of availability, attention and listening. For example caregivers, who during a treatment, have a personal conversation from which the patient is excluded;
precipitation: the patient needs time to gather his ideas, find the right words and check that he has been understood. Otherwise, he will often prefer to be silent;
the medical jargon sometimes used by caregivers;
a statement by the patient considered uninteresting;
a failure at the start of a relationship where too much is asked of him, where he feels devalued.
How to optimize communication
Any cognitive stimulation can be conducive to inducing speech, especially in the case of sessions with a speech therapist. Other therapies (music therapy, relaxation techniques, art therapy) facilitate communication by strengthening self-esteem. Pleasant body treatments such as massages or cosmetic treatments can encourage the patient to talk.
How to stimulate the patient’s attention and motivation
Communication should never be forced. Caregivers to create a climate of trust and stimulate attention and motivation. For this it is necessary to:
go to a quiet place to create together a common space of exchange;
face the sick at the same level as him;
stimulate his attention by touch and gaze;
make simple sentences containing only one idea at a time;
say ‘I’ and avoid using ‘we’ which is too vague and impersonal;
ask questions to elicit ‘yes’ or ‘no’ answers, not ask for choices;
reformulate the patient’s words if necessary to check their content;
possibly help him find the missing words.
Patients suffering from dementia have a verbal expression that becomes more and more difficult. However, until the advanced phase of the disease, before the stage of mutism, the majority of them seek communication to express their experience, to speak in pairs, etc. When the verbal mode of communication disappears, the word is always possible, especially if a favorable climate favors it. Furthermore, It is necessary that the entourage is convinced that exchanging with them makes sense and that their words make sense.
• Vitamin B6, B12 or folic acid deficiency • Hypo or hyperthyroidism • Alcoholism • Heavy metals (mercury, lead) • Drugs or drug intoxication • Dehydration, hyperthermia
Metabolic or toxic agent-related dementias
Dementia subgroups
The medical community divides dementia into two subgroups based on their causes:
Degenerative dementias: Alzheimer’s disease, Lewy body dementia, frontotemporal lobar dementia (degeneration), dementia associated with Parkinson’s disease or extrapyramidal syndrome
Secondary dementias: vascular dementia, neurosyphilis, hypothyroidism, normal pressure hydrocephalus, central nervous system infections, meningitis, encephalitis, HIV, metabolite or toxic attacks (alcohol), Creutzfeldt-Jakob disease.
The causes can be reversible (depression, delirium, harmful side effects of drugs, infections, etc.) or irreversible (dementia of the Alzheimer type, vascular dementia, frontotemporal dementia, dementia with Lewy bodies, dementia associated with Parkinson’s disease, etc.).
The prevalence of dementias
Dementia of the Alzheimer type is the most common dementia (accounting for approximately 50% to 70% of cases), followed by vascular dementia (approximately 20%) and dementia with Lewy bodies. Vascular dementia is often associated with dementia of the Alzheimer type: we then speak of mixed dementia.
Before the age of 65, half of dementia is due only to Alzheimer’s disease, reaching 70% after the age of 65.
Alzheimer’s disease
Dementia with Lewy bodies
Frontotemporal dementias
Vascular dementia
Prevalence
50-70%
0-25%
8-10%
15-30%
The treatment of dementia
There is currently no cure for most dementias (such as Alzheimer’s disease). However, cognitive symptoms are treated by alleviating them and/or delaying their progression. It is sometimes possible to treat or reduce non-cognitive symptoms (aggressiveness, agitation, sleep disturbances, depression, hallucinations, etc.) using medication or by modifying the living environment (for example lowering the sound and light level in the House). Some dementias are treatable, such as those linked to a vitamin B deficiency or following alcoholism.
The anatomical location of dementias
Some of these dementias primarily affect either the cortical areas or the areas located under the cortex (subcortical areas); they can, as in the case of vascular dementia, affect both.
Symptoms associated with dementia
Their nature, severity and progression differ depending on the type of dementia. For example, the symptoms associated with dementia with Lewy bodies (Lewy bodies are cells that ubiquitously affect the brain) resemble those of Alzheimer’s disease (memory loss, difficulty speaking, etc.) but generally progress faster. Behavioral disorders appear earlier and more often in frontotemporal dementia or Lewy body dementia than in Alzheimer-type dementia or vascular dementia.
Clinical characteristics of dementias
The discovery of biomarkers or the contribution of neuroimaging (e.g., MRI, scanner) made it possible to confirm the clinical diagnosis of each of the tomes of dementia.
Alzheimer’s disease
Dementia with Lewy bodies
Frontotemporal dementias
Vascular dementia
First signs
Insidious cognitive losses (short-term memory)
Insidious cognitive and behavioral disturbances (hallucinations)
Behavioral and language disorders, apathy, disinhibition
Sudden onset (with neurological disorder for example)
Evolution
progressive
progressive
progressive
Stepwise worsening
Accompanying signs
Motor disorders, hallucinations, hypersensitivity to neuroleptics
Avoir un logo reconnaissable est la marque de l’entreprise par laquelle les personnes la connaissent. Vous pouvez le dessiner vous-même, vous pouvez commander le développement à un designer, ou vous pouvez utiliser l’un des services en ligne spéciaux qui seront discutés dans cet article.
Qu’est-ce qu’un logo ?
Le logo est considéré comme l’un des éléments les plus importants de l’identité d’une entreprise. Il forme une image de marque reconnaissable, facilite le choix des produits par les clients et joue un rôle important dans la communication. La nécessité de créer un logo est un fait dont on ne parle plus depuis longtemps.
Comment faire un logo en ligne ?
Il y a littéralement dix ou quinze ans, une personne qui décidait de dessiner un logo par elle-même n’avait d’autre choix que de prendre un crayon et du papier. Mais aujourd’hui, l’ensemble du processus est beaucoup plus facile. Il existe de nombreux services en ligne polyvalents comprenant une large gamme d’instruments pour créer un logo en ligne.
Quelques conseils pour ceux qui décident de créer leur propre logo
Que doit être le logo ? Simple, unique, reconnaissable, mémorable. Et il doit être apprécié non seulement par le développeur, mais aussi par le public cible. Pour créer un tel logo :
Familiarisez-vous avec les bases de la psychologie de la perception des couleurs et des formes ;
utilisez des références – elles aident à formuler les exigences de votre logo ;
apprenez des exemples de bons logos, mais ne les copiez pas ;
réalisez une enquête sur les réseaux sociaux pour connaître les préférences du public cible ;
essayez de rester dans les tendances de la catégorie, tout en étant unique ;
ne surchargez pas le logo avec de nombreux détails – ils nuisent à la mémorisation et rendent le logo peu évolutif.
En conclusion. Prendre un modèle et le modifier légèrement ne signifie pas concevoir un logo. Il est important de comprendre que le logo généré est une solution temporaire et qu’un rebranding sera certainement nécessaire à l’avenir. Si vous voulez retarder ce moment autant que possible, utilisez les services du service en ligne Turbologo.
Créateurs de logos en ligne
En utilisant l’un d’entre eux, vous pouvez dessiner votre propre image de marque. Pour ce faire, les concepteurs proposent un vaste ensemble d’outils, d’icônes prêtes à l’emploi et d’autres éléments. En les combinant d’une manière ou d’une autre, vous pouvez créer vous-même un logo à partir de zéro dans des créateurs de logos tels que Logotypemaker, Looka, etc.
Pour : dans la grande majorité des cas, le développement d’un logo dans un concepteur en ligne ne nécessite pas de compétences avancées en matière de conception ou la capacité de travailler avec des outils spécifiques.
Canva
Canva est un éditeur de logos en ligne gratuit qui vous permet de créer des logos à partir de rien ou d’utiliser des modèles prêts à l’emploi. Des modèles prêts à l’emploi, convenant aussi bien aux professionnels qu’aux débutants dans ce domaine. Le service dispose d’une grande bibliothèque d’éléments divers (autocollants, formes, lignes, images), de polices de caractères, y compris celles en langue russe, de mises en page et de modèles.
L’inscription est nécessaire pour créer un projet. Il est possible de réaliser non seulement des logos, mais également diverses bannières, cartes postales, cartes de visite, ainsi que de monter récemment des vidéos.
La version gratuite est suffisante pour un travail confortable, un abonnement sera plus probablement nécessaire pour les grandes entreprises ou ceux qui créent des designs de manière professionnelle.
Générateurs de logos pour les marques ou les produits.
Les générateurs ressemblent à un constructeur avec des fonctionnalités réduites. En règle générale, ils proposent de désigner le domaine d’activité et le nom de l’entreprise. Après cela, il ne reste plus qu’à cliquer sur le bouton et obtenir un certain nombre de logos générés automatiquement. Le logo qui vous plaît peut légèrement être modifié manuellement, mais le choix des outils est généralement limité.
Exemples de générateurs : Turbologo, Logoshi.
Avantages : prix relativement bas. En outre, il existe des générateurs de logos gratuits pour les entreprises. Le processus le plus simple.
Conclusion
Le logo est l’élément central de l’identité visuelle de l’entreprise et le fondement sur lequel la marque elle-même est construite. Le logo parfait ne vieillit pas, il est pour toujours.
Écartez les options trop décorées. Le design doit être modérément sobre, mais intéressant et mémorable. Le plus important est de maintenir la qualité des services de votre marque afin que les consommateurs puissent avoir confiance en elle.
The diagnosis of dementia is based on an interview with the patient and a physical examination (cognitive functions, neurological examination, autonomy, mental health).
Examinations: the first step in diagnosing dementia
It includes basic and clinical examinations. The clinical examination includes a history and physical examination.
A. Maintenance
From the start of the interview, the doctor must establish a climate of trust, by speaking directly to the patient whenever his condition allows. The physician must ensure that the patient hears, sees and speaks clearly (wearing glasses, hearing aids and dental appliances). The office in which the interview takes place should be quiet and well lit. Questioning of a relative is necessary if the patient has problems communicating. The doctor must nevertheless continue to question the patient, interpreting his words.
The anamnesis consists of questioning the patient to collect as much information as possible:
What is the personal history provided by the subject and corroborated by those around him: does he have a medical history, has he stayed in hospital, does he have a family history of illness (for example Does a close relative have Alzheimer’s disease A medical history may be important if a late-onset genetic disease such as Alzheimer’s disease is suspected.
Does he take any prescribed or over-the-counter medications? Certain medications (anxiolytics, hypnotics, certain antidepressants) are likely to cause cognitive disorders.
What are the symptoms frequently reported by the patient?
Is the patient still independent (eg does he take the bus alone?, does he dress himself)? A test is carried out to assess the subject’s ability to perform tasks of daily living (using the questionnaire on instrumental activities of daily living) and self-care (questionnaire on functional activities).
Has he lost weight, does he feel listless, depressed or confused, does he have memory loss, has he recently had falls, does he suffer from gait disturbances and posture, sleep (nightmares)? The observations made by the patient’s entourage describing, for example, the patient’s cognitive decline, are taken seriously into account by the doctor.
B. Physical examination
The doctor assesses mental and functional abilities (that is, their ability to perform physical activities of daily living).
B1. Examination of cognitive functions
It is done using the following assessment tests:
The Mini-Mental State Examination
The clock test
The five-word test
Grober and Buschke’s free and cued recall tests
B2. Neurological examination
The neurological examination includes examination of motor skills, tone and posture. The search for a parkinsonian syndrome (tremor at rest, akinesia, parkinsonian hypertonia) or frontal syndrome (reduction of the capacity to carry out a simple movement up to akinesia) must be sought systematically. The tremors at rest – at the rate of 5 per second – appear for a certain degree of muscular relaxation, and disappear during the execution of a voluntary movement.
It is at the end of the upper limb that it begins and is most characteristic. Akinesia is characterized for example by a rarity of blinking and the reduction of mimicry, giving the patient a frozen appearance, and a reduction in the associated swinging of the arms when walking. The examination highlights a difficulty in executing alternating movements quickly. For example, in strumming movements, there is a rapid decrease in the amplitude of the movements which can lead to a real blockage. Akinesia can disappear temporarily under the influence of an emotion.
The examination of reflexes is not only an exploration of the afferent and efferent elements of the reflex arc. It is also a test that evaluates the responsiveness of the nervous system as a whole at a specific time. Inhibition of Achilles tendon reflexes, caused by stimulation of the Achilles tendon, is frequently observed in very old people. It does not necessarily indicate peripheral neuropathy.
B3. Autonomy Review
The most widely used tool for assessing autonomy is the Activities of Daily Living (ADL) scale , which explores the basic activities of daily living: continence, eating, dressing , hygiene care, mobility, grooming.
If the subject appears to be autonomous, the doctor pursues the evaluation further using the scale of instrumental activities of daily living (AIVQ, in English Instrumental Activities of Daily Living or AIDL).
In France, the AGGIR grid (gerontological autonomy, isoresource groups) gives an overall score of autonomy that includes the patient’s ability to carry out the various acts of daily living independently. Patients are thus divided into six ‘iso-resource’ groups (GIR), which allows them to receive a social benefit for autonomy. The AGIR grid does not make it possible to accurately assess the specific needs of the patient.
The presence of a loss of functional autonomy must evoke the existence of dementia because these daily utilitarian tasks (for example managing one’s finances, using one’s telephone, driving a car, making purchases, etc.) are essentially governed by functions cognitive (judgment, language, orientation, calculation, memory, praxis, planning).
B4. Mental examination
The doctor must detect the signs reflecting a mood disorder or anxiety, corroborated by the patient’s entourage.
Psychiatric disorders are also important to identify because they are often inaugural in certain dementias such as frontotemporal dementia, Alzheimer’s disease and vascular dementia.
For example, anosognosia (the individual’s lack of knowledge of his disease) appears in certain Alzheimer’s patients (it is detected by the Cornell scale) and in other dementias.
Depression is considered a differential diagnosis of dementia (a condition called pseudodementia). It is often difficult to differentiate the two pathologies (dementia and pseudodementia) characterized by the presence of depressive symptoms. The diagnosis is made by prescribing the patient an antidepressant (if the antidepressant has no effect on cognitive disorders, this means that the patient suffers from dementia).
The existence of behavioral disorders should prompt the physician to assess cognitive functions and make a diagnosis of dementia. But he must avoid mistakenly evoking dementia. Indeed, some disorders such as hallucinations can be caused by drugs or delusional manifestations (eg Charles Bonnet syndrome with visual hallucinations).
The rating scales are:
Penn State Questionnaire
Geriatric depression scale
Hamilton Depression Scale
The Neuropsychiatric Inventory
Dementia diagnostic criteria
The diagnostic criteria of the Diagnostic and Statistical Manual of Mental Disorders 4th Edition (DSM IV) will be used to make the diagnosis dementia, or to make a differential diagnosis, that is to say find another cause at the origin of these cognitive disorders /behavioural; mental confusion may be one such cause.
A. Appearance of multiple cognitive deficits, as evidenced by both:
Memory impairment (impaired ability to learn new information and/or recall previously learned information).
One (or more) of the following cognitive disturbances:
Aphasia: language disturbance (difficulty naming objects or people, vague speech with long convolutions, and excessive use of imprecise words like “thing” and “it.
Apraxia: Impaired ability to perform motor activity despite intact motor functions (subjects cannot imitate gestures such as combing their hair or correctly perform symbolic gestures such as waving their hands. Apraxia can lead to impossibility of essential independent tasks dressing, etc.).
Agnosia: inability to recognize or identify objects despite intact sensory functions (objects, family members or even their own image in the mirror).
Disturbance of executive functions (making plans, organizing, ordering in time, thinking abstractly).
Both criteria A1 and A2 cognitive deficits cause significant impairment in social or occupational functioning and represent a significant decline from the previous level of functioning.
Demonstration from the history of the disease, physical examination or complementary investigations that the disturbance is the direct physiological consequence of one of the general medical conditions listed below.
Deficits do not occur exclusively during the course of delirium.
Reference: American Psychiatric Association, DSM-IV, Diagnostic and Statistical Manual of Mental Disorders. French translation, Paris, Masson, 1996.
The etiology of dementia
When the diagnosis of dementia is made, it is necessary to seek the etiology, that is to say the causes and factors at the origin of the disease.
The doctor studies the way in which the disorders have settled, their evolution since the appearance of the first signs, the existence of accompanying signs (motor, psychological and behavioral disorders such as hallucinations), the profile of the neuropsychological disorders, the detection of cerebral abnormalities by neuroimaging (in particular cerebrovascular lesions characteristic of vascular dementia). Other complementary examinations can be useful for the etiological diagnosis.
Dementia Diagnosis Reliability
If the doctor is in doubt about the diagnosis of dementia, another diagnosis can be made within 6 months.
However, and despite a rigorous clinical approach, a quarter of the diagnoses made are invalidated by the post-mortem study of the patient’s brain, which ultimately determines the diagnosis.
Other possible causes of memory problems
If the patient complains of isolated and unproven memory problems (i.e. not validated by neuropsychological tests), the doctor must screen for the existence of anxiety and/or depressive disorders, or ask the patient about the taking harmful drugs.
In those suffering from mild cognitive decline with memory impairment validated by neuropsychological tests, the patient must be followed regularly because the evolution towards dementia is frequent (about 15% of patients suffering from mild cognitive impairment develop dementia per year). In some cases, these memory disorders can also be caused by drugs with side effects by a mood disorder.
Here is a list of drugs that can cause cognitive and behavioral disorders:
Medicines with anticholinergic effects such as antiparkinsonians (e.g. amantadine).
Benzodiazepines (treatment longer than one month).
Benzodiazepine withdrawal.
Cimetidine (an antihistamine which can cause confusion, especially in the elderly and in cases of severe renal insufficiency).
Corticosteroids.
Isoniazid (antibiotic that can cause confusion).
Lithium (mood stabilizer that can cause confusion and memory loss).
Muscle relaxants (e.g. baclofen; cause rare disturbances such as hallucinations, euphoria and mental confusion).
Antipsychotics (confusion, memory problems).
Opiates (morphine).
Quinidine (visual or auditory disturbances).
Theophylline.
Alcohol abuse (or alcohol withdrawal).
Laboratory tests and neuroimaging
Neuroimaging and laboratory examinations are then carried out when the diagnosis of dementia is probable, in order to exclude any reversible form of cognitive decline and to refine the diagnosis.
The laboratory tests thus are intended to identify the reversible causes, the focal (localized) lesions affecting the white matter observed in vascular dementia, as well as atrophy of the medial temporal lobe characteristic of Alzheimer’s disease.
MRI (magnetic resonance imaging) makes it possible to observe cerebral atrophy whose topography points to the etiology of dementia:
Frontotemporal dementia: Marked frontal and anterior temporal atrophy, as well as less medial temporal atrophy than seen in Alzheimer’s disease.
Vascular dementia: presence of focal lesions (lacunae), small bleeding (infarction) and white matter signal abnormalities.
Positron emission tomography (PET) or single photon emission tomography (SPECT) are useful in differentiating Alzheimer’s disease (marked functional abnormalities of the bilateral parietal and temporal lobes) from normal aging or degeneration fronto-temporal (Figure 3), but they are not necessary in the assessment of dementia. Single-photon emission tomography (SPECT) and PET are also used to distinguish dementia with Lewy bodies from Alzheimer’s disease, using the dopamine transporter (DATscan®).
Behavioral and psychological symptoms of dementia (BPSD) are defined as the signs and symptoms suggestive of disturbances in perception, thought content, mood and behavior.
These behavioral and psychological symptoms have been divided into two groups:
symptoms assessed usually and primarily by patient and family interviews. These symptoms are: anxiety, depressed mood, hallucinations and delusions and
symptoms usually detected by observation of the patient’s behavior: aggression, shouting, restlessness, restlessness, wandering, culturally inappropriate behavior, sexual disinhibition, hoarding, cursing.
In addition to suffering, behavioral disorders aggravate the loss of functional autonomy, disturb those around them and are often the cause of hospitalization and permanent institutionalization.
They increase health care costs and greatly affect the quality of life of the patient, their family and other health care providers.
In institutions, the appearance of these disorders represents a real challenge for the medical team who must diagnose them in time in order to better treat them with a dual therapeutic and behavioral approach.
Finding the cause is important because it allows you to choose the most appropriate treatment (antidepressant, antipsychotic). The doctor will look for the existence of triggering factors.
Type and frequency of behavioral and psychological symptoms
These symptoms can be categorized according to the type of symptoms:
Emotional and motivational disorders : emotional disturbances, apathy/indifference, regressive behavior
Behavioral disorders
Psychomotor instability, compulsion,
disinhibition, agitation/aggressiveness.
Psychotic manifestations
Delusions (e.g. theft, harm,
persecution), hallucinations
Identification disorders
Basic conduct disorders
The behavioral and psychological symptoms of dementia can be classified according to their frequency and difficulties in management:
Symptoms
Frequency and support
Delusions Hallucinations depression and anxiety insomnia physical aggression wandering and restlessness
The most frequent and the most difficult to manage
The problems of identification Restlessness, inappropriate behavior and sexual disinhibition The tendency to pace Screams
Relatively frequent which can also be stressful
The cries swear words The lack of enthusiasm Repetitive questions The relentless pursuit of a loved one
Less common and for which treatment remains possible
About 60-90% of patients with dementia related to Alzheimer’s disease show behavioral problems during the course of the disease. According to the REAL study, the frequency of behavioral and psychological symptoms is very high ranging from 84% for people with an MMSE test score of 21 to 30 to 92.5% of patients when the MMSE test score is between 11 and 20 .
Behavioral and psychological symptoms occur at different stages of the disease. Affective symptoms are more likely to appear at an early stage of the disease. Agitated and psychotic behaviors are common in patients with moderate cognitive impairment; however, they become less apparent in advanced stages of dementia, most likely due to deterioration in the patient’s physical condition.
It is often the nurses and caregivers who generally notice them first. The earliest are signs of depression, anxiety, apathy, disinterest, social withdrawal. Then follow the noisiest: agitation, aggressiveness, disinhibition.
Symptoms depend on the type of dementia
Psychological behavioral symptoms are not the same in all dementias: emotional disturbances and apathy often dominate in Alzheimer’s disease, while hallucinations are present in 80% of people with Lewy body dementia; agitation, aggressiveness, apathy dominate in vascular dementias, and impulsive behaviors, agitation, disinhibition characterize frontotemporal dementias.
Some psychological behavioral symptoms are more persistent than others. For example, over a 2-year observation period, one study demonstrated that wandering and restlessness are the longest-lasting behavioral symptoms in patients with Alzheimer’s disease.
Similar profiles have been found in patients with Alzheimer’s disease and vascular dementia. All symptom levels were high (30% of all patients had three or more psychiatric symptoms) and increased with the severity of dementia. Agitation was the most frequent symptom, followed by symptoms of depression, apathy and aberrant behavior.
Other studies have also reported that there was no difference in behavioral profile between patients with Alzheimer’s disease and those with vascular dementia.
Visual hallucinations are more commonly seen in patients with dementia with Lewy bodies than in those with Alzheimer’s or Parkinson’s disease.
These hallucinations occur in nearly 80% of patients with dementia with Lewy bodies, compared to nearly 20% in those with Alzheimer’s disease.
Frequency and evolution of psychological behavioral symptoms in Alzheimer’s disease
Symptoms
Frequency
Depression
5 to 25%
Anxiety
50%
Psychosis
50%
Agitation/aggressiveness
50 to 75%
Apathy
100%
Psychological behavioral symptoms in frontotemporal and Lewy body dementia
Frontotemporal dementias
Dementia with Lewy bodies
physical neglect
Visual hallucinations
Disinhibition
Sleep disorder
Psychic rigidity
Depression and apathy
Hyperorality
Psychomotor restlessness
Stereotypy
Delusions, often elaborate
Usage behavior
Distractibility
Frontotemporal dementias
Dementia with Lewy bodies
Impulsiveness
Anosognosia
Depression
Anxiety
Ideal fixation
Delirium
Hypochondria
Somatic complaints
emotional indifference
Apathy
Inertia
Depression and apathy
Depression represents a differential diagnosis of dementia but it can also be part of the clinical picture of Alzheimer’s disease (early stage) .
Depressive symptoms affect a sizable minority of patients with dementia at some stage of development. Most studies, conducted with patients with Alzheimer’s disease, have shown that depressed mood is found more frequently (40-50% of patients), while depressive disorders are less common (10-20%) . A 5-year longitudinal study of patients with Alzheimer’s disease found relapse rates of 85% for depressive symptoms over a one-year period.
The diagnosis of depression can be difficult to establish, particularly in patients with moderate or severe dementia. In the early stage of dementia, depressed mood and its symptoms can usually be determined during the medical consultation.
As dementia progresses, it becomes more difficult to establish the diagnosis of depression due to the increasing presence of speech and communication difficulties, apathy, weight loss, sleepiness and restlessness that accompany dementia.
A depressive disorder should therefore be considered if:
pervasive depressed mood and anhedonia (inability to feel positive emotions caused by a feeling of disinterest);
self-devaluation and desire to die;
personal or family history of depression prior to the onset of dementia.
The prescription of an antidepressant -without anticholinergic properties- is justified.
Apathy and its associated symptoms affect up to 50% of patients in the early and middle stages of Alzheimer’s disease and other forms of dementia. Patients with apathy present with:
lack of interest in activities of daily living and self-care;
decreased social interaction;
a decrease in facial expressiveness;
a more monotonous voice;
decreased emotional reactivity;
loss of initiative.
Symptoms of apathy can be confused with those of major depression. Both states can manifest as a decline in general interest, psychomotor retardation, lack of energy and insight.
Although a lack of motivation also accompanies the states of apathy and depression, the apathy syndrome denotes a lack of motivation without the vegetative symptoms of depression being present.
The drug treatment of apathy is different from that of depression (the doctor will prescribe an antidepressant to a depressed patient, and a psychostimulant in case of apathy).
Anxiety
Anxiety is with depression the affective disturbance most frequently found (50% of cases) in demented patients. It can precede cognitive disorders to the point that its recent appearance in an individual should lead to the evaluation of his cognitive functions. Demented patients with anxiety will express previously unmanifest concerns and fears about their finances, future, and health (including memory), and fears about events and activities, like being away from home.
The patient may incessantly repeat questions relating to an upcoming event (this is Godot’s syndrome). Another symptom of anxiety characteristic of the demented patient is the fear of being left alone. This form of phobia can manifest itself as soon as the carer goes to another room in the home (the patient follows the carer in his movements) and can be expressed by the patient’s repeated requests not to be left alone. Other phobias may also appear, such as a fear of travelling, being left in the dark, or taking a bath.
It is often difficult to spot anxiety in a person with dementia. Indeed, aphasia can prevent the verbal communication of affects and transform the expression of emotional disorders. Similarly, emotional responses may be maladaptive due to misinterpretation of a stimulus.
Apraxia can also impair nonverbal emotional expression abilities.
Sometimes, the demented subject adopts defense mechanisms against anxiety: for example, he will invoke a relative as if he were still alive. When the dementia is more pronounced, the anxiety presents itself in a more physical form: aggressiveness, running away, incessant wanderings…
Agitation and aggression
They are relatively infrequent but can create a climate of disorganization in an institution. Agitation is defined as inappropriate verbal, vocal or motor activity that is not the result of an immediate need or a state of confusion. Interventions must be immediate and based on the intensity of the crisis.
Aggression is a feared behavioral disorder because it is difficult to control. A distinction must be made between verbal aggression (anger, insults, shouting, etc.) and physical aggression (violent gestures). This type of behavior generally takes shape during exchanges between the patient and the carer (toilet, dressing, meals, bedtime).
There are four forms of agitation/aggressiveness:
Non-aggressive physical behavior :
repetitive gestures (eg hoarding objects – pencils, cups, toiletries, clothes – that are in the room of other patients;
comings and goings;
wandering around, entering other patients’ rooms or bathrooms, lying in their beds;
attempts to travel to another location;
hiding objects, tearing things up, throwing them in the toilet;
inappropriate urination or defecation, including handling stool;
wearing inappropriate clothing;
knocking over food trays, eating from other patients’ trays;
take objects belonging to other patients and staff members (glasses, teeth, pants).
* Cries most often occur when the person is alone in their room, in the evening or at night. Their appearance is more important at the time of interventions carried out in a situation of constraint for the person (toilet, dressing). It is necessary to determine whether these cries come from pain or are the cause of a manifestation of fear, a depressive state or moral suffering that cannot be expressed in words. The installation of bed rails generally increases the intensity and duration of these cries in some patients. It is therefore important to stimulate the patient as much as possible by providing him with activities adapted to his condition (playing of musical tapes, video-cassettes evoking family memories).
Dementia by itself does not completely explain the agitation phenomenon. Indeed, other factors – medical, psychological and environmental – are involved in agitation.
It is important to have a calm attitude and voice, to try to appease the patient by word and gesture, not to be more than three people involved (the patient may feel threatened). It is sometimes necessary to isolate the patient in his room, knowing that there is a risk of a panic attack. Restraints are to be avoided, as they often increase the intensity of the crisis.
The causes and triggering factors depend on the personality of the patient and his situation: it may be, for example, a reaction to pain, an annoyance (loss of a life habit), a reaction to an over -stimulation (sensory or cognitive) for the patient who no longer has the ability to respond.
For example, a patient may be aggressive or even violent if the nursing staff refuses her access to her bathroom after her meal. An interview with one of her children will make it possible to understand the reasons for her anger: she had the habit of brushing her teeth after each meal, a habit which had not been followed when she entered an institution (this is an annoyance relating to that of care which must take place in an institution at specific times).
Delusional Disorders
The frequency of delusions in people with dementia is between 10% and 73% depending on the population studied. About a third of patients with probable Alzheimer’s disease suffer from delusions, and are considered to be at risk for physical aggression. According to one study, 80% of participants with high levels of physical aggression (>1 episode/month) also suffered from delusions. Patients with Lewy body dementia very often present with delusional pathology (about 80%).
Delusional disorders at the beginning of the dementia stage revolve mainly around forgetfulness. They lead to ideas of displacement and theft of objects, intrusion into the home…
They may be accompanied by aggressiveness in a person with a psychiatric history. As the disease worsens, the delusions aim to fill the growing void caused by the memory loss, and draw on the remnants of memories still intact in the patient. The most frequent delusional themes are theft, prejudice and infidelity and jealousy (15% of cases).
The Behavioral Pathologic Rating Scale for Alzheimer’s disease is a behavioral disorder rating scale that describes the different forms of delirium in Alzheimer’s disease. These forms are classified by theme (theft, impostor syndrome, etc.):
The patient believes that he has had things stolen (frequency = 18% to 43%)
Likely explanation: the patient does not remember the location of their personal items. He will therefore be led to blame a third party for their disappearance. In serious forms, he is convinced that people break into his home with the aim of hiding or stealing objects.
The patient believes he is living in a home that is not his
The patient does not remember or recognize his home. He may end up leaving it to return « home » (this is the phenomenon of wandering).
The spouse (or the relative in charge of the patient) is perceived as an impostor
The patient no longer recognizes his loved one. He can also consider the family caregiver or his doctor as an impostor, leading to a reaction of mistrust, even of great violence. This may be similar to an identification disorder (this is Capgras syndrome).
A feeling of abandonment (frequency = 3% to 18%)
The patient is convinced that he has been abandoned, that he will be placed in an institute, and that a plot is being organized for this purpose. This feeling stems from the fact that he realizes the burden he represents for those around him because he retains a certain awareness of his condition. Relatives feel guilty for this feeling of abandonment experienced by the patient.
A feeling of infidelity (frequency = 1% to 9%)
This feeling mainly concerns the spouse or caregivers.
Although the diagnosis of delirium is sometimes difficult in a person with dementia (indeed people with dementia already have a disorder of ideation and confusion), it is possible, with some experience, to tell the difference between delirium and dementia.
Indeed, delirium is usually accompanied by:
a sudden onset of symptoms;
a decrease or increase in alertness in the patient already presenting with dementia, or a marked fluctuation in symptoms;
visual hallucinations accompanied by agitation;
impaired psychomotor activity;
language disorders (slurred speech, slowing or speeding up of speech);
of tremors.
Once the diagnosis of delirium is made, the choice of treatment will depend on identifying the cause. These causes are many and include:
medication side effects;
malnutrition;
an infection;
cerebral pathologies (e.g. subdural hematomas);
endocrine disorders (eg hyperthyroidism);
metabolic diseases (eg certain kidney or liver conditions);
patient environmental factors;
Hypoxia caused by pneumonia, congestive heart failure or sleep apnea;
Urinary retention or fecal impaction (accumulation of feces).
Hallucinations
Hallucinations interfere with the patient’s ability to understand the outside world. This phenomenon can alter their ability to perform activities of daily living and can affect their relationship with caregivers. The frequency of hallucinations in people with dementia ranges from 12 to 49%.
Visual hallucinations are the most common (up to 30% of patients with dementia), and they are more common in moderate dementia than in mild or severe dementia. Auditory hallucinations are present in 10% of dementia cases. Other forms of hallucinations – tactile and olfactory – are rare.
A common type of visual hallucination involves observing the presence of certain people in one’s home, when in fact they are not there. In the case of Lewy’s dementia, the interpretation disorders are based above all on gnosic deficits, and to a lesser extent on mnesic disorders and sensory perception defects.
The most frequently observed gnosic deficits are prosopagnosia (the patient does not recognize his relatives; 12% of cases), self-agnosia (4% of cases), intrusion of people into the patient’s life ( 6% of cases), or the ghost companion syndrome (the patient is convinced that a person is hiding in his home (17% of cases). These hallucinations sometimes require treatment when they are a source of stress. This treatment depends on the etiology.
There seems to be a relationship between a decrease in visual acuity and hallucinations (Bonnet syndrome is quite common). Indeed, a significant percentage of people with dementia have visual impairment (e.g. visual agnosia characterized by difficulty recognizing faces or objects), and many experience difficulty in contrast perception, especially at low frequencies. In these patients, the line of demarcation between the areas of shadow and light appears blurred, which partly explains the high rate of identification disorders and visual hallucinations. It is therefore important to assess the visual perception functions of patients with dementia and suffering from hallucinations. From a practical point of view,
An attitude of empathy from the nursing staff is often the most effective approach when dealing with a patient suffering from delusion of flight. The nurse can then look with him for the objects supposed to have disappeared, which has the effect of reassuring him.
Wandering
Wandering (also called motor hyperactivity) describes a set of manifestations during which the patient follows and seeks caregivers throughout the day. This wandering can be aimless or will be characterized on the contrary by comings and goings between two very specific places. These manifestations, specific to demented states but whose causes are complex, would be the expression of boredom, sadness or anxiety. Here are some causes that would be at the origin of this type of behavior: hyperactivity, orientation disorders leading to seek new landmarks, attempt to escape or escape isolation,
These problems are the most delicate to manage at home, which obliges the relative to hospitalize the patient.
It should be noted that the ambulation associated with akathisia can be caused by the side effects of antipsychotics, effects which persist for several months after stopping treatment.
The management of ambulation primarily involves non-drug treatments. Physical restraints should be avoided because on the one hand they have no effect and on the other hand they raise an ethical problem. The architects have provided spaces in the institutions allowing unrestricted walking. These spaces should be bright, spacious, attractive, risk-free and create a sense of security.
The planning of physical activities (gymnastics, walks) must complete the care program, with dance and singing programs as well. It is strongly advised to monitor the nutritional status of these people, whose energy expenditure and water loss are significant. The room must be arranged so that the patient can find familiar landmarks (small pieces of furniture, family and home photographs, etc.).
Repetitive behaviors
Repetitive behaviors are, like wandering, specific to demented states and often concern the same people. There are two forms of repetitive behavior:
1. Risk-free behaviors
These people are constantly doing and undoing (eg emptying and filling a cupboard, making and undoing a bed, etc.). These activities are favored by boredom and the absence of activities (which is somewhat paradoxical…). The programs proposed to reduce ambulation are quite suitable in this case.
2. Risky behaviors
These are often behaviors of self-mutilation (eg repeated scratching of the face with the fingernails) whose intention is difficult to understand. This behavior can temporarily stop if an affective derivative is offered: caresses, massages or animal therapy.
Runaways are behavioral problems feared by health professionals. They occur most often in the weeks following entry into an institution and tend to subside after a period of adaptation. It would seem that the patient wants to flee a place because it is inhospitable to him, even hostile. However, it is difficult to offer closed structures for security reasons (risk of fire) and psychological reasons (feelings of being in a prison). Some institutions suggest placing exits in dark areas or concealing them. We can also prevent the risk of running away by arranging the environment with familiar objects to familiarize the patient with his environment and improve his well-being.
Disinhibition
It results in impulsive and inappropriate behavior. The patient cannot maintain the type of social behavior he had before. Symptoms include:
the cries;
euphoria;
verbal abuse;
physical violence directed at others or objects;
self-destructive behaviors;
sexual disinhibition;
motor restlessness;
intrusive behaviors;
impulsiveness;
wandering.
Shoplifting, gambling addiction, compulsive shopping can lead to financial and social problems in patients with disinhibition. Patients with impaired judgment are more likely to consume alcohol or drugs excessively.
Intrusive behaviors
The intrusive behavior manifests itself in gestures of protest, impatience, clinging or jostling. These behaviors have a prevalence of about 40% in dementia, and do not seem to depend on the severity of the cognitive and functional deficit.
Sleep disorders
These disorders end up exhausting the family caregivers, leading them to place the patient in a medical institution. The doctor must inform the relative of the physiological changes in sleep (lengthened sleep onset, jerky sleep, reduced quality of sleep, etc.) that accompany dementia. Difficulties falling asleep are caused by anxiety disorders, while early awakenings evoke depression.
The use of sleeping pills should be temporary and of short duration. Some non-drug measures can improve sleep disturbances.
Vocalization behaviors
These verbal expressions, often brief but repetitive, are more frequent when the patient is isolated, in the evening between meals and going to bed, or in the morning between waking up and getting up. It is difficult for caregivers to determine the causes and reasons for these cries. In the absence of specific causes (pain, hallucinations, etc.), the cries are attributed to dementia.
Treating these symptoms is difficult. Certain non-drug approaches sometimes prove effective: correction of sensory deficits, reduction of sensory stimulation (noise, light, cognitive activity), getting the patient to talk about the difficulties he is experiencing in the institution (his daily life, his isolation) , adopt a calm and reassuring attitude, communicate non-verbally (caress the patient’s hands and face), offer massage sessions.
Atypical antipsychotics present but seem, according to some doctors, to be less well tolerated than selective serotonin reuptake inhibitor type antidepressants (eg Paxil).
The problems of identification
Unlike hallucinations (which occur in the absence of external stimuli), identification disorders are due to errors in the perception of external stimuli. There are four forms:
‘ghost boarder’ syndrome: presence of people inside the patient’s home (about 5% are convinced that people appearing on television are present in the room);
self-identification disorder: it is often manifested by the fact that the subject does not recognize himself in a mirror; (about 4% of patients);
other people’s identification disorder: the patient takes people for other people (about 12%);
misidentification of events.
In 1990, Ellis and Young distinguished three forms of delusional identification disorder: Capgras syndrome .
Fregoli syndrome is a type of hyperidentification characterized by the fact that patients are convinced that individuals wishing to harm or influence them disguise themselves to assume the identity of other people. In many ways, Fregoli syndrome can be like the normal experience.
When a non-demented subject expects to meet a person, he may, for a brief moment, take a stranger for the person he was expecting, but this perception is quickly corrected by noting divergent facts. A patient with Fregoli syndrome will attribute these divergent facts to the notion of a disguise.
Intermetamorphosis delusion describes a situation in which one person’s physical appearance is perceived as that of another.
Many parents and caregivers find their own ways to cope with the difficulties of identification. It is important to understand that what works well with one person may not be as effective with another. In some cases, humor will be preferred while in others, comfort or diversion will lead to greater success.
Care
What are the actions to take when faced with a person suffering from dementia accompanied by psychological and behavioral disorders?
Anxiety
Anxiety often causes physical manifestations related to anxiety. In some cases, it can result in delusional states during which the elder will accuse those around them of theft, for example.
In the patient, anxiety results in momentary disturbances which concern both the body and the consciousness. It becomes abnormal when it occurs for no apparent reason and permanently.
At the psychological level, it results in a feeling of inner malaise, internal tensions, difficulty concentrating, threat or depersonalization. At the physical level, anxiety results in an increase in heart rate, respiratory rate and sweating. From the behavioral point of view, we observe agitation, incessant movements, clenching of the hands and aggressiveness.
How to deal with this type of problem? – Prescribe a short-lived anxiolytic. – Use relaxation techniques to relax the muscles and the mind. – Identify the triggering factor. – Reassure the person by speaking calmly. – Try to determine the origin of the agitation, taking into account the person’s biography.
Agitation and aggression
Agitation is manifested above all by incessant wanderings, diurnal or nocturnal, messy activities, akathisia, running away caused by temporal and spatial disorientation, repeated manual movements (eg scratching, folding of clothes). Two-thirds of patients show signs of institutional agitation. This percentage reaches 90% in those suffering from dementia.
Aggression, whether verbal or physical, often has an unusual character that surprises those around you. The latter dreads it, because it is often unpredictable and difficult to control. Aggression refers to all activities aimed at harming an individual or an object directly or indirectly. This behavioral disorder generally takes place during a relational exchange, in particular when washing, dressing, eating or going to bed.
A distinction must be made between aggressive and non-aggressive behaviors, which are expressed verbally and non-verbally (physically): – motor agitation: aimless walk, nocturnal wandering; – physical aggression: throwing or destroying objects, making inappropriate gestures, biting, spitting, scratching, kicking, beating another person; – verbal aggression: speaking loudly, shouting, swearing, using rude words, threatening, accusing, making unusual noises, complaining, whining, making repeated demands; – passive aggression: delaying, avoiding or refusing help.
How to prevent or mitigate this type of behavior? – Prescribe pharmacological treatment (neuroleptics, lithium, carbamazepine, buspirone, antidepressants, short-lived benzodiazepines), particularly in the event of an acute situation of agitation and/or aggressiveness accompanying dementia. – Try to restore an activity during the day (in case of nocturnal restlessness). – Adopt a serene attitude and a soothing tone of voice. – Stand facing the person and at the same height. – Express yourself in simple words. – Establish physical contact by staying close to the patient or holding their hand, especially when there are visitors in the house. – Listen and decode non-verbal language. – Name yourself and name the person. – Give a snack at the time of the crisis of nocturnal agitation. – Leave a little light in the room in case of nocturnal agitation. – Install the person in a room for two to reduce anxiety in the event of nocturnal restlessness in an institution. – Propose a relaxing activity (walking, listening to music, isolation for some time in the room). – Try to establish routines in the activities of daily living (bathing, meals). – Avoid placing the patient near a source of noise (washing machine, washing machine) which could cause agitation. – De-dramatize situations that could humiliate or make the patient feel guilty (in the event of aggressive behaviour).
Don’ts: – Do not insist if the person refuses to cooperate. – Avoid using the subject “on”. – Do not respond to aggression with aggression. – Do not adopt an infantilizing attitude. – Do not try to reason with the patient with arguments that go beyond the possibilities of understanding. – Do not argue with her by contradicting her. – Do not offer him things that are too difficult for his remaining abilities. – Do not express your frustration. – Do not re-discuss events that led to aggressive behavior.
After having tackled the problem, you must try to determine the origin of the conflict: look for the triggering elements showing significant frustration (agitation and/or aggressiveness in a demented state). Look for bodily, psychological or psychosocial causes and then treat them.
Here are some questions to ask yourself in order to identify the factors if possible: – Does the patient have a headache? – Is he particularly tired? – Is he constipated? – Does he sleep badly? – Are his clothes inappropriate (too warm, tight)? – Is he too stimulated by the environment (continuous television, radio at too high a volume)? – Is he upset by a new environment (bedroom, kitchen) in which he has lost his bearings? – Is he more restless when he is alone or, on the contrary, when there are people around? – Does he feel compelled to perform an activity against his will?
Agitation, confusional states and end of life
Agitation is common in elderly subjects at the end of life. The causes come from several factors, hence the need to seek a curable cause or poorly relieved pain, or to remove drugs that are not essential.
Elements to look for in a patient at the end of life: – Unrelieved pain. – Urinary retention. – Dry mouth, dehydration. – Confusion caused by medication (neuroleptics, morphine, antiemetic, anticholinergic). – Metabolic disorders (hypoglycaemia). – Neurological origin (eg brain metastasis). – Psychological origin (anxiety, conflict).
Psychological Behavioral Symptoms References
Pancrazi MP, Métais P. Alzheimer’s disease, diagnosis of psychological and behavioral disorders. Medical Press 2005; 34(9): 661-6.
Brocker P et al. Psychological and behavioral symptoms of dementia: description and management. The Journal of Geriatrics, Volume 30, N°4 April 2005
Cummings JL, Mega MS, Gray K, Rosemberg-Thompson S, Gornbein T. The Neuropsychiatric Inventory: comprehensive assessment of psychopathology in dementia. Neurology 1994;44:2308-14.
Conn D, Thorpe L. Assessment of behavioral and psychological symptoms associated with dementia. Can J Neurol Sci. 2007 Mar;34 Suppl 1:S67-71.
Benoit M, Staccini P, Brocker P, Benhamidat T, Bertogliati C, Lechowski L, Tortrat D, Robert PH. Behavioral and psychological symptoms in Alzheimer’s disease: results of the REAL.FR study Rev Med Interne. 2003 Oct;24 Suppl 3:319s-324s.
Delirium – a confusional syndrome or state – is characterized by changes in the level of consciousness, disorientation and behavioral problems.
It is common after surgery.
Do not confuse delirium (literally translated from Latin as outside the groove) and delirium, which is a disturbance of logical reasoning.
History of delirium
1881: Lasègue describes for the first time delirium tremens in alcoholics.
1892: Chaslin describes mental confusion and already notes clinical heterogeneity.
1911: Regis describes secondary post-dream delusions.
1935: Wolff and Curran describe it as a mental condition caused by malnutrition and poor blood supply in the brain.
Epidemiology of delirium
The highest incidence rates were observed in intensive care, postoperative and palliative care units. Since many of these studies excluded patients with cognitive impairment or dementia at baseline, these rates are likely underestimated.
In general medicine and geriatrics departments, the prevalence of delirium (present on admission) varies from 18 to 35%. More specifically, the prevalence of delirium in hospitalized elderly patients varies from 15 to 25% after major surgery and is estimated at 50% after high-risk surgery in the elderly, such as a hip fracture or heart surgery. Confusional state is therefore common after surgery.
The prevalence of delirium in the community setting is relatively low (1–2%), but its onset usually brings the patient to urgent care. It is present in 8 to 1% of elderly people admitted to the emergency room. Its prevalence at the end of life is around 85% in palliative care.
In intensive care units, delirium usually begins within the first few days of hospitalization and usually lasts 24 to 48 hours. The prevalence is 70%.
Delirium is consistently associated with an increased mortality rate in all non-surgical patient populations (e.g. medical, geriatric, and intensive care units). 15-18% of those who develop delirium in general or geriatric medicine wards are 1.5 times more likely to die within a year. In older patients with dementia, delirium is associated with increased rates of cognitive decline, institutionalization, and mortality.
The causes of delirium
While a single factor can lead to delirium, most often delirium is multifactorial. There are predisposing factors that are associated with etiological factors: advanced age, male sex, presence of dementia or psychiatric illness, consumption of drugs (eg benzodiazepines, sedatives, hypnotics) , alcohol dependence and decreased visual acuity.
In patients who are highly vulnerable to delirium, such as those with underlying dementia and several other disorders, a simple intake of medication – such as a single dose of sleeping pills – may be enough to precipitate delirium.
Conversely, in a healthy young patient, delirium will only develop after exposure to a series of major events, such as general anesthesia, major surgery, taking multiple psychotropic drugs, a stay in intensive care and sleep deprivation.
Clinically, the implication of multiple causative factors is that treatment of a single risk factor is unlikely to resolve delirium, and that multiple approaches will be most effective in both preventing and treating delirium. .
somatic ailments (retention of urine, fecal impaction);
psychological causes;
neoplasms;
taking multiple medications or using psychoactive drugs (risk increased by 4.5 times).
Withdrawal from a substance or medication (benzodiazepines, opiates, psychostimulants, alcohol).
Dementia is, along with mental confusion and depression, one of the etiological factors of delirium. Confusional episodes are common after an operation caused, for example, by postoperative hypoxia or hypotension. Tricyclic antidepressants , antipsychotics and antiparkinsonens (which have anticholinergic properties), benzodiazepines and barbiturates, drug withdrawals (benzodiazepines, barbiturates) also cause delirium, as do morphine-type analgesics, some antihypertensives (beta-blockers, calcium channel blockers, etc.).
Pathophysiology
Delirium is undoubtedly the consequence of the involvement of several causal factors which, taken one by one, seem minor but which, when accumulated, trigger the disease.
It is likely that several sets of interacting biological factors lead to disruption of neural networks and decreased metabolism in certain regions of the brain, leading to cognitive dysfunction. Other causal mechanisms interfere more indirectly with neurotransmission. For example, the inflammatory response seen in sepsis can lead to a cascade of local neuroinflammation in the brain triggered by cytokines, leading to decreased blood flow and neuronal death. Neuroinflammation can lead to glial cell activation, which in turn leads to neuron death.
Changes in the sleep-wake cycle have been reported in patients with sleep disturbances and sundowning syndrome. The cholinergic system is affected in delirium, a hypothesis supported by the fact that drugs with anticholinergic properties (ie, which cause a drop in acetylcholine levels) cause acute confusional episodes. Other neurotransmitters (GABA, dopamine) may also be involved.
The risk factors are:
age
Madness
The male gender
Previous history of delirium
Vitamin deficiencies
Depression, anxiety
Pain, fractures. dehydration.
Diagnostic criteria for delirium
Delirium is a clinical diagnosis, often misunderstood and easily overlooked. Recognition requires a brief cognitive screening and clinical observation.
Key diagnostic features include sudden and fluctuating onset of symptoms, inattention, altered level of consciousness, and impaired cognition (eg, disorientation, memory impairment, impaired language).
Perceptual disturbances (hallucinations or illusions), delusions, psychomotor disorders (hypo- or hyper-activity), inappropriate behaviors and emotional lability. The current reference standard diagnostic criteria are the American Psychiatric Association’s Diagnostic and Statistical Manual (DSM-IV) and the World Health Organization’s International Classification of Diseases (ICD-10).
The DSM-IV defines delirium as a mental disorder due to a general medical condition consisting of the combination of the following diagnostic criteria:
disturbance of attention and consciousness;
modification, impairment of cognitive performance (memory, orientation, language, perception disorders);
alteration of sensory perceptions manifested by visual or auditory illusions or hallucinations that affect the patient’s behavior, with the possibility of panic reactions (fugue, aggression, etc.);
the disturbance sets in in a short time (a few hours to a few days), with a fluctuating course throughout the day;
evidence from the anamnesis, clinical and paraclinical examinations, that the disturbance is due to the direct physiological consequences of a general medical condition.
For the CIM-10 , the criteria are substantially the same, except that according to the CIM-10, the onset is usually sudden, the evolution is fluctuating during the day and the total duration of the episode is less than six month. In addition to these main criteria, there are:
psychomotor disorders (hypo- or hyperactivity, prolonged reaction time, increased or decreased verbal flow, exaggerated startle reaction);
sometimes vegetative disorders (tachycardia, sweats, fever…);
incoherent, logorrheic language.
More than 24 instruments have been used in scientific studies to identify delirium. The most widely used is the Confusion Assessment Method (CAM), with a sensitivity of 94% and a specificity of 89%. Cognitive testing is recommended for optimal use of the CAM. The CAM has been adapted for use in intensive care units, emergency rooms and nursing homes.
Clinical forms
Perceptual abnormalities include hallucinations, delusions, and misinterpretations. They are mostly visual and accompanied by emotional reactions. A sudden onset and a short and limited duration are necessary criteria for the diagnosis of the confusional episode, but the ICD-10 admits a maximum duration of the table over six months. The confusional episode can either reveal the presence of an undiagnosed dementia, or aggravate an already installed dementia.
Differential diagnosis
One `two-thirds of delirium diagnoses are missed due to the fluctuation of the clinical picture throughout the day.
Acute confusional state can be confused with the following diseases:
1. Dementia. Unlike delirium, the onset of dementia is insidious, vigilance is intact, hallucinations are rare, psychomotor activity is normal, mood is little altered, delusions are infrequent, speech is most often normal during early disease and neurological signs are often absent.
2. Wernicke’s aphasia. This disorder is marked by disorders of oral comprehension, verbal productions characterized by jargon or paraphasias. In some cases, the patient is difficult to channel, which can lead to a confusing diagnosis. A CT scan will help differentiate Wernicke’s aphasia from delirium.
3. Amnesic stroke. The patient suffers from isolated amnesia, of sudden and transient installation (a few hours). The other cognitive functions are intact.
4. Gayet-Wernicke encephalopathy. This vitamin B1 deficiency results in a confusional syndrome with hypersomnia and hallucinations, and concerns people who are undernourished. Early treatment, before neurological signs appear, is critical.
Clinical evaluation of delirium
The most important step in the assessment is to learn the history from an informed observer (eg, a family member or caregiver) and perform a brief cognitive assessment.
A brief cognitive screening should be done using cognitive screening tests such as the short MMSE or the Montreal Cognitive Assessment. If time is extremely limited, orientation assessment with an attention task provides a baseline screening.
The electroencephalogram (EEG) has limited sensitivity and specificity in the diagnosis of delirium. However, delirium is characterized by increased theta and delta activity.
The tools for assessing the severity of delirium are the Delirium index and the Confusional State Evaluation, while the main screening tool is the Confusion Assessment Method. Other neuropsychological tests are also useful: the mini-mental exam, the clock test, and the tracing test.
Given the high mortality rates, any suspicious or uncertain cases (including those with lethargy or unable to complete an interview) should be treated as delirium until proven otherwise. Initial management focuses on three concurrent priorities: (1) maintaining patient safety; (2) investigate causes; and (3) management of symptoms of delirium.
To maintain patient safety, efforts should focus on protecting the airway, maintaining hydration and nutrition, preventing falls, and avoiding restraints that increase the risk and persistence of delirium.
A complete medical check-up is necessary: blood sugar, drug dosage, vitamin B12, thyroid function test, albumin, sodium, potassium, magnesium, etc.
Treatments
Delirium must be treated because it increases morbidity and the risk of institutionalization and length of hospitalization.
1. Stimulation of the patient during the day, by encouraging him and facilitating references to his natural living environment. Avoid isolation which promotes sensory deprivation. Reassure the patient by speaking calmly. Avoid noise and commotion.
2. Pharmacological treatment with psychotropic drugs with sedative effects: Clometiazole, carbamates, antipsychotics without anticholinergic effects (haloperidol, risperidone, clozapine, quetiapine, benzamides). It is absolutely necessary to avoid barbiturates and benzodiazepines, drugs which are often the cause of delirium, except in the case of delirium due to withdrawal from alcohol or benzodiazepines.
It is preferable to prescribe psychotropic drugs in a single evening dose, in order to restore the sleep-wake cycle. Repeated treatment during the day can cause a paradoxical on-off phenomenon with sudden aggravation of symptoms.
3. Parental treatment with vitamins B1, B6 and PP in alcoholics.
4. Sleep a lot, stay well hydrated and eat well.
5. Ensure that the patient sees and hears well.
6. Make sure loved ones are present. They must be well informed about the disease.
7. Communicate succinctly.
Does delirium lead to dementia?
A major area of controversy is whether delirium is simply a factor in dementia vulnerability, or whether delirium itself leads to dementia. It is likely that both hypotheses are true. There is little doubt that the occurrence of an episode of delirium may reflect brain vulnerability and an increased risk of future dementia. In some cases, delirium can cause previously undiagnosed cognitive disorders to be treated. Delirium and dementia usually coexist, with dementia being a major risk factor for delirium, i.e. increasing the risk of delirium by 2-5 times.
A meta-analysis involving two studies with a total of 241 patients demonstrated that delirium was associated with an increased rate of dementia (+457%). In a sample of 225 cardiac surgery patients, delirium caused a severe one-time decline in cognitive functioning, followed by recovery over 6 to 12 months in most patients. However, a significant proportion, particularly those with prolonged delirium, never return to their original functioning. In 263 patients with Alzheimer’s disease, delirium caused an accelerated rate of cognitive decline after hospitalization. The decline is estimated to occur three times faster.
In summary
The confusional state (or delirium) is easy to overlook without a formal cognitive assessment. A brief cognitive examination can help identify delirium, with appropriate management. Additionally, older adults often take multiple psychoactive medications that increase the risk of delirium. Falling and loss of appetite are often warning signs.
Assess delirium in all older hospitalized patients: using a simple cognitive screening and confusional state rating scale. Be sure to get the history from someone close to the patient.
Reduce psychoactive drugs.
Use non-pharmacological approaches to manage sleep, anxiety, and restlessness.
Have pharmacological approaches for patients with severe agitation, who are at risk for self-harm, or who have psychotic symptoms (eg, hallucinations, delusional states).
Invite family members to care, especially for redirection and prevention of self-harm.
Encourage mobility.
Make sure patients wear their glasses, hearing aids and dentures. Being able to see, hear and eat is important.
Let patients know their schedule and keep them involved in their care. Communicate regularly with patients and their families.
Source: Sharon K et al. Delirium in elderly people. Lancet. 2014 March 8; 383(9920): 911–922.
Clinical case #1
Mrs. M, 71 years old, was diagnosed with Alzheimer’s disease (mild stage) after a physical examination and a cognitive performance evaluation (her Mini-Mental State Examination score is 22). In addition to medication for high blood pressure, Ms. M has been taking lorazepam 1 mg, 3 times a day, for more than 15 years to treat anxiety. Mrs. M has been getting more confused at home for a few days, and her daughter is taking her to her doctor for an evaluation. Recognizing that benzodiazepines can contribute to acute confusional state (delirium), the doctor stops the lorazepam. Three days later, Mrs. M’s confusion worsens and she develops nausea and tremors.
She is taken to the emergency room where she is admitted for benzodiazepine withdrawal, to find out if the delirium is caused by Alzheimer’s disease or the medication.
Although delirium is caused by several factors, drugs are frequent predisposing and aggravating factors and contribute to approximately 12% to 39% of cases of delirium.
Benzodiazepines and opioids are the drugs most often associated with an increased risk of delirium, as are drugs with strong anticholinergic properties (i.e. lowering acetylcholine in the brain).
In general, there are no strict rules on how to reduce and discontinue potentially risky medications. The steps to consider are:
Consider tapering and discontinuing benzodiazepines in a patient taking more than the minimum expected doses for at least two weeks, especially after eight weeks of treatment.
In the case of opioids, consider reducing them in a patient taking more than the minimum expected dose for more than a few days. When trying to eliminate symptoms of delirium, reduce opioids as quickly and as safely as possible, with a recommended reduction of less than 20% per day to prevent withdrawal symptoms.
The onset and duration of withdrawal symptoms depend on the half-life of a drug. Withdrawal occurs earlier when one stops taking drugs with short elimination half-lives (usually within 1-2 days), while it may not appear until 3-8 days after stopping drugs with a half-life greater than 24 hours.
Abruptly stopping a sedative – both hypnotic and opioid – can lead to intolerable withdrawal symptoms.
Benzodiazepine withdrawal should be avoided because of the risk of severe complications, such as seizures and delirium.
Withdrawal seizures are particularly common with alprazolam due to its short half-life.
Therefore, greater caution should be exercised when phasing out this drug.
In general, withdrawal from opioids or anticholinergics is not life-threatening, but should be done with caution.
Key points
Medications strongly implicated in delirium should be withdrawn or changed.
Consider the dosage and duration of treatment, the half-life of the drug, and the nature of withdrawal symptoms when determining how quickly the dose of a drug should be tapered.
A gradual reduction in activity over 2 or 3 days may be considered in hospitalized patients.
Clinical case #2
An octogenarian woman arrives at the emergency room for behavioral problems (agitation and visual and auditory hallucinations) accompanied by falls.
She is being treated for high blood pressure and has no psychiatric history. She is being treated with Plavix .
She has no confusion during the examination because she is aware of her hallucinations, which is the source of her anxieties.
Two diagnostic hypotheses are proposed:
A confusional state that occurs during dementia;
A confusional state with a transient organic cause.
The following additional examinations were requested:
A scanner having reported neither signs of hemorrhage or cerebral ischemia, nor of cerebral atrophy.
An electrocardiogram.
An electroencephalogram, which revealed heart rhythm disorders, with fluctuation of alertness.
A mini-mental exam score of 24 out of 30, suggesting cognitive decline.
No chronic mental illness according to the psychiatrist.
1st diagnosis _
A diagnosis of confusional state with hallucinations is made. Treatment with seresta (benzodiazepine) is provided.
The low MMSE score suggests the presence of mild cognitive decline .
An anomaly on the ECG suggests a heart problem, a hypothesis supported by the observation of an irregular heartbeat on the electroencephalogram.
A second, more in-depth electrocardiogram is undertaken.
2nd diagnosis
Medical staff diagnose:
Atrial fibrillation treated in particular with anti-vitamin K (anticoagulant) and an antiarrhythmic.
An enlarged left ventricle, characteristic of heart disease (heart disease is present in 70% of cases of atrial fibrillation)
This so-called paroxysmal atrial fibrillation (that is to say that it manifests itself in the form of an acute crisis for about a week and then ends spontaneously) leads to an acute confusional syndrome with a decrease in cerebral blood flow.
Onset of very moderate cognitive impairment with preserved autonomy.
Following the implementation of the treatments, no recurrence was observed.
