Dementia diagnosis situations in elderly patient are extremely frequent.
The prevalence of dementia and very important concerns, according to the latest estimates, approximately 800,000 French. Dementia is present the doctor as impairment of mental functions with memory impairment and decreased independence in activities of daily living.
DEFINITION AND DIAGNOSIS DEMENTIA:
definition:
The definition of insanity retained by the DSM-IV (4th version of the Diagnostic and Statistical Manual of Mental Disorders) was the basis for the realization of most of the scientific work and the establishment of the HAS recommendations.
This definition is based on three criteria:
– Evidence of disorders of the upper functions combining both memory impairment and at least one of the four disorders in aphasia, agnosia, apraxia and executive dysfunctions;
– Certainty that these altered higher functions represent a decline from a previous situation and the impact of all of these disorders on social or occupational functioning of the patient is significant;
– Exclusion of delirium as the cause of all troubles.
These three criteria make up what may be called the core insane. Finally, other criteria are related to the condition causing the dementia itself.
Memory disorders and premium features:
These disorders include always the weakening of memory capacity of episodic memory and semantic, recent or old, antegrade or retrograde, and achieving a higher functions among the praxis, the gnosis, language, or executive functions.
Poor memory:
If patient anosognosia, memory disorders can be initially apprehended by exploring the complaint expressed by the patient or the environment. Immediate memory can be efficiently explored through the ordeal of three words learned and recalled in the test of the MMS (Mini Mental State) or by asking the patient to recall the constituents of his last meal. Exploring the temporospatial orientation also gives a good idea of the state of immediate memory. The longer term can be explored for example by asking the patient to reconstitute the main elements of the health of its previous years.
Aphasia:
Language disorders can take many forms. First of all, there is a lack of the word that is very often a spontaneous complaint or the first and only complaint of the patient. This complaint is often referred to erroneously moment by the patient or the entourage of memory disorders. In the lack of the word, the doctor attempts to distinguish a difficulty in accessing lexical stock of impaired lexical stock itself. Naming tests and designation images or simple objects, help the distinction. Language disorders may also result in a loss of speech and a significant use circumlocutions in order to overcome the lack of words. written language disorders can also occur with dysgraphia and loss of syntactic and grammatical rules.
Do write a complete sentence to the patient, as in the test of MMS, is very important in the evaluation of higher functions. At an advanced stage of Alzheimer’s disease, disorders are similar willingly to those observed during a Wernicke aphasia type and the final stage, there may be a complete silence.
Apraxia:
Praxis is defi ned by the ability to perform more or less complex actions, with or without known objects or actions.
These deficits exclude motor deficits, while coordination disorder, disorder tone and any disorder related to abnormal movements.
Sensory loss, damage pyramidal, extrapyramidal, cerebellar ataxia, presence of tremor and other movement disorders such as chorea be excluded before concluding apraxia. Apraxia and primarily reflects an inability to perform a sequence of movements. It restricts usually patient autonomy in activities of daily living and requires the help of the entourage. When neuropsychological assessment, different types of apraxia will be sought.
Ideomotor apraxia:
It concerns actions not involving the manipulation of a utensil or tool. It may be symbolic gestures like using your hand to hitchhike or military salvation, not symbolic gestures as representing intertwined rings with your thumbs and index finger of both hands.
Ideational apraxia:
It relates the gestures involving the manipulation of a tool or utensil. The defi cit can thus affect the action of opening a lock with a key, to screw with a screwdriver or a fruit peel with a knife.
Dressing apraxia:
It can cause difficulties to tie a shoelace, tie a tie, buttoning a shirt or put on a sweater.
Constructive apraxia:
It results in difficulty represented by drawing a cube in perspective or to reproduce a complex geometric figure.
Agnosia:
The agnosia is the inability to recognize and identify an object with one of the senses, and this without any sensory defi cit. Thus, especially in the elderly, we must ensure that no hearing loss or visual impairment before asserting the existence of a hearing or visual agnosia. Exploring agnosia requires consideration of possible language disorders.Indeed, a patient who presents an object to be recognized can be bothered to name because of a language disorder prevents to find the expected name.
In the absence of language disorder, the patient may be unable to identify the object as an element known for its difficulty integrating sensory stimuli, visual in this example.
Aurally, agnosia preventing the patient to recognize a series of syllables as a phrase and words can mimic perfectly a language disorder and lead to misdiagnosis. The search for a agnosia is usually tricky especially as more frequent cognitive impairment are entangled. In patients with advanced dementia disorders with significant short-term memory, forgetting the setpoint delivered only seconds before another difficulty.
In general, it is useful to perform recognition tests with two successive presentations of the “target” associated with distracters, resembling the target for example.
Visual agnosia can result in an inability to recognize faces, even familiar.
Dysexecutive syndrome:
Executive functions are the ability to plan in time a complex task such as mental arithmetic, to complete a tax return sheet, or to arrange an appointment to see her doctor, for example. Difficulties in planning a task may involve the motor sequences and programming time. Thus the dysexecutive syndrome can result in severe complications for dressing, grooming or preparing a meal. Executive functions also include all of volition with motivation and initiative for action. A dysexecutive syndrome goes easily unnoticed if it is not systematically searched. Scales such as rapid battery evaluation of executive functions (BREF) are being validated on large samples, and its routine use in routine is probably relevant.
Progression of cognitive disorders and their impact:
The key is to establish the novelty of these disorders of the upper functions. His absence is very often an obstacle for a diagnosis of dementia that would differentiate these disorders eg an old psychiatric illness such as schizophrenia or a congenital condition such as hypothyroidism. A history made with the environment used to document the existence of these conditions.
In addition, these cognitive disorders should have resulted in a significant impact on the social or professional activities.
The patient in full professional activity in difficulties in achieving work, a lack of promotion, repetition of penalties, failures or even dismissal.
On the social level, the patient may experience marital difficulties and see its surrounding social fabric is disintegrating gradually.
We must also search for discontinued operations because of memory problems or superior functions. These varied activities can be cultural, artistic or entertainment. Typically, patients with Alzheimer’s disease can not, from the early stage, follow the daily reading of a book, oblivious to progressively reading the previous pages. These latter points are particularly difficult to establish when one is faced with very elderly patients tend to be progressively excluded from social connection to their age. Nevertheless, these elderly patients can also drop some activities due to disabilities such as trouble walking, vision, hearing, or even during a hard-felt mourning. It is then able to distinguish from the reduction of activities related mainly to the evolution of possible dementia.
At a more advanced stage, activities hampered by these disorders are the most basic activities of daily living such as those that allow an autonomous social life: making shopping, managing a heritage, a domestic budget, using a telephone, travel arrangements or a receipt, keep a proper interior clean condition, etc. These activities can be easily assessed by the IADL scale (Instrumental Activities of Daily Living) Lawton. Finally, the basic activities of daily life, capacity to ensure one’s own personal hygiene and diet will be useful and easily evaluated by ADL scale (Activities of Daily Living) Katz. In an ultimate degree of dementia, the patient may become incontinent and bedridden.
The progression disorders can be relatively easy to document. Just in general to refer to the patient’s level of education, its academic or professional qualification to certify that the observed disorders represent a decline from a previous situation. However, in case of former psychomotor disability or old psychotic condition, the notion of decline is much more difficult. The repetition of assessments, with an annual frequency as recommended by the HAS, can highlight this increase in disorders and sign a progressive disease process.
Excluding a delirium:
Before you can say that all the troubles, its evolutionary characteristics and scale, is explained by a dementia syndrome, the hypothesis of delirium as the principal diagnosis should be lifted. Indeed, the delirium, responsible for higher functions and disorders affecting willingly identical to that population affected by dementia syndromes differs from the latter by the presence of impaired consciousness, as well as its evolutionary mode. The onset is often sudden and evolution marked by fluctuations. Its resolution is finally upon systematic disappearance of the cause of delirium.
However frequently, especially in hospitalized patients, these two syndromes may coexist, the dementia is one of the conditions conducive to the occurrence of delirium. One challenge is then to diagnose since firstly to confirm the diagnosis of dementia according to DSM-IV delirium as a principal diagnosis should be excluded, and secondly, to make a diagnosis of syndrome confusion according to the same DSM-IV, it is necessary to say that the observed disorders are not due to the evolution of a dementia syndrome. The importance of a rigorous examination and a medical history is fundamental in these patients. This highlights the interest of a geriatric followed in which a balance of higher functions is performed, and if appropriate, a diagnosis of dementia posed.
The demented core is thus formed by these initial criteria: impairment of mental function and memory, a significant impact of these disorders, the exclusion of a delirium. Then the different etiologic diagnoses may be considered.
Other etiologies and diagnostic criteria:
These criteria will be considered depending on the diagnosis.
Alzheimer’s disease:
For the diagnosis of Alzheimer’s disease will require more of the dementia core join three other criteria (Box 1):
Box 1. Diagnostic Criteria for Alzheimer’s disease according to DSM-IV
1. The development of multiple cognitive deficits, as evidenced by both:
A. a memory impairment (impaired ability to learn new information or to recall previously learned information)
B. one (or more) of the following cognitive disturbances:
– Aphasia (language disturbance)
– Apraxia (impaired ability to perform a motor activity, despite intact motor function)
– Agnosia (inability to recognize or identify objects despite intact sensory function)
– Disturbance in executive functioning (making plans, organizing, sequencing time, abstracting)
2. The cognitive deficits in criteria 1A and 1B are both the cause of significant impairment in social or occupational functioning and represent a significant decline from a previous level of functioning
3. The course is characterized by gradual onset and continuing cognitive decline
4. cognitive deficits challenge criteria 1A and 1B are not due:
A. other central nervous system that can lead to memory deficits and cognitive functioning (cerebrovascular disease, Parkinson’s disease, Huntington’s disease, subdural hematoma, normal-pressure hydrocephalus, brain tumor)
B. general conditions that can cause dementia (hypothyroidism, folate deficiency, B12, pellagra, hypercalcemia, neurosyphilis, HIV infection)
C. to drug or toxic substances.
5. The deficits do not occur exclusively during the course of a delirium (confusion)
6. The disturbance is not better accounted for by a mental disorder (major depressive disorder, schizophrenia)
– The first of these three criteria is an evolution of disorders characterized by gradual onset and continuing cognitive decline;
– The second criterion is the exclusion of certain pathologies. These are the central nervous system such as cerebrovascular disease, Parkinson’s disease, Huntington’s disease, a subdural hematoma, normal pressure hydrocephalus or brain tumors. These conditions can also be such a general disease as hypothyroidism, vitamin B12 or folate, a pellagra, hypercalcemia, neurosyphilis, infection HIV (HIV) or a disease induced a drug or toxic substance;
– The third criterion is to show that the entire clinical picture can not be better explained by a psychiatric disorder such as schizophrenia or major depressive disorder.
Other diagnostic criteria defi ned in 1984 by NINCDS–ADRDA (National Institute of Neurological and Communicative Disorders and Stroke-Alzheimer’s Disease and Related Disorders Association) allow to consider the diagnosis
of Alzheimer’s disease according to three different degrees of certainty. We talk about Alzheimer’s “possible”, “probable” and “some”. The diagnosis of Alzheimer’s disease “some” is worn with a confi rmation pathological brain, to which we must associate the clinical history and diagnosis of probable Alzheimer’s disease.
The diagnostic criteria for DSM-IV and NINCDS–ADRDA are now routinely used in all biomedical research. The major drawback of these criteria is impossible, from these, to bring an early diagnosis of Alzheimer’s disease, including a “preclinical” phase during which only abnormalities on neuropsychological examination, which come in addition to memory complaints, are strong arguments to talk about Alzheimer’s disease. The balance sheet at this stage does not usually detected any impact and then it is not possible to speak of dementia, while the patient may have a genuine Alzheimer’s disease at a stage “pre-dementia”.
Vascular dementia:
According to the DSM-IV, to lead to a diagnosis of vascular dementia, the only necessary additional criterion is the presence of symptoms and focal neurological signs. Are considered as an exaggeration of the tendon reflexes, a cutanéoplantaire reflex extension, pseudobulbar palsy, gait disturbances, weakness of the extremities, or the demonstration by neuroimaging cerebrovascular disease with the presence multiple infarcts in the cortex and subcortical white matter (Box 2). The term vascular dementia are met actually several pathologies since includes holders of multi-infarct patients, diffuse gaps in subcortical regions, or a CADASIL disease, genetic condition.
Box 2. Diagnostic criteria for vascular dementia according to DSM-IV
1. Emergence of multiple cognitive deficits challenge, as evidenced by both:
A. a memory impairment (impaired ability to learn new information or to recall previously learned information)
B. one (or more) of the following cognitive disturbances:
– Aphasia (language disturbance)
– Apraxia (impaired ability to perform a motor activity, despite intact motor function)
– Agnosia (inability to recognize or identify objects despite intact sensory function)
– Disturbance in executive functioning (making plans, organizing, sequencing time, abstracting)
2. The cognitive deficits in criteria 1A and 1B are both the cause of significant impairment in social or occupational functioning and represent a significant decline from a previous level of functioning
3. Demonstration of focal neurological signs and symptoms (exaggeration of ROT, Babinski, pseudobulbar palsy, gait disturbances, weakness of an extremity) or highlighted by the diagnostic tests cerebrovascular disease (multiple infarcts on the cortex and white matter subcortical) causally related judged with dementia
4. The deficits do not occur exclusively during the course of a delirium (confusion)
Dementia with Lewy bodies:
This is a condition characterized by the combination of a dementia syndrome with a neuropsychological profile both cortical and souscortical very fluctuating an extrapyramidal syndrome and hallucinations. This makes for an entity that approaches an association between Alzheimer’s disease and Parkinson’s disease. The diagnostic criteria are based on those defined by McKeith in 1996 (Box 3).
Box 3. Diagnostic criteria of McKeith for dementia with Lewy bodies diffuse
Essential for diagnosis
progressive cognitive decline of sufficient severity to interfere with social or occupational activity
memory disorders, transient or permanent, may miss early in the disease, but occur during evolution
Attention disorders and visuospatial abilities, and a sub-corticofrontal syndrome may dominate
potential illness: one or two of the following; Probable disease: at least two of the following
intellectual functions fl uctuantes, with pronounced variations in attention and alertness
recurrent visual hallucinations, typically rich
spontaneous parkinsonism
Other diagnostic evidence supporting the diagnosis
repeated falls
syncopations
transient loss of consciousness
Sensitivity to neuroleptic
systematized delusions
non visual hallucination
The diagnosis is unlikely
In the presence of stroke (focal clinical or brain imaging).
In the presence of a somatic disease or other brain disease that could explain the symptomatology
In the presence of dementia core, the diagnosis of diffuse Lewy body dementia is likely if at least two of the following three criteria are met:
– The first criterion is the presence of parkinsonism;
– The second is the presence of fluctuations in cognition with variations in attention and alertness;
– The third, the presence of recurrent visual hallucinations.
Only one of these three criteria associated with dementia t enough to pose the possibility of a diagnosis of dementia with Lewy bodies.
Other symptoms for diagnosis.
This is the occurrence of repeated falls, syncope, brief loss of consciousness, a frank sensitivity to neuroleptics, systematized delirious ideas and hallucinations other than visual.
Finally, the presence of a stroke argues against the diagnosis.
The memory problems may be subtle early in evolution while attentional cit challenge is often greater. On the other hand, visuospatial abilities are altered early. Parkinsonism is willing bilateral, insensitive to L-DOPA, usually composed of bradykinesia and a hypertension that quakes. Evolution is typically progressive and can be glazed by accident when prescribing antipsychotic motivated by frequent visual hallucinations. Indeed, the poor tolerance of neuroleptics, sometimes fatal, is very characteristic of the disease.
Frontotemporal dementias:
It is a neurodegenerative dementia associated with a progressive atrophy of the frontal lobes, the anterior portions of the temporal lobes and basal ganglia. The expression of this pathology is essentially of psycho-behavioral disorders.Cognitive disorders are not in the foreground. The diagnostic criteria currently in force are derived from those of Lund and Manchester and grouped by Lebert and Pasquier into four disorders (Box 4)
Box 4. frontal malfunction scale for the
diagnosis of frontotemporal dementia (Lebert and according
Pasquier, 1998)
self-control disorders (grade 1 or 0)
overeating
alcoholic behavior
verbal disinhibition
behavioral disinhibition
Irritability, anger
Control Disorders emotions: laughter or tears
restlessness
Physical neglect from previous patterns (grade 1 or 0)
Personal hygiene
Clothing (harmony, cleanliness, indifference to the tasks)
Hair (cut, cleanliness)
Mood disorders (grade 1 or 0)
apparent sadness
emotional indifference
hyperemotivity
Exaltation
Manifestations of decreased interest (grade 1 or 0)
daytime sleepiness
Apathy
social disinterest
perseverations ideational
Each symptom must represent a change from the previous character.
This symptom causes a scoring 1 for the category to which it belongs. A total score of at least 3 of 4 is very supportive of a diagnosis of frontotemporal dementia for patients with mild dementia (MMS> 18)
– The first category includes self-control disorders, with alcoholic behavior, irritability, behavioral disinhibition or verbal;
– The second category covers disorders with physical neglect hygiene and keeping defective with respect to habits;
– The third category is represented by mood disorders, with emotional indifference, an exaltation, an arousal or an apparent sadness;
– The fourth category of disorders is a declining interest characterized by daytime sleepiness, social indifference, apathy or ideational perseveration.
In a patient whose score Mini Mental- State is greater than 18, the presence of an element of at least three of the previous four categories is very supportive of the diagnosis of frontotemporal dementia.
Prion diseases:
These neurological diseases characterized by the accumulation of PrPsc protein, derived from its precursor, PrPc.Several forms are distinguished with mostly sporadic, representing over 80% of cases, genetic forms, and more rarely, communicable forms. Furthermore Creutzfeldt-Jakob disease (CJD) is the most widespread, we must note the emergence of variant CJD, which is from the transmission to humans of bovine spongiform encephalopathy. It is likely that we are witnessing an outbreak of plague whose predictable magnitude is not well known. Variant CJD differs from the classical form between each other by a middle-aged younger at death (29 years against 66 years for CJD) and a duration of average change of 14 months against 4 months. Clinically, the variant CJD is characterized by disorders psychobehavioural with dysphoria, anxiety, instability, insomnia and apathy, gait disturbances, extrapyramidal syndrome, incontinence, visual disturbances, and a terminal stage, akinetic mutism.
DIAGNOSIS ETIOLOGICAL:
A detected dementia, then consider the etiological diagnosis. This will be based primarily on an accurate history, documented history, clinical examination and neuropsychological explorations.
The different diagnoses to consider and their main mechanisms are shown in Box 5.
Box 5. Main dementia diagnoses
degenerative dementia
Alzheimer’s disease
Dementia with Lewy bodies
frontotemporal dementia
Lobar atrophy focal
corticobasal degeneration
Multiple system atrophy
Progressive supranuclear palsy (Steel disease
Richardson Olszewski)
dementia arteriopathy
CADASIL
Binswanger disease
Infarcts in strategic areas
State lacunar
multiinfarct
infectious dementias
Syphilis
progressive multifocal leukoencephalopathy HIV
Prion diseases:
– Creutzfeldt-Jacob
– Variant Creutzfeldt-Jacob
– Disease Gerstmann-Straussler-Scheinken
vitamin deficiencies
Vitamin B12 deficiency
Deficiency of vitamin B9
general diseases
Hypothyroidism
hyperthyroidism
hypercalcemia
Other
Normal pressure hydrocephalus
chronic subdural hematoma
Differential diagnosis
Depression
delirium
background:
The antecedents are of major importance for a diagnosis of dementia. Indeed, the number of Alzheimer’s disease, frontotemporal dementia or diffuse Lewy body dementia have a family nature, and the existence of dementia in a family member may be the element that motivated the patient to consult.
There are exceptional families where the prevalence of Alzheimer’s disease is very important.
This is called autosomal dominant forms associated with gene mutations, some of which are known. Moreover, the homozygous epsilon-4, encoding apolipoprotein E, is associated with an increased risk of developing Alzheimer’s disease from 8 to 30 times compared to those with no epsilon-4 allele. According Daw, patients homozygous for the epsilon 4 allele commence their Alzheimer’s disease almost 18 years before the patient whose genotype is epsilon-2 / epsilon-3.
The frontotemporal dementias have an important genetic support linked to chromosome 17. These forms are associated with age at onset of disease relatively young, with an extrapyramidal syndrome and to a larger family character than for other forms of frontotemporal dementia.
Some dementias prion such as Creutzfeldt-Jakob also the subject of rare familial descriptions.
Personal history is also important to consider. The complete inventory of all cardiovascular risk factors, high cholesterol, smoking, high blood pressure or diabetes, helps when at questioning of vascular dementia.
Psychiatric history such as episodes of depression, the accuracy of their mode of evolution and the age of their occurrence, are also important to address. An old psychotic illness, bipolar disorder, for example, or an aged paranoid schizophrenia can also cause symptoms that motivate a consultation for memory disorders. On the other hand, frontotemporal dementias willingly reveal a psychiatric inaugural table in appearance that can combine multiple front-end problems such as disinhibition, irritability, aggressiveness, a dysexecutive syndrome with loss of mental flexibility, while memory problems are minor or absent.
Physical examination :
Several items will be searched systematically besides the already mentioned neuropsychological disorders. This is such myoclonus or convulsive episodes whose presence makes evoke a Creutzfeldt-Jakob disease or Alzheimer’s disease at an advanced stage. Looking for orthostatic hypotension is a case for multi-system atrophy diagnosis, including Shy-Drager syndrome. Hypotension can be the source of severe multiple falls marking even frequently the disease onset. An oculomotor deficit on upward gaze fact evoke progressive supranuclear palsy.
A frontal lobe syndrome evidently evokes a frontotemporal dementia but also Alzheimer’s disease especially after a long evolution.
It also should not deviate too hastily progressive supranuclear palsy or vascular dementia. Piriformis syndrome to suspect vascular dementia but remains consistent with Alzheimer’s disease at an advanced stage. A cit systematized neurological challenge is one of the signs that can retain the diagnosis of vascular dementia provided they can attribute all or most of the problems constituting the dementia observed with vascular lesions seen in brain imaging.
The sign most “profitable” for the clinician is undoubtedly the extrapyramidal syndrome whose presence makes evoke a range of pathologies despite its possible appearance in an authentic Alzheimer’s disease. Indeed, Parkinson’s disease can obviously be marked in its evolution by the occurrence of a true dementia. We simply distinguish a diffuse Lewy body disease whose differential diagnosis is always difficult. Typically in dementia with Lewy bodies diffuse the onset of dementia follows that of extrapyramidal syndrome of less than a year. This feature allows to use the criteria of McKeith for diagnosis.
However, a recent review of these criteria allowed to admit a lag longer time between the occurrence of these two syndromes. In the disease Lewy bodies, extrapyramidal symptoms are typically symmetrical and insensitive to L-DOPA in contrast to Parkinson’s disease.
Similarly, the detection periods “onoff” under L-DOPA sign rather the diagnosis of Parkinson’s disease. Highlighting hallucinations, particularly visual, moving towards the diagnosis of dementia with Lewy bodies.
It must then be wary of the occurrence of hallucinations in the treatment of Parkinson’s disease with L-DOPA.
The impact of hypertensive heart disease in target organs such as the retina, kidneys or the brain is an important argument for the diagnosis of vascular dementia.
The identification of these elements also help with start-up decision of a secondary preventive treatment. Even if the treatment of vascular dementia today is not codified, these are often accompanied by other cardiovascular disease that will certainly deal.
Depression is common in diseases accompanied by a dementia syndrome. In Alzheimer’s disease, for example, this one is truly present half the time.
The mechanisms are not well known. These may be both of the land on which more easily develop Alzheimer’s disease, the absence of anosognosie in other patients causing a depression in response to the finding of cognitive disorders or disorders in psychobehavioural compared with limbic reached. Anyway, it is of major importance to recognize depression in these patients and the risk of suicide because the impact on the environment is therefore probably important and depressive symptoms significantly involved in patient discomfort .
Neuropsychological assessment:
It aims to highlight and clarify memory disorders, language disorders, apraxia, agnosia and executive function disorders. This initial assessment should be systematic and directed by a psychologist with special expertise in neuropsychology.
It helps to clarify the patient’s personality traits and integrate in the observed disorders, which result from both brain damage present and previous character traits. To date, there is no consensus on the various tests to be performed as part of a diagnosis of dementia, but some tests are frequently carried out.
A change of character is usually filled by those close to the patient.
This change in character sometimes is the most light symptom even as memory impairment may be at the same time moderate or absent. Similarly, the MMS can be normal and falsely reassure the doctor in the presence of a patient whose socio-cultural level and education level are high. This change of character is also often the cause of suffering for those around who feel rub a very different person.
These character changes may also result in reaction, a change in behavior of the family or professional entourage no apparent reason.
Paraclinical data:
Different conditions cause a dementia syndrome does not translate into a specific biological anomaly. It has no useful marker in routine clinical. The standard biology has two main roles. The first is to look for arguments to a delirium which can be itself an expression of anemia, or a dysnatrémie dyscalcemia. The second reason to practice is standard laboratory tests to detect the maximum the different comorbidities associated with neuropsychiatric disorder.
Indeed, comorbidity probably has a poor outcome in the presence of dementia. Similarly, it is likely that one or comorbidities are derogatory factors in terms of evolution of dementia.
Recommended examinations are complete blood count, the electrolytes, serum calcium, TSH, albumin and blood sugar. Other assays are performed according to the clinical context, eg liver function tests, HIV serology, a syphilis serology and lumbar puncture. Vitamin B12 and folate assays are classic, but in practice, it is exceptional that a vitamin B12 deficiency or folate is the cause of true dementia. Recently, a strong link between hyperhomocysteinemia and the onset of Alzheimer’s disease has been demonstrated, but the systematic dosage of homocysteine is not currently part of the recommendations.
Brain Imaging:
Brain scan:
Any alteration of higher functions motivates almost automatically performing a brain imaging, a dementia syndrome has already been diagnosed or not. The purpose of this imaging is twofold. First, it is sometimes remove a curable disease such as a subdural hematoma, normal pressure hydrocephalus or brain tumor. Moreover, this imagery can sometimes help the etiological diagnosis with the identification of multiple infarction, myocardial single serving in a strategic area, abnormalities of white matter in favor of cerebrovascular disease, or localized atrophy or not.
On the HAS working group recommended the implementation of a brain imaging that is at least a CT scan without injection, and at best a magnetic resonance imaging (MRI), nuclear. Most of the time, a brain scan without iodinated contrast injection is enough.
However, when a vascular disease is suspected, an MRI is systematic in order not to disregard lacunar ischemic lesions. Finally, in many cases, initial imaging is sufficient and repeat later adds nothing to the etiological diagnosis.According to these recommendations, there is no need to repeat these exams. The extent of brain imaging in the diagnosis of dementia approach remains limited since it never brings a positive diagnosis. The detection of diffuse cortical atrophy can in no case allow a diagnosis of Alzheimer’s disease.
MRI:
This examination is recommended by the HAS in the diagnostic process of dementia syndromes.
The key elements for achieving this examination are essentially the ease of vascular ischemic lesions are identified and safety in the absence of indication-cons. The main limitations of MRI are sometimes limited access, the relatively high cost and finally anxiety induced by the harsh conditions of the examination in the patient with superior functions disorders. Indeed, claustrophobic patients can become a major obstacle. Localized atrophy seahorses temporal and entorhinal cortex is an important argument for the early diagnosis of Alzheimer’s disease as well as the discovery of a predominant atrophy in the frontal lobes will confirm a diagnosis of frontotemporal dementia.
Instead of other imaging techniques and functional tests:
The HAS recommendations refer to techniques such as PET scans, SPECT, fMRI, EEG quantified as diagnostic tests not recommended in clinical practice, but reserved primarily for research. Functional studies of patients with lesional processes such as Alzheimer’s disease, also provide fundamentals on understanding the normal functioning of the human brain.
Lumbar puncture:
The realization of a lumbar puncture before a dementia syndrome is justified to date as in the case of a neuromeningeal suspected infection such as syphilis, or in the case of a suspected disease Creutzfeldt-Jakob disease.In the latter case, 14-3-3 is sought systematically while having in mind that this research can be positive in such situations that Alzheimer’s disease or a stroke.
Genetics:
Genotyping of apolipoprotein E is not recommended by the HAS as a screening test.
It is made in the context of biomedical research. Its systematic fulfillment would create significant ethical problems, especially against diseases with curative treatments are zero
Brain biopsy and necropsy:
Achieving a cerebral biopsy in living patients is exceptional and reserved for young patients for whom a clinical diagnosis is difficult or insufficient. The practice of autopsies is today largely neglected while a confirmation neuroanatomical help the clinician specifically in clinical skills, an accurate diagnosis can be useful for the offspring, especially if a family risk exists, finally, that his contribution to biomedical research is very relevant.
BOOM SYNDROME DEMENTIA:
The problems of the clinician and the patient does not stop at the etiologic diagnosis of dementia. Whatever the disease in question, let alone where geriatric Alzheimer’s disease, dementia with Lewy bodies, frontotemporal dementia and diffuse are the most commonly discussed diagnoses, the repercussions and complications should be systematically studied and screened to you can initiate a quality management.
Behavioral and psychological symptoms:
Psychological or behavioral symptoms, called it a few years ago behavioral disorders are common regardless of the diagnosis and evolutionary stage. Early onset of behavioral problems may be the only clinical sign and can be a mode of entry into dementia.
In mild Alzheimer’s disease to moderately severe, these disorders affect about 85% of patients. For frontotemporal dementias, it is not only the main mode of revelation but also the symptom that predominates throughout the course of the disease, even as memory impairment may be mild or absent. Vascular dementias are also characterized by the intensity of behavior problems. Generally, any pathology involving an attack of the frontal lobes, afferent or efferent pathways of the frontal structures such as striatofrontales connections, is accompanied by behavioral disorders. This is mostly for the adjustment difficulties of patient to the environment, when changing the place of living (residential or hospital room change) or interpersonal environment.
A healthy person adapts the gains made most often during childhood.
These achievements are such modesty,
politeness, attitudes to food.
At the front patient it is as if these gains were lost or forgotten. Thus reactions of aggressiveness can develop when the triggering event should be all the more irritating to a healthy subject.
These behavioral problems can be assessed by a tool such as the NPI (Neuro-Psychiatric Inventory). The NPI and evaluate the frequency, severity and impact on the environment of 12 different behaviors. These behaviors are pathological depression / dysphoria, sleep disorders (insomnia or hypersomnia), appetite disorders (anorexia or bulimia), apathy / indifference, aggression / agitation, delusions, hallucinations, aberrant motor behaviors, anxiety, disinhibition, irritability / lability, exaltation of mood / euphoria.
Mood disorders depend on the relationship between the frontal structures and limbic structures. These disorders are frequently associated with a syndrome with someone dysexecutive particular volition and motivation process. These behavioral disorders are closely related to the environment and entourage. ‘Educational’ programs for the entourage often possible to reduce behavioral problems while on the contrary therapeutic such as neuroleptics can have very modest effects seen zero in view of the adverse effects that often result.
The assessment of behavioral disorders is a full component of the care of these patients, since these disorders can increase a patient’s suffering, resound heavily on the environment, cause a loss of autonomy for the activities of daily life, cause repeated hospitalizations, an iatrogenic when neuroleptics are prescribed and even a risk of institutionalization.
Loss of independence with activities of daily living:
The loss of autonomy is the inability to realize oneself the daily life activities.
These activities can be distinguished in elementary acts such as toileting, dressing, toileting, moving unaided, eat and be continent, or more elaborate such as instrumental activities activities.
It may be then the capacity to go shopping, to handle money, perform household chores or properly use a phone.
Participate in this frail various disorders of cognitive functions such as progressive impairment of praxis, impaired executive functions that make it difficult task scheduling in time, memory disorders that hinder the patient to go shopping, the language disorders that prevent proper use of the phone or the occurrence of agnosia.
The loss of autonomy rarely spontaneously identified by the environment can be a major pain for the patient. Often unnoticed also for the patient, it is even denied the most long anosognosiques such as subjects with Alzheimer’s disease.
It is unfortunately too often accepted that loss of independence is one of the inevitable consequences of aging.
Yet she remains totally abnormal and allows to differentiate, for example, memory problems simply age-related and qualified so benign, a true dementia.
Thus, the Bordeaux Paquid identified four activities for which simultaneous involvement was highly predictive of Alzheimer’s disease. It was the ability to use a phone to use public transport, to manage his medication, and manage its budget and current affairs.
accurately determine the degree of autonomy of an elderly patient with tools such as the Katz ADL and IADL Lawton is essential in any geriatric assessment. If this loss of autonomy is insufficiently anticipated and mitigated, it is a source of acute crises and emergency hospitalizations. Finally, all dementias evolve towards a progressive and total loss of autonomy.
Suffering of the entourage:
The suffering of those around the dementia is very common. The reasons are multiple although poorly identified to date.
This pain may be the result of the alteration of the character and personality, the appearance of behavioral disorders, loss of independence with activities of daily living, social repercussions of the disease. This pain may result from medical-decisions for the patient, such as the introduction of drug treatment to treat behavioral disorders, institutional placement.
This suffering is finally a source of abuse. The impact of the disease on the environment can be measured and monitored by means of the scale of Zarit.
It is well established that the person identified as “primary caregiver”, within or outside the family, is an increased risk in terms of morbidity as mortality.
For these subjects, the prevalence of depression is greater than in the rest of the general population.
It is also likely that the suffering of the primary caregiver influences the behavior of the demented patient with such symptoms like depression, agitation or aggression.
Identify this suffering and try to reduce it is fully part of the care of the patient, or more precisely the main patient-caregiver couples. This needs to be clearly envisaged from the diagnostic phase.
A pathology such as frontotemporal dementia where behavioral disorders predominate, is associated with a particularly significant suffering for the entourage, with sometimes tragic consequences such as divorce or abandonment.
Undernutrition:
Undernutrition that can develop in patients with dementia is an important element in more ways than one. This malnutrition is a particularly high prevalence in Alzheimer’s disease but also in most dementia without the mechanisms involved are clearly identified. One of the causes can be eating disorders, then identified as a behavioral disorder.
It can be also consequences of the loss of autonomy making impossible the effectuation of shopping or preparing a meal. In Alzheimer’s disease, hypercatabolism inherent in the disease is not completely excluded even if the recent literature does not formally plead to this mechanism.
This malnutrition is clearly part of the problem of comorbidity and frailty in dementia with all the ensuing difficulties, both for identification and for its management. Track the risk of malnutrition using a tool such as the Mini Nutritional Assessment (MNA) is so important in the care of such patients, malnutrition is associated with a poor outcome. The early screening and dietary intervention can prevent the onset of malnutrition.
Comorbidities:
Take stock of comorbidities, particularly in older individuals, fragile and multiple pathologies, is essential during the balance of a dementia syndrome. Indeed, dementia associated with decompensation of another chronic condition is very often the cause of delirium resulting in support for decompensated pathology heavier, increasing the length of stay, morbidity and increased mortality, and decreased independence in activities of daily life that persists even remotely of the confounding episode. Moreover, the simultaneous existence of another disease with dementia is probably many examples an aggravating factor the evolution of dementia.
TERMS OF MANAGEMENT:
The management of dementia is at once global and fits in time, in order, until the patient’s death. It therefore directly affects the general practitioner and geriatrician.
The support includes both the own evolution of the dementia syndrome, the occurrence of complications and its repercussions, the crises, in order, marked by the appearance of delirium, the decision of social measures such as institutionalization, and management of comorbidities.
Getting drug load of dementia:
Specific drugs:
To date, only the Alzheimer disease benefits from the existence of specific drug treatments covered by the authorization to place on the market two drug classes: the cholinesterase molecules and inhibitors of the nicotinic receptors.
These two drug classes have been many clinical trials. These have shown efficacy on patients in the evolution of cognitive disorders, loss of independence in activities of daily life, and evolution of behavioral and psychological symptoms. Studies on the delay in the decision of institutionalization on the financial impact of the management and reduction of suffering of caregivers exist, but are still too few to conclude a net profit of these molecules on these nevertheless essential to the management.
On the one hand, the use of these molecules is marked by the occurrence of quite significant adverse effects on the individual level. Moreover, the high cost of these drugs requires continuous reflection of the therapist about their prescription.
Nevertheless, the fact that they are the only drugs specifically indicated in Alzheimer’s disease makes them essential requirement in the treatment of these numerous patients.
Cholinesterase inhibitors are the most prescribed drugs because they are given at the earliest stage of the disease, regardless of the observed shape. These drugs are donepezil, rivastigmine and galantamine. Their introduction should be graded challenge to limit the occurrence of adverse effects and increase patient compliance. Their side effects are mainly gastrointestinal with nausea, vomiting, transit disorders (diarrhea mainly), abdominal pain.
They can also cause sleep disturbance and agitation episodes.
A research conduction disorders and cardiac rhythm should be performed, and if appropriate, the approval of a cardiologist should be obtained to allow their prescription. To facilitate progress doses, they are marketed in several dosages.
Donepezil (Aricept):
There are tablets 5 mg and 10 mg. He initially prescribed at 10 mg per day taken once daily. The said therapeutic dose of 10 mg is obtained after at least 4 weeks of a prescription 5 mg / d. Some trials showed efficacy for 5 mg / day.
Rivastigmine (Exelon):
It combines its action on cholinesterase action on butyrylcholinesterase, which gives it after the pharmaceutical company an advantage over competing drugs.
However, this action is a misjudged clinically interest. Finally, recent clinical trials have shown clinical efficacy in patients with dementia with Lewy bodies diffuse, and more recently on patients with dementia associated with Parkinson’s disease. An extension of its marketing authorization for dementia associated with Parkinson’s disease (Parkinson’s disease and dementia with Lewy bodies) is expected very soon. It exists for the drug four strengths: 1.5 mg, 3 mg, 4.5 mg and 6 mg. He prescribed one gel./2x/j. The therapeutic dose is in order at least 9 mg / day and most preferably 12 mg / day.
Dose escalation is done every 4 weeks. Some studies suggest efficacy of the molecule at 6 mg / day.
Galantamine (Reminyl®)
It exists in three different strengths: 4 mg, 8 mg and 12 mg. He prescribed one tablet morning and night. The therapeutic dose is 16 mg / day and most preferably 24 mg / day. Dose escalation is done in steps of 4 weeks also.
Clinical trials have evaluated the efficacy of these three drugs over a period of at least 12 weeks, and most often 24 weeks.
This efficiency is judged primarily on the performance of cognitive functions. But there is also an efficiency of behavioral disorders and declining autonomy for activities of daily living. Thus, the action of behavioral disorders can be beneficial in the sense that the joint limitation neurotropic such as antidepressants, anxiolytics and neuroleptics may be reduced or stopped, which is an advantage given the adverse effects of these drugs.
The effectiveness of these molecules on these elements makes decisions more difficult because doctor before judging these molecules in the patient, must be clearly defined treatment goals.
Memantine (Ebixa):
It is the only drug in the class of inhibitors of the nicotinic receptors. Its authorization for the market exists for forms of Alzheimer disease stage moderately severe to severe. It exists in two dosage forms: 5 mg and 10 mg. He prescribed one tablet morning and night and increasing doses is also done after 4 weeks of treatment with 5 mg morning and evening. This increase can only be done if there is kidney failure, and if it is severe memantine is against inappropriate. The effectiveness of this molecule exists on cognitive function and behavioral disorders.
Specific drug prescribing rules:
Cholinesterase inhibitors are given from the stage “mild” Alzheimer’s disease. Their effectiveness is evident over a long period of several years. However, a semi-annual assessment must be made to seek inefficient prescribed cholinesterase inhibitor, which exists in almost 40% of patients.
If appropriate, the interruption of the administered molecule must be pursued by prescribing another drug anticholinestérasique.
The discussion of treatment should be renewed especially in case of institutionalization of the patient, since treatment goals change on this occasion. In institutions, their prescription may be motivated to maintain patient autonomy in activities of daily life and to treat behavioral disorders.
Memantine may be prescribed in combination with a cholinesterase inhibitor to increase the effectiveness of cognitive and behavioral disorders.
However its authorization for the market exists for moderately severe to severe forms.
Finally, when dementia is very advanced, ethical discussion should be conducted as regards the interruption of cholinesterase inhibitors and memantine, in order not to unnecessarily expose the patient to frequent side effects against the benefits generally limited at this stage of the disease.
Non-specific drugs:
The treatment of dementia and Alzheimer’s disease in this case is not limited to the so-called specific drugs.
In many cases, neurotropic such as anxiolytics need to be prescribed. There is no rule as to the choice of a benzodiazepine for short or long half-life. But the side effects of benzodiazepines are numerous and frequent in these patients and their prescription is subject to increased surveillance.
Depression is commonly associated with Alzheimer’s disease and other dementias, and must justify the prescription of an antidepressant. Because of the very common side effects and many tricyclic, they are avoided to the maximum.The choice of an inhibitor of the serotonin receptor is in this case much better. Efficacy of these molecules on the apathy was recently highlighted, although not having undergone rigorous clinical trial of object.
Finally the only psychotic symptoms must discuss a neuroleptic. For some years, limited anticholinergic effects of so-called “atypical” antipsychotics have made these drugs particularly useful class of drugs in the armamentarium of physicians. However, the occurrence of major adverse events (stroke) in patients with Alzheimer’s disease has led to exclude “atypical” antipsychotics prescriptions of these patients. Thus, only the neuroleptic “classic” can still be recommended when psychotic symptoms are a significant morbidity for patients and their families. However, their prescription must be done with great caution because of adverse effects.
Despite the existence of psychotic symptoms in dementia with Lewy bodies, the prescription of neuroleptics should be banned because of the high sensitivity to these drugs in this disease.
The introduction of a neuroleptic should be considered at very low doses, very progressive and in a specialized hospital.
Vascular dementia has been the subject of clinical trials for the use of cholinesterase inhibitors but which are so insufficient quality day to justify prescription of these molecules in this disease. It is the same for dementia “mixed”, that is to say, Alzheimer’s disease associated with a vascular component objectified by clinical and paraclinical.
The drugs listed in Parkinson’s disease can be discussed in the management of dementia with Lewy bodies.
Getting no drug load:
The management of dementia patients, regardless of etiology, is not limited to only drugs, far from it. Support is immediately focused on the GP.
This must be supported by the geriatrician, neurologist or psychiatrist who introduced the special treatment if necessary. It is essential to include in the care caregivers, ensure its effectiveness, and also suffer more frequently than the general population, depression. Recent studies have also shown increased mortality of caregivers.
The medical care must propose the mobilization of referring doctor the patient’s home, as the disease progresses, move the patient can become increasingly difficult.
This support must also offer specialized monitoring by a geriatrician, neurologist or psychiatrist, surrounded by a multidisciplinary team. This monitoring allows the discussion of specific treatment and a multidisciplinary assessment including the evaluation of cognitive, behavioral and psychological symptoms, nutritional status, autonomy for activities of daily living, the load carried by the caregiver , research and management of comorbidities.
Ideally semi and obviously includes the caregiver.
Behavioral adaptation:
This concept must be part of education issued by the doctor to the patient’s entourage.
It is considered that the patients suffer from a disease of adaptation to the environment.
If the bedrock of behavioral and psychological symptoms is actually organic, manifestation of disorders and symptoms is most often caused by an inability of the patient to adapt to a changing environment. It may be a change of place of living, environment, a change of pace meals, bedtimes and rise, a change of stakeholders such as formal support (nurses, aides caregivers, patients guards), hospitalization, a room change at the hospital, the occurrence of concomitant illness, the occurrence of adverse effects of a drug, a mourning of an abuse, physical or psychological.
The patient can not by itself easily adapt to the environment, it must help develop and with great gentleness changes in his life, it can be anticipated or not. Often this behavioral adaptation allows the amendment or mitigation of behavioral and psychological symptoms.
These principles also apply to the entourage encouraged to accept the symptoms such as aggression, irritability, anxiety, depression, wandering, so, for example, react as little as possible aggression by aggression, or raise spending time to reassure an anxious patient.
This behavioral adaptation concerns of course the patient’s environment, but also all health stakeholders involved with the patient.
Nutrition:
Most dementias are associated with a major risk of malnutrition. The environment must be particularly attentive to the patient’s diet, ensuring the normal supply of food, proper and rigorous management of the refrigerator, and the ability of the patient to benefit from daily meals which he need. Monitoring of alcohol consumption should also be done without deleting it completely.
The advice of a dietician are sometimes useful and prescription nutritional supplements may be necessary.
Regular evaluation of nutritional status and risk of undernutrition can usefully be done through the MNA scale.
Social care:
The diagnosis of dementia, regardless of the etiology restraint must be a demand supported by social security to 100% for long-term conditions.
The intervention of the social sector is desirable in order to benefit the patient and the entourage of stakeholders such as care assistants, home care services, and home care systems whether maintaining the home is preferred.
The financing of these interventions can be provided in part by the personal autonomy allowance (APA), allocation determined by the degree of dependence and the patient’s income.
Finally, the discussion of institutionalization in nursing home, in institutions specializing in the treatment of dementia, or in long term care units in the hospital should be considered by the doctor with the environment and patient . The ideal is to avoid by anticipating any situation which led to the institutionalization emergency, because of the trauma represented by such a decision for the patient and his entourage, and because of the difficulty to “find a place “in urgent situations. We must therefore include this discussion in the project of life of the patient, and establish the greatest institution in anticipation of admission records left to push iteratively admission when it is offered by the host institution .
Some facilities offer “day care” (formerly known sometimes as “day hospital”). This solution allows the reception of the patient certain days of the week, particularly to relieve the immediate surroundings. Some structures may also propose yearly hospitalizations of “respite” for a few weeks.
Support for the loss of autonomy in activities of daily living:
The loss of autonomy for the daily activities is inevitable and a source of great suffering for the patient and his relatives.
Regular evaluation with the environment adapts to the best place of the patient’s life, with the intervention eg an occupational therapist.
The prescription of physical therapy, ideally at home, for example facilitates the maintenance of walking as long as possible quality.
Delirium:
The delirium is a major risk for patients with dementia.
Triggers are many and can sometimes be as simple as a bereavement, a change in the environment, in subsidies, a change of place of life (stay for the holidays, for example), or changing a drug administration. Diagnostic and therapeutic issues are discussed in a specific chapter of this book.
The occurrence of delirium should be suspected if they develop disorders of vigilance, a sudden impairment of mental ability, or the sudden onset of behavioral and psychological symptoms.
She materially strike the patient’s functional capacity and usually requires hospitalization in emergency near a specialized structure.
CONCLUSION:
The aging of the population, lack of easily controllable risk factor and cure and the progress made in the field of early diagnosis of diseases such as Alzheimer’s disease, make dementia a clinical entity encountered and cause it to be encountered more and more often. The forecast of the evolution of the number of cases in France for decades to make dementia a major public health issue.
Recognition of this syndrome should not be reserved only for geriatricians or only to neurologists. Today there are tools that lead easily to an accurate diagnosis and therefore to initiate a multidisciplinary approach. This diagnostic approach, rather than technical, requires above all time and human resources. It must immediately go beyond the identification of pathology and include the balance sheet impact of dementia on the environment and on patient autonomy in activities of daily living, as well as the balance of complications caused by nutritional disorders, behavioral disorders and interactions with co-morbidities.
It is the indispensable prerequisite for support which certainly include the current specific drug therapies and future and all other measures to accompany the medico consequences of dementia.
