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Cortical Atrophy

Alzheimer’s Disease

Alzheimer’s disease (AD) is a primary degenerative dementia that manifests in steady regress of memory, intellectual activity and other higher cortical functions and leading to total dementia. Usually, the onset of the disease is diagnosed after 65.

Stages:

  • Initial stage. Cognitive disorders, intellectual and memory degradation, difficulties in time orientation, deteriorated social and professional activity, frequent events of fixation amnesia increase, neuropsychological symptoms, such as aphasia, apraxia, agnosia. Emotional and personality disorders: egocentrism, subdepressive reactions to the person’s own failures, delusional disorder. At this stage of AD, the patients evaluate their condition critically and try to correct their increasing incapacity.
  • Stage of moderate dementia. Parieto-temporal neuropsychological syndrome; amnesia increases, disorientation in time and space increases. Intellectual functions are strongly disordered (impaired analysis and synthesis, disordered judgment), as well as instrumental functions, such as speech, praxis, gnosis, visual-spatial activity. The interests of the patients are extremely limited; they require constant care and are unable to cope with their professional duties. Still, at this stage the patients retain their main personal features, the sense of their own insufficiency and adequate emotional reaction to the disease.
  • Stage of severe dementia. Complete disintegration of memory, the personality judgment is discrepant. Total care must be constantly provided as the patients lose their independence. Agnosia progresses to the extreme. The speech is disintegrated according to the type of the total sensory aphasia.

Reasons. Genetic predisposition is supposed to be the key reason for the AD.

The defects of mitochondrial DNA are found in 50% of the patients (502500, MTND1 gene). Early onset is associated with defects in АРР, PSEN1, PSEN2 genes.

The disease can be viewed as the cluster of the diseases that have various origin but common pathogenesis: all known genetic defects modify the processing of the amyloid precursor protein (the amyloid hypothesis) leading to the development of neurotoxic types of proteins. The AD leads to the formation of multiple plaques which are the accumulations of the amyloid protein that induce degeneration of neurons.

The symptoms of Alzheimer’s Disease

  • Gradual onset and progressing course
  • Memory disorder.
  • Intellectual regress
  • Abstract thinking disorder
  • Disorder of cortical functions (speech, writing, counting recognition)
  • Reduced critical attitude to the patient’s own psychic state. At early stages of the disease, the critical attitude is preserved and it may lead to the depressions and suicidal tendencies.  
  • Personality changes
  • Disorientation in place, space and personality (manifest at early stage of disease)
  • Psychomotor excitement manifested in anxiety, restlessness, bustling
  • Social and professional maladjustment
  • The association of the disorder with other psychic diseases is absent
  • Hallucinations and delirium are possible 
  • Late symptoms include cramps and paroxysms

Diagnostics

  • Blood tests (clinical and biochemical tests, B12 and folic acid levels)
  • Examination of the thyroid gland functions
  • Tests for syphilis and HIV
  • ECG
  • EEG – diffuse slowing
  • CT, MRI – cortex atrophy, increased size of ventricular cavities. Hydrocephaly, infarction, hematomas and tumors must be ruled out
  • The increased levels of amyloid in CSF
  • Excessive papillary dilation after administration of mydriatics.
  • Defective E4 allele of ApoE gene.

Treatment

So far there is no specific treatment of Alzheimer’s disease. The drugs that can be used are minimal due to poor tolerance. They include:

  • Glutamatergic modulators.
  • Nootropic, cerebroportective and vascular therapy.
  • Stimulants of neuro-muscular transfer (cholinesterase inhibitors)
  • Selective inhibitors of neuronal serotonin uptake.
  • Tranquilizers for hallucinations and delusions.

The treatment begins with minimal doses and they are gradually increased to become effective

  • Carbamazepine 100 mg two or three times per 24 hours for excitation and aggression
  • Antihistamines should be avoided

Pick’s Disease

Pick’s Disease (PD) is the atrophy of the brain cortex that involves the neurons in frontal, or more rarely in frontotemporal lobes of the brain. It is rarer than Alzheimer’s disease and females after 50 suffer from it more often than males after 50.

The reasons. The reasons are not clear; the supposed reason is tau protein build up in the brain. Sometimes, the hereditary predisposition is observed.

Main Characteristics:

  • Change in the emotional sphere and personality: severe personality disorders, complete absence of criticism, passive behavior, impulsiveness, rudeness, hyper-sexuality, foul language; the patient is unable to properly evaluate the situation.
  • Changes in the cognitive sphere: disordered thinking, while automatic skills (count, writing, professional stereotypes) retain for a long time. Systemic perseveration in the speech and praxis. Memory disorders manifest much later than the personality disorders and are not so severe as in AD and vascular dementia.

The Symptoms of Pick’s Disease

Behavior disorders:

  • rudeness;
  • foul language;
  • absence of criticism of one’s own behavior;
  • impulsiveness;
  • neglected social norms;
  • coarse humor;
  • hyper-sexuality;
  • disordered food behavior, craving for sweet food and proneness to obesity;
  • neglected hygiene.

Emotional disorders:

  • hostility;
  • aggressiveness.

Speech disorders:

  • terseness;
  • reduced tempo;
  • difficulties in naming things;
  • answers in monosyllabic words and phrases;
  • multiply repetition of the last syllables in every word;
  • repetition of the phrases and words after the other party (echolalia) .

Postural disorders:

  • Shuffling gait, instability, frequent falls.

Bladder control problems.

Diagnostics

  • Complains for asocial behavior of the patient.
  • Gradually increasing signs of mental disability.
  • Typical signs detected in the examination of the psychologist or psychiatrist:
  1. Disordered health habits;
  2. No critical attitude to patient’s own behavior;
  3. Impulsiveness;
  4. Coarse humor; hyper-sexuality.
  • Neurological examination:
  1. Disorders of speech (terseness, monosyllabic answers);
  2. Shuffling gait, frequent falls.
  • MRI (magnetic resonant tomogram) of the brain is the method that allows for a layer-by-layer study of the brain. In Pick Disease the atrophy (thinning) of the brain cortex is in frontal and temporal areas.

Pick Disease Treatment

The treatment that can slow down the diseases is absent.

In case of severely disordered behavior and psyche the patient may need psychiatric assistance, and sometimes admission to the hospice.

Complications and Consequences

  • Severe dementia (inability to orient in space, time and personality).
  • Complete immobility (absence of motivation).

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С уважением,
доктор медицинских наук
профессор
А. С. Брюховецкий

НейроВита

Адрес Клиники

Россия Москва
Каширское шоссе 23

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