ALZHEIMER DISEASE (A. Alzheimer, is mute. the pathologist and the psychiatrist, 1864 — 1915) — the disease which is characterized by disintegration of the highest cortical functions and leading to weak-mindedness as a result of a diffusion atrophy of a brain; treats group of so-called presenile dementias. It is for the first time described in 1907. However disputable still is a question of relationship between Alzheimer Bolesnyue and senile dementia (see. Weak-mindedness ).
Some authors, ranking Alzheimer Bolesn as senile dementia as early, heavier (and long) its form, Diseases were based on similarity of morphological changes and patterns of development of both diseases, and also on overseeing by accession characteristic of Alzheimer. focal frustration in certain cases senile dementia [«aljtsgeymerization» according to J. Ajuriaguerra]. The authors considering Alzheimer Bolesn as an independent disease proceed from characteristic clinical and genetic features.
Data on Alzheimer's prevalence of the Disease insufficient. Among the patients coming to insane hospitals, this disease makes from 0,3 to 0,9% [E. Ya. Shternberg, Bini, Shegren (L. Bini, N. of Sjögren), etc.], and among the mental diseases which began aged is more senior than 50 years — about 5% (Shegren). From all pathoanatomical openings made from 1960 to 1970 in the Moscow insane hospitals, about 4% are made by patients And.
Average age of the beginning of a disease of 54 — 56 years, its average duration — 8 — 10 years. Considerable deviations from these average figures are observed, however; were described both early (juvenile), and late (senile) forms, and also cases with very long current (10 — 15 years and more). Women get sick much more often — according to literary data by 3 — 5 times, on E. Ya. Shternberg's observations — almost by 8 times.
The clinical picture
In typical cases of Alzheimer Bolesn proceeds as the progreduated, leading to weak-mindedness process, to Krom in different terms, is more often in the first 2 — 3 years, symptoms of disintegration of the highest cortical functions join (speeches, a praksisa, accounts, recognitions, etc.).
The central place in development of dementia at And. occupy early the beginning progressing disturbances of memory, disorder of orientation in space at preservation of awareness of the mental insolvency. Gradually there comes the full anamnestic disorientation, and the autopsikhichesky disorientation can reach extent of not recognition of own image in a mirror. At the same time in much smaller degree, than at senile dementia, an anamnestic syndrome (see. Amnesia ) is followed by revival of former experience (shift of a situation in the past). Early the accruing disorders of attention, comprehension and perception surrounding act.
Idiosyncrasy of the developing dementia — the progressing loss of usual skills, including and the most automated. Disturbance gradually passes it into clear apraxia (see), and such development of initial special lines of dementia into neurologic frustration, e.g. forgetfulness in anamnestic aphasia (see) or disturbances of optical attention in optical agnosia (see), can be considered as idiosyncrasy of dynamics of dementia at And.
Disintegration of the speech is defined by dominance of anamnestic and touch aphasia; usually a little later there come disturbances of the motor speech among which are especially characteristic violent repetition of separate syllables and letters (logoclonism) and speech excitement. As a rule, the letter (agraphia) and the account (acalculia) are especially deeply violated. Apraksin reaches sometimes extreme degree; at the same time the picture of the general motor confusion or a full apraktichesky obezdvizhennost can be observed.
Psychotic frustration — rather frequent phenomenon at And. They arise in different terms, is more often in the first years. Usually it is rudimentary crazy and hallucinatory syndromes or conditions of the dulled consciousness. In some cases there are long paranoiac psychoses which are observed usually at konstitutsionalno predisposed persons at a small progrediyentnost of the process leading to weak-mindedness.
In 25 — 30% of cases at Alzheimer of the Disease epileptic seizures, usually generalized, sometimes abortal are observed. However the progreduated current of an epileptic syndrome meets seldom. Often there are also various neurologic, in particular subcrustal, symptoms (gipokineticheski-hypertensive and various hyperkinesias, are more often choreiform). In an initial stage of a disease are observed: violent crying and laughter, oral and prehensile avtomatizm and reflexes, dominance of a forced (embryonal) pose, a cachexia, bulimia, endocrine frustration, including signs of a masculinization at women (see. Virilescence ). Studying of heredity yielded contradictory results. According to many authors, And. treats polygenic diseases. One authors describe cases, transferred on dominant type, others — group of probands (see. Medical genetics ), in which families there is the known accumulation of patients And. or senile dementia, and considerable number of sporadic cases of Alzheimer of the Disease.
according to most of authors, pathoanatomical changes at Alzheimer Diseases qualitatively do not differ from those at senile dementia. The diffusion atrophy of a brain, preferential bark and in a smaller measure of gray subcrustal educations is observed. However at Alzheimer macroscopic and microscopic changes are expressed to the Disease much more, than at senile dementia, and differ in a bigger ochagovost.
In typical cases the intravital diagnosis And. it is quite possible. Its clinical current differs from senile dementia in considerably bigger expressiveness of focal frustration. Difficulties are presented sometimes by so-called psevdoaltsgeymerovsky forms of a disease (see. Gakkebusha-Geyera-Geymanovicha syndrome ), at which the current happens less smooth and progreduated. And. it is necessary to differentiate with a disease of Peak (see. Peak disease ), at a cut into the forefront rough changes of the personality act, and dysmnesias are expressed less.
the Forecast is adverse: the disease progresses, and patients perish in various terms from the beginning of a disease. Effective methods of treatment and prevention it is not developed.
Zhislin S. G. Sketches of clinical psychiatry, M., 1965; Shternberg E. Ya. Clinic of dementia presenilyyugo age, L., 1967; Alzheimer A. Über eine eigenartige Erkrankung der Hirnrinde, Zbl. Nervenheilk., Bd 30, S. 177, 1907 u. Allg. Z. Psychiat., Bd 64, S. 146, 1907; Bini L. Le demenze preseni-li, Rome, 1948; Braunmühl A. Alzheimersche Krankheit, Handb. spez. path. Anat. u. Histol., hrsg. v. F. Henke u. O. Lubarsch, Bd 13, T. 1, S. 482, V., 1957; Sjögren T., Sjögren H. Lindgren A. Morbus Alzheimer and morbus Pick, Stockholm, 1952; Wolstenholme G. E. W. a. O'Cоnnor M. Alzheimer's disease and related conditions, L., 1970.
E. Ya. Shternberg.