PEAK DISEASE

From Big Medical Encyclopedia

PEAK DISEASE (A. Pick, Czech psychiatrist and neuropathologist, 1851 — 1924; synonym atrophy of Peak) — the progressing disease which is characterized by development of total weak-mindedness and disintegration of the speech, first of all expressional.

The disease is for the first time described in 1892. Peak which considered it not an independent disease of and an early, atypical form senile dementia (see). Nosological independence of a disease was clinically proved researchers in 20 — the 30th is near to 20 century. The most reasonable would be P.'s reference. to so-called system atrophies — group of hereditary and degenerative diseases of a nervous system with progreduated character patol, process and its various localization. At classification of mental diseases of late age of P. usually combine in group of presenile dementias with others, the atrophic processes which are also demonstrating preferential at presenile age and leading to weak-mindedness: with Alzheimer's disease (see. Alzheimer disease ), Jacob's disease — Kreyttsfeldta (see. Kreyttsfeldta — Jacoba a disease ), a chorea of Gentington (see. Gentington chorea ). Such association, despite nosological distinctions between them, is represented lawful in view of the general similarity their clinical (a tendency to development of weak-mindedness and disturbance of the highest cortical functions) and morphological (development of atrophic changes in bark and subcrustal formations of a brain) manifestations.

Average age of patients, in Krom is observed manifestation of a disease, 53 — 55 years (cases of earlier and later beginning of a disease meet, however).

The clinical picture

the Disease develops, as a rule, gradually and gradually. In the prevailing most cases of P. begins with weakening of the highest forms of intellectual activity and with changes of the personality which nature correlates with preferential localization of atrophic process. At the isolated or preferential defeat of a pole and camber of frontal lobes the increasing slackness, indifference, apathy, an aspontannost, emotional obtusion, the general impoverishment of a mental, speech and physical activity prevail, and dissociation between fading of spontaneous motives and relative safety of their excitability from the outside is characteristic. At expressiveness of an atrophy of orbital (basal) bark observe loss of feeling of a step and distance, disinhibition of the lowest inclinations, euphoria, loss of criticism with relative safety of memory and orientation (a pseudoparalytic syndrome). At dominance of atrophic changes in temporal shares early there are stereotypies of the speech, acts and movements. From the very beginning the intelligence, in particular the most difficult, the abstracting, generalizing, integrating processes suffers. Steadily productivity and mobility of thinking, the critic and level of judgments decrease. In process of a further progrediyentnost of a disease everything develops more total weak-mindedness (see). Only then there is a true easing of memory while at early stages of a disease loss of memory is the pas of the essence «seeming», i.e. result of indifference and passivity of patients.

Rather seldom the following atypical initial syndromes meet: asthenic and astheno-depressive syndromes with complaints to weakness, headaches, dizziness, a sleep disorder; syndromes with an unusual prematurity of focal, in particular aphotic, frustration (see. Aphasia ); syndromes with dominance of psychotic frustration at the beginning of process — most often with a persecution complex, jealousy, damage (see. Nonsense ), what in connection with the wrong behavior of patients quite often causes suspicion on existence of schizophrenic process; syndromes at which true disturbances of memory are found very much early. Despite atypical character of an initial picture of a disease, the further current usually significantly does not deviate the stereotype typical for P.

In developed a wedge, a picture of the disease (called by some researchers the second stage of development) disorders of the highest cortical functions and different, generally Extrapyramidal, nevrol, symptoms join steadily progressing weak-mindedness. At the same time would remain characteristic of a dekhmention at P. its «focal» coloring — in the form of the reached maximum expressiveness of an aspontannost, in the form of a performance into the forefront of a pseudoparalytic or stereotyped behavior.

Among focal frustration the main place is taken by the disintegration of the speech differing in the following features: the speech gradually becomes scanty, there comes the full speech aspontannost («the seeming dumbness» after the previous stage «unwillingness to speak»). There are losses (usually moderate) anamnestic-afatiche-skiye, and in the field of the impres-sivny speech the hl suffers. obr. semantic understanding at safety of the repeated speech (transcortical type of touch aphasia). Characteristic manifestations of disintegration of the speech at P. — the accruing echolalia sometimes in combination with an ekhopraksiya (see. Catatonic syndrome ) also scorched-liyey (an alalia in the form of repeated repetition of separate phrases, words or syllables) and the speech stereotypies taking the increasing place in the speech of patients, so-called «standing» turns turning eventually into the only speech products of patients (the automated «standing» turns in the form of the speech formulas deprived of communicative value). «Standing» turns arise also in a written language, in motility and behavior (motor stereotypies). Apraktichesky frustration are observed (see. Apraxia ), not reaching usually that expressiveness which is characteristic of Alzheimer's disease. Also the cases proceeding with the expressed navyazchivost meet. Unlike other atrophic processes psychotic episodes exogenous (delirious, amental, etc.) type happen extremely seldom.

Except disorders of the highest cortical functions, in developed a wedge, a picture at P. symptoms are observed also various nevrol (attacks of «loss of a tone» without spasms and a loss of consciousness); amyostatic - hyperkinetic syndromes of different expressiveness; various subcrustal hyperkinesias (see), including horeo-both atetozopodobny, and pyramidal symptomatology (see. Pyramidal system ), is more often unilateral, upon transition of atrophic process to a front central crinkle.

For a final stage of P. symptoms are characteristic pseudobulbar paralysis (see) — violent crying, laughter or grimacing, and also various prehensile and oral avtomatizm and reflexes. In an end-stage comes cachexia (see), patients become absolutely helpless, unavailable to any contact; completely the speech and any other signs of mental activity disappears.

Etiology and pathogeny

Aetiology and P.'s pathogeny. are insufficiently studied. Many researchers carry it to hereditary diseases, others consider that concerning a considerable part of cases hereditary character of suffering is not proved. Numerous cases of family P. are described. with preferential accumulation of sekundarny cases in generation of a proband. Families in which, except P. are known., also other hereditary and degenerative forms were observed (including a chorea of Gentington). External factors, napr, injuries, infections, play, apparently, the role only provoking or making heavier.

Pathological anatomy

Fig. 1. Macrodrug of a brain at a disease of Peak: the expressed atrophy of crinkles of bark of frontal lobes (it is specified by shooters) and reduction of the sizes of the right cerebral hemisphere.

Pathoanatomical changes at P. are characterized by a selective atrophy of hl. obr. bark and white matter of cerebral hemispheres. Sometimes the atrophy in much smaller degree takes subcrustal nodes and trunk departments. Distinguish an atrophy of preferential frontal (fig. 1) or temporal shares, it is frequent — the combined their defeat. Often parietal lobes, however the isolated their defeat as well as the atrophy of occipital shares, meets extremely seldom are involved in process. At an atrophy of frontal lobes pretsentralny crinkles usually remain safe. The atrophy develops, as a rule, in symmetric sites of both hemispheres, sometimes with bigger expressiveness in left and is extremely rare — in the right hemisphere.

Fig. 2. Microdrug of a cerebral cortex at a disease of Peak: sharp reduction of number of cells in upper (II, IIIA) layers of a cerebral cortex; coloring across Nisslgo; x 18.
Fig. 3. Microdrugs of a cerebral cortex at a disease of Peak: and — a ballonovidny nervous cell with the central chromatolysis and an ectopia of a kernel; coloring across Nissl; X400; — spherical argentofilny inclusion (the little body of Peak, is specified by an arrow) in cytoplasm of a nervous cell; impregnation across Bilyiovsky; h400.

The progressing hron, dystrophic changes of nervous cells in the form of wrinkling, the atrophies, a lipofustsi-new degeneration which are coming to an end are histologically noted cytolysis (see) and bringing to sharp diffusion, and sometimes and to focal devastation, a cut is followed by the expressed replaceable gliosis due to proliferation generally of astrocytes and oligodendroglyocites (see. Gliosis ). Cellular devastation is especially noticeable in top coats (II — IIIA) cerebral cortices (fig. 2). Characteristic signs of P. — emergence of the bulked-up ballonovidny cells with the central chromatolysis and an ectopia of a kernel (fig. 3, a) reminding the neurons with primary irritation at axonal reaction sometimes turning in the superbulked-up homogenized cells, and also emergence of neurons with argentofil-ny cytoplasmic spherical inclusions — little bodies of Peak (fig. 3, b). According to Esku-roll (R. Escourolle, 1956), the bulked-up cells would meet approximately in 60% of cases of P., whereas argentofilny inclusions — in 20%. Preferential localization of the bulked-up cells — the III—V layers of bark, neurons with argentofit ny inclusions — most often a hippocampus. However these changes can meet in all formations of a brain which underwent an atrophy, and even in cells of a brainstem.

The electronic and microscopic picture of the bulked-up cells and neurons with argentofilny inclusions is characterized differently. One researchers do not find ultrastructural distinctions between these two types of defeat of neurons, others specify that argentofilny inclusions are formed by chaotic networks of proliferating neurofilaments and granules of lipofuscin, and the ultrastructure of cytoplasm of the bulked-up neurons is presented by the extensive zones containing granular material, scattered neurotubules and the changed mitochondrions at almost total absence of neurofilaments (see. Nervous cell ).

Changes of myelin fibers are expressed in spindle-shaped and spherical inflation of myelin covers, disintegration of a myelin and demyelination, swelling of axial cylinders is sometimes noted. The opinion on primary defeat of fibers at P. would be expressed. Vascular changes are shown in the form of disturbance of permeability of walls of vessels, fibrosis of small arteries and capillaries.

The diagnosis

the Diagnosis is in most cases based on the analysis a wedge, features and a stereotype of development of a disease. From paraclinic methods for an exception of tumoral process it is shown pneumoencephalography (see): over atrofichny shares of a brain accumulation of air in combination with internal hydrocephaly comes to light. On the electroencephalogram (see. Elektroentsefalografiya ), which has smaller value, than at Alzheimer's disease, low-amplitude curves with smoothing of regional distinctions prevail. In cerebrospinal liquid (see) unsharply expressed changes are found in a part of patients (moderate increase in the general protein content, positive globulinovy reactions, small teeth at Lange's reaction).

Differential diagnosis carry out with other atrophic processes and first of all with Alzheimer's disease (see. Alzheimer disease ), at which already in an initial stage memory, orientation or the account are progrediyentno broken, at P. these types of mental activity remain usually long. At the early and expressed development subcrustal nevrol, symptoms (expressiveness of an amyostatic syndrome, existence of horeo-like twitchings and other hyperkinesias) there can be a need for differentiation with Parkinson's disease (sy. Trembling paralysis) and a chorea of Gentington (see. Gentington chorea ). In the presence of atypical initial frustration of P. differentiate with diffusion atherosclerotic process, at Krom, as a rule, depth of changes of the personality is not expressed.

Treatment

At the initial stages of a disease a temporary favorable effect is reached course (within 2 — 3 months) by use of Aminalonum (to 3 — 4 g a day) or nootropil (to 2 — 3 g a day). At psychotic forms of a disease (crazy or hallucinatory-dilision states, the wrong behavior) appoint small doses of neuroleptics (aminazine, neuleptil).

Forecast adverse; average duration of P. — 6 — 8 years. Working capacity is lost early and completely; already at rather early stages of disease patients need constant stay in a psychiatric hospital. In an end-stage the lethal outcome comes being able marasmus (see) or from intercurrent diseases.

Prevention is not developed.

See also Pathopsyhosis , Psychoorganic syndrome .


Bibliography: The multivolume guide to pathological anatomy, under the editorship of A. I. Strukov, t. 2, page 601, M., 1962; V. S. K of a patomorfologiya of a disease of Peak, Zhurn, a neuropath, and psikhiat is civil., t. 57, century 4, page 534, 1957, bibliogr.; Shternberg E. Ya. Clinic of dementias of presenile age, L., 1967; it, Gerontological psychiatry, M., 1977; Bini L. Le demenze prese-nili, Roma, 1948; B r i o n S., M i k o 1 J. a. P s i m a r a s A. Resent findings in Pick’s disease, Progr. Neuropath., v. 2, p. 421, 1973; Constantinidis J., Richard J. Tissot R. Pick's disease, Europ. Neurol., v. 11, p. 208, 1974, bibliogr.; Handbuch der speciellen pathologischen Anatomie und Histologie, hrsg. v. O. Lubarsch u. a., Bd 13, T. 1, Bandteil A. S. 614, B. u. a., 1957; Pick A. tiber die Beziehungen der seni-len Hirnatrophie zur Aphasie, Prag. med. Wschr., S. 165, 1892; Sjogren T., Sjogren H. u. Lindgren A.G.H. Morbus Alzheimer und morbus Pick, Stockholm, 1952; Spatz H. Die «systema-tischen Atrophien», Arch. Psychiat. Nervenkr., Bd 108, S. 1, 1938; Stertz G. Pick’s atrophy, Z. Neurol. Psvchiat., Bd 101, S. 729, 1926.


E. Ya. Shternberg; P. B. Kazakova (stalemate. An.).

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