The peak of disease

The peak of disease (A. Pick; synonym Pikovsky, or limited, atrophy of the brain)- motologichesky independent form presenilny endogenous-degenerative brain with focal atrophic changes. Peak believed that they allocated syndrome, which is only a special variation of dementia; however, currently nosological independence of the disease Peak is recognised by most researchers. Published hundreds of cases of P. b., but the true frequency, undoubtedly, much higher, especially as lifetime diagnosis is still fraught with difficulties. There P. B. about 4 times less other atrophic process of a brain of Alzheimer's disease.
The etiology of the disease peaked yet not clear. Some foreign authors refer it to hereditary diseases, pointing to frequent family cases. More often, however, the disease occurs sporadically, so attributing P. B. genetic diseases in the strict sense, cannot be justified. Usually early in manifesting and somewhat atypical in their manifestations family cases are observed more frequently disease brothers and sisters, rather than transmission of the disease from generation to generation. Received recognition concept facilities disease Peak to the so-called system atrophy - a group of endogenous degenerative-atrophic diseases, varying mostly preferred localization atrophic process. There are combinations P. B. other forms of system atrophy, in which also there are hereditary (family) and sporadic cases. Exogenous factors (first of all craniocerebral trauma) have only accelerating or weighting effect.
Pathological changes in P. B. are atrophic process, affecting both the grey and white matter of the brain. Although there always diffuse changes, atrophy is macroscopically strictly limited to one or more of the brain; it is symmetric or more pronounced in the same hemisphere. Distinguished mainly frontal, temporal or combined localization (occipital lobe is affected very rarely). In each lobe atrophy begins in a typical "centres" (orbital surface of the temporal pole, and so on); often affects the subcortex. Histologically diagnosed clean atrophy; characteristic believe "sverhdorogie" cells and argentophile inclusion. Senile plaques and change neurofibril, as a rule, are absent.
In its clinical picture of the disease Peak is a chronic disease that can only be detected more often in presenilny age (average 55 years), but sometimes the disease starts in earlier or in old age. The duration of illness varies from 1 year to 15 years, an average of about 6 years. Women get sick more often. Beginning of the disease is slow and subtle. Gradually increasing dementia with a characteristic clinical features. At the beginning of its development profound personality changes prevail over violations "instrumental functions of intelligence (memory, attention, orientation); suffer the most complex forms of intellectual activity (criticism, judgment, complex mental operations). At mainly frontal lobe atrophy depending on the primary lesion of the orbital cortex or convexity of shares there are two types of personality changes: psevdomatematicheskoe syndrome (disinhibition, euphoria, expansive-non-critical behavior) and the syndrome of spontannosti with affective and intellectual impoverishment. As of progression of the process develops deep (globalnoe) dementia with extreme impoverishment of all forms of mental activity and activity; in the final period of disease occurs and total devastation of memory resources.
Mandatory for disease Peak should be considered as a combination of dementia with a gradually increasing violations of higher cortical functions and primarily by the collapse of speech.
The symptoms typical of the collapse of speech in P. b., include: gradual impoverishment of speech and fall of speech activity (up to an "apparent silence and unwillingness to speak); development of amnestic and followed her sensory aphasia (see), first "transcortical" (that is, with the prevalence of violations of the semantic understanding); the appearance against this background speech stereotipy ("standing of revolutions") and ehrliches speech. Simultaneously observed violations of reading and writing; usually later joins and apraxia (see).
Sometimes there psychotic disorders, as a rule, are rudimentary and short-term; they occur as the initial syndrome, on later stages of the course as an expression of acceleration of the rate of atrophic process. Occasionally there are paroxysmal States "the loss of tone that is different from epileptic seizures characteristic of Alzheimer's disease. In the neurological status meet pyramidal symptoms, only extremely rarely; with frequent transition atrophy subcortical formations often see extrapyramidal symptoms (parkinsonizma or hyperkinesia). General condition of patients remains usually for a long time satisfactory, insanity comes late.
The prognosis of the disease Peak adverse, death occurs from "cerebral insufficiency", at the extreme severity of all forms of P. B. (globalna dementia, total aphasia, prakticheskaya immobility and so on)without the addition of other somatic diseases.
Any effective treatment is found.
Patients need, as a rule, long-term care in a psychiatric hospital.