Limited atrophy of the brain
Limited atrophy of the brain (Pick’s disease, frontotemporal dementia, frontotemporal lobar degeneration) is a rare hereditary disease of the central nervous system. The pathological gene is located on the 17th chromosome. The pathologoanatomical basis of the Pick’s disease is the cortex atrophy, mostly of the frontal brain sections and particularly mediobasal cortical area and frontal lobes. Atrophic process usually spreads to the white matter of the frontal parts of the brain hemispheres, the structure of the corpus callosum and subcortical nodes. Sometimes, the Pick’s bodies, the protoplasmic inclusions of microtubular tau protein, are detected in the cortical neurons and glial cells. Meanwhile, the amyloid plaques that are typical for Alzheimer’s disease are absent in the brain tissue in this case. Frontotemporal dementia belongs to the group of neurogenerative diseases that characterizes by the progressing changes in behavior, dysfunction of goal-oriented activity and speech disorder induced by degeneration of the medial prefrontal and frontal-insular cortex. Four clinical subtypes of Pick’s disease have been identified: semantic dementia, progressing slow aphasia, behavioral variation of the frontotemporal dementia and atrophy of the right temporal lobe. The disease was first described by Arnold Pick, the Czech psychoneurologist (1851-1924).
Slow beginning with progressive reduction of intellectual functions is typical for a clinical picture, as well as frontal symptoms that manifest in the combination of the following signs: emotional flatness, social behavior roughness, disinhibition, anxiety or apathy, speech disorders of efferent motor aphasia type. In the beginning, the memory and the function of the parietal sections of a large hemisphere are relatively intact. Obvious disorders of memory are preceded by loss of tact, asocial, hypersexual and silly behavior. With the time, the patients become absent-minded, unclean, scruffy; apathy and absence of initiative increase; the signs of subcortical pathology are represented by akinetic rigid syndrome. The answers to the questions often acquire the character of echolalia, paraphasia is frequent. The patient has no critical opinion of their condition.
As the mental disorder increases, the patients become more and more helpless, lose their independency, the signs of oral automatism appear and epileptic fits may start.
The Pick’s disease is rarer that the Alzheimer’s disease, usually it manifests after sixty with slow progressing changes in intellectual, mnestic and emotional spheres. The disease is characterized by earlier than Alzheimer’s onset, by more pronounced behavioral disorders as compared to cognitive functions and the signs of selective atrophy of the frontal lobes at CT and MRI images. With the time, the intellectual and mnestic changes become general and total dementia comes.
Life expectancy after the diagnosis varies from 6 to 8 years. Usually, the patents die from cachexia induced by the intercurrent diseases.
