Frontotemporalny dementia
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Description:
Frontotemporalny dementia belongs to sporadic hereditary diseases at which frontal and temporal lobes, including the Peak disease are surprised.
Frontotemporalny dementia (FTD) makes up to 10% of all of dementia. The beginning of this neurologic disease belongs to younger age (from 55 to 65 years), than the beginning of a disease of Alzheimer's disease. Frontotemporalny dementia men and women equally often suffer. The Peak disease as one of options of frontotemporalny dementia from the pathomorphologic point of view is characterized by the expressed brain substance atrophy, loss of neurons, a gliosis, emergence of abnormal neurons (the Peak cell) containing inclusions (the Peak little body).
Reasons of frontotemporalny dementia:
About 1/2 cases of frontotemporalny dementia are hereditary, the most significant mutations arise in a chromosome 17q21-22 that leads to disturbances in structure of a tauprotein therefore frontotemporalny dementia designate a kaktaupatiya. Some experts carry the progressing nadjyaderny paralysis and a kortikobazalny degeneration to frontotemporalny dementia as in their basis the similar pathological changes and genetic mutations damaging таупротеин take place. The symptomatology can not always correspond to genetic mutations and pathological displays of a disease and vice versa. For example, similar mutations cause displays of frontotemporalny dementia in one family, and in members of other family - symptomatology of a kortikobazalny degeneration, cells of Peak can be absent at patients with typical displays of a disease of Peak.
Symptoms of frontotemporalny dementia:
In general frontotemporalny dementia more affects personal features of the patient, his behavior and speech functions (syntax and smoothness) and less memory in comparison with Alzheimer's disease. The abstract thinking and attention (keeping and a pereklyuchayemost) are lost, responses will be disorganized. Orientation is kept, but reproduction of information can be weakened. Locomotory skills are, as a rule, kept. Patients experience difficulties with the sequence of performance of tasks in spite of the fact that performance of visual and space and constructive tasks suffers to a lesser extent.
Signs of a disinhibition of frontal bark (prehensile phenomena, sucking, hobotkovy reflexes, superciliary (glabellar), palmomentalny (palmar) reflexes) appear in later stages of a disease, but can be also present and at other types of dementia. At some patients the clinical picture of a disease of a motor neuron with a generalized muscular atrophy, weakness, fastsikulyation, bulbar symptoms develops (including a dysphagy, a dysphonia, chewing difficulty) that increases risk of aspiration pneumonia and early death.
Frontal option of frontotemporalny dementia. Due to the defeat of structures of the basis of a frontal lobe the social behavior and personal features of the patient suffer. Patients become impulsive and lose control over social restraining factors (including they can become inclined to thefts), neglect personal hygiene. Some have manifestations of a syndrome of Klyuvera-Byusi including emotional dullness, hyper sexuality, hyper orality (including, bulimia, suction and a prishlepyvaniye lips), visual agnosias. There is a decline in the ability to concentration, a divergence and intellectual rigidity. The behavior becomes stereotypic (the patient can go to the same place every day). Patients can collect and manipulate accidental objects (utilization behavior). Verbal products decrease, there is an echolalia, perseverations (inappropriate repetition of answers to questions) and finally the mutism develops.
Primary progressing aphasia. Speech functions are lost in connection with asymmetric (more than left-side) an anterolateralny temporal atrophy; the hippocampus and memory suffer moderately. Most of patients have difficulties in selection of words. The attention (including, the consecutive digital account) can be significantly broken. Many patients have an aphasia with decrease in smoothness of the speech and difficulty of understanding of speech designs, the uncertainty in speech products and a dysarthtia also often meets. One patients have an aphasia as a monosymptom develops 10 and more years later from the beginning of a disease, at others global deficit arises within several years.
Semantic dementia represents one of types of primary progressing aphasia. In cases when the left cerebral hemisphere is damaged more, ability to understanding of words is progressively lost. The speech remains smooth, but in it otstutstvut sense (for example, similar or connected terms are used despite existence of specific names of objects). In cases when the right hemisphere more suffers, patients have a progressing anomy (inability to call a subject, and a prozopagnoziya (inability to distinguish familiar faces). They cannot remember topographical interrelations. Some patients with semantic dementia have an Alzheimer's disease.
Diagnosis:
Diagnosis is based on establishment of typical clinical signs of a disease. As well as at other types of dementia, at patients cognitive deficit is estimated. The KG and MRT are carried out for clarification of localization and degree of manifestation of an atrophy of a brain and an exception of other possible reasons of its emergence (including brain tumors, abscesses, a stroke). Frontotemporalny dementia is characterized by the expressed brain atrophy, sometimes extent of thinning of furrows of a temporal and frontal lobe reaches thickness of the sheet of paper. As MPT and KT can not reveal zones of the prevailing cerebral cortex atrophy up to late stages of development of frontotemporalny dementia, neurovisualization can be less useful to an Alzheimer's disease exception (at which in early stages the hippocampus and parietal lobes are preferential injured), however clinical distinctions between these diseases allow to distinguish them. For example, primary progressing aphasia differs from Alzheimer's disease in safety of memory and space and visual function when losing a syntactic component of the speech and its smoothness
Treatment of frontotemporalny dementia:
Specific treatment does not exist. The supporting treatment is usually carried out.
