Temporal insanity fronto by change of gene GRN

The temporal insanity fronto by change of the GRN or temporal insanity fronto with ubiquitine-positive inclusions involve a progressive Aphasie and a extra-pyramidal Syndrome with syndrome parkinsoninen or cortico-basal person in charge of abnormal movements. The disease starts between 35 and 87 years. The neurological signs are very varied at the same time in the same family or between different families.

The behavioral problems are the earliest signs followed by a progressive aphasia. Disorders of the higher functions appearing by anomalies of judgment or a loss of the assets. The first signs of the aphasia are a difficulty of finding its words, a loss of the comprehension of the language and a difficulty of indicating the objects. Later, the individuals completely lose their aptitude for a verbal communication. The first signs of the parkinson's syndrome are rigidity, of the slow movements or the disappearance of the movements, a dystonie of the members, an impossibility to carry out voluntary mouvments and disorders of balance. The latest signs are the Myoclonie S and the disorders of swallowing. The majority of the people lose the possibility of going.

The death occurs between 3 and 12 years after the beginning of the disease

Sources

Ging-Yuek Robin Hsiung, Howard H Feldman, GRN-Related Frontotemporal Dementia in GeneTests: Medical Genetics Information Resource (database online). Copyright, University off Washington, Seattle. 1993-2007 ==Références==

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