Insanity frontotemporale

The insanity frontotemporale is a Syndrome resulting from the atrophy of the frontal lobes and temporal of the Cerveau. The signs most constant are behavioral problems associated with speech difficulties with relative safeguarding with the functions with memorizing. Neurological signs belonging to the parkinson's syndrome and the amyotrophic side Sclérose can coexist the principal signs.

The Diagnostic is done on the private clinic and the observations of the medical imagery.

The examination anatomopathologic of the brain makes it possible to distinguish several types of insanity frontotemporale and remains still currently the only means of distinguishing the various types.

Its Prévalence is estimated between 3,6 and 15 case for 100.000 people. It appears in a sporadic way in a family or can be in connection with a genetic mutation (25 to 40% of the patients). The two sexes are reached equally.

Diagnosis

Private clinic

The criteria of diagnosis of this disease were the subject of a consensus

Imagery

The Imagerie by magnetic resonance highlights an atrophy prevailing at the frontal and temporal lobes former of the brain. This examination makes it possible to eliminate a vascular insanity of origin.

Tests neuropsychology

These tests especially highlight the absence of important amnesia and the absence of confusion temporospatiale.

Biology

The blood biological examinations and of the liquid céphalorachidien eliminate an inflammatory or metabolic cause. They are on the other hand unable to differentiate the insanity frontotemporale from the other types of neurodégénérative insanity.

Examination neuropathologic postmortem

It remains, to date, the only examination making it possible to carry a final diagnosis of insanity frontotemporale and to know its under type.

Differential

One can discuss a Maladie of Huntington.

Various types

Insanity frontotemporale with inclusions tau

Four standard pennies of insanity frontotemporale with inclusions tau are known:

Disease of Pick

Insanity frontotemporale with parkinsonism

See also: fronto-temporal Insanity related to the chromosome 17

Degeneration corticobasale

Progressive paralysis supra nuclear

Insanity frontotemporale with ubiquitine-positive inclusions

Several insanities frontotemorales with inclusions ubiquitines are known

Temporal insanity fronto by change of gene GRN

Temporal insanity fronto by change of gene CHMP2B

Syndrome myopathy with inclusions - disease of Paget - fronto-temporal insanity

Insanity frontotemporale with neurofilament-positive inclusions

Insanity frontotemporale without characteristic anatomopathologic lesions

Sources

Doctor Manuela Neumann, Frontotemporal dementia in Orphanet

References

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