[Early manifestation of fronto-temporal dementia]

The term frontotemporal dementia is used to describe a primary degenerative form of dementia, which is characterized by typical clinical, neuropsychological, radiological and neuropathological features. Its onset is usually before the age of 65 years; manifestations before the age of 30 years have rarely been described. We report the case of a 22-year old man, who showed symptoms of behavioural disorder such as social retreat, lack of initiative, mental rigidity, progressive reduction of speech, and stereotyped behaviour. The neuropsychological examination revealed disorders of the executive functions. The cerebral MRI investigations showed bifrontal atrophies corresponding with hypoperfusion areas on the SPECT. Other investigations including EEG, evoked potentials, duplex ultrasonography, cerebral angiography, laboratory tests and cerebrospinal fluid were normal. In the present case report we discuss the clinical presentation of frontotemporal dementia with early onset.