A case of frontotemporal lobar degeneration with ubiquitin-positive intraneuronal inclusions.

The case of a 57-year-old man is reported who has been treated several times because of his relatively expedite mental decline which has begun four years before his death. His first complaints were forgetfulness, mild changes in his behaviour, confusion and difficulty in speech. The neuropsychiatric examinations displayed a mild difficulty in naming and sometimes comprehension of words, although his speech was grammatically correct. Furthermore the patient presented a very severe decrease in short term memory with dementia and confusion. These symptoms together with the results of CT and test examinations established the diagnosis of Alzheimer's disease. Finally pneumonia afflicted the patient during the last hospitalization and he died. Histopathological examinations of the brain showed a severe, mainly temporofrontal atrophy caused by an extensive cortical neuronal loss and gliosis without neurofibrillary degenerations and senile plaques which characterize the Alzheimer's disease. Tau-positivity Pick- and Lewy-bodies may not be found. The loss of neurons associated in some places with spongiosity of laminar form. The ubiquitin-positive intracytoplasmic inclusions proved to be the most characteristic feature in the swollen neurons. These mainly occurred in the gray matter of the mediobasal part of the temporal lobe. The positivity of GFAP immunocytochemistry revealed a definite astrocytosis in the affected parts of the gray matter. In the temporal and frontal cortex scattered ballooned cells (achromatic or Pick cells) were seen in alpha B-crystallin immunohistochemistry. These findings confirmed the diagnosis of the case of frontotemporal lobar degeneration with ubiquitin-positive intraneuronal inclusions (FTLD-U) without tau-positivity. The separation of the different forms in the group of the frontotemporal dementias is recommended by means of the modern immunocytochemical examinations.