A case of Pick's disease presenting with progressive nonfluent speech

ABSTRACT

BACKGROUND AND SIGNIFICANCE: Frontotemporal dementia is a behavioral disorder arising from nonAlzheimer's disease atrophy of frontal and anterior temporal lobe. Clinical manifestations include frontal lobe dysfunction. Kluver-Bucy syndrome or progressive language impairments. Two types of histological change underline, the atrophy. The commoner pathology is nerve cell loss and spongiform change with astrocytic gliosis. The second one is typical Pick-type histology characterized by intraneuronal inclusion body and astrocytic gliosis. We report a case with biopsy proved Pick's disease presenting with progressive nonfluent speech. CASE A 41-years, old right-handed woman developed progressive language impairment over a period of 6 months. Brain MRI revealed asymmetric frontotemporal cortical atrophy more severe on the left side and 18F-FDG-brain, PET showed hypometabolism in the same area. Neuropsychological test including Korean version-Western Aphasia Battery revealed non-fluent speech as well as frontal lobe, dysfunction. A biopsy from left frontal lobe, demonstrate neuronal loss and diffuse astrogliosis. In the cytoplasm of remaining neurons are eosinophilic inclusion bodies which are neurofilament-positive with immunostaining. Senile plaque, neurofibrillary tangle and cortical Lewy body were absent. COMMENT: We report a case presenting with progressive nonfluent speech whose imaging and pathological findings are compatible with Pick's disease, which may be the first biopsy proven case in Korea.