[An autopsy case of corticobasal degeneration mimicking frontal Pick's disease]

A pathologically proven case of corticobasal degeneration (CBD) with marked psychiatric symptoms was reported. A 59-year-old female was admitted to our hospital in October 1989 because of her "forgetfulness". Her abnormal behavior began in August 1989, and she had no psychiatric signs before that time. On this admission she was diagnosed to have panhypopituitarism due to Sheehan syndrome. Cortisone and levothyroxine were administered, but her mental symptoms deteriorated. Strange behaviors such as buying same materials every day and wandering around all day long fully dressed with all of her jewels were observed. She could not perform her housework after March 1991. She was in a bed ridden state after 1992 and died in March 1993, at the age of 62. The total duration of her illness was 3 years and 7 months. Her clinical course resembled frontal Pick's disease. At autopsy advanced gastric cancer without metastasis and infarct of the pituitary gland were found. The weight of the brain was 987g and atrophy of the bilateral frontal lobes was evident on macroscopical examination. Neuronal loss, proliferation of the glia, and spongy state were observed in the superficial layer of the frontal cerebral cortex. Widespread appearance of ballooned neurones was also observed in the deep layers of the frontal cerebral cortex. Neuronal loss and gliosis were found in the striatum, pallidum, thalamus, and substantia nigra. There was neither senile plaques nor Pick bodies. Numerous argyrophilic threads were found by Gallyas-Braak method and all these pathological findings were compatible with the previously reported cases of CBD. Although CBD is considered as predominantly a motor disorder, there are a few reports of pathologically proven cases of CBD mimicking Pick's disease. These cases suggest clinical diversity of CBD and further investigations are essential to establish the disease entity of CBD and Pick's disease.