線条体黒質変性症の1症例

特にその痴呆症状に注目して

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Striato-nigral degeneration (SND) was first described by Adams et al, in 1960 as a new nosological unit. In the present paper, the authors have studied a case of SND with a special reference to the associated mental deteriorations.
A 49-year-old female without any family history had been suffering from various progressive extrapyramidal symptoms such as tremor, rigidity, hypo- and brady-kinesia and mild disturbance of oral speech for five years. She had also been noticed cerebellar ataxia and vesical disturbance. A cerebellar atrophy was pointed out on cranial X-ray CT. At the age of 46, she was clinically diagnosed as SND. Besides the typical neurological symptoms she presented several psychic symptoms, mental deteriorations in particular, during the long term drug treatment.
It is well known that SND shows the extrapyramidal disturbances usually accompanied by cerebellar disorders and autonomic failures, but in some cases there are more or less intellectual impairments as are in other spinocerebellar degenerations. Though the memory impairment, characterized by the word “forgetfulness”, is easily noticed in the clinical observation of SND, it seems not so easy to find out mental deteriorations such as motivational disturbance, bradyphrenia and uncontrollable moodiness as seen in our patient. These mental deteriorations could be considered as “subcortical dementia”. How ever, there are certain difficulties, which have not yet been solved, in clinical diagnosis of SND and in intellectual evaluation. Therefore, we have to take account not only of the main neurological symptoms like extrapyramidal disturbances but also of the associated psychiatric symptoms including mental deteriorations in the case of patient with SND.