[A 56-year-old woman with parkinsonism and dementia with the age of onset at 41 years].

We report a 56-year-old woman with parkinsonism and dementia who died of respiratory failure. The patient was well until the age of 41 when she noted insidious onset of difficulty in moving around. Soon after, she noted tremor in both her hands and gait disturbance. She received stereotaxic right thalamotomy when she was 46-year-old; after thalamotomy, improvement was noted in her tremor, rigidity, and in gait. However, a few months later, she started to experience motor fluctuations with worsening of her symptoms in the afternoon. This worsening was temporarily relieved by increasing her levodopa/benserazide dose. She started to show visual hallucination and agitation when she was 54-year-old. Her symptoms had progressively become worse with marked motor fluctuations and she was admitted to our hospital when she was 56-year-old. On admission, she was alert and general physical examination was unremarkable. Neurologic examination revealed that she was disoriented to time and place; memory was markedly disturbed and calculation was poor. Hasegawa dementia scale was 7/30. Higher cerebral functions appeared intact. She showed masked face, small voice, and some dysphagia. Other cranial nerves were intact including ocular movements. She was unable to walk by herself; when supported she walked in small steps with marked disturbance in the righting reflex. Mixed rigidity and Gegenhalten was noted in her four limbs and in the neck. Tremor was absent. She showed marked akinesia.(ABSTRACT TRUNCATED AT 250 WORDS)