ABSTRACT

CASE PRESENTATION A right-handed woman was examined for the first time in 1986 at age 47 years. She complained of tremor, interfering with writing and other activities of daily life. She had a family history of an unknown movement disorder with probable autosomal dominant transmission (her father and a sister). Tremor was first noticed in the right arm at age 41 and gradually worsened, involving both sides within 1 year. On examination, the tremor was bilateral and complex, mainly affecting posture and action with a mild rest component which was more obvious in the lower limbs. Mild right hemi-parkinsonism was also present, with cogwheel rigidity at the wrist and loss of right arm swing while walking. Reflexes were pathologically brisk. There were no prominent cerebellar signs at this stage, except mild slurring of speech and a broad-based gait. Sensory examination, eye movements, and cognition were normal. The patient complained of several episodes of depression in past years and was depressed at the time of first examination.