ABSTRACT
CASE PRESENTATION A 71-year-old man had been healthy until his early 30s when he noticed tremor in his right hand while writing and in both hands when holding objects. The tremor gradually worsened. In his 40s he developed tremor of the head while upright that resolved when supine. Tremor of jaw and tongue appeared later. He was treated with beta-blockers (propranolol 80 mg twice a day or nadolol 20 mg daily) and a carbonic anhydrase inhibitor (methazolamide 50 mg three times a day) for years, without obvious benefit. He experienced increased difficulty coordinating hand movements and was no longer able to write legibly. He required assistance cutting food, frequently spilled from glasses, and required a straw to drink. His speech became progressively slurred and he eventually developed dysphagia. In his 50s, the patient developed mild gait unsteadiness which progressed. He frequently steadied himself on walls or furniture. Prominent upper limb tremor and incoordination prevented effective use of a cane or walker. He fell frequently. In his mid-60s, family members observed slight memory impairment that progressed. In his 70s he developed bowel and bladder incontinence and light-headedness. He was admitted to hospital following a syncopal episode for which no cause, other than his neurological disorder, was found. He was transferred to a nursing home and was no longer able to walk. He required support while seated to prevent him from falling. His brain magnetic resonance imaging (MRI) 6 years ago and head computed tomography (CT) following syncope revealed diffuse atrophy of cerebral cortex and cerebellum with compensatory ventriculomegaly.
