Cortical cerebellar degeneration associated with a specific disorder of standing and locomotion.
The three patients presented showed a rhythmic bobbing of the body when standing with flexed legs. It was produced by slow, coarse, synchronous extensions-flexions in the legs. Platform and accelerometer records demonstrated an almost clockwork regularity of rate in the 2.5--3.5 c/sec range. In Romberg's test there occurred slow rhythmic extensions-flexions of the feet. The patients walked with a peculiar stiff "heel-gait", which was not conspicously broad-based, unsteady or trembling. On ascending a platform they displayed a slow leg tremor and a marked disorder of forward-vertical movement. This very uniform motor syndrome retained its specific features over the years. An upper limb involvement was observed in one of the patients. Post-mortem examination in one patient, a chronic alcoholic, showed a pronounced atrophy of the superior cerebellar vermis. Tomographic pneumoencephabgrams demonstrated the superior vermis atrophy in the two other patients.
D000328:Adult; D000437:Alcoholism / Q000150:complications; D001284:Atrophy; D002525:Cerebellar Cortex; D002526:Cerebellar Diseases / Q000150:complications*; D004185:Disability Evaluation; D005260:Female; D005500:Follow-Up Studies; D005528:Foot; D005684:Gait; D006801:Humans; D007866:Leg; D008297:Male; D008875:Middle Aged; D009069:Movement Disorders / Q000209:etiology*; D009748:Nutrition Disorders / Q000150:complications; D011187:Posture; D014202:Tremor / Q000209:etiology*