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.