CORTICAL CEREBELLAR DEGENERATION ASSOCIATED WITH A SPECIFIC DISORDER OF STANDING AND LOCOMOTION

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Abstract
The 3 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 Hz range. In Romberg''s test there were slow rhythmic extensions-flexions of the feet. The patients walked with a peculiar stiff heel-gait, which was not conspicuously 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 1 patient. Postmortem examination in 1 patient, a chronic alcoholic, showed a pronounced atrophy of the superior cerebellar vermis. Tomographic pneumoencephalograms demonstrated the superior vermis atrophy in the 2 other patients.