Progressive unilateral tremor associated with large confluent perirolandic juxtacortical lesions in multiple...

Multiple sclerosis (MS)-related tremor is classically attributed to infratentorial lesions.

To characterize unilateral tremor associated with perirolandic lesions in MS.

This retrospective study included Mayo Clinic patients diagnosed with MS who had: 1) Progressive unilateral tremor; and 2) Contralateral perirolandic juxtacortical T2-lesion. Medical records, neuroimaging and movement laboratory studies were evaluated.

Of 12 patients, 8 were female. Median age of tremor onset was 36.5 years (range, 23-49), with diagnosis of MS a median of 101 months (range, 5-238) after. Tremor was the first MS symptom in 10 and all had progression. Most had tremors (10/12) in the 4.5 - 7 Hz range, while 2 had Holmes tremors in 3 - 5 Hz range. The perirolandic lesions were often large and confluent. All 3 with acute/subacute MRIs (within 4 months of onset) had enhancement. There was lesional atrophy in 6/11 with follow-up imaging available. Detailed treatment outcomes are described.

Progressive unilateral tremor with contralateral perirolandic lesion is a novel phenotype of progressive MS and the pathogenesis may reflect disruption of cerebello-thalamo-cortical circuitry.