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

BACKGROUND

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

OBJECTIVE

To characterize unilateral tremor associated with perirolandic lesions in MS.

METHODS

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.

RESULTS

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.

CONCLUSION

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.