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October 2004

Spinocerebellar Ataxia and Peripheral Neuropathy

Arch Neurol. 2004;61(10):1625-1626. doi:10.1001/archneur.61.10.1625-c

We read with great interest the recent excellent article by Dr van de Warrenburg et al1 concerning peripheral nerve involvement in patients with spinocerebellar ataxias. In their series, which particularly focused on spinocerebellar ataxia 6 (SCA6), 2 of 7 patients had paresthesia and numbness. Motor weakness was also recognized in 2 patients. Furthermore, electrophysiologic abnormalities were observed in 1 patient and interpreted as axonal sensorimotor neuropathy; this was not disease related. We would like to introduce our limited experience concerning electrophysiologic studies of SCA6. We evaluated 4 patients with SCA6 (2 men and 2 women, aged 50 to 70 years). None of our patients had motor or sensory symptoms and signs. Results of nerve conduction studies and needle electromyography were normal, and repeated studies showed no evidence of neuromuscular abnormalities in these patients. Did the authors perform repeated studies or single-fiber electromyography?