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The "split hand" phenomenon

Evidence of a spinal origin

From the Departments of Neurology (Drs. Schelhaas, van de Warrenburg, and Kremer) and Clinical Neurophysiology (Dr. Zwarts), University Medical Center Nijmegen, the Netherlands.

Address correspondence and reprint requests to Dr. H.J. Schelhaas, University Medical Center Nijmegen, Department of Neurology, Reinier Postlaan 4, P.O. Box 9101, 6500 HB Nijmegen, The Netherlands; e-mail: h.schelhaas{at}neuro.umcn.nl

The clinical phenomenon of a split hand, dominant muscle atrophy in the thenar as compared to the hypothenar complex, has been used to support the theory of primary cortical degeneration in amyotrophic lateral sclerosis. However, the same phenomenon, both clinically and electrophysiologically, was observed in three diseases with a second but not first motor neuron affection: autosomal dominant spinal muscular atrophy, spinocerebellar ataxia type 3, and juvenile muscular atrophy. Neurogenic loss in a split hand distribution points to a spinal instead of cortical origin.

Received April 28, 2003. Accepted in final form August 13, 2003.