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Compression syndromes due to hypertrophic nerve roots in hereditary motor sensory neuropathy type I

Department of Neurology, The Johns Hopkins University School of Medicine, Baltimore, MD (Drs. Rosen, Cornblath, and Hurko)
Department of Radiology, The Johns Hopkins University School of Medicine, Baltimore, MD (Dr. Wang)
Department of Neurosurgery, The Johns Hopkins University School of Medicine, Baltimore, MD (Dr. Uematsu)

Three patients with hereditary motor sensory neuropathy type I developed neurologic deficits attributable to hypertrophic nerve roots. Compression of the cervical spinal cord by enlarged nerve roots occurred in our index patient. Multilevel decompressive laminectomies relieved the myelopathy. An unrelated patient who had syncope precipitated by neck rotation had hypertrophied nerve roots that eroded into the transverse foramina in juxtaposition to the vertebral arteries. In a 3rd patient, compression of hypertrophied nerve roots within the the cal sac and neural foramina was associated with spinal claudication and radiculopathy, respectively.

Address correspondence and reprint requests to Dr. Rosen, South Shore Neurologic Associates, 280 East Main Street, Bay Shore, NY 11706.

Received February 23, 1989. Accepted for publication in final form April 7, 1989.

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