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

Abstract

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 thecal sac and neural foramina was associated with spinal claudication and radiculopathy, respectively.