M Hahn1, A Hirschfeld, H Sander. 1. Department of Neurosurgery, St. Vincent's Hospital, New York, New York 10011, USA.
Abstract
BACKGROUND: Hereditary motor and sensory neuropathy types I and III usually lead to enlargement of peripheral nerves. Rarely, spinal nerve roots may also be involved, leading to radiculopathy and/or myelopathy. METHODS: This 44-year-old man with back and lower extremity radicular pain and distal lower extremity weakness and numbness was found to have a nonenhancing intradural mass that caused a nearly complete myelographic block from L1-L4. He underwent a decompressive laminectomy with intradural exploration. RESULTS: Hypertrophic but otherwise normal-looking nerve roots were observed. Subsequent electrodiagnostic testing and sural nerve biopsy confirmed that this patient had a previously unsuspected hereditary motor and sensory neuropathy (HMSN). His pain resolved, but at latest follow-up his weakness and numbness persisted. CONCLUSIONS: Nonenhancing spinal intradural mass lesions may represent enlarged nerve roots, which have a number of potential etiologies. Electrodiagnostic studies and peripheral nerve biopsy are instrumental in establishing the diagnosis of HMSN.
BACKGROUND:Hereditary motor and sensory neuropathy types I and III usually lead to enlargement of peripheral nerves. Rarely, spinal nerve roots may also be involved, leading to radiculopathy and/or myelopathy. METHODS: This 44-year-old man with back and lower extremity radicular pain and distal lower extremity weakness and numbness was found to have a nonenhancing intradural mass that caused a nearly complete myelographic block from L1-L4. He underwent a decompressive laminectomy with intradural exploration. RESULTS:Hypertrophic but otherwise normal-looking nerve roots were observed. Subsequent electrodiagnostic testing and sural nerve biopsy confirmed that this patient had a previously unsuspected hereditary motor and sensory neuropathy (HMSN). His pain resolved, but at latest follow-up his weakness and numbness persisted. CONCLUSIONS: Nonenhancing spinal intradural mass lesions may represent enlarged nerve roots, which have a number of potential etiologies. Electrodiagnostic studies and peripheral nerve biopsy are instrumental in establishing the diagnosis of HMSN.