Hahn M, Hirschfeld A, Sander H
Department of Neurosurgery, St. Vincent's Hospital, New York, New York 10011, USA.
Surg Neurol. 1998 May;49(5):514-8; discussion 518-9. doi: 10.1016/s0090-3019(97)00160-2.
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.
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.
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.
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.
I型和III型遗传性运动和感觉神经病通常会导致周围神经增粗。很少情况下,脊神经根也可能受累,导致神经根病和/或脊髓病。
该44岁男性有背部和下肢神经根性疼痛以及下肢远端无力和麻木,检查发现硬膜内有一不强化的肿块,导致L1 - L4节段几乎完全性脊髓造影阻塞。他接受了减压性椎板切除术及硬膜内探查。
观察到神经根增粗但外观正常。随后的电诊断测试和腓肠神经活检证实该患者患有此前未被怀疑的遗传性运动和感觉神经病(HMSN)。他的疼痛缓解了,但在最近一次随访时,其无力和麻木症状仍持续存在。
硬膜内不强化的肿块病变可能代表增粗的神经根,其有多种潜在病因。电诊断研究和周围神经活检有助于确立HMSN的诊断。