Allan H Ropper1, Cenk Ayata, Lester Adelman. 1. Neurology Service, St Elizabeth's Medical Center, Tufts University School of Medicine, Boston, Mass. 02135, USA. aropperm@cchcs.org
Abstract
BACKGROUND: Vasculitis of the central nervous system is rare but well described. It affects the cerebral hemispheres predominantly and only exceptionally involves the spinal cord. OBJECTIVE: To describe a case of spinal cord vasculitis with unusual pathologic changes. DESIGN: Case report with clinicopathologic correlation. Case Description A young man developed leg weakness and sensory symptoms over several weeks. He had an asymmetric paraparesis with impaired vibration sense in the feet and a Romberg sign but no sensory level. The cerebrospinal fluid contained 123 white blood cells x103/ micro L, mostly lymphocytes, and a protein concentration of 52 mg/dL; oligoclonal bands were not detected, but the illness simulated multiple sclerosis. Magnetic resonance imaging scans of the spinal cord and brain were normal. His condition improved on several occasions with intravenous infusions of corticosteroid agents, but his neurologic signs gradually worsened over several months, and he acquired a thoracic sensory level and sphincteric abnormalities. An explosive preterminal illness occurred with paraplegia, nystagmus, and coma. The findings of a pathologic examination showed numerous ischemic areas in the spinal cord, some cavitated, and a vasculitis of the leptomeningeal branches of the anterior spinal artery and of subpial vessels. The vessel walls were not necrotic, but many of their lumens were occluded by fibrinous material. There were similar findings in regions of cerebral hemorrhagic infarction. CONCLUSIONS: A destructive and vasculitic process should be considered in cases of subacute myelopathy with persistent cellular reaction in the cerebrospinal fluid and clinical responsiveness to corticosteroid therapy. The magnetic resonance imaging scan of the spinal cord may be normal.
BACKGROUND:Vasculitis of the central nervous system is rare but well described. It affects the cerebral hemispheres predominantly and only exceptionally involves the spinal cord. OBJECTIVE: To describe a case of spinal cord vasculitis with unusual pathologic changes. DESIGN: Case report with clinicopathologic correlation. Case Description A young man developed leg weakness and sensory symptoms over several weeks. He had an asymmetric paraparesis with impaired vibration sense in the feet and a Romberg sign but no sensory level. The cerebrospinal fluid contained 123 white blood cells x103/ micro L, mostly lymphocytes, and a protein concentration of 52 mg/dL; oligoclonal bands were not detected, but the illness simulated multiple sclerosis. Magnetic resonance imaging scans of the spinal cord and brain were normal. His condition improved on several occasions with intravenous infusions of corticosteroid agents, but his neurologic signs gradually worsened over several months, and he acquired a thoracic sensory level and sphincteric abnormalities. An explosive preterminal illness occurred with paraplegia, nystagmus, and coma. The findings of a pathologic examination showed numerous ischemic areas in the spinal cord, some cavitated, and a vasculitis of the leptomeningeal branches of the anterior spinal artery and of subpial vessels. The vessel walls were not necrotic, but many of their lumens were occluded by fibrinous material. There were similar findings in regions of cerebral hemorrhagic infarction. CONCLUSIONS: A destructive and vasculitic process should be considered in cases of subacute myelopathy with persistent cellular reaction in the cerebrospinal fluid and clinical responsiveness to corticosteroid therapy. The magnetic resonance imaging scan of the spinal cord may be normal.