Progressive Necrotizing Myelopathy – a Case Report

Česká verzia

Authors: I. Lisá ¹; V. Belan ²; D. Podhoranská ¹
Authors place of work: II. Neurologická klinika LF UK a FNsP Bratislava, 2Radiodiagnostická klinika SZU a FNsP Bratislava ¹
Published in the journal: Cesk Slov Neurol N 2009; 72/105(3): 265-269
Category: Case Report

Summary

Progressive necrotizing myelopathy is an extremely rare disease, even considered among non-compressive myelopathies. Spinal cord symptoms are characteristic of it, with the peripheral motor neuron severely affected. A typical cerebrospinal fluid finding is increased total protein without oligoclonal bands, sometimes with mild pleocytosis. A specific MRI criterion is the involvement of at least three or more neighbouring vertebral spinal cord segments with hypersignal in the TW2 picture and gadolinium enhancement, evolving over months into atrophy, which corresponds to spinal cord necrosis confirmed on bioptic examination. The range of differential diagnosis is wide. Apart from other more common diseases (multiple sclerosis, inflammatory, neoplastic, systemic, vascular and metabolic causes of myelopathy), rare paraneoplastic spinal cord involvement must also be excluded. Recently, autoimmune pathogenetic mechanisms related to neuromyelitis optica have been considered, based on the occurrence of specific NMO IgG antibodies against aquaporin 4. The disease has a progressive course, although immunomodulatory treatment may possibly prove therapeutic. The authors present the case report of a 67-year-old man with noticeably atopic terrain revealed on immunological examination, fulfilling clinical and MRI criteria for progressive necrotizing myelopathy. Treatment with corticoids had no effect and was therefore followed by intravenous immunoglobulins, which resulted in a temporary improvement in the clinical condition and an improvement in the serious motor deficiency. Two years after the first manifestation of neurological symptoms, a conventional type of carcinoma was diagnosed in the patient from gr. II WHO kidney cells. As a consequence, in spite of repeated negative findings for antineuronal antibodies, we have to consider a possible paraneoplastic origin for the progressive necrotizing myelopathy.

Key words:
progressive necrotizing myelopathy – neuromyelitis optica – paraneoplastic syndrome – intravenous immunoglobulins

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