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  • Updated 06.25.2023
  • Released 05.08.1995
  • Expires For CME 06.25.2026

Transverse myelitis



Transverse myelitis is inflammation spanning both sides of at least one segment of the spinal cord. The inflammation injures the myelin that protects the nerve fibers. There are multiple etiologies for transverse myelitis. In this updated article, the author describes new developments in the diagnosis of subgroups of myelitis caused by myelin oligodendrocyte glycoprotein (MOG)-Ab and its comparison to multiple sclerosis and neuromyelitis optica.

Key points

• Acute transverse myelitis, as defined here, is idiopathic. It excludes other forms of myelitis such as myelin oligodendrocyte glycoprotein-associated disease (MOGAD), neuromyelitis optica (NMO, Devic disease), multiple sclerosis, postinfectious and postvaccinal myelitis, etc.

• Cord symptoms evolve over hours to days and then typically resolve over several weeks or months.

• The pathology in the spinal cord is similar to lesions in multiple sclerosis, but only 40% will develop multiple sclerosis.

• Cord lesions in MOGAD, NMO, and CNS Sjögren disease are longitudinal, down the center of the cord; in multiple sclerosis the lesions are shorter and acentric.

• High-dose glucocorticosteroids hasten recovery from an attack but may have no long-term benefit.

Historical note and terminology

Transverse lesions of the spinal cord were historically ascribed to thrombosis from arteriosclerosis, syphilis, and other infections (ie, Bastian in the 1880s) (49), and this concept persisted well into the next century. Foix and Alajouanine believed that transverse myelitis had a vascular etiology (48). They described two patients with subacute necrosis in the sacral or thoracolumbosacral cord associated with massive dilatation and endomysial hypertrophy of the extramedullary veins and similar but less marked changes in the intramedullary vessels. The lumens were not obliterated, and the arteries were not involved. The term "angiodysgenetic necrotizing myelopathy" is sometimes applied to this condition (49). A similar syndrome is believed to be caused by spinal dural arteriovenous fistulae (109). Paine and Byers were the first to report a large clinical series with follow-up exams and used the term “transverse myelopathy” (123). They postulated a vascular cause but presented no pathological evidence. In most of these early reports, one or two patients were studied, and the autopsies were performed months to years after the original illness (71).

Several large series describing idiopathic transverse myelitis appeared in the 1960s and 1970s and seldom showed vascular alterations (03; 100; 140; 18). Some assumed the condition was an inflammatory myelitis from an antigen-antibody reaction (131) or a hypersensitivity reaction to infection or vaccination (113). A consensus evolved that acute transverse myelitis was an inflammatory demyelinating disease of the cord. It sometimes followed infections but usually had no antecedent. In a series of 33 cases of acute or subacute noncompressive myelopathy, 46% were parainfectious, 12% were associated with cord ischemia, 21% were multiple sclerosis exacerbations, and 21% were idiopathic (74).

Acute transverse myelitis, as defined here, is an idiopathic disorder caused by inflammation of the spinal cord, associated with marked demyelination and often with significant axonal loss. The specific process that instigates the inflammation in transverse myelitis is unknown. (This includes the third of transverse myelitis patients with antecedent virus infections.)

Diagnostic criteria for transverse myelitis include (1) neurologic symptoms attributable solely to the spinal cord, negative brain MRI, and no clinical brain symptoms; (2) bilateral signs or symptoms; (3) clearly defined sensory level; (4) MRI exclusion of compression; (5) inflammation in the spinal cord (CSF white cells or elevated IgG index, or MRI gadolinium enhancement) seen within seven days of onset; (6) progressive worsening with maxima from 4 hours to 21 days after onset; and (7) no other etiology such as infection, neoplasm, compressive, trauma, embolus, degeneration, or vitamin deficiency (164). With these strict criteria, only 16% of 288 patients with acute transverse myelitis from all causes have idiopathic acute transverse myelitis (37). Scott and colleagues counter that inflammation is less severe in transverse myelitis than in multiple sclerosis and, thus, inflammation should not be used as a criterion (151). Acute transverse myelitis can be split into complete or partial transverse myelitis, each with a different outcome (148).

These definitions are undergoing revision. Subsets of transverse myelitis are now associated with antibodies against myelin oligodendrocyte glycoprotein (MOG) and aquaporin-4 (“NMO-IgG”; neuromyelitis optica [NMO], Devic disease), partially overlapping with Sjögren disease (72). These differ markedly from multiple sclerosis. Unfortunately, many of the classic treatises on idiopathic transverse myelitis likely included patients with these disorders.

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