Myelopathy: overview Myelopathy is a disorder of the spinal cord.

Myelopathies may be characterized as intrinsic or intramedullary (lesions are always intradural), or extrinsic or extramedullary (lesions may be intradural or extradural). It may be possible to differentiate intramedullary from extramedullary lesions on clinical grounds, although this distinction is never absolute because of clinical overlap. Pathologies recognized to cause myelopathy include: Intrinsic: Inflammatory disease: myelitis. Primary/Idiopathic: clinically isolated syndrome (transverse myelitis), may be harbinger of multiple sclerosis. Secondary: Post-infectious, e.g., varicella, EBV, mycoplasma, Brucella. Postvaccination (e.g., influenza). Multiple sclerosis, neuromyelitis optica (Devics syndrome). Neurosarcoidosis. Collagen vascular diseases: Systemic lupus erythematosus, Sjgrens syndrome, antiphospholipid syndrome, giant cell arteritis. Infection: HTLV-1 infection, HIV-related vacuolar myelopathy (often discovered incidentally at postmortem), tabes dorsalis. Tumor: primary, secondary. Syringomyelia. Infarction, e.g., anterior spinal artery syndrome. Metabolic causes: vitamin B 12

deficiency producing subacute combined degeneration of the cord, adrenoleukodystrophy (adreno myeloneuropathy). Extrinsic: Prolapsed disc, osteophyte bar. Tumor (primary, secondary). Arteriovenous malformation, dural arteriovenous fistula, hematoma. Abscess.474 A-Z of Neurological Practice Clinical features Intrinsic/intramedullary myelopathy: dependent on the extent to which the cord is involved, the following features may occur: some pathologies have a predilection for posterior columns, central cord, etc. Motor: lower motor neurone signs may be prominent and diffuse; upper motor neurone signs tend to occur late (spastic paraparesis below level of lesion). A combination of upper and lower motor neurone signs is much more likely to reflect intrinsic than extrinsic pathology. Sensory: symptoms of central (funicular) pain may occur; dissociated sensory loss (spinothalamic > dorsal column involvement, or vice versa), suspended sensory loss, and sacral sparing are characteristic of intramedullary lesions; a Brown-Squard syndrome may occur. Vibratory sensibility is more often affected than proprioception. Sphincters: bladder involvement common, often early and slow to recover. Extrinsic/extramedullary myelopathy:

Motor: sequential spastic paraparesis below the level of the lesion; upper motor neurone signs occur early; lower motor neurone signs are unusual and have a segmental (radicular) distribution if present. Sensory: symptoms of pain may be radicular (e.g., secondary to a neu- rofibroma) or vertebral (e.g., secondary to neoplastic or inflammatory processes); sensory signs are not usually marked until the later stages, and all modalities are often involved. A Brown-Squard syndrome may be more common in extrinsic than intrinsic myelopathies. Sphincters: may have bladder urgency, impotence. Investigation Bloods: dependent upon context, may require FBC, vitamin B 12 , autoantibodies, very long-chain fatty acids; serology for HIV, HTLV-1, syphilis, and EBV. Neuroimaging with MR is often helpful in defining the cause of myelopathy. CSF: may be required, for cell count, protein, glucose, oligoclonal bands, and serology. References Andersen O. Myelitis. Curr Opin Neurol. 2000;13:311316 Larner AJ. Diseases of the spinal cord. In: Cox TM, et al., eds. Oxford Textbook of Medicine. 5th ed. Oxford: Oxford University Press; 2010:50395045 Wong SH, Boggild M, Enevoldson TP, Fletcher NA. Myelopathy but normal MRI: where next? Pract Neurol. 2008;8:90102