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Discriminating long myelitis of neuromyelitis optica from sarcoidosis
Author(s) -
Flanagan Eoin P.,
Kaufmann Timothy J.,
Krecke Karl N.,
Aksamit Allen J.,
Pittock Sean J.,
Keegan B. Mark,
Giannini Caterina,
Weinshenker Brian G.
Publication year - 2016
Publication title -
annals of neurology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 4.764
H-Index - 296
eISSN - 1531-8249
pISSN - 0364-5134
DOI - 10.1002/ana.24582
Subject(s) - medicine , neuromyelitis optica , myelitis , transverse myelitis , sarcoidosis , optic neuritis , spinal cord , magnetic resonance imaging , lymphocytic pleocytosis , cerebrospinal fluid , multiple sclerosis , pathology , radiology , immunology , encephalitis , virus , psychiatry
Objective To compare longitudinally extensive myelitis in neuromyelitis optica spectrum disorders (NMOSD) and spinal cord sarcoidosis (SCS). Methods We identified adult patients evaluated between 1996 and 2015 with SCS or NMOSD whose first myelitis episode was accompanied by a spinal cord lesion spanning ≥3 vertebral segments. All NMOSD patients were positive for aquaporin‐4–immunoglobulin G, and all sarcoidosis cases were pathologically confirmed. Clinical characteristics were evaluated. Spine magnetic resonance imaging was reviewed by 2 neuroradiologists. Results We studied 71 patients (NMOSD, 37; SCS, 34). Sixteen (47%) SCS cases were initially diagnosed as NMOSD or idiopathic transverse myelitis. Median delay to diagnosis was longer for SCS than NMOSD (5 vs 1.5 months, p < 0.01). NMOSD myelitis patients were more commonly women, had concurrent or prior optic neuritis or intractable vomiting episodes more frequently, had shorter time to maximum deficit, and had systemic autoimmunity more often than SCS ( p < 0.05). SCS patients had constitutional symptoms, cerebrospinal fluid (CSF) pleocytosis, and hilar adenopathy more frequently than NMOSD ( p < 0.05); CSF hypoglycorrhachia (11%, p = 0.25) and elevated angiotensin‐converting enzyme (18%, p = 0.30) were exclusive to SCS. Dorsal cord subpial gadolinium enhancement extending ≥2 vertebral segments and persistent enhancement >2 months favored SCS, and ringlike enhancement favored NMOSD ( p < 0.05). Maximum disability was similar in both disorders. Interpretation SCS is an under‐recognized cause of longitudinally extensive myelitis that commonly mimics NMOSD. We identified clinical, laboratory, systemic, and radiologic features that, taken together, help discriminate SCS from NMOSD. ANN NEUROL 2016;79:437–447