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.
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 myelitispatients 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.
Authors: Divyanshu Dubey; Sean J Pittock; Karl N Krecke; Padraig P Morris; Elia Sechi; Nicholas L Zalewski; Brian G Weinshenker; Eslam Shosha; Claudia F Lucchinetti; James P Fryer; A Sebastian Lopez-Chiriboga; John C Chen; Jiraporn Jitprapaikulsan; Andrew McKeon; Avi Gadoth; B Mark Keegan; Jan-Mendelt Tillema; Elie Naddaf; Marc C Patterson; Kevin Messacar; Kenneth L Tyler; Eoin P Flanagan Journal: JAMA Neurol Date: 2019-03-01 Impact factor: 18.302
Authors: Nicholas L Zalewski; Alejandro A Rabinstein; Karl N Krecke; Robert D Brown; Eelco F M Wijdicks; Brian G Weinshenker; Timothy J Kaufmann; Jonathan M Morris; Allen J Aksamit; J D Bartleson; Giuseppe Lanzino; Melissa M Blessing; Eoin P Flanagan Journal: JAMA Neurol Date: 2019-01-01 Impact factor: 18.302
Authors: Eoin P Flanagan; Philippe Cabre; Brian G Weinshenker; Jennifer St Sauver; Debra J Jacobson; Masoud Majed; Vanda A Lennon; Claudia F Lucchinetti; Andrew McKeon; Marcelo Matiello; Nilifur Kale; Dean M Wingerchuk; Jay Mandrekar; Jessica A Sagen; James P Fryer; Angala Borders Robinson; Sean J Pittock Journal: Ann Neurol Date: 2016-04-04 Impact factor: 10.422
Authors: Nicholas L Zalewski; Alejandro A Rabinstein; Waleed Brinjikji; Timothy J Kaufmann; Deena Nasr; Michael W Ruff; Eoin P Flanagan Journal: JAMA Neurol Date: 2018-12-01 Impact factor: 18.302