title: Longitudinal ultra-extensive transverse myelitis as a manifestation of neurosarcoidosis.
abstract: OBJECTIVE
  To analyze the clinical characteristics and outcome of patients with neurosarcoidosis manifesting as longitudinal transverse myelitis spanning 6 or more spinal segments.
  
  
  METHODS
  Retrospective analysis of 7 cases from a single institution.
  
  
  RESULTS
  Four males and 5 African-American were included. The mean onset age for neurological symptoms was 49.1 years old. Only 1 patient had a prior diagnosis of sarcoidosis. In all patients, spinal MRI showed contiguous cervical and/or thoracic cord lesions predominantly in a central or centrodorsal location, associated with parenchymal or leptomeningeal gadolinium enhancement. Cerebral spinal fluid (CSF) pleocytosis was present in all and hypoglycorrhachia in 3 patients. Angiotensin-converting enzyme (ACE) level was elevated in the serum of 1 patient while being normal in the CSF of all 4 cases tested. Chest imaging facilitated the diagnosis of sarcoidosis in all cases. The use of corticosteroid and immunosuppressive agents including infliximab and methotrexate led to improved outcome.
  
  
  CONCLUSIONS
  Neurosarcoidosis should be considered in the differential diagnosis of longitudinal ultra-extensive myelitis, even in the absence of previously diagnosed sarcoidosis. Timely usage of corticosteroid and immunosuppressive agents improves the clinical outcome of patients with ultra-extensive spinal cord sarcoidosis.
authors.affiliation: Department of Neurology, Second Artillery General Hospital, Beijing 100088, China.
authors.forename: Lei
authors.initials: L
authors.lastname: Wang
authors.affiliation: Department of Neurology, Cleveland Clinic, Cleveland, OH 44195, USA. Electronic address: liy@ccf.org.
authors.forename: Yuebing
authors.initials: Y
authors.lastname: Li
mesh_terms: D000328:Adult; D001741:Black or African American; D002493:Central Nervous System Diseases / Q000175:diagnosis / Q000503:physiopathology* / Q000628:therapy; D003937:Diagnosis, Differential; D005074:Evoked Potentials, Visual; D005260:Female; D006801:Humans; D007074:Immunoglobulin G / Q000097:blood; D007155:Immunologic Factors / Q000627:therapeutic use; D008137:Longitudinal Studies; D008279:Magnetic Resonance Imaging; D008297:Male; D008875:Middle Aged; D009188:Myelitis, Transverse / Q000175:diagnosis*; D007703:Peptidyl-Dipeptidase A / Q000097:blood; D012189:Retrospective Studies; D012507:Sarcoidosis / Q000175:diagnosis / Q000503:physiopathology* / Q000628:therapy; D013116:Spinal Cord / Q000473:pathology*; D041623:Tomography, Optical Coherence
chemical_list: D007074:Immunoglobulin G; D007155:Immunologic Factors; D007703:Peptidyl-Dipeptidase A
keywords: Immunosuppression; Myelopathy; Neurosarcoidosis; Sarcoidosis; Spinal cord sarcoidosis; Transverse myelitis