Spinal cord neurosarcoidosis.
BACKGROUND
Spinal cord neurosarcoidosis (SN) is problematic to diagnose because it mimics other inflammatory neurologic diseases. The authors report the clinical features of 29 SN cases.


METHODS
They retrospectively reviewed the medical records of 29 histologically proven sarcoidosis patients with spinal cord involvement seen at 3 university medical centers. They collected clinical data including laboratory and radiological findings. Clinical outcomes were assessed retrospectively using the modified Rankin scale.


RESULTS
The cohort included high number of African Americans (16/29, 55%). The lung and intrathoracic lymph nodes were the most common confirmatory biopsy sites (18/29, 62%), whereas the spinal cord was a relatively uncommon one (4/29, 14%). The most common presenting symptoms were lower extremity weakness and paresthesias. Thoracic segment was most frequently involved (21/27, 78%). Lesions were mostly intramedullary (22/27, 81%), although nearly half involved the leptomeninges (13/27, 48%). The average size of a lesion spanned 3.9 spine segments (range, 1-9); 17 of 22 (77%) intramedullary patients had ≥3 spine segments involved. Angiotensin-converting enzyme levels in cerebrospinal fluid were elevated in only 2 of 11 (18%) patients. All patients received glucocorticosteroids. Additional immune-modulating agents were used in 24 of 29 (83%) patients. Scores on the modified Rankin scale at the final follow-up visit were improved.


CONCLUSIONS
Most SN cases were diagnosed indirectly based on extraneural tissue biopsy. Extended spinal cord lesion (≥3 spine segments) may be useful to distinguish SN from multiple sclerosis. Cerebrospinal fluid analysis was of limited value. Most patients experienced clinical improvement with immunosuppressive treatment, but many required combination therapy.
Division of Neurology, Department of Neurosciences (MS, KN), Medical University of South Carolina, Charleston, South Carolina; Pulmonary, Allergy, and Critical Care Medicine (DAC), Cleveland Clinic Foundation, Cleveland, Ohio; Division of Pulmonary and Critical Care Medicine, Department of Internal Medicine (MAJ), Medical University of South Carolina, Charleston, South Carolina; Department of Neurology (TFS), Drexel University College of Medicine, Allegheny Campus, Pittsburgh, Pennsylvania; Neuromuscular Center (JT), Cleveland Clinic Foundation, Cleveland, Ohio; and Division of Pulmonary and Critical Care Medicine (MAJ), Albany Medical College, Albany, New York.
Mimi
M
Sohn
Daniel A
DA
Culver
Marc A
MA
Judson
Thomas F
TF
Scott
Jinny
J
Tavee
Kenkichi
K
Nozaki
D000305:Adrenal Cortex Hormones / Q000627:therapeutic use; D000328:Adult; D002493:Central Nervous System Diseases / Q000175:diagnosis* / Q000188:drug therapy; D005260:Female; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D008168:Lung / Q000473:pathology; D008198:Lymph Nodes / Q000473:pathology; D008297:Male; D008875:Middle Aged; D011859:Radiography; D012507:Sarcoidosis / Q000175:diagnosis* / Q000188:drug therapy; D013116:Spinal Cord / Q000000981:diagnostic imaging / Q000473:pathology; D055815:Young Adult
D000305:Adrenal Cortex Hormones; D007166:Immunosuppressive Agents