Sarcoidosis of the spinal cord is a rare disease. The aims of this study are to describe the features of spinal cord sarcoidosis (SCS) and identify prognostic markers. We analyzed 20 patients over a 20-year period in 8 French hospitals. There were 12 men (60 %), mostly Caucasian (75 %). The median ages at diagnosis of sarcoidosis and myelitis were 34.5 and 37 years, respectively. SCS revealed sarcoidosis in 12 patients (60 %). Eleven patients presented with motor deficit (55 %) and 9 had sphincter dysfunction (45 %). The median initial Edmus Grading Scale (EGS) score was 2.5. The cerebrospinal fluid (CSF) showed elevated protein level (median: 1.00 g/L, interquartile range (IQR) 0.72-1.97), low glucose level (median 2.84 mmol/L, IQR 1.42-3.45), and elevated white cell count (median 22/mm(3), IQR 6-45). The cervical and thoracic cords were most often affected (90 %). All patients received steroids and an immunosuppressive drug was added in 10 cases (50 %). After a mean follow-up of 52.1 months (range 8-43), 18 patients had partial response (90 %), 7 displayed functional impairment (35 %), and the median final EGS score was 1. Six patients experienced relapse (30 %). There was an association between the initial and the final EGS scores (p = 0.006). High CSF protein level showed a trend toward an association with relapse (p = 0.076). The spinal cord lesion was often the presenting feature of sarcoidosis. Most patients experienced clinical improvement with corticosteroids and/or immunosuppressive treatment. The long-term functional prognosis was correlated with the initial severity.

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Background and objectives:Spinal cord sarcoidosis is a rare manifestation of sarcoidosis with a consequent risk of neurological sequelae for the patient. We investigated prognostic factors and efficacy of immunosuppressive treatments in a longitudinal cohort.Methods:We retrospectively studied patients with spinal cord sarcoidosis followed between 1995 and 2021 in seven centers in France. Patients with a definite, probable or possible spinal cord sarcoidosis according to the Neurosarcoidosis Consortium Consensus Group criteria and with a spinal cord involvement confirmed by MRI were included. We analyzed relapse or progression rate with a Poisson model, initial Rankin score with a linear model and change in the Rankin score during follow-up with a logistic model.Results:A total of 97 patients were followed for a median of 7.8 years. Overall mean relapse or progression rate was 0.17 per person-year and decreased over time. At last visit, 46 (47.4%) patients had a loss of autonomy (Rankin score ≥ 2). The main prognostic factors significantly associated with relapse or progression rate were gadolinium enhancement (relative rate [95% CI]: 0.61 [0.4, 0.95]) or meningeal involvement (relative rate [95% CI]: 2.05 [1.31, 3.19]) on spinal cord MRI, and cell count (relative rate [95% CI] per 1 log increase: 1.16 [1.01, 1.33]) on CSF analysis. Relapse or progression rate was not significantly associated with initial Rankin score or EDSS. TNF α antagonists significantly decreased relapse or progression rate compared with corticosteroids alone (relative rate [95% CI]: 0.33 [0.11, 0.98]). Azathioprine was significantly less effective than methotrexate on relapse or progression rate (relative rate [95% CI]: 2.83 [1.04, 7.75]) and change in Rankin score (mean difference [95% CI]: 0.65 [0.23, 1.08]).Discussion:Regarding the relapse or progression rate, meningeal localization of sarcoidosis was associated with a worse prognosis; TNF α antagonists resulted in a significant decrease compared to corticosteroids alone; and methotrexate was more effective than azathioprine.Classification of Evidence:This study provides Class IV evidence that in individuals with spinal cord neurosarcoidosis, TNF α antagonists were associated with decreased relapse or progression rate compared to corticosteroids alone, but other therapies showed no significant benefit.