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Clinical features and prognostic factors of spinal cord sarcoidosis: a multicenter observational study of 20 BIOPSY-PROVEN patients.
- Source :
-
Journal of neurology [J Neurol] 2016 May; Vol. 263 (5), pp. 981-990. Date of Electronic Publication: 2016 Mar 23. - Publication Year :
- 2016
-
Abstract
- 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.
- Subjects :
- Adrenal Cortex Hormones therapeutic use
Adult
Aged
Female
Follow-Up Studies
France epidemiology
Humans
Immunosuppressive Agents therapeutic use
Magnetic Resonance Imaging
Male
Middle Aged
Prognosis
Retrospective Studies
Sarcoidosis drug therapy
Sarcoidosis epidemiology
Severity of Illness Index
Spinal Cord diagnostic imaging
Spinal Cord drug effects
Spinal Cord Diseases drug therapy
Spinal Cord Diseases epidemiology
Treatment Outcome
Young Adult
Sarcoidosis diagnosis
Sarcoidosis physiopathology
Spinal Cord Diseases diagnosis
Spinal Cord Diseases physiopathology
Subjects
Details
- Language :
- English
- ISSN :
- 1432-1459
- Volume :
- 263
- Issue :
- 5
- Database :
- MEDLINE
- Journal :
- Journal of neurology
- Publication Type :
- Academic Journal
- Accession number :
- 27007482
- Full Text :
- https://doi.org/10.1007/s00415-016-8092-5