Clinical features and prognostic factors of spinal cord sarcoidosis: a multicenter observational study of 20 BIOPSY-PROVEN patients

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.

A practical approach to the diagnosis of spinal cord lesions

“…80 Intramedullary lesions can affect the cervical or thoracic cord73 75 with a mean lesion length of 3.9 segments (range1–9) 72 74. Additional supporting features are overlying leptomeningeal involvement72 (especially with meningomyelitis in the context of sarcoid meningitis75), nerve root involvement81 or persistent enhancement of the lesion on follow-up MRI for more than 2 months despite treatment 52. Clues to additional brain involvement are leptomeningeal enhancement, hydrocephalus (if basal meninges involved), cranial neuropathies (particularly VII and VIII) and involvement of the hypothalamic-pituitary axis or, more rarely, the cavernous sinus 75 82…”

The Burden of Neurosarcoidosis: Essential Approaches to Early Diagnosis and Treatment

“…13 An overview of NS manifestations is displayed in ►Table 1. [2][3][4][5]8,9,[12][13][14][15][16][17][18][19][20][21][22][23][24][25][26]…”

“…►Table 2 provides an overview of presenting symptoms/ signs and associated manifestations. 2,6,13,15,17,18,20,[27][28][29][30][31][32] Multiple simultaneous cranial neuropathies strongly suggest NS and are often associated with basal aseptic meningitis. The onset of spinal cord involvement can be acute and progressive, with early diagnosis and immediate initiation of appropriate treatment being crucial to averting progressive damage-related disability.…”

“…3. Spinal cord disease/myelitis [16][17][18]24 16-28% Subpial intramedullary lesions or leptomeningeal involvement, typically longitudinally extensive; predilection cervicothoracic.…”

Neurosarcoidosis: unusual presentations and considerations for diagnosis and management

“…16 Occasionally hypesthesia and paraesthesia are present 17 with motor dysfunction in over 50% of cases. 18 Patients with neurosarcoidosis affecting the intraventricular space present with memory problems, confusion, nausea and speech disturbances. 19 Endoscopic biopsy has been shown to be the most effective in making a clear diagnosis.…”