Spinal cord sarcoidosis: report of seven cases

Eur J Neurol. 2009 Mar;16(3):289-96. doi: 10.1111/j.1468-1331.2008.02409.x.

Abstract

Background: Spinal cord involvement in sarcoidosis is rare, occurring in <1% of patients with sarcoidosis.

Methods: We report seven cases of spinal cord sarcoidosis, seen in two French hospitals over a 13-year period. Presentation of disease, methods of diagnosis and response to treatment, with quantification according to the reduction of the modified Rankin scale (MRS), were noted.

Results: Six patients presented insidious paresthesias or weakness and one a sudden paraplegia. Average MRS at diagnosis was to 2. Spine MRI showed one or several intramedullary lesions in all cases. Diagnosis was confirmed by extra-neural tissue biopsies in all cases, including mediastinoscopy (two patients), coelioscopy (one patient), bronchoscopy (one patient), salivary gland biopsy (one patient) and skin biopsy (two patients). The average follow-up for the group was 49.4 months. All patients responded to corticosteroid therapy with a median reduction of MRS of one point. Five patients received immunosuppressive therapy: cyclophosphamide (two patients), methotrexate (two patients), azathioprine (one patient), mycophenolate mofetyl (one patient), with an inconstant benefit. Patients who received cyclophosphamide presented severe fungaemia.

Conclusion: Based on our study and literature analysis, we propose an algorithm for treatment of spinal cord sarcoidosis.

Publication types

  • Case Reports

MeSH terms

  • Adrenal Cortex Hormones / therapeutic use*
  • Adult
  • Aged
  • Azathioprine / therapeutic use
  • Cyclophosphamide / adverse effects
  • Cyclophosphamide / therapeutic use
  • Female
  • Fungemia / etiology
  • Humans
  • Immunosuppressive Agents / therapeutic use*
  • Magnetic Resonance Imaging
  • Male
  • Methotrexate / therapeutic use
  • Middle Aged
  • Mycophenolic Acid / analogs & derivatives
  • Mycophenolic Acid / therapeutic use
  • Sarcoidosis* / diagnosis
  • Sarcoidosis* / drug therapy
  • Sarcoidosis* / physiopathology
  • Severity of Illness Index
  • Spinal Cord / drug effects
  • Spinal Cord / pathology
  • Spinal Cord Diseases / diagnosis
  • Spinal Cord Diseases / drug therapy*
  • Spinal Cord Diseases / physiopathology

Substances

  • Adrenal Cortex Hormones
  • Immunosuppressive Agents
  • Cyclophosphamide
  • Mycophenolic Acid
  • Azathioprine
  • Methotrexate