Single Center Experience with Isolated Spinal Cord Neurosarcoidosis

World Neurosurg. 2021 Dec:156:e398-e407. doi: 10.1016/j.wneu.2021.09.089. Epub 2021 Sep 25.

Abstract

Introduction: Isolated spinal cord neurosarcoidosis is extremely rare. The potential implications of long-term immunosuppressant therapy make correct diagnosis imperative. However, there are challenges inherent in isolated spinal cord involvement that require a multidisciplinary approach. Here we present the largest series of definite and possible isolated spinal neurosarcoidosis and discuss our institutional experience in managing this rare but morbid condition.

Methods: A retrospective review was performed to identify all neurosarcoidosis cases starting from 2002 to 2020 at our institution. Patients were screened for cases of isolated spinal neurosarcoidosis. A descriptive analysis was performed for each case.

Results: A total of 64 cases of neurosarcoidosis were identified. The spine was involved in 26 (40.6%) patients. Only 4 (6.3%) cases had isolated spinal cord involvement. A full medical and imaging workup was performed in determining isolated spinal cord involvement. Three patients subsequently underwent surgical biopsy, and 1 did not undergo biopsy because of patient preference. One of the patients who underwent biopsy had an initial nondiagnostic biopsy and had a repeat biopsy. Corticosteroids were employed in all cases with additional immunosuppressive agents for maintenance therapy and refractory cases. All showed radiographic improvement and were clinically stable to improved.

Conclusion: Isolated spinal cord involvement of neurosarcoidosis is rare and can present challenges in diagnosis. A biopsy can be performed when necessary. However, a biopsy of the spinal cord carries inherent risks and may not always be possible or result in a nondiagnostic sample. In the setting of high clinical suspicion, maximal medical therapy is still employed.

Keywords: Biopsy; Isolated; Neurosarcoidosis; Spinal cord.

MeSH terms

  • Adrenal Cortex Hormones / adverse effects
  • Adrenal Cortex Hormones / therapeutic use
  • Biopsy
  • Central Nervous System Diseases / diagnosis
  • Central Nervous System Diseases / epidemiology
  • Central Nervous System Diseases / therapy*
  • Combined Modality Therapy
  • Drug Resistance
  • Female
  • Humans
  • Immunosuppressive Agents / adverse effects
  • Immunosuppressive Agents / therapeutic use
  • Magnetic Resonance Imaging
  • Male
  • Middle Aged
  • Neurosurgical Procedures / methods
  • Retrospective Studies
  • Sarcoidosis / diagnosis
  • Sarcoidosis / epidemiology
  • Sarcoidosis / therapy*
  • Spinal Cord Diseases / diagnosis
  • Spinal Cord Diseases / epidemiology
  • Spinal Cord Diseases / therapy*

Substances

  • Adrenal Cortex Hormones
  • Immunosuppressive Agents

Supplementary concepts

  • Neurosarcoidosis