Abstract
Introduction
Sarcoidosis is a multisystem disease characterized histologically by noncaseating granulomas. Localization of sarcoidosis to the CNS is termed neurosarcoidosis, a complex and rare neuroinflammatory form of sarcoidosis. When the spinal cord is involved, lesions are often intradural. Here, we present a rare case of progressive myelopathy secondary to multifocal spinal extradural neurosarcoidosis with spinal cord compression and without pulmonary involvement.
Case presentation
A 29-year-old African American female presented to the emergency department with numbness and paresthesia of 2-month duration in her left lower extremity and 2-week duration in her right lower extremity. The patient reported difficulty ambulating, paresthesia below the umbilicus, and back pain radiating to bilateral lower extremities. She endorsed 9-month history of cough, subjective fevers, night sweats, and unintentional 15 kg weight loss. Examination revealed 4/5 strength in the left lower extremity. MRI of the brain and spinal cord revealed enhancing extradural lesions, with spinal cord compression at T8 measuring 1.3 × 1.9 cm. Lumbar puncture demonstrated oligoclonal bands and increased CSF neutrophils, lymphocytes, monocytes, and protein. T8 laminectomy with resection of the epidural lesion was performed. Histology showed granulomas, consistent with neurosarcoidosis. At follow-up, repeat spinal MRI revealed disease progression with intramedullary involvement. Long-term immunosuppressive treatment was eventually initiated with satisfactory response.
Discussion
This is a rare case of myelopathy secondary to spinal extradural neurosarcoidosis. Spinal neurosarcoidosis is predominantly an intradural process. Our review of the literature identified only seven cases of extradural neurosarcoidosis presenting with compressive myelopathy. Additional insight into management and rehabilitation following pathological diagnosis is of clinical significance.
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Acknowledgements
The authors wish to extend special thanks to Haroon Majoka, MD, and Anum Riaz, MD. This case report would not have been possible without their guidance in the initial hospital management of this patient.
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RS proposed the concept for this article. Material preparation and literature search were performed by RS, OS and LR. The first draft of the manuscript was written by RS, OS and LR. Editing and proofreading were performed by SS and JVM. GC and DI supplied the histology slides and interpretation. SN guided the revisions of the subsequent versions of the manuscript. All authors read and approved the final article.
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Shields, R., Sagan, O., Roebke, L. et al. Rare case of multifocal extradural and intramedullary neurosarcoidosis without pulmonary involvement: a case report and literature review. Spinal Cord Ser Cases 7, 89 (2021). https://doi.org/10.1038/s41394-021-00450-1
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DOI: https://doi.org/10.1038/s41394-021-00450-1
