Cervical epidural neurosarcoidosis: A case report and literature review
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Abstract
Background:
Neurosarcoidosis is a rare disease. In the spine, it commonly presents as an intramedullary lesion. Epidural spinal lesions are extremely rare.Case Description:
A 29-year-old patient presented with a 22-month history of progressive neck, upper limb pain, and myelopathy. The cervical MRI showed a large epidural mass infiltrating the paraspinal soft tissue. After an open biopsy, the diagnosis of neurosarcoidosis was established and was followed-up by appropriate medical management.Conclusion:
To manage cervical epidural neurosarcoidosis, first, you must obtain a tissue diagnosis and then follow with appropriate medical management.INTRODUCTION
Sarcoidosis is a multisystem disease attributed to an accelerated immune response of unknown etiology.[7] Systemic sarcoidosis occurs in 1–3/100,000 individuals; only 5% of lesions involve the nervous system (e.g., neurosarcoidosis). Just 10% of those with neurosarcoidosis, which have spinal lesions that are typically located within the cord itself (i.e., intramedullary).[7]
Here, we present a 29-year-old female with left-sided C2–C7 epidural cervical neurosarcoidosis diagnosed utilizing an open biopsy, followed by appropriate medical management.
CASE REPORT
A 29-year-old female presented with a 22-month history of progressive dysphagia, neck pain, and cervical myelopathy. Although she had no motor deficit, she exhibited impaired pin appreciation on the left side from C2–C7, bilateral Hoffman’s signs, and diffuse upper and lower extremity hyperreflexia.
The computed tomography (CT) and full neuraxis enhanced MR studies showed a left-sided epidural enhanced mass from C2 to C7 compressing the spinal cord and displacing it to the right with a paravertebral extension [Figure 1a-d].

Three days after the presentation, a CT-guided biopsy was performed. However, due to the initiation of steroids (dexamethasone 4 mg every 6 h), the tumor had significantly decreased in size (e.g., repeat MRI showed that the lesion had profoundly regressed), and a biopsy was no longer feasible/ reliable [Figure 2a-d].

The next MR performed 2 weeks after cessation of steroids revealed significant tumor recurrence [Figure 3a and b]. The repeated CT-guided biopsy revealed a granuloma in a background of nonneoplastic skeletal muscular and fibrous tissues. This was followed by an open anterior C5–C6 biopsy of the large recurrent extradural lesion; the latter confirmed the diagnosis of neurosarcoidosis (i.e., nonnecrotizing granulomatous lymphadenitis) neurosarcoidosis [Figure 4a and b]. She was later discharged on 60 mg prednisone/day and placed on an 8-week taper. Three months later, she exhibited substantial clinical improvement.


DISCUSSION
Sarcoidosis is a chronic systemic inflammatory disease characterized by noncaseating granulomas on pathological examination. It most commonly affects the lungs and mediastinal lymph nodes.[7] Only approximately 5% of all sarcoidosis patients have central nervous system involvement; most of these lesions are intracranial.[7,6]
Just 10% of neurosarcoidosis involve the spine. Seven similar cases found in the literature. Of interest, most are intramedullary lesions, and few involve the extradural compartment [Table 1].[2,5,6]

Most cases of neurosarcoidosis have normal levels of ACE, and the histology demonstrates nonnecrotizing granulomas with an inflammatory background [Table 1].[1,7,8]
Although medical management is the standard of care for neurosarcoidosis, most patients initially undergo biopsy\ surgical decompression or debulking to obtain pathology [Table 1]. Medical management usually includes steroids (e.g., in some cases, require a prolonged course of steroid utilization) along with immunosuppressive medications(e.g., methotrexate).[3,4]
CONCLUSION
Epidural spinal neurosarcoidosis is very rare. Establishing a pathological diagnosis warrants cessation of steroids (i.e., if previously administered), and the performance of a biopsy\ or open surgical debulking. Once pathology has been established, these lesions can be typically managed with short-\long-term steroids and/or other medical regimens.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent.Financial support and sponsorship
Nil.Conflicts of interest
There are no conflicts of interest.REFERENCES
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