Abstract

Background: Sarcoidosis, a multisystem inflammatory non-caseating granulomatous disease, can present with neurologic lesions in up to 10% of patients.

Case Description: A 57-year-old male presented with three months of worsening upper extremity radicular pain associated with dysmetria, hyperreflexia, bilateral Hoffman’s, and positive Babinski signs. The contrast magnetic resonance imaging (MRI) showed a diffuse T2 signal hyperintensity and T1-enhancing 2.5 cm lesion extending sagittally between C4 and C6. The cerebrospinal fluid analysis showed a high protein level and lymphocytic pleocytosis. A cardiac positron emission tomography scan was consistent with the diagnosis of cardiac sarcoidosis. With the diagnosis of multisystemic/probable neurosarcoidosis, the patient was unsuccessfully treated with intravenous methylprednisolone, followed by infliximab. Due to severe cord compression/myelopathy, a C3–C6 laminectomy and C3–C7 posterior spinal fusion were performed. Postoperatively, the patient developed a transient right-sided hemiparesis. Over nine postoperative months, the patient had four relapses of transient repeated episodes of paresis, although follow-up cervical MRI scans revealed adequate cord decompression with a stable intramedullary hyperintense lesion.

Conclusion: Patients with neurosarcoidosis respond unpredictably to surgical decompression and require prolonged medical care, which is often unsuccessful.

Keywords: Cervical intramedullary lesion, Laminectomy, Neurosarcoidosis

INTRODUCTION

Sarcoidosis, a chronic multisystem inflammatory non-caseating granulomatous disease of unknown etiology, can present with neurologic lesions in up to 10% of patients, including intramedullary or extramedullary spinal cord lesions.[ 12 ] As patients with spinal sarcoidosis may exhibit inconsistent responses to steroids and other medications, surgery is often warranted. Here, we described a patient with a C4–C6 intramedullary cervical sarcoid lesion and the patient’s variable responses to surgical decompression and subsequent medical management.

CASE PRESENTATION

Clinical findings

A 47-year-old African American male with a past medical history of glucose-6-phosphate dehydrogenase deficiency presented with three months of worsening bilateral upper extremity radicular pain/numbness and tingling radiating to both shoulders. On examination, he had bilateral hyperreflexia, bilateral Hoffmann’s and Babinski’s signs, all reflecting the presence of significant cervical myelopathy.

Radiological diagnosis of sarcoidosis

The cervical magnetic resonance imaging (MRI) demonstrated congenital spinal stenosis and a 2.5 cm intramedullary C4–C6 spinal cord lesion that enhanced with contrast on the T2 image and was diffusely hyperintense on the T2-weighted studies [ Figures 1 - 4 ]. The abdominal-chest computed tomography and endobronchial ultrasound-guided fine-needle aspiration (i.e., of mediastinal hilar lymphadenopathy) revealed non-caseating granulomas consistent with sarcoidosis. The cardiac positron emission tomography scan also confirmed cardiac sarcoidosis characterized by a patchy abnormal increased signal u in the left ventricle. Further, the cerebrospinal fluid (CSF) protein level was high (>390) [ Table 1 ].

Figure 1:

(a) Preop axial T1-weighted magnetic resonance imaging (MRI) of the cervical spine at C5. (b) Preop sagittal T1 weighted MRI of the cervical spine with an enhanced intramedullary spinal cord lesion from C4 to C6.

Figure 2:

(a) Preoperative axial T2-weighted magnetic resonance imaging (MRI) of the cervical spine at C5. (b) Preoperative sagittal T2-weighted MRI of the cervical spine revealed spinal cord hyperintensity from the corticomedullary junction to the T1 level.

Figure 3:

(a) Postoperative C3–C7 fusionanterior–posterior X-ray of the cervical spine. (b) Postoperative C3–C7 fusion lateral X-ray of the cervical spine.

Figure 4:

Postoperative mid-sagittal T2-weighted magnetic resonance imaging of the cervical spine at C5.

Table 1:

Patient-specific clinical findings suggestive of neurosarcoidosis.

Initial failed medical management

With the initial diagnosis of multisystemic sarcoidosis/probable neurosarcoidosis, the patient was started on daily IV methylprednisolone (dose of 1 g). After developing a left punctate pontine stroke (i.e., resulting in left internuclear ophthalmoplegia), vertical nystagmus, and mild left ptosis, he was started on clopidogrel and aspirin. However, as the subsequent contrast brain MRI revealed pachymeningitis, leptomeningitis, and a T2-hyperintense lesion in the pons, the patient was subsequently started on infliximab (5 mg/kg) and referred to neurosurgery with the diagnosis of severe cervical myelopathy secondary to a cervical C4–C6 intramedullary neurosarcoid lesion.

Cervical surgery

To decompress but not biopsy the C4–C6 intramedullary mass, the patient underwent a C3–C6 laminectomy and C3– C7 posterior spinal fusion. Although one day postoperatively, he developed a new right-sided motor hemiparesis that lasted for two weeks; the repeat MRI scan did not show any new cord lesions or increased intrinsic/extrinsic cord compression. Nine months later, the patient experienced four transient relapses of hemiparesis/quadriparesis (i.e., none of which warranted or were treated surgically due to no new MR findings) for which he received varying doses of rituximab, methotrexate, and corticosteroids [ Table 2 ].

Table 2:

Summary of clinical events experienced by the patient.

DISCUSSION

Diagnosis of neurosarcoidosis and systemic sarcoidosis

The diagnosis of neurosarcoidosis is confirmed by laboratory evidence of central nervous system inflammation (i.e., elevated levels of CSF protein and/or cells) and MRI-documented neural lesions (i.e., T2 enhancement of meningeal or parenchymal tissues). In addition, patients may have evidence of systemic sarcoidosis and demonstrate cardiopulmonary, dermatologic, or ophthalmologic symptoms/signs and/or elevated inflammatory laboratory markers reflective of sarcoidosis requiring further medical management.[ 12 , 15 ]

No consensus regarding pharmaceutical management of neurosarcoidosis

There is presently no consensus regarding the pharmaceutical management of neurosarcoidosis. The first line of treatment is typically the administration of corticosteroids (i.e., varying dosage, duration, and method). However, if this fails, alternative immunomodulatory therapies are offered (i.e., tumor necrosis factor alpha inhibitors, infliximab). For instance, in Moravian and Segal’s series, seven patients with glucocorticoid-refractory neurosarcoidosis responded favorably to infliximab.[ 9 ]

Surgery

A decline in neurological function has been reported following extensive decompressive surgeries, underscoring the unpredictable course of neurosarcoidosis.[ 4 , 7 ] Patients with cervical neurosarcoidosis variably respond to surgical biopsy, decompression alone, laminoplasty alone, or laminectomy with fusion in addition to preoperative/postoperative corticosteroids and immunotherapy.[ 1 , 2 , 4 , 6 , 7 , 10 , 11 , 13 , 14 ] In one study, 4 of 12 patients with cervical neurosarcoidosis improved for two months following cervical decompression alone (i.e., without biopsy); notably, preoperative nonmyelopathic patients had better outcomes.[ 13 ] Another series also successfully managed intramedullary neurosarcoidosis/cervical stenosis with decompression only.[ 10 ] In a review study, 50% of patients undergoing decompressions without biopsy worsened postoperatively versus a lower 28% in worsening after laminectomy with biopsy.[ 7 ] Furthermore, cervical laminoplasty without biopsy performed for neurosarcoidosis also resulted in new postoperative neurologic deficits that failed to respond to corticosteroids.[ 11 ] Our patient, following a decompressive cervical laminectomy without biopsy, developed fluctuating postoperative neurological deterioration that was not controlled/mitigated by the administration of corticosteroids and/or immunotherapy. Posterior instrumented fusion was performed to preserve the cervical alignment and avoid post-laminectomy kyphosis.[ 8 ] Table 3 summarizes the literature of intramedullary cervical neurosarcoidosis treated with decompression without biopsy.

Table 3:

Summary of the literature on cases of intramedullary cervical neurosarcoidosis treated with decompression and no biopsy.

CONCLUSION

Patients with neurosarcoidosis may respond unpredictably, with marked fluctuations/exacerbations and remissions, to either medical (i.e., primarily to corticosteroids and secondarily to immunotherapies) and/or surgical management (i.e., decompression with/without fusion).

Ethical approval

Institutional Review Board approval is not required.

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 conflict of interest.

Use of artificial intelligence (AI)-assisted technology for manuscript preparation

The authors confirm that there was no use of artificial intelligence (AI)-assisted technology for assisting in the writing or editing of the manuscript and no images were manipulated using AI.

Disclaimer

The views and opinions expressed in this article are those of the authors and do not necessarily reflect the official policy or position of the Journal or its management. The information contained in this article should not be considered to be medical advice; patients should consult their own physicians for advice as to their specific medical needs.

References

1. Chen HI, Lang SS, Coyne TM, Malhotra NR, Schuster JM. Intramedullary spinal sarcoidosis masquerading as cervical stenosis. World Neurosurg. 2013. 80: e375-80

2. Hayat GR, Walton TP, Smith KR, Martin DS, Manepalli AN. Solitary intramedullary neurosarcoidosis: Role of MRI in early detection. J Neuroimaging. 2001. 11: 67-70

3. Jefferson M. Sarcoidosis of the nervous system. Brain. 1957. 80: 540-56

4. Kasliwal MK, Harbhajanka A, Nag S, O’Toole JE. Isolated spinal neurosarcoidosis: An enigmatic intramedullary spinal cord pathology-case report and review of the literature. J Craniovertebral Junction Spine. 2013. 4: 76-81

5. Kwon DH, Lee SH, Kim ES, Eoh W. Intramedullary sarcoidosis presenting with delayed spinal cord swelling after cervical laminoplasty for compressive cervical myelopathy. J Korean Neurosurg Soc. 2014. 56: 436-40

6. Maroun FB, O’Dea FJ, Mathieson G, Fox G, Murray G, Jacob JC. Sarcoidosis presenting as an intramedullary spinal cord lesion. Can J Neurol Sci. 2001. 28: 163-6

7. Mathieson C, Mowle D, Ironside J, O’Riordan R. Isolated cervical intramedullary sarcoidosis--a histological surprise. Br J Neurosurg. 2004. 18: 632-5

8. McAllister BD, Rebholz BJ, Wang JC. Is posterior fusion necessary with laminectomy in the cervical spine?. Surg Neurol Int. 2012. 3: S225-31

9. Moravan M, Segal BM. Treatment of CNS sarcoidosis with infliximab and mycophenolate mofetil. Neurology. 2009. 72: 337-40

10. Nurboja B, Chaudhuri A, David KM, Casey AT, Choi D. Swelling and enhancement of the cervical spinal cord: When is a tumour not a tumour?. Br J Neurosurg. 2012. 26: 450-5

11. Oe K, Doita M, Miyamoto H, Kanda F, Kurosaka M, Sumi M. Is extensive cervical laminoplasty an effective treatment for spinal cord sarcoidosis combined with cervical spondylosis?. Eur Spine J. 2009. 18: 570-6

12. Pirau L, Lui F, editors. Neurosarcoidosis. StatPearls. Treasure Island, FL: StatPearls Publishing; 2023. p. Available from: https://www.ncbi.nlm.nih.gov/books/NBK534768 [Last accessed on 2023 Jul 02]

13. Sakai Y, Matsuyama Y, Imagama S, Ito Z, Wakao N, Ishiguro N. Is decompressive surgery effective for spinal cord sarcoidosis accompanied with compressive cervical myelopathy?. Spine. 2010. 35: E1290-7

14. Saleh S, Saw C, Marzouk K, Sharma O. Sarcoidosis of the spinal cord: Literature review and report of eight cases. J Natl Med Assoc. 2006. 98: 965-76

15. Zajicek JP, Scolding NJ, Foster O, Rovaris M, Evanson J, Moseley IF. Central nervous system sarcoidosis--diagnosis and management. QJM. 1999. 92: 103-17