2395 Case report: neurosarcoidosis presenting with bizarre gait and extensive pulmonary emboli

Objectives & Methods Neurosarcoidosis accounts only for 5–10% of Sarcoidosis cases,¹ neurological complaints however maybe the first prompt for medical review.

This case report from a tertiary neurology centre illustrates that Neurosarcoidosis may present in an advanced state with a slow progressive course and neurological findings inconsistent with a single lesion location.

Results We present a 55-year-old female, referred to Neurology clinic with a six-month history of progressive neurological symptoms including transient diplopia, gait disturbance and reduced balance with left lowerlimb weakness, ataxia, altered sensation and proprioception.

MRI of the brain and spine demonstrated diffuse enhancing nodular lesions along the dura and leptomeninges in the brain, cervical and thoracic spine including the pontine and interpeduncular cisterns, medulla and cervicomedullary junction.

Further investigations with CT and PET revealing extensive pulmonary thromboembolic disease and multiple prominent, FDG avid lymph nodes. Serum and CSF ACE were significantly elevated with samples demonstrating no evidence of malignancy or infection.

Biopsy of the supraclavicular node demonstrated granulomatous lymphadenitis where well-formed granulomas replaced nodal parenchyma. These demonstrated chronic features having undergone regression and fibrosis. Findings were consistent with a diagnosis of Sarcoidosis.

The patient was initially commenced on short course of intravenous corticosteroids, with Infliximab² added early due to the extensive disease burden.

Conclusions This case highlights the potential varied clinical presentation of Neurosarcoidosis even in the context of diffuse and longstanding disease burden. With cranial nerve neuropathies being the most common presentation of Neurosarcoidosis³; this bizarre presentation emphasises the need for targeted work-up in atypical cases.

References

1. Ungprasert P, Matteson EL. Neurosarcoidosis. Rheum Dis Clin North Am. 2017;43(4):593–606.

2. Fritz D, Timmermans WMC, van Laar JAM, et al. Infliximab treatment in pathology-confirmed neurosarcoidosis. Neurol Neuroimmunol Neuroinflamm. 2020;7(5):e847.

3. Kidd DP. Neurosarcoidosis: clinical manifestations, investigation and treatment. Practical Neurology 2020;20:199–212.