Abstract Details

Sarcoidosis is a multisystemic inflammatory disorder that can lead to neurological manifestations in approximately 5-10% of individuals. Typical neurological presentations include cranial neuropathies, aseptic meningitis, peripheral neuropathy, and myopathy. Stroke is an uncommon manifestation of neurosarcoidosis, often affecting young patients without traditional vascular risk factors. Diagnostic criteria for neurosarcoidosis are based on clinical and imaging evidence of central nervous system involvement, with histopathological evidence of non-caseating granulomas in neural tissue, and with exclusion of alternative causes.

We present a case of a 42-year-old African American male with HIV who presented with an insidious onset headache followed by acute development of dysarthria and unilateral facial weakness. Neuroimaging was notable for intraparenchymal and sulcal subarachnoid hemorrhages with evidence of diffuse leptomeningeal and dural enhancement. Meningeal biopsy revealed granulomatous inflammation while lymph node biopsy revealed necrotizing granulomas. Workup included serum interleukin-2 receptor elevation with unrevealing infectious and paraneoplastic panels. Treatment included a three-day course of intravenous methylprednisolone 1000mg daily followed by oral prednisone at 60mg daily with taper to 10mg daily. He was then started on Infliximab 5 mg/kg every four weeks. Neuroimaging four months after treatment initiation revealed significant improvement in disease activity as noted by FLAIR non-suppression resolution. 

Although uncommon, stroke is a recognized manifestation of neurosarcoidosis, which should always be included in differential diagnoses for a young patient presenting with stroke. Necrotizing granulomas are present in a small minority of neurosarcoidosis cases, with noncaseating granulomas being the defining and overwhelmingly predominant histopathologic feature. Over the last few decades, there have been a growing number of cases published with necrotizing sarcoid granulomatosis, suggesting a continuous spectrum of necrosis associated with sarcoidosis, ranging from minimal to extensive.