Neurosarcoidosis, an uncommon manifestation of extrapulmonary sarcoidosis, presents a diagnostic and therapeutic challenge because it is a rare disease with diverse clinical presentations.

We report the case of a middle-aged African American female with a history of idiopathic intracranial hypertension and hydrocephalus with ventriculoperitoneal shunt who presented with progressive predominantly left-sided gait instability, weakness, and paresthesia. Cerebrospinal fluid (CSF) findings showed elevated lymphocytes, red blood cells, oligoclonal bands, and kappa free light chains, prompting concern for multiple sclerosis (MS). Serum and CSF Aquaporin-4 Antibody tests were negative, effectively eliminating Neuromyelitis Optica Spectrum Disorder as a possible diagnosis. All other labs were normal, and cultures yielded negative results. Imaging studies revealed characteristic findings of longitudinally extensive spinal cord lesion consistent with neurosarcoidosis, including the “trident sign” appearance on contrast-enhanced spinal MRI. Although her CSF findings raised suspicion for MS, her normal brain imaging and longitudinally extensive spinal cord lesion were more consistent with neurosarcoidosis, establishing the presumptive diagnosis and treatment for spinal cord neurosarcoidosis. Management included corticosteroid therapy with SoluMedrol, leading to partial symptomatic improvement. Follow-up imaging studies and evaluations demonstrated continued spinal cord involvement despite treatment.

This case emphasizes the importance of recognizing neurosarcoidosis in patients with atypical neurological symptoms and highlights the challenges in its diagnosis and management. A multidisciplinary approach, including neurological, rheumatological, and radiological expertise, is essential for optimal patient care and tailored treatment strategies. Further research is warranted to establish evidence-based guidelines for the management of neurosarcoidosis.