Hypertrophic pachymeningitis in conjunction with transverse myelitis is a rare inflammatory disease process that includes diffuse thickening of the dura mater and transverse myelitis. Neurosarcoidosis is a granulomatous disease of the nervous system typically associatied with systemic sarcoidosis, leading to neurological manifestations affecting the brain, spinal cord, cranial nerves, meninges, and peripheral nerves. Here, we present a unique case of transverse myelitis and hypertrophic pachymeningitis for possible consideration of neurosarcoidosis in the absence of systemic sarcoidosis symptoms.

We present a 42 year old male presenting with a chief complaint of tingling and paresthesia in his upper extremities that are more pronounced with exercise. Neurologic exam was notable for a mild right sided paretic gait. MRI of the thoracic spine revealed longitudinally extensive transverse myelitis spanning multiple spinal levels from C7-T2 while brain MRI showed diffuse pachymeningeal enhancement without parenchymal lesions. Differential diagnoses (DDx) included neuromyelitis optica, neurosyphilis, vasculitis, and idiopathic hypertrophic pachymeningitis. HIV, syphilis, aquaporin-4 IgG, and MOG antibody testing were negative. CSF analysis revealed a mildly elevated ACE level of 3.2 (normal limit: 0.0-2.5U/L) however, serum ACE levels and chest CT did not reveal granulomatous disease. Planned meningeal biopsy was not performed due to scheduling constraints. Follow up brain MRI showed resolution of the pachymeningitis while thoracic cord T2 hyperintensity  persisted. Due to presumptive neurosarcoidosis, oral prednisone 20 mg PO daily was started with stable neurologic symptoms at last follow up.