A Rare Neuroinflammatory Overlap: Hypertrophic Pachymeningitis and Longitudinal Transverse Myelitis as a Possible Manifestation of Neurosarcoidosis
Lydia Ta1, Sabeen Wazir2, Grace Lee1, Ariel Antezana3
1Edward via College of Osteopathic Medicine - Louisiana Campus, 2Edward Via College of Osteopathic Medicine - Louisiana Campus, 3NeuroMedical clinic of cenla
Objective:

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.


Background:

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.


Design/Methods:
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Results:
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Conclusions:
Hypertrophic pachymeningitis in conjunction with longitudinal extensive transverse myelitis (HP) is exceedingly rare, with current knowledge derived primarily from isolated case reports and small case series. HP/TM in neurosarcoidosis has rarely been reported and presented as a confounding factor in this patient. We hope this case expands the differential diagnoses for patients presenting with pachymeningitis and adds to the limited literature on this phenomenon.
Generative AI Usage
No, did not use generative AI in the drafting or editing in this abstract.
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