(Kiosk 4) Progressive Neurologic Deficits Due to NMO Spectrum Disorder: A Case Presentation

Case Diagnosis: Progressive neurologic deficits due to NMO spectrum disorder

Case Description or Program Description: A previously independent 47 year old female presented in February 2023 from an outside hospital for escalation of care. She initially presented in December with pain radiating down the left arm. She was found to have a C3-C4 intramedullary lesion. One month later, she developed left sided weakness; MRI showed enlargement of the lesion from C2-T1 and she underwent C2-7 laminectomy and duraplasty. She was admitted to inpatient rehabilitation (IPR) but developed new onset tingling, paresthesias, ataxia, and weakness in the right upper extremity. MRI brain and cervical/thoracic spine revealed progression of the cervical intramedullary lesion with multiple enhancing intramedullary thoracic cord nodularities, suspicious for developing metastasis. She was transferred at that time for escalation of care.

Setting: Major academic and referral center with Level I adult trauma

Assessment/Results: The patient underwent a complete workup revealing elevated NMO/aquaporin antibody. Lumbar puncture was done; CSF revealed elevated protein and glucose with negative oligoclonal bands. She underwent posterior cervical laminectomy for biopsy of intradural and intramedullary tumor and repair of pesudomeningocele with dural graft. Pathology was non-neoplastic with features of demyelination, suspicious for neuromyelitis optica spectrum disorder (NMOSD). She completed a course of Decadron and plasmapheresis was initiated. She was transferred to IPR requiring maximal assistance for bed mobility, transfers, and ADLs.

Discussion (relevance): This case demonstrates progressive neurologic deficits, including weakness, paresthesias, and ataxia due to neuromyelitis optica. NMO is a rare disorder associated with aquaporin-4 water channel antibody, which is expressed in the brain, spinal cord, and optic nerves. This antibody causes damage to astrocytes, resulting in demyelination. In the case of this patient, spinal cord and medullary involvement are likely given her deficits. Longitudinally extensive transverse myelitis, the most specific presentation of NMOSD, occurs with spinal cord involvement and results in para or tetraplegia based on the level of spinal cord involvement.

Conclusions: In the case of rapidly progressive neurologic deficits with bilateral involvement, it is important to consider central etiologies such as NMOSD especially in those with history of autoimmune disease. Prompt diagnosis is essential to guide early treatment and prevent severe morbidity and mortality.