Longitudinal Extensive Transverse Myelitis: A Diagnostic and Therapeutic Challenge

Date of submission: 11-07-2020 | Date of acceptance: 09-12-2020 | Published: 03-04-2021

Abstract

Neuromyelitis optica spectrum disorders (NMOSD) are inflammatory disorders of the central nervous system characterized by immune-mediated demyelination and axonal damage predominantly targeting optic nerves and spinal cord.

We report a case of an 11-year-old boy, admitted with suspected viral meningitis. 24 hours after admission he developed bladder dysfunction and progressive ascendant sensory loss. The brain and spinal cord MRI showed extensive myelitis. Broad-spectrum antibiotics and immunoglobulin were started. Neurologic symptoms continued to progress and fundoscopy showed bilateral optic papilitis. MRI on eighth day showed progression of the lesions and involvement of the optic nerve, suggesting NMOSD. High dose corticoids, followed by plasma exchange were started. Gradually there was some neurologic recovery. The aquaporin-4 antibodies were negative and infectious and other autoimmune causes were excluded.

Although rare, pediatric-onset NMOSD can have an exuberant presentation with severe sequela. The clinical and imagiological picture should prompt an early diagnosis and aggressive immunosuppressive therapeutic.