A Case-Report on Double Seronegative Neuromyelitis Optica Spectrum Disorder (DN NMOSD)

Abstract

Neuromyelitis optica spectrum disorder, formerly known as neuromyelitis optica or Devic's disease, affects roughly 0.3 to 4.4 individuals per 100,000. NMOSD is a peculiar inflammatory disease of the central nervous system that manifests clinically as optic neuritis, transverse myelitis, and specific brainstem and brain-inside syndrome. Here we presented a 55-year-old patient who had been normal months prior to developing numbness of the right half of the face and a history of swaying to the right side. He initially received treatment elsewhere with a five-day course of methylprednisolone pulse therapy, resulting in symptom improvement. However, three months later, he experienced a recurrence and came for further management after receiving a second pulse of steroids. The investigations revealed left-sided facial nerve involvement, sensory loss, and impaired joint position sense and vibration sense in the toes, positive Romberg's sign, and gait ataxia. The magnetic resonance imaging showed T2/FLAIR hyperintense lesions, and cerebrospinal fluid analysis supported a possible diagnosis of neuromyelitis optica spectrum disorder. However, the absence of serum Aquaporin-4 and Myelin Oligodendrocyte Glycoprotein antibodies complicates the diagnosis. The patient was treated effectively for the condition with appropriate glycemic control and is under monitoring for recurrence and progression for any modifications in the treatment as needed. This case highlights the complexity of diagnosing the disease, especially in the context of negative antibody tests, and underscores the need for careful differentiation from other central nervous system disorders.