1King George Medical University, Lucknow, India
Neuromyelitis optica spectrum disorder is a debilitatively relapsing autoimmune neurological disorder affecting young adults. With the advent of steroids, plasma exchange and immunomodulator therapy, the course of the disorder can be controlled upto a comprehensive extent. But the real question is : how long?
Material(s) and Method(s):
In this case report, i present a 27 year old lady suffering from neuromyelitis optica spectrum disorder, relapsing after 8 years of disease onset, just 15 days after stopping azathioprine therapy. This 27 year old vegan, unmarried, non-smoker, non-diabetic lady was diagnosed as neuromyelitis optica spectrum disorder for last 8 years presenting with weakness of both lower limbs along with bladder symptoms and blurring of vision in both eyes for 2 days. She had longitudinally extensive transverse myelitis from thoracic spinal cord d5 to lumbar spinal cord l5 levels and prolonged visual evoked potentials bilaterally. She was found negative for anti-aquaporin antibody and myelin oligodendrocyte glycoprotein antibody. She was treated with pulse dose methyl prednisolone followed by oral steroids with azathioprine initially for 3 months with subsequent monotherapy with azathioprine. She was improving gradually and within 5 years, she attained a modified rankin score score of 0 from an initial score of 4. She took the immunomodulator drug for another 3 years and then stopped from herself after which she developped dense paraplegia with bowel and bladder involvement along with altered mentation.
She was diagnosed with relapse of neuromyelitis optica spectrum disorder with involvement of spinal cord and brain axes. She was treated with standard pulse steroids with plasma exchange therapy followed by immunomodulator therapy but recovery was poor with modified rankin score of 4 remaining unchanged.
Neuromyelitis optica spectrum disorder has an unpredictable time course of relapse. The debilitative nature of the subsequent relapse(s) in this disorder is very grave. The need for standardisation of lifelong therapy with immunomodulatory agents in neuromyelitis optica spectrum disorder can prudentialise prevention of catastrophic neurological outcome.