Objective: Highlight a complex hyperkinetic movement disorder in Neuromyelitis optica spectrum disorder exacerbation with remission on fingolimod therapy.

Background: Neuromyelitis optica spectrum disorder (NMOSD) can present with hyperkinetic movement disorders, such as dystonia, myoclonus, and chorea/ballism. Occasionally, the movements are complex, posing a diagnostic and therapeutic challenge. NMOSD can present with hyperkinetic movement disorders, such as dystonia, myoclonus, and chorea/ballism. Occasionally, the movements are complex, posing a diagnostic and therapeutic challenge.

Method: A 57-year-old woman diagnosed with seronegative NMOSD of three years duration presented with involuntary, nonrhythmic, non-suppressible, purposeless movements affecting the right upper extremities, accompanied by weakness, numbness, and impaired proprioception. The hyperkinetic movements were noted more with postural maintenance and varied in speed and amplitude. The movements were seemingly complex and did not meet the classic definition of tremor, myoclonus, chorea, or dystonia.

Results: Cranial MRI and electroencephalogram were unremarkable. Cervical MRI showed an ill-defined hyperintense intramedullary lesion at the level of C1-C6. Given the current extent of the spine lesion, which previously just affected the C1-C2 levels, a relapse of her NMOSD was considered. Initial treatment with anti-epileptic medications, haloperidol, and muscle relaxants did control her abnormal movements. High dose methylprednisolone was given for five days but with no signs of improvement. Second-line therapy with fingolimod for 30 days afforded complete remission of the hyperkinetic movements and sensory loss

Conclusion: This case report highlights the importance of recognizing movement disorders as a presenting symptom of NMOSD exacerbation. The hyperkinetic movements can be complex and are vaguely termed “spinal movement disorders” associated with NMOSD. Voluntary compensation for weakness and a consequence of sensory deafferentation of proprioceptive feedback are the purported mechanism behind these disabling abnormal movements. To date, available therapeutic options for these movements are scarce. Herein, we have demonstrated the potential use of fingolimod as a treatment option for movement disorders in NMOSD, stressing the need for immunosuppression when dealing with these involuntary movements.

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MDS Abstracts - https://www.mdsabstracts.org/abstract/fingolimod-responsive-complex-hyperkinetic-movement-disorder-in-neuromyelitis-optica-spectrum-disorder/