Objective: Movement disorders secondary to spinal cord demyelination are the most frequent movement disorders in multiple sclerosis (MS). Retrospective studies and case series suggest that spinal movement disorders (SMDs), especially tonic spasms, are even more prevalent in neuromyelitis optica spectrum disorder (NMOSD). There is a pressing need for better symptomatic treatments for SMDs.

Background: Retrospective studies suggest that spinal movement disorders, especially tonic spasms, are prevalent in NMOSD. However, there have been no prospective studies evaluating spinal movement disorders in NMOSD, MOGAD, and idiopathic transverse myelitis (ITM).

Method: Patients referred to a tertiary neuroimmunology clinic for spinal cord demyelination (excluding MS) were evaluated. All patients answered a movement disorders survey and underwent a movement disorder-focused exam. Movement disorders were compared among patients with NMOSD with and without AQP4-IgG, MOGAD, and ITM. Patients with and without involuntary movements were also compared to identify predictors of spinal movement disorders.

Results: Sixty-three patients were evaluated from 2017 to 2021 (71% females, average age 48+/-14 years, average disease duration 4.5+/-8 years). Of the total, 49% had ITM, 21% had NMOSD without AQP4-IgG, 19% had NMOSD with AQP4-IgG, and 11% had MOGAD. Movement disorders were present in 73% of the total patients and were most frequent in NMOSD with AQP4-IgG (92%) and least frequent in MOGAD (57%).  The most frequent spinal movement disorders were tonic spasms (57%), focal dystonia (25%), spinal tremor (16%), spontaneous clonus (9.5%), secondary restless limb syndrome (9.5%), and spinal myoclonus (8%).  Multivariate analysis showed that longitudinally-extensive myelitis and AQP4-IgG are independent risk factors for the development of spinal movement disorders while MOG-IgG and African American race were independent protective factors.

Conclusion: Spinal movement disorders are highly prevalent in non-MS demyelinating disorders of the spinal cord. Prevalence rates exceed those reported in MS and retrospective NMOSD studies.

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