Paroxysmal painful tonic spasms (PTS) are common in patients with neuromyelitis optica spectrum disorder (NMOSD) that suffer longitudinal extensive transverse myelitis that can be successfully treated with carbamazepine, according to a case report recently published in eNeurologicalSci.
Although paroxysmal movement disorders in association with immune neurological disorders were first described over 60 years ago, there is limited literature regarding the link between paroxystical movement disorders and NMOSD, with studies reporting an incidence that varies between 14% and 95%, the researchers noted.
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The case involved a 38-year-old female admitted due to the acute onset of tetraparesis associated with hypoesthesia, fecal and urinary incontinence, dysesthesia, and allodynia. Magnetic resonance imaging revealed gadolinium enhancement at C1 and T1 levels and increased signal in the left anterolateral bulb and the area postrema on T2. Based on imaging findings, the patient was started on corticosteroids and plasmapheresis.
Blood testing was remarkable for anti-aquaporin 4 positivity together with rheumatoid factor and antinuclear antibody positivity and positive anti-SSA/Ro and anti-SSB/La. The laboratory findings led to the diagnosis of NMOSD and Sjogren syndrome, and the patient was started on rituximab.
Five weeks after symptom onset, the patient presented with painful tonic spasms involving the four limbs. The spasms were associated with strong emotions and sudden movements and occurred approximately 10 times a day.
Initial therapeutic attempts with antispastics pregabalin and baclofen failed, which led to the use of 100 mg of carbamazepine which quickly led to complete remission. A transitory discontinuance of carbamazepine led to a relapse and was promptly reinstated. Medication was discontinued after 10 months, and the patient persisted symptom-free 3 years since then.
“We reported and recorded a case of PTS during the recovery phase after an NMOSD relapse. Our goal is to increase awareness on this not so infrequent symptom appearing in association with NMOSD and responding to CBZ administration,” the authors concluded.
Reference
Lucas S, Lalive PH, Lascano AM. Paroxysmal painful tonic spasms in neuromyelitis optica spectrum disorder. eNeurologicalSci. Published online January 13, 2023. doi:10.1016/j.ensci.2023.100443
