To characterize the semiology of paroxysmal symptoms (PS) in Neuromyelitis Optica Spectrum Disorder (NMOSD).

An anonymous survey on REDCap was distributed via the ‘NMO Clinic’ Facebook page.Participants were asked general demographic and disease-related characteristics and detailed PS history.

206 responders reported having received the diagnosis of NMOSD from their clinician. 150 responders (72.8%) were US residents, 158 (77.1%) were white, 180 (87.4%) were female; mean age was 47.39 ±13.9 years, and mean disease duration was 4.5±0.5 years. 125 responders (63.1%) reported ‘positive’ AQP4 Ab test, 61 (30.8%) were seronegative and 12 (6.1%) didn’t know their serostatus.

147 responders (71.4%) reported ≥1 type of PS lasting ≤2 minutes. The most common symptom was tingling/numbness sensation (103, 70.1%), followed by involuntary muscle contractions/abnormal posture (91, 61.9%), hot/cold/burning sensations (82, 55.8%), and shock-like sensations along the spine or limbs (73, 49.7%). 146 responders (70.8)reported paroxysmal pain, with a severity of ³ 8/10 in 79 responders with PS patients (54.1%) and severity of 10/10 in 38 patients (26.0%). PS were most commonly aggravated by fatigue (102, 71.8%), physical activity (83, 58.5%), and neck bending (35, 24.6%). Only 80 patients (55.2%) reported having been prescribed medications for PS. The most frequently prescribed medications were gabapentin (53, 67.9%), baclofen (49, 62.9%), pregabalin (33, 42.3%) and carbamazepine (21, 26.9%). Less than 50% reported a significant benefit from these medications. 

This survey highlights that NMOSD patients very frequently experience disabling, often very painful and not adequately treated PS. Although limited by self-reported diagnosis of NMOSD and possible selection bias related to media accessibility, this survey represents a novel method to learn more about a rare disease from the patient’s perspective.