Neuromyelitis optica spectrum disorder history and symptoms
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Julinka Auta Fernandes
Overview
- Neuromyelitis optica spectrum disorder (NMOSD) usually presents with acute or subacute attacks involving the optic nerves, spinal cord, area postrema, brainstem, diencephalon, or cerebrum. The two clinical hallmarks are optic neuritis and acute myelitis; they may occur simultaneously but more often occur successively.[1]
- Common symptoms of NMOSD include painful visual loss, blurred or impaired color vision, limb weakness, sensory disturbance, neuropathic pain, painful tonic spasms, and bladder or bowel dysfunction. Persistent otherwise unexplained nausea, vomiting, or hiccups may represent area postrema syndrome.[1][2]
- Less common symptoms of NMOSD include eye-movement disturbance, facial weakness or numbness, imbalance, excessive sleepiness, narcolepsy, headache, cognitive symptoms, and seizures. Symptom severity can range from mild tingling or blurred vision to blindness, severe weakness, paralysis, or respiratory difficulty.[1]
History
Temporal profile and attack history
- Neuromyelitis optica spectrum disorder typically produces discrete acute or subacute neurologic attacks. Symptoms within one attack may involve more than one anatomic region and may appear simultaneously or successively.[1]
- A history should document the onset, duration, maximum severity, recovery, and residual symptoms of every suspected attack. Previous episodes may have been diagnosed as isolated optic neuritis, transverse myelitis, unexplained vomiting, or another brainstem or cerebral syndrome.[1]
- NMOSD attacks may be mild or severe. Mild tingling, subtle weakness, or blurred vision does not exclude the disorder.[1]
- A relapsing history is characteristic. A steadily progressive neurologic decline unrelated to attacks is atypical for NMOSD.[1]
Focused history
- Neuromyelitis optica spectrum disorder history-taking should specifically address the following symptom domains:[1][3]
- Previous unilateral or bilateral visual loss, blurred vision, impaired color vision, visual-field disturbance, flashes of light, headache, or pain behind the eye or with eye movement.
- Previous limb weakness, paralysis, numbness, tingling, abnormal painful sensations, band-like truncal tightness, painful spasms, itching, stiffness, gait difficulty, respiratory difficulty, or bladder, bowel, and sexual dysfunction.
- Otherwise unexplained prolonged hiccups, nausea, or vomiting, including whether symptoms were constant or episodic and whether they occurred alone or together.
- Eye-movement disturbance, facial weakness or numbness, imbalance, incoordination, excessive sleepiness, sudden sleep attacks, confusion, headache, cognitive change, or seizures.
- Fatigue, depressed mood, and persistent cognitive complaints.
- The past medical history should record coexisting autoimmune disorders, particularly systemic lupus erythematosus, Sjögren syndrome, antiphospholipid syndrome, myasthenia gravis, celiac disease, and autoimmune thyroid disease, because these may coexist with NMOSD.[1]
Common Symptoms
Optic neuritis symptoms
- Neuromyelitis optica spectrum disorder-related optic neuritis may affect one or both eyes and commonly produces:[1][2]
- Rapidly developing blurred vision or reduced visual acuity.
- Partial or complete visual loss.
- Reduced color intensity or color desaturation.
- Missing or blurred areas within the visual field.
- Retrobulbar pain or pain worsened by eye movement.
- Headache.
- Phosphenes, described by the patient as flashes or sparks of light.
Acute myelitis symptoms
- Neuromyelitis optica spectrum disorder-related acute myelitis may produce motor, sensory, autonomic, respiratory, and pain symptoms, including:[1][2]
- Weakness or paralysis affecting the legs, arms, or all four limbs.
- Gait difficulty or inability to walk.
- Numbness, tingling, burning, electric-shock sensations, or other dysesthesias.
- A tight or girdle-like sensation around the trunk.
- Neuropathic pain, hyperalgesia, or allodynia.
- Painful tonic spasms, muscle stiffness, or spasticity-associated pain.
- Neuropathic itching.
- Urinary urgency, retention, incomplete emptying, or incontinence.
- Constipation, fecal urgency, or fecal incontinence.
- Sexual dysfunction.
- Shortness of breath or respiratory weakness when the upper cervical spinal cord is involved.
Area postrema syndrome symptoms
- Neuromyelitis optica spectrum disorder may present with area postrema syndrome (APS), characterized by otherwise unexplained:[3][1][2]
- Persistent or intractable nausea.
- Recurrent or intractable vomiting.
- Persistent or intractable hiccups.
- APS symptoms may be acute or subacute, isolated or combined, and constant or episodic. Otherwise unexplained symptoms persisting for at least 48 hours are particularly suggestive of APS.[1]
Less Common Symptoms
Other brainstem symptoms
- Neuromyelitis optica spectrum disorder may less commonly cause a brainstem syndrome other than APS. Patient-reported symptoms may include:[3][1]
- Double vision or another disturbance of eye movement.
- Facial numbness or facial weakness.
- Imbalance, unsteadiness, or incoordination.
Diencephalic symptoms
- Neuromyelitis optica spectrum disorder may rarely cause a symptomatic diencephalic syndrome, including:[3][1]
- Marked excessive sleepiness.
- Hypersomnolence or narcolepsy, including sudden sleep attacks.
Cerebral and neuropsychiatric symptoms
- Neuromyelitis optica spectrum disorder may less commonly produce cerebral or neuropsychiatric symptoms, including:[3][1]
- Headache.
- Confusion or encephalopathic symptoms.
- Cognitive impairment or subjective cognitive decline.
- Fatigue.
- Depressed mood.
- Seizures, which are uncommon in NMOSD.
References
- ↑ 1.00 1.01 1.02 1.03 1.04 1.05 1.06 1.07 1.08 1.09 1.10 1.11 1.12 1.13 1.14 1.15 Jarius S, Aktas O, Ayzenberg I, et al. Update on the diagnosis and treatment of neuromyelitis optica spectrum disorders—revised recommendations of the Neuromyelitis Optica Study Group (NEMOS). Part I: Diagnosis and differential diagnosis. Journal of Neurology. 2023;270(7):3341-3368. doi:10.1007/s00415-023-11634-0. PMID: 37022481.
- ↑ 2.0 2.1 2.2 2.3 Giorgi L, Marignier R, Pique J, et al. French guidelines for the diagnosis and management of neuromyelitis optica spectrum disorder. Revue Neurologique (Paris). Published online July 21, 2026. doi:10.1016/j.neurol.2026.06.004. PMID: 42481362.
- ↑ 3.0 3.1 3.2 3.3 3.4 Wingerchuk DM, Banwell B, Bennett JL, et al. International consensus diagnostic criteria for neuromyelitis optica spectrum disorders. Neurology. 2015;85(2):177-189. doi:10.1212/WNL.0000000000001729. PMID: 26092914.