She experienced frequent micturition for 4 months prior to presentation but no other bladder/bowel symptoms. There were no symptoms or signs of systemic lupus erythematosus or Sjögren syndrome. [jamanetwork.com]

Although the two usually present concurrently, it is not uncommon for one to precede the other by up to several weeks 3. Additionally, it is now recognized that some patients present with unilateral optic nerve involvement. [radiopaedia.org]

Five months later, she presented with gait difficulties, repeated falls and distal paresthesias. [clinmedjournals.org]

APS is the initial presenting feature of NMOSD in approximately 12% of cases.11 Acute brainstem syndromes overlap with APS but also include patients who present with oculomotor dysfunction (eg diplopia and nystagmus), or other cranial nerve palsies, depending [rcpjournals.org]

• Pain

  The most common side effects observed were the common cold (nasopharyngitis), headache, upper respiratory tract infection, inflammation of the lining of the stomach, rash, joint pain, extremity pain, fatigue and nausea. [news-medical.net]

  Optic neuritis Optic neuritis may manifest as vision impairment or vision loss, often associated with eye pain or pain on eye movement Transverse myelitis Spinal cord inflammation than can lead to muscle weakness or paralysis, reduced sensation, or loss [chordtherapeutics.com]

  Symptoms of myelitis may include: Headaches and back pain Pain in the arms, legs or spine Mild to severe paralysis (called paraparesis when it’s partial paralysis and paraplegia when it’s a complete loss of sensation and motor function) Loss of bladder [verywellhealth.com]

  Nerve pain: NMOSD attacks can cause long-term pain, numbness and other nerve issues. Our team can recommend medications and help you find therapies such as acupuncture and meditation. [ohsu.edu]

• Epilepsy

  J Neuroinflammation 15:134. https://doi.org/10.1186/s12974-018-1144-2 CAS Article PubMed PubMed Central Google Scholar Ogawa R, Nakashima I, Takahashi T, Kaneko K (2017) MOG antibody-positive, benign, unilateral, cerebral cortical encephalitis with epilepsy [link.springer.com]

  Epilepsy Res. 2012;101(1–2):166–173. 60. Nakano H, Tanaka M, Kinoshita M, et al. Epileptic seizures in Japanese patients with multiple sclerosis and neuromyelitis optica. Epilepsy Res. 2013;104(1–2):175–180. 61. [dovepress.com]

  GEN SEQUENCING: HEREDITARY IgA NEPHROPATHY PANEL Nx GEN WHOLE EXOME SEQUENCING, TRIO Nx GEN SEQUENCING: CATARACT Nx GEN SEQUENCING: HEREDITARY INCLUSION MYOPATHY PANEL Nx GEN SEQUENCING: AMYOTROPHIC LATERAL SCLEROSIS Nx GEN SEQUENCING: COMPREHENSIVE EPILEPSY [lalpathlabs.com]

  MOG antibody-positive, benign, unilateral, cerebral cortical encephalitis with epilepsy. Neurol Neuroimmunol Neuroinflamm 2017;4:e322. [PMC free article] [PubMed] [Google Scholar] 45. Hacohen Y, Palace J. [ncbi.nlm.nih.gov]

• Constitutional Symptom

  Children Although median age of onset is 30 to 40 years, 3% to 5% of patients with NMOSD present as children, which can be clinically challenging.16 The most common presenting features of NMOSD in children include visual, motor, and constitutional symptoms [practicalneurology.com]

• Respiratory Insufficiency

  In the non-blinded stage of the study, two patients died, one due to respiratory insufficiency caused by disease progression, the other due to inflammatory diseases of the central nervous system with unknown aetiology, and an association between death [spandidos-publications.com]

  Respiratory insufficiency caused by a recent NMOSD attack was considered to be the most likely cause of death and was not considered related to inebilizumab [30]. [futuremedicine.com]

  One was most likely due to an NMOSD relapse-related respiratory insufficiency, and the second resulted from an encephalopathy of unknown origin. [dovepress.com]

• Vomiting

  […] or cyclical vomiting syndrome. [rcpjournals.org]

  […] horizontal half of the visual field (altitudinal visual field defect) or loss of acuity (20/200 or worse) A complete spinal cord syndrome, particularly with paroxysmal tonic spasms An area postrema syndrome, causing intractable hiccups or nausea and vomiting [msdmanuals.com]

  A 58-year-old woman without prior medical history developed acute left facial pain, vomiting, and hiccups. MRI showed an intraparenchymal lesion extending from the medulla oblongata to cervical cord with high T2-weighted signal intensity. [jstage.jst.go.jp]

  Area postrema syndrome (APS) manifesting as intractable nausea, vomiting, or hiccups (INVH) is also often present. [chordtherapeutics.com]

• Nausea

  The most common side effects observed were the common cold (nasopharyngitis), headache, upper respiratory tract infection, inflammation of the lining of the stomach, rash, joint pain, extremity pain, fatigue and nausea. [news-medical.net]

  Area postrema syndrome (APS) manifesting as intractable nausea, vomiting, or hiccups (INVH) is also often present. [chordtherapeutics.com]

  An episode of hiccups or nausea/vomiting with no other cause. A brain syndrome with lesions visible on an MRI that are consistent with NMOSD. [ohsu.edu]

  […] patients with: A history of life-threatening infusion reaction to UPLIZNA Active hepatitis B infection Active or untreated latent tuberculosis WARNINGS AND PRECAUTIONS Infusion Reactions: UPLIZNA can cause infusion reactions, which can include headache, nausea [neurologylive.com]

• Hiccup

  A 58-year-old woman without prior medical history developed acute left facial pain, vomiting, and hiccups. MRI showed an intraparenchymal lesion extending from the medulla oblongata to cervical cord with high T2-weighted signal intensity. [jstage.jst.go.jp]

  Area postrema syndrome (APS) manifesting as intractable nausea, vomiting, or hiccups (INVH) is also often present. [chordtherapeutics.com]

  It can rarely affect the brainstem (base of the brain) causing intractable vomiting, hiccups, sleep attacks, and other rare symptoms. NMOSD most often strikes middle aged women but it can also affect men, children and the elderly. [uhhospitals.org]

  Neuromyelitis optica can cause blindness in one or both eyes, weakness or paralysis in the legs or arms, painful spasms, loss of sensation, uncontrollable vomiting and hiccups, and bladder or bowel dysfunction from spinal cord damage. [mayoclinic.org]

  Vomiting and hiccups were more common followed by occulomotor dysfunction, pruritis, deafness, facial palsy, vertigo, trigeminal neuralgia and other cranial nerve signs. [annalsofian.org]

• Visual Impairment

  Vision problems with MS usually affect one eye at a time and result in mild visual impairment while NMOSD may affect both eyes at the same time and may cause severe visual loss. [uhhospitals.org]

  Approximately 50% of patients with NMOSD have permanent visual impairment and paralysis caused by NMOSD attacks. Estimates vary, but NMOSD is thought to impact approximately 4,000 to 8,000 Americans. [fda.gov]

  Some children are left with lasting effects such as visual impairment, weakness in bowel and bladder function and movement problems. Preventive maintenance treatment aims to decrease the risk of disability. [gosh.nhs.uk]

  Paresthesia and visual impairment were the most common clinical symptoms. Almost all of the patients had visual impairment, except patient 5 who had normal binocular vision and patient 7 in which eye examination was not performed. [bjbms.org]

• Leg Weakness

  She presented to the Neurology Department at Wayne State University with bilateral leg weakness, ascending paresthesias, and decreased sensation. Within days, she was unable to walk without assistance. [jamanetwork.com]

  If the swelling is in the lower back, then symptoms can include back pain, weakness of both legs and bowel and bladder problems. [gosh.nhs.uk]

  KEYWORDS Neuromyelitis optica aquaporin-4 antibody Case vignette A 24-year-old male developed nausea, vomiting, and pain between his shoulder blades with leg weakness. [rcpjournals.org]

  KEYWORDS: Neuromyelitis optica, aquaporin-4, antibody Case vignette A 24-year-old male developed nausea, vomiting, and pain between his shoulder blades with leg weakness. [ncbi.nlm.nih.gov]

• Muscle Weakness

  It can also include pain or discomfort along neck, back or abdomen, or cause area postrema syndrome Eye pain and vision impairment/loss Muscle weakness in the arms and legs Nausea, vomiting and hiccups Paralysis / Inability to walk Depression Fatigue, [chordtherapeutics.com]

  In rare cases, muscle weakness can cause breathing difficulties that require the use of an artificial ventilator. Treatments may help prevent episodes and resulting disability. [medicalnewstoday.com]

  [citation needed] The most common initial manifestation of the disease is inflammation of the spinal cord (myelitis).[4] Myelitis causes spinal cord dysfunction, which can result in muscle weakness, paralysis in the limbs, lost or reduced sensation, spasms [en.wikipedia.org]

  […] wasting, slow muscle weakness, fatigue and depression, and even raise functional ambulation and FIM scores.[ 35 215 240 ] Urinary function A recent study focusing on urinary dysfunction in NMO patients found detrusor-sphincter dyssynergia and detrusor [surgicalneurologyint.com]

• Aggressive Behavior

  Monophasic presents with constant relapses, a torpid progression, and aggressive behavior, with neurological disability and a poor prognosis.[ 293 ] The most important factor for survival and prognosis in NMO is relapse.[ 99 164 222 292 294 ] Approximately [surgicalneurologyint.com]

• Seizure

  […] prognosis compared with patients without seizures.60 A possible explanation for the association between epileptic seizures and NMOSD is that severe prominent inflammation and axonal damage in NMOSD may cause epileptic seizures. [dovepress.com]

  Children can have confusion, seizures or coma with NMO. Neuromyelitis optica flare-ups might be reversible, but they can be severe enough to cause permanent visual loss and problems with walking. [mayoclinic.org]

  […] recovery following an attack.7 MOG-positive patients are also more likely to have brainstem involvement.8 The most frequent presentations of MOG-positive disease are acute disseminated encephalomyelitis in children under 7 years of age, which can provoke seizures [rarediseaseadvisor.com]

  Long term video EEG monitoring was performed and showed evidence of diffuse encephalopathy only, without any evidence of seizure activity. [clinmedjournals.org]

  Although motor outcomes after myelitis are also better, patients can be left with significant sphincter and erectile dysfunction.41 Seizures can also occur as part of an encephalitis-like presentation with cortical involvement, more commonly than with [rcpjournals.org]

• Myelopathy

  Paraneoplastic isolated myelopathy: clinical course and neuroimaging clues. Neurology. 2011;76(24):2089-2095.PubMedGoogle ScholarCrossref 15. Cross SA, Salomao DR, Parisi JE, et al. [jamanetwork.com]

  Sjögren’s syndrome myelopathy: spinal cord involvement in Sjögren’s syndrome might be a manifestation of neuromyelitis optica. [scielo.br]

  Herein, we describe a patient with serologic and radiologicconfirmed SS who showed a serum anti AQP4-IgG without evidences of myelopathy, optic neuropathy and any central nervous system involvement. [austinpublishinggroup.com]

  Autoimmune myelopathy associated with collapsin response-mediator protein-5 immunoglobulin G. Ann Neurol. 2008;63(4):531-4. Nakashima I, Fukazawa T, Ota K, et al. [rarediseases.org]

• Limb Weakness

  This may involve vision loss and limb weakness or paralysis. While there is no cure, treatments can reduce the likelihood of future episodes. [medicalnewstoday.com]

  Among these patients, 59 had LETM, out of which 38 (64.4%) reported pruritus during the course of their illness, and 16 reported pruritus as an initial symptom followed by limb weakness. [karger.com]

  The resultant disruption of communication along these nerves means that patients with NMO experience temporary or permanent blindness in one or both eyes that is preceded or followed by limb weakness or paralysis and loss of bladder and bowel control. [journals.plos.org]

  […] treatment.[ 125 ] The current physical rehabilitation strategies, as well as pharmacological therapies, employed for NMO patients are similar to those used for MS, as both diseases show varying rates and degrees of impairment to bladder and bowel function, limb [surgicalneurologyint.com]

• Neglect

  Neurologists should be aware that short-segment myelitis is also common in NMOSD; neglecting to consider this is associated with delay in diagnosis and treatment.13 The anterolateral cord syndrome with prominent weakness and spinothalamic tract deficits [practicalneurology.com]

  Although RTX has been on the market for nearly two decades, the risk of progressive multifocal leukopathy, hypogammaglobulinemia, and severe infections cannot be neglected in patients who take RTX for a long time (18,19). [spandidos-publications.com]

PANEL 3 HLA PRE-TRANSPLANT WORKUP SENSITIZED RECIPIENT PANEL 3 HEPATOCELLULAR CARCINOMA (HCC) PANEL HISTOPATHOLOGY KIDNEY: BIOPSY SECOND OPINION HLA PRE-TRANSPLANT WORKUP SENSITIZED RECIPIENT PANEL 4 HEPCIDIN, SERUM HISTOPATHOLOGY LIVER BIOPSY PANEL [lalpathlabs.com]

[…] concerning for APS, myelitis, or ON in patients with systemic autoimmunity should prompt testing for serum AQP4 antibodies.18 Conversely, patients with NMOSD, especially if seronegative, should be asked about systemic symptoms and have a rheumatologic workup [practicalneurology.com]

• White Matter Lesions

  white matter involvement (unlike MS, there are usually no Dawson's fingers) periaqueductal grey matter hypothalamus/medial thalamus dorsal pons/medulla corpus callosum multiple callosal lesions with heterogeneous signal leading to a marbled pattern 7 [radiopaedia.org]

  On MRI, cerebral white matter lesions are uncommon in neuromyelitis optica, unlike in MS. On MRI, morphology and distribution of the lesions differ from those in MS. [msdmanuals.com]

  white matter lesions, OR (b) optic nerve MRI with T2-hyperintense lesion or T1-weighted gadolinium enhancing lesion extending over >1/2 optic nerve length or involving optic chiasm[7] Acute myelitis: requires associated intramedullary MRI lesion extending [eyewiki.aao.org]

  Acute optic neuritis: requires brain MRI showing (a) normal findings or only nonspecific white matter lesions, OR (b) optic nerve MRI with T2-hyperintense lesion or T1-weighted gadolinium-enhancing lesion extending over >1/2 optic nerve length or involving [cureus.com]

  Lesions were classified as NMO-like when surrounding the fourth ventricle, hypothalamus, or aqueduct with or without small deep white matter lesions, as previously described,10 and scans with lesions in deep gray nuclei or fluffy white matter lesions [jamanetwork.com]

During this study, treatment with Enspryng reduced the number of NMOSD relapses by 74% in patients who were anti-AQP4 positive compared to treatment with a placebo (inactive treatment). [fda.gov]

SLE associated with LETM disease modifying agents and NSAIDs are part of the treatment [14]. [clinmedjournals.org]

In participants with NMOSD, treatment with inebilizumab resulted in rapid and consistent depletion of CD20-positive B cells starting at 8 days of treatment [30]. [futuremedicine.com]

The Neuromyelitis Optica Program at UT Southwestern is recognized as a world leader in the research and treatment of NMOSD. [utswmed.org]

Epidemiological factors involved in the prognosis of the disease Several epidemiological factors have been related to the prognosis of the disease, both in terms of disability (visual or motor) and in relation to the risk of relapses (Table IV). [neurologia.com]

Prognosis Patients with NMOSD have a variable prognosis with many patients suffering high levels of disability.[8] One study demonstrated that only 22% of patients had full recovery but 6% showed no recovery at all.[8] Severe visual defects or motor impairment [eyewiki.aao.org]

• Early initiation of immunotherapy significantly improves prognosis. [jns-journal.com]

Patients with a relapsing course have a poorer prognosis 4: blind in one or both eyes: monophasic 22% vs. relapsing 60% monoplegia or paraplegia: monophasic 31% vs. relapsing 52% History and etymology The disease was first described in 1870 by Sir Thomas [radiopaedia.org]

Prognosis and Management of Neuromyelitis Optica Neuromyelitis optica is usually characterized by relapsing episodes, a monophasic course being exceptional. [karger.com]

Disease Entity Neuromyelitis optica (NMO) is an inflammatory disease that causes demyelination of the central nervous system, primarily affecting the optic nerve (optic neuritis) and the spinal cord. [2] [3] Etiology The precise etiology of NMOSD remains [eyewiki.aao.org]

However, this finding is not as specific in children as it is in adults. 17-19 Differential diagnoses may include multiple infectious and reactive etiologies, among them, post-infectious and post-vaccinal etiologies, vascular pathologies, nutritional [redalyc.org]

The demonstration of AQP4 immunoreactivity in this patient’s tumor further supports a paraneoplastic etiology for some NMOSD cases. [jamanetwork.com]

Another repeat LP was done and was negative for infectious etiologies. [clinmedjournals.org]

We aimed in this review to address the current evidence describing the relation of NMOSD to systemic autoimmunity diseases, its controversy of being co-association or the same etiology, and its practical implications. [erar.springeropen.com]

Epidemiological studies based on 2015 criteria General methodology of the studies To date, 13 studies have assessed the epidemiology of NMOSD according to the 2015 criteria. [neurologia.com]

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Part 2: Epidemiology, clinical presentation, radiological and laboratory features, treatment responses, and long-term outcome Journal of Neuroinflammation 2016; 13:280. Jarius et al. [rarediseases.org]

Pathophysiology NMOSD is primarily an astrocytopathy. [eyewiki.aao.org]

Since antibody-producing cells like plasmablasts and plasma cells are derived from B lymphocytes, the role of this very lymphocyte compartment is crucial for the pathophysiology of NMOSD [13]. [encyclopedia.pub]

Consequently, aquaporins may be involved in the pathophysiology of some skin diseases [23]. [karger.com]

In 2004, the identification of antibodies targeting aquaporin-4 (AQP4-Ab), a CNS water channel, in patients with NMO enabled researchers to understand this condition as a disease with a different pathophysiological basis to that described in patients [elsevier.es]

B cells in the pathophysiology of autoimmune neurological disorders: A credible therapeutic target. Pharmacol Ther. 2006;112(1):57-70. Wingerchuk D. Neuromyelitis optica. Int MS J. 2006; 13:42-50. [rarediseases.org]

This update to the Commission presents advances and discusses persisting and new challenges in prevention, clinical care, and research into TBI. [thelancet.com]

Treatments may help prevent episodes and resulting disability. [medicalnewstoday.com]

In order to prevent additional injury from future attacks, it is imperative that people with NMOSD take medication regularly. NMOSD never goes away on its own, so preventive treatment is required lifelong. [massgeneral.org]

The aim is to reduce inflammation and prevent damage to the nervous system. It’s important to receive treatment as soon as possible to prevent long-term effects. After a course of steroids, patients also may undergo plasma exchange therapy, or PLEX. [neuromyelitisnews.com]

Once NMOSD is diagnosed, especially when anti-AQP4 antibodies are present, we develop a treatment plan to prevent relapses. [utswmed.org]