Recent Advances in Neuromyelitis Optica Spectrum Disorder: Pathogenesis, Mechanisms and Potential Treatments

: Neuromyelitis optica spectrum disorder (NMOSD) is an acute or subacute demyelinating disease that affects mainly the optic nerve and spinal cord. A major proportion of NMOSD cases have a relationship with autoimmunity to aquaporin 4 (AQP4) found on the central nervous system. NMOSD can occur repeatedly, causing symptoms such as decreased vision and weakness of limbs. The main goal of current therapy is to relieve acute symptoms and prevent recurrence of the disease. Without timely and appropriate treatment, the recurrence and disability rates are high. In the present work, we review recent advances in the diagnosis and treatment of patients with NMOSD, as well as the pathogenesis and mechanisms of AQP4-IgG-seropositive NMOSD.

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An uncommon cause of vertigo: Neuromyelitis optica spectrum disorder

Ear Nose & Throat Journal ◽

10.1177/01455613211053393 ◽

2021 ◽

pp. 014556132110533

Author(s):

Kuan-Ling Lin ◽

Ching-Yu Yang ◽

Wen-Ko Su

Keyword(s):

Spinal Cord ◽

Central Nervous System ◽

Nervous System ◽

Neuromyelitis Optica ◽

Spectrum Disorder ◽

Atypical Presentation ◽

Neuromyelitis Optica Spectrum Disorder ◽

Positive Serum ◽

The Central Nervous System ◽

Aquaporin 4 Antibody

Neuromyelitis optica spectrum disorder (NMOSD) is an uncommon antibody-mediated disease of the central nervous system. Its classic presentation includes long segments of spinal cord inflammation, optic neuritis with or without intractable vomiting, and hiccups. Here, we described a case of a 39-year-old woman with an atypical presentation of vertigo, which was finally diagnosed as NMOSD by a positive serum aquaporin-4 antibody.

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An acute onset of paraparesis in 65 years old Croatian woman – a case report

NATIONAL JOURNAL OF NEUROLOGY ◽

10.28942/nnj.v1i1(12).50 ◽

2018 ◽

Author(s):

V. Kosta

Keyword(s):

Spinal Cord ◽

Central Nervous System ◽

Nervous System ◽

Case Report ◽

Neuromyelitis Optica ◽

Demyelinating Disease ◽

Acute Onset ◽

Spinal Cord Lesions ◽

The Central Nervous System ◽

High Doses

Neuromyelitis optica (NMO, Devic`sdisease) is a rare inflammatory, demyelinating disease of the central nervous system that predominantly affects the spinal cord and optic nerves. Seropositivity for NMO-IgG (aquaporin 4antybodies) and longitudinally extensive spinal cord lesions (3 or more segments) are characteristics of NMO. We described a 65-year old woman with an acute onset of paraparesis that was not recognized as NMO at the beginning. The diagnosis was made three months later when she was readmitted because of the relapse.Despite the treatment with high doses of methylprednisolone, plasmapheresis and immunoglobulins her condition stayed unchanged – she was paraplegic and incontinent.

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FDA approves first treatment for neuromyelitis optica spectrum disorder, a rare autoimmune disease of the central nervous system

Case Medical Research ◽

10.31525/cmr-163a09f ◽

2019 ◽

Author(s):

Keyword(s):

Central Nervous System ◽

Nervous System ◽

Autoimmune Disease ◽

Neuromyelitis Optica ◽

Spectrum Disorder ◽

Neuromyelitis Optica Spectrum Disorder ◽

The Central Nervous System

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Diagnosis of Multiple Sclerosis

10.1093/med/9780197512166.003.0065 ◽

2021 ◽

pp. 540-547

Author(s):

W. Oliver Tobin

Keyword(s):

Multiple Sclerosis ◽

Central Nervous System ◽

Nervous System ◽

Demyelinating Disease ◽

Acute Disseminated Encephalomyelitis ◽

Myelin Oligodendrocyte Glycoprotein ◽

Spectrum Disorder ◽

Demyelinating Diseases ◽

Neuromyelitis Optica Spectrum Disorder ◽

The Central Nervous System

Multiple sclerosis is the most common idiopathic inflammatory demyelinating disease of the central nervous system (CNS), with a prevalence of 1 in 500 to 1 in 2,000 people, depending on geography and various other factors. Idiopathic inflammatory demyelinating diseases are a group of related disorders that include acute disseminated encephalomyelitis, neuromyelitis optica spectrum disorder, and myelin oligodendrocyte glycoprotein–immunoglobulin G–associated CNS demyelinating disease.

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The onset location of neuromyelitis optica spectrum disorder predicts the location of subsequent relapses

Multiple Sclerosis Journal ◽

10.1177/1352458514528763 ◽

2014 ◽

Vol 20 (14) ◽

pp. 1908-1911 ◽

Cited By ~ 7

Author(s):

Manuella Edler Zandoná ◽

Su-Hyun Kim ◽

Jae-Won Hyun ◽

Boram Park ◽

Jungnam Joo ◽

...

Keyword(s):

Spinal Cord ◽

Optic Nerve ◽

Neuromyelitis Optica ◽

Linear Trend ◽

Spectrum Disorder ◽

Anatomical Location ◽

Neuromyelitis Optica Spectrum Disorder ◽

Initial Event ◽

Event Location ◽

The Central Nervous System

We evaluated whether the location of the initial attack predicted the locations of subsequent events in neuromyelitis optica spectrum disorder (NMOSD). In the retrospective analysis from 164 patients with NMOSD, increased odds of a second attack occurring in the initial event location were seen in all locations (odds ratio [OR] brain: 16.00; brainstem: 4.42; optic nerve: 4.08; and spinal cord: 4.59), as was a positive linear trend when evaluating the number of previous events in the same location as the third event location (OR brain: 62.52; brainstem: 44.55; optic nerve: 6.48; and spinal cord: 2.98). This study suggests early clinical events of NMOSD tend to recur in the same anatomical location within the central nervous system (CNS).

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Pediatric paraneoplastic neuromyelitis optica spectrum disorder associated with ovarian teratoma

Multiple Sclerosis Journal ◽

10.1177/13524585211037582 ◽

2021 ◽

pp. 135245852110375

Author(s):

Lauren Tardo ◽

Cynthia Wang ◽

Veena Rajaram ◽

Benjamin M Greenberg

Keyword(s):

Central Nervous System ◽

Pediatric Patient ◽

Neuromyelitis Optica ◽

Area Postrema ◽

Spectrum Disorder ◽

Aquaporin 4 ◽

Ovarian Teratoma ◽

Neuromyelitis Optica Spectrum Disorder ◽

Inflammatory Condition ◽

The Central Nervous System

Neuromyelitis optica spectrum disorder is an inflammatory condition of the central nervous system typically manifesting as myelitis, optic neuritis, and/or area postrema syndrome. Here, we present a pediatric patient who developed symptoms consistent with area postrema syndrome with positive anti-aquaporin-4 (AQP4) antibodies who was also found to have an ovarian teratoma. Pathological specimens revealed the presence of aquaporin-4. This was felt to be the antigenic trigger that led to the patient’s condition. She suffered no further clinical attacks and seroconverted to negative AQP4 status upon teratoma removal. This case varies from others, in that the paraneoplastic presentation occurred in a pediatric patient and in that the patient has not required maintenance immunotherapy after teratoma removal.

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NEUROMYELITIS OPTICA SPECTRUM DISORDERS: DIAGNOSIS AND TREATMENT, THE EXPERIENCE OF CLINICAL OBSERVATIONS

The Scientific Notes of the I P Pavlov St Petersburg State Medical University ◽

10.24884/1607-4181-2018-25-3-7-13 ◽

2018 ◽

Vol 25 (3) ◽

pp. 7-13 ◽

Cited By ~ 1

Author(s):

V. D. Piven ◽

V. S. Krasnov ◽

A. S. Novikova ◽

F. M. Piven ◽

Ya. B. Kushnir ◽

...

Keyword(s):

Spinal Cord ◽

Central Nervous System ◽

Neuromyelitis Optica ◽

Clinical Observation ◽

Clinical Course ◽

Neuromyelitis Optica Spectrum Disorder ◽

Neuromyelitis Optica Spectrum Disorders ◽

Clinical Observations ◽

The Central Nervous System ◽

Spectrum Disorders

Neuromyelitis optica spectrum disorder (NMOSD) is an aggregate of inflammatory and autoimmune disorders of the central nervous system characterized by recurrent, disabling clinical course and damages predominantly targeting optic nerves, brain stem and spinal cord. NMOSD is stratified into two types: seropositive for aquaporin-4 antibodies (AQP4-IgG) and seronegative, which is reported in 25 % of cases. This article presents modern conceptualizations of NMOSD and describes authors’ own experience of clinical observation of patients.

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Cortical oscillopsia without nystagmus, an isolated symptom of neuromyelitis optica spectrum disorder with anti-aquaporin 4 antibody

Multiple Sclerosis Journal ◽

10.1177/1352458511414149 ◽

2011 ◽

Vol 18 (2) ◽

pp. 244-247 ◽

Cited By ~ 8

Author(s):

Sung-Min Kim ◽

Ji-Soo Kim ◽

Young Eun Heo ◽

Hye-Ran Yang ◽

Kyung Seok Park

Keyword(s):

Spinal Cord ◽

Neuromyelitis Optica ◽

Spectrum Disorder ◽

Aquaporin 4 ◽

Neuromyelitis Optica Spectrum Disorder ◽

Ocular Manifestation ◽

Initial Manifestation ◽

Ocular Symptoms ◽

Head Tremor ◽

Aquaporin 4 Antibody

Neuromyelitis optica (NMO), mainly affecting optic nerve and spinal cord, can also manifest diverse ocular symptoms due to brain abnormalities. We present a cortical oscillopsia without nystagmus or head tremor in a patient with neuromyelitis optica spectrum disorder (NMOSD) with anti-aquaporin 4 antibody. This rare ocular manifestation, which is easily underestimated owing to absence of the typical nystagmus, can be an initial manifestation of NMOSD.

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Neuromyelitis optica spectrum disorder in pediatrics. Case report

Case reports ◽

10.15446/cr.v5n1.74943 ◽

2019 ◽

Vol 5 (1) ◽

pp. 11-18

Author(s):

Jhon Camacho ◽

Sebastian Zuleta ◽

Maria Paula Alba ◽

Andrea Hernandez ◽

Carlos Navas

Keyword(s):

Neuromyelitis Optica ◽

Interesting Case ◽

Spectrum Disorder ◽

Demyelinating Diseases ◽

Inflammatory Disorder ◽

Neuromyelitis Optica Spectrum Disorder ◽

The Central Nervous System ◽

High Doses ◽

Aqp4 Antibody ◽

Timely Treatment

Introduction: Neuromyelitis optica is an inflammatory disorder of the central nervous system that accounts for 5% of demyelinating diseases in pediatrics. Its clinical presentation is variable and associated to the involved area of the central nervous system.Case presentation: This is the case of a 15-year-old patient who consulted several times for nonspecific neurological symptoms. During his last visit to the Clínica Universitaria Colombia in Bogotá, he presented with bilateral optic neuritis, associated with frontal and parietal headache. Immunophenotyping studies were carried out, reporting positive IgG anti-aquaporin 4 antibodies (anti-AQP4 antibody), thus leading to a diagnosis of seropositive neuromyelitis optica spectrum disorder (NMOSD). Management with methylprednisolone pulses was initiated with subsequent outpatient management with rituximab that allowed stabilizing the disease.Discussion: This is an interesting case due to its insidious and uncertain onset in a pediatric patient. It was possible to evaluate clinical and diagnostic differences in relation to its presentation in adults. NMOSD mediated by anti-AQP4 is rare; brain and bone marrow MRI are essential for diagnosis. The treatment of choice for acute conditions consists of high doses of methylprednisolone.Conclusion: This disorder may result in irreversible neurological damage; for this reason, high suspicion is required for early diagnosis and timely treatment.

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