Neuromyelitis Optica

Neuromyelitis optica (NMO), or Devic’s disease is an inflammatory disorder of the central nervous system that preferentially affects the optic nerves and spinal cord. Initially considered a variant of multiple sclerosis (MS), NMO is now clearly recognized to have distinct clinical, radiographic, and pathologic characteristics. Historically, the diagnosis of NMO required bilateral optic neuritis and transverse myelitis; however, the identification of a specific biomarker, NMO-IgG, an autoantibody against the aquaporin-4 (AQP4) water channel, has broadened NMO spectrum disease to include patients with diverse clinical and radiographic presentations. This chapter addresses the diagnosis, pathophysiology, and management of the disease.

First Presentation of an Acquired Demyelinating Syndrome

Journal of Pediatric Neurology ◽

10.1055/s-0037-1606380 ◽

2017 ◽

Vol 16 (03) ◽

pp. 164-170

Author(s):

Rachel Gottlieb-Smith ◽

Amy Waldman

Keyword(s):

Multiple Sclerosis ◽

Central Nervous System ◽

Nervous System ◽

Differential Diagnosis ◽

Optic Neuritis ◽

Clinical Features ◽

Neuromyelitis Optica ◽

Transverse Myelitis ◽

Acute Disseminated Encephalomyelitis ◽

AbstractAcquired demyelinating syndromes (ADS) present with acute or subacute monofocal or polyfocal neurologic deficits localizing to the central nervous system. The clinical features of distinct ADS have been carefully characterized including optic neuritis, transverse myelitis, and acute disseminated encephalomyelitis. These disorders may all be monophasic disorders. Alternatively, optic neuritis, partial transverse myelitis, and acute disseminated encephalomyelitis may be first presentations of a relapsing or polyphasic neuroinflammatory disorder, such as multiple sclerosis or neuromyelitis optica. The clinical features of these disorders and the differential diagnosis are discussed in this article.

Neuromyelitis Optica

10.1093/med/9780199732920.003.0005 ◽

2013 ◽

Author(s):

Aaron E. Miller ◽

Teresa M. DeAngelis

Keyword(s):

Multiple Sclerosis ◽

Spinal Cord ◽

Central Nervous System ◽

Nervous System ◽

Neuromyelitis Optica ◽

Autoimmune Disorder ◽

Therapeutic Approaches ◽

New Concepts ◽

Spectrum Disorders

Neuromyelitis optica (NMO), a chronic inflammatory, demyelinating autoimmune disorder of the central nervous system with a predilection for the optic nerves and spinal cord, has long been confused with classical multiple sclerosis. In this chapter, we review the important clinical and radiographic distinctions of NMO and NMO spectrum disorders, and summarize promising new concepts in pathophysiology and therapeutic approaches.

Neuromyelitis optica with brain stem involvement in a middle-aged Ethiopian woman: a case report and review of literature

Journal of Medical Case Reports ◽

10.1186/s13256-021-03019-6 ◽

2021 ◽

Vol 15 (1) ◽

Author(s):

Rodas Asrat Kassu ◽

Hailu Abera Mulatu ◽

Sisay Gizaw ◽

Henok Fisseha ◽

Amir Musema ◽

...

Keyword(s):

Spinal Cord ◽

Central Nervous System ◽

Nervous System ◽

Case Report ◽

Optic Neuritis ◽

Neuromyelitis Optica ◽

Oculomotor Nerve ◽

Oculomotor Nerve Palsy ◽

Optic Nerves ◽

Abstract Introduction Neuromyelitis optica is a demyelinating disease of the central nervous system that predominantly affects the optic nerves and spinal cord. In neuromyelitis optica, white blood cells and antibodies primarily attack the optic nerves and the spinal cord, but may also attack the brain. Brainstem manifestation has been described recently. So far, neuromyelitis optica is very rare in Ethiopia and there were only two case reports, but this is the first case report of neuromyelitis optica with brainstem involvement. Case presentation A 47-year-old Addis Ababa woman presented to Saint Paul’s Hospital Millennium Medical College with a history of visual loss of 7 years and bilateral lower limb weakness of 4 days duration. She had bilateral oculomotor nerve palsy. Her past medical history showed systemic hypertension for 18 years and dyslipidemia for 1 year. The objective evaluation of the patient revealed right optic nerve atrophy suggesting optic neuritis and flaccid paraplegia with sensory level at the fourth thoracic vertebra. Diagnostic work-up using electromyography and spinal magnetic resonance imaging revealed demyelinating anterior visual pathway dysfunction and signs of extensive cervicothoracic transverse myelitis from the third cervical to lower thoracic vertebrae, respectively. Then a diagnosis of neuromyelitis optica was established. After treatment with high-dose systemic steroid followed by azathioprine, the patient was stable for several months with significant improvement of vision and lower-extremity weakness with no relapse of symptoms. Conclusion The case described here is a rare inflammatory demyelinating disorder of the central nervous system occurring in East Africa. It reminds clinicians to suspect neuromyelitis optica in a patient who presented with unexplained recurrent optic neuritis to make a timely diagnosis and prevention of permanent neuronal damage. Neuromyelitis optica can also be associated with oculomotor nerve involvement.

No association of AQP4 polymorphisms with neuromyelitis optica and multiple sclerosis

Translational Neuroscience ◽

10.1515/tnsci-2016-0012 ◽

2016 ◽

Vol 7 (1) ◽

Cited By ~ 2

Author(s):

Ting-Ting Yang ◽

Yang He ◽

Ya-Juan Xiang ◽

Dong-Hui Ao ◽

Yang-Yang Wang ◽

...

Keyword(s):

Multiple Sclerosis ◽

Central Nervous System ◽

Nervous System ◽

Environmental Factors ◽

Neuromyelitis Optica ◽

Water Channel ◽

Aquaporin 4 ◽

Genetic And Environmental Factors ◽

Demyelinating Disorders

AbstractMultiple sclerosis (MS) and neuromyelitis optica (NMO) are inflammatory demyelinating disorders of the central nervous system (CNS). Various genetic and environmental factors have been identified to contribute to etiology of MS and NMO. Aquaporin 4 (AQP4), is the most abundant water channel in CNS.

The Spectrum of Neuromyelitis Optica (NMO) in Childhood

Journal of Child Neurology ◽

10.1177/0883073812451495 ◽

2012 ◽

Vol 27 (11) ◽

pp. 1437-1447 ◽

Cited By ~ 22

Author(s):

J.M. Tillema ◽

A. McKeon

Keyword(s):

Central Nervous System ◽

Nervous System ◽

Neuromyelitis Optica ◽

Water Channel ◽

Transverse Myelitis ◽

Acute Disseminated Encephalomyelitis ◽

Aquaporin 4 ◽

Simultaneous Occurrence ◽

Clinical Syndromes ◽

The evaluation of inflammatory central nervous system disorders in childhood with predominant involvement of the optic nerves and spinal cord has been greatly enhanced over the last decade with identification of a group of disorders unified by the detection of neuromyelitis optica (NMO)–IgG, an antibody targeting the central nervous system–predominant water channel aquaporin-4. Clinical syndromes are predominated by the relapsing form of NMO but also include encephalopathic variants that can mimic acute disseminated encephalomyelitis. Maintenance immunotherapy is used to prevent relapses in NMO-IgG–seropositive patients. In contrast, NMO-IgG–seronegative children with NMO more commonly have a monophasic course (simultaneous occurrence of optic neuritis and transverse myelitis) and do not require remission-maintaining immunotherapy, but close surveillance is advised. Current clinical, pathological, and pathogenetic knowledge is reviewed with a focus on clinical presentation, neuroimaging findings, serological investigations, and treatment of children with disorders within the spectrum of central nervous system aquaporin-4 autoimmunity.

Recurrent Hypothalamic Dysfunction in Seropositive Neuromyelitis Optica

Neurology Clinical Practice ◽

10.1212/cpj.0000000000001012 ◽

2020 ◽

pp. 10.1212/CPJ.0000000000001012

Author(s):

Mary Clare McKenna ◽

Nuala McNicholas ◽

Conor Fearon ◽

David Bradley

Keyword(s):

Spinal Cord ◽

Central Nervous System ◽

Neuromyelitis Optica ◽

Clinical Manifestations ◽

Inflammatory Disorder ◽

Diverse Range ◽

Hypothalamic Dysfunction ◽

Inappropriate Secretion ◽

Clinical Presentations ◽

Background:Neuromyelitis optica (NMO) is a rare autoimmune inflammatory disorder of the central nervous system1. Pathogenic aquaporin 4 (AQP4) antibodies are present in 65-88% of cases1. The majority of cases follow a relapsing course preferentially involving the optic nerves, spinal cord, brainstem, diencephalon or cerebral regions1, 2. Within the acute diencephalic clinical presentations, symptomatic hypothalamic lesions may have a diverse range of clinical manifestations including homeostatic dysfunction of neuroendocrine systems2-4. We report a case of recurrent hypothalamic dysfunction secondary to NMO manifesting as syndrome of inappropriate secretion of antidiuretic hormone (SIADH), thermal dysregulation, dysautonomia and disorder of alertness.

The clinical spectrum associated with myelin oligodendrocyte glycoprotein antibodies (anti-MOG-Ab) in Thai patients

Multiple Sclerosis Journal ◽

10.1177/1352458515614093 ◽

2015 ◽

Vol 22 (7) ◽

pp. 964-968 ◽

Cited By ~ 28

Author(s):

Sasitorn Siritho ◽

Douglas K Sato ◽

Kimihiko Kaneko ◽

Kazuo Fujihara ◽

Naraporn Prayoonwiwat

Keyword(s):

Multiple Sclerosis ◽

Central Nervous System ◽

Nervous System ◽

Optic Neuritis ◽

Neuromyelitis Optica ◽

Myelin Oligodendrocyte Glycoprotein ◽

Neuromyelitis Optica Spectrum Disorders ◽

Visual Recovery ◽

Central Nervous System Disorders ◽

Spectrum Disorders

Background: Myelin oligodendrocyte glycoprotein (anti-MOG) antibody was reported in anti-aquaporin-4 (anti-AQP4) seronegative neuromyelitis optica spectrum disorders (NMOSD) patients. Objectives: To describe clinical phenotypes associated with anti-MOG. Methods: Seventy consecutive Thai patients with inflammatory idiopathic demyelinating central nervous system disorders (IIDCD) who were previously anti-AQP4 seronegative were tested for anti-MOG. Results: Anti-MOG was positive in six patients, representing 20.7% of the IIDCD anti-AQP4 seronegative patients with a non-multiple sclerosis phenotype, and most had relapses. All first presented with optic neuritis with good visual recovery after treatment. Conclusions: Anti-MOG positive patients may have manifestations that mimic NMOSD but differ in their course and prognosis from anti-AQP4 positive NMOSD.

OPTIC NEURITIS-A SIGN OF MULTIPLE SCLEROSIS OR OTHER DISEASES OF THE CENTRAL NERVOUS SYSTEM

Acta Neurologica Scandinavica ◽

10.1111/j.1600-0404.1974.tb02815.x ◽

2009 ◽

Vol 50 (6) ◽

pp. 690-718 ◽

Cited By ~ 28

Author(s):

E. Nikoskelainen ◽

P. Riekkinen

Keyword(s):

Multiple Sclerosis ◽

Central Nervous System ◽

Nervous System ◽

Optic Neuritis ◽

Multiple Sclerosis

10.32545/encyclopedia202008.0013.v1 ◽

2020 ◽

Author(s):

Amirhossein Azari Jafari ◽

Seyyedmohammadsadeq Mirmoeeni

Keyword(s):

Multiple Sclerosis ◽

Spinal Cord ◽

Central Nervous System ◽

Nervous System ◽

Optic Nerve ◽

Autoimmune Disease ◽

Genetic And Environmental Factors ◽

Chronic Autoimmune Disease ◽

The Brain

Multiple sclerosis (MS) is a chronic autoimmune disease affecting the central nervous system (CNS), caused by genetic and environmental factors. It is characterized by intermittent and recurrent episodes of inflammation that result in the demyelination and subsequent damage of the underlying axons present in the brain, optic nerve and spinal cord [1][2][3].

Acute Disseminated Encephalomyelitis

10.1093/med/9780199937837.003.0089 ◽

2017 ◽

Author(s):

Benjamin M. Greenberg ◽

Allen Desena

Keyword(s):

Spinal Cord ◽

Central Nervous System ◽

Nervous System ◽

Supportive Care ◽

Acute Disseminated Encephalomyelitis ◽

Autoimmune Response ◽

Inflammatory Disorder ◽

The Brain

Acute disseminated encephalomyelitis (ADEM) is a rare inflammatory disorder of the central nervous system (CNS) that can be fatal or lead to long-term disability. Various triggers have been identified in children and adults, which presumably cause an autoimmune response targeting myelin. The resulting inflammation causes demyelination and edema of the brain, spinal cord, and optic nerves. Depending on which portion of the CNS is affected, patients will experience a variety of symptoms including weakness, numbness, ataxia, encephalopathy, and seizures. Treatment is currently focused on reducing the amount of inflammation and supportive care.