Neuromyelitis Optica: Difference between revisions

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[[File:Devic.jpg|thumb]]
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Neuromyelitisoptica, also calledNMO or Devic'sdiseaseis aserious thoughrare autoimmune diseasecondition chiefly affectingthe Central Nervous System (CNS)．The myelin, an insulator of the CNS is the primary focus of the disease. Therefore disease specificallyaffects the optic nerve (CN II)and the spinal cord. The optic nerve isa paired nerve whichconveyvisual impulses to the lateral geniculate nucleus of the brain.Thespinal cordis composedofnervous tissue extending fromthemedulla oblongata (hindbrain) tothelumbar regionof thespinal column. Thediseasethus causes blindness and paralysis as a result of the CNS parts it affects.Kowarik MC, Soltys J. Bennett JL. [https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4208473/ The Treatment of Neuromyelitis Optica]. Journal of Neuro-Ophthalmology. 2014. 34 (1): 70–82In 1894, Eugene Devic and his PhD student, Fernand Gault were able to to describe 16 patients whoso vision got impairedin的眼睛。They were found to develop serious weakness of the extremities, sensory dysfunction and bladder control. They recognized the symptoms to be defects of the optic nerve and spinal cord.Jarius S,Wildemann B．[https://jneuroinflammation．biomedcentral．com/articles/10.1186/1742-2094-10-8 The historyofneuromyelitis optica.]Journal of Neuroinflammation．2013.10(1): 8．Devic E. Myélite subaiguë compliquée de névrite optique (Subacute myelitis complicated with optic neuritis).Bulletinof theMedical Library Association (in French)．1894．8:1033.Murray TJ．Multiple Sclerosis: The History of a Disease．New York: Demos Medical Publishing．2005
NeuromyelitisOptica (NMO)or Devic'sSyndromeis a rare autoimmune diseaseofthe Central Nervous System (CNS)that mainlyaffects the optic nerve (CN II),a paired nerve whichconveysvisual impulses to the lateral geniculate nucleus of the brainSmith AM, Czyz CN．Neuroanatomy, Cranial Nerve 2 (Optic). InStatPearls. StatPearls Publishing. 2022, andspinal cord．It's a demyelinating disorder thus myelin, an insulatorof theCNS istheprimary focusof the diseaseKowarik MC, Soltys J. Bennett JL. [https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4208473/ The Treatment of Neuromyelitis Optica]. Journal of Neuro-Ophthalmology. 2014. 34 (1): 70–82．This condition affects more prevalentinwomen than menname=":1">Barkhof F,Koeller KK (February 2020)．[https://www.ncbi.nlm．nih．gov/books/NBK554329/13 Demyelinating Diseasesofthe CNS (Brain and Spine)] .In: Hodler J, Kubik-Huch RA, von Schulthess GK(eds．).Diseasesof theBrain, Head and Neck, Spine 2020–2023: Diagnostic Imaging．IDKD Springer Series．Cham, Switzerland:Springer．页．165–176．．


== Epidemiology ==
There are two typesof NMO:
Although, prevalenceof NMOis yet to be established because it is unrecognized and confused with its differential diagnosis most especially multiple sclerosis. It is more prevalent in women than men. Patients usually have no family history and therefore it is a nonfamilial condition. 1" />


== Mechanism of Injury/Pathological Process ==
* '''Relapsing form:''' This form is characterized by periodic flare-ups with some recovery and another surge later. 80% of affected patients are female.=":1" />
* '''Monophasic form:''' This form is characterized by a single attack that lasts a month or two months. Men and women get this type equallyNeuromyelitis Optica. Neuromyelitis Optica | Johns Hopkins Medicine. Available at: https://www.hopkinsmedicine.org/health/conditions-and-diseases/neuromyelitis-optica (accessed 17/2/2023)</ref>.


The mechanism of injury is the inflammation and demyelination of the optic nerve (optic neuritis) and the spinal cord (myelitis). =”:1”> Barkhof F,噪鹃KK(2020年2月)。[https://www.ncbi.nlm.nih.gov/books/NBK554329/ 13 Demyelinating Diseases of the CNS (Brain and Spine)] . In: Hodler J, Kubik-Huch RA, von Schulthess GK (eds.). Diseases of the Brain, Head and Neck, Spine 2020–2023: Diagnostic Imaging . IDKD Springer Series. Cham, Switzerland: Springer. pp. 165–176.
== Pathological Process ==


== Types ==
Neuromyelitis Opticaiscaused by a highly specific serum immunoglobulin (Ig)G autoantibody (NMO-IgG) which targets the most abundant astrocytic water channel aquaporin-4 (AQP4) causing the activation of other parts of the immune system and thus resulting in the inflammation and damage to cells as well as the demyelination of the optic nerve (optic neuritis) and the spinal cord (myelitis)Paul F, Jarius S, Aktas O, Bluthner M, Bauer O, Appelhans H, Franciotta D, Bergamaschi R, Littleton E, Palace J, Seelig HP. Antibody to aquaporin 4 in the diagnosisofneuromyelitis optica. PLoS medicine. 2007 Apr;4(4):e133．1" />．NMO-IgG/AQP4-antibodies are present in up to80% of patientswith NMOWaters P, Jarius S, Littleton E, Leite MI, Jacob S, Gray B, Geraldes R, Vale T, Jacob A, Palace J,Maxwell S．Aquaporin-4 antibodies in neuromyelitis opticaandlongitudinally extensive transverse myelitis. Archives of neurology. 2008 Jul 14;65(7):913-9.．
Itis oftwo types．0" />
* '''Relapsing form:''' This is characterised by periodic flare-ups with some recovery and another surge later.80% ofaffectedpatientsare female.name=":1" />
* '''Monophasic form''',which involves a single attack that lasts a month or 2．Menandwomen get this type equally．


== Clinical Presentation ==
== Clinical Presentation ==


Themainsymptomsof NMOarevision and spinal cord dysfunction.
Patients with NMO experience attacks that come and go. These attacks vary from mild to severe and can last from days to months ad in some cases these attacks may last and become permanent.The symptoms arebroken down into three categories:


Optic neuritismay present as; 1" />
Optic neuritisoccurs due to the inflammation of one or both of the optic nerves. Some of the optic neuritis symptoms include:
* Decreased visual acuity
* Decreased visual acuity
* Visual field defects
* Visual field defects
*Colourblindness
*Colorblindness
Spinalcorddysfunction manifests in;
* Eye pain
Myelitis which occur due to the inflammation of the spinalcordand some of it's symptoms include:
* Muscle weakness
* Muscle weakness
* Decreased sensation
* Decreased sensation

Revision as of 18:53, 17 February 2023

Original Editor-Kehinde Fatola
Top Contributors-Kehinde Fatola,Reem Ramadan,Nikhil Benhur Abburi,Kim JacksonandLeana Louw

Introduction[edit|edit source]

Devic.jpg

Neuromyelitis Optica (NMO) or Devic's Syndrome is a rare autoimmune disease of the Central Nervous System (CNS) that mainly affects the optic nerve (CN II), a paired nerve which conveys visual impulses to the lateral geniculate nucleus of the brain[1], and spinal cord. It's a demyelinating disorder thus myelin, an insulator of the CNS is the primary focus of the disease[2]．This condition affects more prevalent in women than men[3]．

There are two types of NMO:

  • Relapsing form:This form is characterized by periodic flare-ups with some recovery and another surge later. 80% of affected patients are female.[3]
  • Monophasic form:This form is characterized by a single attack that lasts a month or two months. Men and women get this type equally[4]．

Pathological Process[edit|edit source]

Neuromyelitis Optica is caused by a highly specific serum immunoglobulin (Ig)G autoantibody (NMO-IgG) which targets the most abundant astrocytic water channel aquaporin-4 (AQP4) causing the activation of other parts of the immune system and thus resulting in the inflammation and damage to cells as well as the demyelination of the optic nerve (optic neuritis) and the spinal cord (myelitis)[5][3]．NMO-IgG/AQP4-antibodies are present in up to 80% of patients with NMO[6]．

Clinical Presentation[edit|edit source]

Patients with NMO experience attacks that come and go. These attacks vary from mild to severe and can last from days to months ad in some cases these attacks may last and become permanent. The symptoms are broken down into three categories:

Optic neuritis occurs due to the inflammation of one or both of the optic nerves. Some of the optic neuritis symptoms include:

  • Decreased visual acuity
  • 视野缺陷
  • Color blindness
  • Eye pain

Myelitis which occur due to the inflammation of the spinal cord and some of it's symptoms include:[3][7]

  • Muscle weakness
  • Decreased sensation
  • Incontinence
  • Paraparesis
  • Quadriparesis
  • Vomiting
  • Pains

Diagnostic Procedures[edit|edit source]

The following can be used to diagnose the condition;[3]

  • Neurological examinations to examine movement, muscle strength, coordination, sensation, memory and thinking (cognitive) functions, and vision and speech. An ophthalmologist also may be involved in the examination.
  • Magnetic Resonance Imaging (MRI) of the brain and spinal cord.
  • Blood test / spinal tap (lumbar puncture) to test for antibodies which might indicate the presence of the disease.
  • Stimuli response test to learn how well the brain responds to stimuli such as sounds, sights or touch.

The tests helps to find lesions or damaged areas in the nerves, spinal cord, optic nerve, brain or brainstem.

Differential Diagnosis[edit|edit source]

  1. Multiple sclerosis
  2. Guillain Barre Syndrome
  3. Disc herniation
  4. Transverse myelitis
  5. Parainfectious myelitis
  6. Tumour
  7. Epidural or subdural hematoma
  8. Epidural and/or paraspinal abscess

Medical Management / Interventions[edit|edit source]

The disease is not curable, however, proper management may ensure the patient live good quality life. Medical intervention may involve;[7]

  • Intravenous corticosteroids
  • Immunosuppressants
  • Plasmapheresis

Physiotherapy Management[edit|edit source]

As stated earlier, no known cure has been found for the disease yet. However, Physiotherapy management is essential for possible future remission as interventions focuses on the clinical presentations which are most times physical disabilities. Physiotherapy interventions may include;

  1. Gait training
  2. Wheelchair training
  3. Pain reduction / management
  4. Transfers
  5. Reduce risk of pressure ulcers
  6. Aid control to spasticity
  7. Strengthening programme
  8. Stretching programme

References[edit|edit source]

  1. ↑Smith AM, Czyz CN. Neuroanatomy, Cranial Nerve 2 (Optic). InStatPearls. StatPearls Publishing. 2022
  2. ↑Kowarik MC, Soltys J. Bennett JL.The Treatment of Neuromyelitis Optica．Journal of Neuro-Ophthalmology. 2014. 34 (1): 70–82
  3. ↑3.03.13.23.33.4Barkhof F, Koeller KK (February 2020).13 Demyelinating Diseases of the CNS (Brain and Spine)．In: Hodler J, Kubik-Huch RA, von Schulthess GK (eds.). Diseases of the Brain, Head and Neck, Spine 2020–2023: Diagnostic Imaging . IDKD Springer Series. Cham, Switzerland: Springer. pp. 165–176.
  4. ↑Neuromyelitis Optica. Neuromyelitis Optica | Johns Hopkins Medicine. Available at: https://www.hopkinsmedicine.org/health/conditions-and-diseases/neuromyelitis-optica (accessed 17/2/2023)
  5. ↑Paul F, Jarius S, Aktas O, Bluthner M, Bauer O, Appelhans H, Franciotta D, Bergamaschi R, Littleton E, Palace J, Seelig HP. Antibody to aquaporin 4 in the diagnosis of neuromyelitis optica. PLoS medicine. 2007 Apr;4(4):e133.
  6. ↑Waters P, Jarius S, Littleton E, Leite MI, Jacob S, Gray B, Geraldes R, Vale T, Jacob A, Palace J, Maxwell S. Aquaporin-4 antibodies in neuromyelitis optica and longitudinally extensive transverse myelitis. Archives of neurology. 2008 Jul 14;65(7):913-9.
  7. ↑7.07.1Wingerchuk DM, Lennon VA, Pittock SJ, Lucchinetti CF, Weinshenker BG.Revised Diagnostic Criteria for Neuromyelitis Optica．Journal of Neurology. 2006. 66 (10): 1485–9.
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