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{{Infobox_Disease |
  Name          = {{PAGENAME}} |
  Image          = |
  Caption        = |
  DiseasesDB    = 158 |
  ICD10          = {{ICD10|G|04|0|g|00}} |
  ICD9          = {{ICD9|323.61}} |
  ICDO          = |
  OMIM          = |
  MedlinePlus    = |
  MeshID        = D004673 |
}}
{{Acute disseminated encephalomyelitis}}
{{Acute disseminated encephalomyelitis}}
{{CMG}}
{{CMG}}; {{AE}} {{Sujaya}} {{SHM}}


{{SK}} ADEM
'''''Synonyms and Keywords:''''' post infectious [[encephalomyelitis]]; [[Autoimmune]] [[demyelinating disease]] of [[central nervous system]]


== History and Symptoms ==


===Presentation===
==[[Acute disseminated encephalomyelitis overview|Overview]]==
It has an abrupt onset and a monophasic course. Symptoms usually begin 1-3 weeks after infection or vaccination. Major symptoms include [[fever]], [[headache]], drowsiness, [[seizure]]s and [[coma]]. Although initially the symptoms are usually mild, later in the course of the disease patients may even die, if they are not treated properly. Some patients recover completely, while others have permanent neurological impairments.
* The disease presents with progressive, often abrupt, neurologic deterioration.  The clinical course is quite variable, and in some cases, rapid progression occurs over hours to days.
* ADEM classically follows a monophasic course, though there are now discussions in the literature about “multiphasic ADEM”
* ''Features'' include:
*:* Somnolence, confusion, lethargy
*:* Fever – that had previously resolved if there was a precipitating illness
*:* Headache
*:* Meningismus
*:* Motor features may include ataxia, myoclonic movements, and choreoathetosis
*:* Seizures
*:* Decerebrite rigidity may develop in severe cases
*:* Paraplegia, quadraplegia, absent deep tendon reflexes (DTRs), sensory levels, and bladder or bowel involvement suggest prominent spinal cord involvement
*:* Coma
* '''Signs of disseminated neurologic disease''' are usually present
*:* Motor findings: 
*:*:* Hemiparesis
*:*:* Quadriparesis
*:*:* Extensor plantar responses
*:* DTR’s may be lost initially, and later become hyperactive
*:* Sensory findings are commonly present
*:* Brainstem involvement may be present
*:* Cerebellar involvement is particularly prominent in ADEM that occurs as a consequence of chickenpox
* In ''post-exantham'' disease, the rash usually appears 2-4 days before the neurologic manifestations, and is typically fading at the onset of neurologic disease. 
* Though most cases demonstrate widespread disease (brain, spinal cord, optic nerves, etc), neurologic features may be limited to spinal cord features (transverse myelitis), cerebellar features, etc.


==[[Acute disseminated encephalomyelitis historical perspective|Historical Perspective]]==
==[[Acute disseminated encephalomyelitis classification|Classification]]==
==[[Acute disseminated encephalomyelitis pathophysiology|Pathophysiology]]==


== Laboratory Findings ==  
==[[Acute disseminated encephalomyelitis causes|Causes]]==


=== Electrolyte and Biomarker Studies ===
==[[Acute disseminated encephalomyelitis differential diagnosis|Differentiating Acute disseminated encephalomyelitis from other Diseases]]==
* CSF protein is usually modestly elevated (50-150 mg/dl)
* CSF lymphocytic pleocytosis is present, usually <200 cells/ul.
* Serum white count may be normal or elevated


==[[Acute disseminated encephalomyelitis epidemiology and demographics|Epidemiology and Demographics]]==
[[Category: (name of the system)]]


=== MRI and CT ===
==[[Acute disseminated encephalomyelitis risk factors|Risk Factors]]==
* Radiographic features:
*:* MRI
*:*:* Extensive gadolinium enhancement of white matter of the brain and spinal cord
*:*:* Often extensive and relatively symmetric, often also involving the posterior fossa
*:*:* Most lesions enhance with gadolinium, suggestive that all lesions are active, and that the disease is therefore monophasic.
*:* CT is less sensitive and is sometimes falsely negative


==[[Acute disseminated encephalomyelitis screening|Screening]]==


== Risk Stratification and Prognosis==  
==[[Acute disseminated encephalomyelitis natural history, complications and prognosis|Natural History, Complications and Prognosis]]==
* Prognosis varies with the severity of the neurologic disease.  Some patients will not survive the acute illness, and some will be left with neurologic sequelae.  Some remarkably recover completely.
* The disease is characteristically monophasic, but descriptions of multiphasic disease have been reported.
* Affected children will often suffer from persistent seizures and behavioral and learning disorders.  Adults are somewhat less likely than children to have neurologic sequelae.


== Treatment ==
==Diagnosis==
* There are case reports of improvement with treatment with plasma exhange and intravenous immunoglobulin in patients who have not responded to steroids.
[[Acute disseminated encephalomyelitis history and symptoms|History and Symptoms]] | [[Acute disseminated encephalomyelitis physical examination|Physical Examination]] | [[Acute disseminated encephalomyelitis laboratory findings|Laboratory Findings]] | [[Acute disseminated encephalomyelitis electrocardiogram|Electrocardiogram]] | [[Acute disseminated encephalomyelitis x ray|X Ray]] | [[Acute disseminated encephalomyelitis CT|CT]] | [[Acute disseminated encephalomyelitis MRI|MRI]] | [[Acute disseminated encephalomyelitis echocardiography or ultrasound|Echocardiography or Ultrasound]] | [[Acute disseminated encephalomyelitis other imaging findings|Other Imaging Findings]] | [[Acute disseminated encephalomyelitis other diagnostic studies|Other Diagnostic Studies]]
* The first treatment is usually [[steroid]]s and [[intensive care]] is often required.
== Pharmacotherapy ==


=== Acute Pharmacotherapies ===  
==Treatment==
* Most patients are treated with pulse intravenous methylprednisolone with subsequent taper.
[[Acute disseminated encephalomyelitis medical therapy|Medical Therapy]] | [[Acute disseminated encephalomyelitis surgery|Surgery]] | [[Acute disseminated encephalomyelitis primary prevention|Primary Prevention]] | [[Acute disseminated encephalomyelitis secondary prevention|Secondary Prevention]] | [[Acute disseminated encephalomyelitis cost-effectiveness of therapy|Cost-Effectiveness of Therapy]] | [[Acute disseminated encephalomyelitis future or investigational therapies|Future or Investigational Therapies]]


----
==Case Studies==
== Acknowledgements ==
[[Acute disseminated encephalomyelitis case study one|Case #1]]
The content on this page was first contributed by: ELLISON L. SMITH, M.D.


==Related Chapters==
==Related Chapters==
*[[Acute hemorrhagic leukoencephalitis]]
*[[Acute hemorrhagic leukoencephalitis]]


== External links ==
==External links==
 
*[http://www.adem.org Acute Disseminated Encephalomyelitis (ADEM)Information page-> including pathogenesis/aetiology/treatment/prognosis] at adem.org
*[http://www.adem.org Acute Disseminated Encephalomyelitis (ADEM)Information page-> including pathogenesis/aetiology/treatment/prognosis] at adem.org
* [http://www.myelitis.org/adem.htm Acute Disseminated Encephalomyelitis (ADEM)] at myelitis.org
*[http://www.myelitis.org/adem.htm Acute Disseminated Encephalomyelitis (ADEM)] at myelitis.org
* {{cite journal |author=Murthy J |title=Acute disseminated encephalomyelitis |journal=Neurology India |volume=50 |issue=3 |pages=238-43 |year=2002 |pmid=12391446 |url=http://www.neurologyindia.com/article.asp?issn=0028-3886;year=2002;volume=50;issue=3;spage=238;epage=43;aulast=Murthy}}
*{{cite journal |author=Murthy J |title=Acute disseminated encephalomyelitis |journal=Neurology India |volume=50 |issue=3 |pages=238-43 |year=2002 |pmid=12391446 |url=http://www.neurologyindia.com/article.asp?issn=0028-3886;year=2002;volume=50;issue=3;spage=238;epage=43;aulast=Murthy}}




{{Multiple sclerosis}}
==References==
{{reflist|2}}
 
{{Diseases of the nervous system}}
{{Diseases of the nervous system}}


[[Category:Neurological disorders]]
[[Category:Neurology]]
[[Category:Neurology]]


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Latest revision as of 10:02, 8 December 2022

Acute disseminated encephalomyelitis Microchapters

Home

Patient Information

Overview

Historical Perspective

Classification

Pathophysiology

Causes

Differentiating Acute disseminated encephalomyelitis from other Diseases

Epidemiology and Demographics

Risk Factors

Screening

Natural History, Complications and Prognosis

Diagnosis

Diagnostic Study of Choice

History and Symptoms

Physical Examination

Laboratory Findings

Electrocardiogram

X Ray

CT

MRI

Echocardiography or Ultrasound

Other Imaging Findings

Other Diagnostic Studies

Treatment

Medical Therapy

Surgery

Primary Prevention

Secondary Prevention

Cost-Effectiveness of Therapy

Future or Investigational Therapies

Case Studies

Case #1

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Directions to Hospitals Treating Acute disseminated encephalomyelitis

Risk calculators and risk factors for Acute disseminated encephalomyelitis

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Sujaya Chattopadhyay, M.D.[2] Shameera Shaik Masthan MBBS, DLO, DNB[3]

Synonyms and Keywords: post infectious encephalomyelitis; Autoimmune demyelinating disease of central nervous system


Overview

Historical Perspective

Classification

Pathophysiology

Causes

Differentiating Acute disseminated encephalomyelitis from other Diseases

Epidemiology and Demographics

Risk Factors

Screening

Natural History, Complications and Prognosis

Diagnosis

History and Symptoms | Physical Examination | Laboratory Findings | Electrocardiogram | X Ray | CT | MRI | Echocardiography or Ultrasound | Other Imaging Findings | Other Diagnostic Studies

Treatment

Medical Therapy | Surgery | Primary Prevention | Secondary Prevention | Cost-Effectiveness of Therapy | Future or Investigational Therapies

Case Studies

Case #1

Related Chapters

External links

  • Acute Disseminated Encephalomyelitis (ADEM)Information page-> including pathogenesis/aetiology/treatment/prognosis at adem.org
  • Acute Disseminated Encephalomyelitis (ADEM) at myelitis.org
  • Murthy J (2002). "Acute disseminated encephalomyelitis". Neurology India. 50 (3): 238–43. PMID 12391446.


References

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