Acute disseminated encephalomyelitis history and symptoms: Difference between revisions

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{{Acute disseminated encephalomyelitis}}
{{Acute disseminated encephalomyelitis}}
{{CMG}}; {{AE}}
{{CMG}}; {{AE}} {{Sujaya}}


==Overview==
==Overview==
ADEM 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.
Classic [[ADEM]] is [[monophasic]], with a history of usually a preceding [[illness]] or less commonly, a [[vaccination]]. It is characterised by an [[acute]] onset of [[focal]] [[neurologic]] [[symptoms]], often with rapid deterioration of [[consciouness]], after a variable [[latent]] period of several days to few months.


==History and Symptoms==  
==History==
* 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.
Classic [[ADEM]] is [[monophasic]], with a history of usually a preceding [[illness]] or less commonly, a [[vaccination]]. It is characterised by an [[acute]] onset of [[focal]] [[neurologic]] [[symptoms]], often with rapid deterioration of [[consciouness]], after a variable [[latent]] period of several days to few months.
* ADEM classically follows a monophasic course, though there are now discussions in the literature about “multiphasic ADEM”
 
* ''Features'' include:
==Symptoms==
*:* Somnolence, confusion, lethargy
===Typical===
*:* Fever – that had previously resolved if there was a precipitating illness
====Prodromal symptoms====
*:* Headache
*Identical in [[adults]] and [[children]]
*:* Meningismus
*[[Headache]]
*:* Motor features may include ataxia, myoclonic movements, and choreoathetosis
*[[Fever]], [[malaise]]
*:* Seizures
*[[Irritability]]
*:* Decerebrite rigidity may develop in severe cases
*[[Nausea]] and [[vomiting]]
*:* Paraplegia, quadraplegia, absent deep tendon reflexes (DTRs), sensory levels, and bladder or bowel involvement suggest prominent spinal cord involvement
*General [[neurological]] symptoms after 2-5 days<ref name="pmid27572859">{{cite journal| author=Pohl D, Alper G, Van Haren K, Kornberg AJ, Lucchinetti CF, Tenembaum S | display-authors=etal| title=Acute disseminated encephalomyelitis: Updates on an inflammatory CNS syndrome. | journal=Neurology | year= 2016 | volume= 87 | issue= 9 Suppl 2 | pages= S38-45 | pmid=27572859 | doi=10.1212/WNL.0000000000002825 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=27572859  }} </ref>
*:* Coma
====Altered mental status====
* '''Signs of disseminated neurologic disease''' are usually present
*Features of [encephalopathy]] comprising changes in [[behaviour]] and [[consciousness]] (46-73% of [[pediatric]] [[patients]] and 20-56% of [[adult]] cases)<ref name="pmid23325908">{{cite journal| author=Marchioni E, Ravaglia S, Montomoli C, Tavazzi E, Minoli L, Baldanti F | display-authors=etal| title=Postinfectious neurologic syndromes: a prospective cohort study. | journal=Neurology | year= 2013 | volume= 80 | issue= 10 | pages= 882-9 | pmid=23325908 | doi=10.1212/WNL.0b013e3182840b95 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=23325908  }} </ref> ranging in [[severity]] from [[lethargy]] to [[coma]]. Presence of [[encephalopathy]] differentiates [[pediatric]] [[ADEM]] from [[Multiple Sclerosis]]<ref name="pmid23572237">{{cite journal| author=Krupp LB, Tardieu M, Amato MP, Banwell B, Chitnis T, Dale RC | display-authors=etal| title=International Pediatric Multiple Sclerosis Study Group criteria for pediatric multiple sclerosis and immune-mediated central nervous system demyelinating disorders: revisions to the 2007 definitions. | journal=Mult Scler | year= 2013 | volume= 19 | issue= 10 | pages= 1261-7 | pmid=23572237 | doi=10.1177/1352458513484547 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=23572237  }} </ref>.
*:* Motor findings: 
*[[Confusion]]
*:*:* Hemiparesis
*[[Psychosis]]
*:*:* Quadriparesis
 
*:*:* Extensor plantar responses
====Focal neurological symptoms====
*:* DTR’s may be lost initially, and later become hyperactive
*Damage to [[occipital]] [[lobes]]: [[Homonymous]] [[visual]] [[field]] [[defects]], [[cortical]] [[blindness]]
*:* Sensory findings are commonly present
*[[Agraphia]], [[aphasia]], [[alexia]], [[acalculia]]
*:* Brainstem involvement may be present
*[[Sensory]] [[symptoms]]: [[Astereognosis]] ,[[agraphesthesia]], loss of [[proprioception]], [[vibration]] and [[temperature]] [[sensation]]
*:* Cerebellar involvement is particularly prominent in ADEM that occurs as a consequence of chickenpox
*[[Brainstem]] [[involvement]] (carries a poorer [[prognosis]] and a higher risk of [[fulminant]] [[disease]] course<ref name="pmid18272282">{{cite journal| author=Tenembaum SN| title=Disseminated encephalomyelitis in children. | journal=Clin Neurol Neurosurg | year= 2008 | volume= 110 | issue= 9 | pages= 928-38 | pmid=18272282 | doi=10.1016/j.clineuro.2007.12.018 | pmc=7116932 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=18272282  }} </ref>): [[Diplopia]], [[dysphagia]], [[dysarthria]], [[vertigo]], [[hearing]] loss, loss of [[taste]] and [[smell]]<ref name="pmid27572859">{{cite journal| author=Pohl D, Alper G, Van Haren K, Kornberg AJ, Lucchinetti CF, Tenembaum S | display-authors=etal| title=Acute disseminated encephalomyelitis: Updates on an inflammatory CNS syndrome. | journal=Neurology | year= 2016 | volume= 87 | issue= 9 Suppl 2 | pages= S38-45 | pmid=27572859 | doi=10.1212/WNL.0000000000002825 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=27572859 }} </ref>
* 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.
====Meningism====
Caused by [[lymphocytic]] [[infiltration]] of the [[meninges]] in 26-31% of [[cases]]
===Atypical symptoms===
Other conditions should be excluded in the presence of the following [[atypical]] features of [[ADEM]]<ref name="pmid27572859">{{cite journal| author=Pohl D, Alper G, Van Haren K, Kornberg AJ, Lucchinetti CF, Tenembaum S | display-authors=etal| title=Acute disseminated encephalomyelitis: Updates on an inflammatory CNS syndrome. | journal=Neurology | year= 2016 | volume= 87 | issue= 9 Suppl 2 | pages= S38-45 | pmid=27572859 | doi=10.1212/WNL.0000000000002825 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=27572859  }} </ref>:
*[[Progressive]] [[onset]]
*Persistent [[headache]]
*[[Stroke]]-like events
*Recurrent [[seizures]] (predominant in [[pediatric]] cases)
*[[Neuropsychiatric]] [[symptoms]]


==References==
==References==
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[[Category:Disease]]
[[Category:Disease]]
[[Category:Infectious disease]]
 
[[Category:Neurology]]
[[Category:Neurology]]


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Latest revision as of 10:13, 8 December 2022

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Sujaya Chattopadhyay, M.D.[2]

Overview

Classic ADEM is monophasic, with a history of usually a preceding illness or less commonly, a vaccination. It is characterised by an acute onset of focal neurologic symptoms, often with rapid deterioration of consciouness, after a variable latent period of several days to few months.

History

Classic ADEM is monophasic, with a history of usually a preceding illness or less commonly, a vaccination. It is characterised by an acute onset of focal neurologic symptoms, often with rapid deterioration of consciouness, after a variable latent period of several days to few months.

Symptoms

Typical

Prodromal symptoms

Altered mental status

Focal neurological symptoms

Meningism

Caused by lymphocytic infiltration of the meninges in 26-31% of cases

Atypical symptoms

Other conditions should be excluded in the presence of the following atypical features of ADEM[1]:

References

  1. 1.0 1.1 1.2 Pohl D, Alper G, Van Haren K, Kornberg AJ, Lucchinetti CF, Tenembaum S; et al. (2016). "Acute disseminated encephalomyelitis: Updates on an inflammatory CNS syndrome". Neurology. 87 (9 Suppl 2): S38–45. doi:10.1212/WNL.0000000000002825. PMID 27572859.
  2. Marchioni E, Ravaglia S, Montomoli C, Tavazzi E, Minoli L, Baldanti F; et al. (2013). "Postinfectious neurologic syndromes: a prospective cohort study". Neurology. 80 (10): 882–9. doi:10.1212/WNL.0b013e3182840b95. PMID 23325908.
  3. Krupp LB, Tardieu M, Amato MP, Banwell B, Chitnis T, Dale RC; et al. (2013). "International Pediatric Multiple Sclerosis Study Group criteria for pediatric multiple sclerosis and immune-mediated central nervous system demyelinating disorders: revisions to the 2007 definitions". Mult Scler. 19 (10): 1261–7. doi:10.1177/1352458513484547. PMID 23572237.
  4. Tenembaum SN (2008). "Disseminated encephalomyelitis in children". Clin Neurol Neurosurg. 110 (9): 928–38. doi:10.1016/j.clineuro.2007.12.018. PMC 7116932 Check |pmc= value (help). PMID 18272282.

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