Acute disseminated encephalomyelitis history and symptoms: Difference between revisions

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Latest revision as of 10:13, 8 December 2022

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

Identical in adults and children
Headache
Fever, malaise
Irritability
Nausea and vomiting
General neurological symptoms after 2-5 days^[1]

Altered mental status

Features of [encephalopathy]] comprising changes in behaviour and consciousness (46-73% of pediatric patients and 20-56% of adult cases)^[2] ranging in severity from lethargy to coma. Presence of encephalopathy differentiates pediatric ADEM from Multiple Sclerosis^[3].
Confusion
Psychosis

Focal neurological symptoms

Damage to occipital lobes: Homonymous visual field defects, cortical blindness
Agraphia, aphasia, alexia, acalculia
Sensory symptoms: Astereognosis ,agraphesthesia, loss of proprioception, vibration and temperature sensation
Brainstem involvement (carries a poorer prognosis and a higher risk of fulminant disease course^[4]): Diplopia, dysphagia, dysarthria, vertigo, hearing loss, loss of taste and smell^[1]

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]:

Progressive onset
Persistent headache
Stroke-like events
Recurrent seizures (predominant in pediatric cases)
Neuropsychiatric symptoms

References

↑ ^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.
↑ 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.
↑ 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.
↑ 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.

Template:WS Template:WH

[pmid27572859-1] 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.

[pmid23325908-2] 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.

[pmid23572237-3] 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.

[pmid18272282-4] 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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[2]

[3]

[4]

@@ Line 1: / Line 1: @@
 __NOTOC__
 {{Acute disseminated encephalomyelitis}}
-{{CMG}}; {{AE}}
+{{CMG}}; {{AE}} {{Sujaya}}
 ==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==
 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]]===
+==Symptoms==
-====[[Prodromal]] [[symptoms]]====
+===Typical===
+====Prodromal symptoms====
 *Identical in [[adults]] and [[children]]
 *[[Headache]]
@@ Line 17: / Line 18: @@
 *[[Nausea]] and [[vomiting]]
 *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>
-====[[Altered]] [[mental]] [[status]]====
+====Altered mental status====
 *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>.
 *[[Confusion]]
 *[[Psychosis]]
-====[[Focal]] [[neurological]] [[symptoms]]====
+====Focal neurological symptoms====
+*Damage to [[occipital]] [[lobes]]: [[Homonymous]] [[visual]] [[field]] [[defects]], [[cortical]] [[blindness]]
+*[[Agraphia]], [[aphasia]], [[alexia]], [[acalculia]]
+*[[Sensory]] [[symptoms]]: [[Astereognosis]] ,[[agraphesthesia]], loss of [[proprioception]], [[vibration]] and [[temperature]] [[sensation]]
+*[[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>
+====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==