Acute disseminated encephalomyelitis

Infobox_Disease
Name = Acute disseminated encephalomyelitis

Caption =
DiseasesDB = 158
ICD10 = ICD10|G|04|0|g|00
ICD9 = ICD9|323.61
ICDO =
OMIM =
MedlinePlus =
eMedicineSubj = neuro
eMedicineTopic = 500
MeshID = D004673

ADEM produces multiple inflammatory lesions in the brain and spinal cord, particularly in the white matter. Usually these are found in the subcortical and central white matter and cortical gray-white junction of both cerebral hemispheres, cerebellum, brainstem, and spinal cord [cite journal |author=Wingerchuk DM |title=Postinfectious encephalomyelitis |journal=Curr Neurol Neurosci Rep |volume=3 |issue=3 |pages=256–64 |year=2003 |month=May |pmid=12691631 |doi= |url=] , but periventricular white matter and gray matter of the cortex, thalami and basal ganglia may also be involved.

Causes, Antecedent History

Presentation

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, seizures and coma. Although initially the symptoms are usually mild, they worsen rapidly over the course of hours to days, with the average time to maximum severity being about four and a half days.

Treatment

No controlled clinical trials have been conducted on ADEM treatment, but aggressive treatment aimed at rapidly reducing inflammation of the CNS is standard. The widely accepted first-line treatment is high doses of intravenous corticosteroids [cite journal |author=Shahar E, Andraus J, Savitzki D, Pilar G, Zelnik N |title=Outcome of severe encephalomyelitis in children: effect of high-dose methylprednisolone and immunoglobulins |journal=J. Child Neurol. |volume=17 |issue=11 |pages=810–4 |year=2002 |month=Nov |pmid=12585719 |doi= |url=] , such as methylprednisolone or dexamethasone, followed by 3-6 weeks of gradually lower oral doses of prednisolone. Patients treated with methylprednisolone have shown better outcomes than those treated with dexamethasone. Oral tapers of less than three weeks duration show a higher chance of relapsing [cite journal |author=Dale RC, de Sousa C, Chong WK, Cox TC, Harding B, Neville BG |title=Acute disseminated encephalomyelitis, multiphasic disseminated encephalomyelitis and multiple sclerosis in children |journal=Brain |volume=123 Pt 12 |issue= |pages=2407–22 |year=2000 |month=Dec |pmid=11099444 |doi= |url=http://brain.oxfordjournals.org/cgi/pmidlookup?view=long&pmid=11099444] [cite journal |author=Anlar B, Basaran C, Kose G, "et al" |title=Acute disseminated encephalomyelitis in children: outcome and prognosis |journal=Neuropediatrics |volume=34 |issue=4 |pages=194–9 |year=2003 |month=Aug |pmid=12973660 |doi=10.1055/s-2003-42208 |url=] , and tend to show poorer outcomesFact|date=March 2008. Other antiinflamatory and immunosuppressive therapies have been reported to show beneficial effect, such as plasmapheresis, high doses of intravenous immunoglobulin (IVIg)cite journal |author=Shahar E, Andraus J, Savitzki D, Pilar G, Zelnik N |title=Outcome of severe encephalomyelitis in children: effect of high-dose methylprednisolone and immunoglobulins |journal=J. Child Neurol. |volume=17 |issue=11 |pages=810–4 |year=2002 |month=Nov |pmid=12585719 |doi= |url=] [cite journal |author=Ravaglia S, Piccolo G, Ceroni M, "et al" |title=Severe steroid-resistant post-infectious encephalomyelitis: general features and effects of IVIg |journal=J. Neurol. |volume=254 |issue=11 |pages=1518–23 |year=2007 |month=Nov |pmid=17965959 |doi=10.1007/s00415-007-0561-4 |url=] , mitoxantrone and cyclophosphamide. These are considered alternative therapies, used when corticosteroids cannot be used, or fail to show an effect.

There is some evidence to suggest that patients may respond to a combination of methylprednisolone and immunoglobulins if they fail to respond to either separately [cite journal |author=Straussberg R, Schonfeld T, Weitz R, Karmazyn B, Harel L |title=Improvement of atypical acute disseminated encephalomyelitis with steroids and intravenous immunoglobulins |journal=Pediatr. Neurol. |volume=24 |issue=2 |pages=139–43 |year=2001 |month=Feb |pmid=11275464 |doi= |url=http://linkinghub.elsevier.com/retrieve/pii/S0887-8994(00)00229-0] In a study of 16 children with ADEM, 10 recovered completely after high-dose methylprednisolone, one severe case that failed to respond to steroids recovered completely after IVIg; the five most severe cases -with ADAM and severe peripheral neuropathy- were treated with combined high-dose methylprednisolone and immunoglobulin, two remained paraplegic, one had motor and cognitive handicaps, and two recovered. A recent review of IVIg treatment of ADEM (of which the previous study formed the bulk of the cases) found that 70% of children showed complete recovery after treatment with IVIg, or IVIg plus corticosteroids [cite journal |author=Feasby T, Banwell B, Benstead T, "et al" |title=Guidelines on the use of intravenous immune globulin for neurologic conditions |journal=Transfus Med Rev |volume=21 |issue=2 Suppl 1 |pages=S57–107 |year=2007 |month=Apr |pmid=17397768 |doi=10.1016/j.tmrv.2007.01.002 |url=] . A study of IVIg treatment in adults with ADEM showed that IVIg seems more effective in treating sensory and motor disturbances, while steroids seem more effective in treating impairments of cognition, consciousness and rigor [cite journal |author=Ravaglia S, Piccolo G, Ceroni M, "et al" |title=Severe steroid-resistant post-infectious encephalomyelitis: general features and effects of IVIg |journal=J. Neurol. |volume=254 |issue=11 |pages=1518–23 |year=2007 |month=Nov |pmid=17965959 |doi=10.1007/s00415-007-0561-4 |url=] . This same study found one subject, a 71 year old man who had not responded to steroids, that responded to a IVIg treatment 58 days after disease onset.

Prognosis

Full recovery is seen in 50 to 75% of cases, ranging to 70 to 90% recovery with some minor residual disability (typically assessed using measures such as mRS or EDSS), average time to recover is one to six months. The mortality rate may be as high as 5%.. Poorer outcomes are associated with unresponsiveness to steroid therapy, unusually severe neurological symptoms, or sudden onset. Children tend to have more favorable outcomes than adults, and cases presenting without fevers tend to have poorer outcomes [cite journal |author=Lin CH, Jeng JS, Hsieh ST, Yip PK, Wu RM |title=Acute disseminated encephalomyelitis: a follow-up study in Taiwan |journal=J. Neurol. Neurosurg. Psychiatr. |volume=78 |issue=2 |pages=162–7 |year=2007 |month=Feb |pmid=17028121 |doi=10.1136/jnnp.2005.084194 |url=] . The latter effect may be due to either protective effects of fever, or that diagnosis and treatment is sought more rapidly when fever is present.

Motor deficits

Residual motor deficits are estimated to remain in about 8 to 30% of cases, the range in severity from mild clumsiness to ataxia and hemiparesis .

Neurocognitive

Patients with demylinating illnesses, such as MS, have shown cognitive deficits even when there is minimal physical disability [cite journal |author=Foong J, Rozewicz L, Quaghebeur G, "et al" |title=Executive function in multiple sclerosis. The role of frontal lobe pathology |journal=Brain |volume=120 ( Pt 1) |issue= |pages=15–26 |year=1997 |month=Jan |pmid=9055794 |doi= |url=http://brain.oxfordjournals.org/cgi/pmidlookup?view=long&pmid=9055794] . Research suggests that similar effects are seen after ADEM, but that the deficits are less severe than those seen in MS. A study of six children with ADEM (mean age at presentation 7.7 years) were tested for a range of neurocognitive tests after an average of 3.5 years of recovery. [cite journal |author=Hahn CD, Miles BS, MacGregor DL, Blaser SI, Banwell BL, Hetherington CR |title=Neurocognitive outcome after acute disseminated encephalomyelitis |journal=Pediatr. Neurol. |volume=29 |issue=2 |pages=117–23 |year=2003 |month=Aug |pmid=14580654 |doi= |url=http://linkinghub.elsevier.com/retrieve/pii/S0887899403001437] . All six children performed in the normal range on most tests, including verbal IQ and performance IQ, but performed at least one standard deviation below age norms in at least one cognitive domain, such as complex attention (one child), short-term memory (one child) and internalizing behaviour/affect (two children). Group means for each cognitive domain were all within one standard deviation of age norms, demonstrating that, as a group, they were normal. These deficits were less severe than those seen in similar aged children with a diagnosis of MS [cite journal |author=Banwell BL, Anderson PE |title=The cognitive burden of multiple sclerosis in children |journal=Neurology |volume=64 |issue=5 |pages=891–4 |year=2005 |month=Mar |pmid=15753431 |doi=10.1212/01.WNL.0000152896.35341.51 |url= |doi_brokendate=2008-08-27] .

Another study compared nineteen children with a history of ADEM, of which 10 were five years of age or younger at the time (average age 3.8 years old, tested an average of 3.9 years later) and nine were older (mean age 7.7y at time of ADEM, tested an average of 2.2 years later) to nineteen matched controls [cite journal |author=Jacobs RK, Anderson VA, Neale JL, Shield LK, Kornberg AJ |title=Neuropsychological outcome after acute disseminated encephalomyelitis: impact of age at illness onset |journal=Pediatr. Neurol. |volume=31 |issue=3 |pages=191–7 |year=2004 |month=Sep |pmid=15351018 |doi=10.1016/j.pediatrneurol.2004.03.008 |url=] . Scores on IQ tests and educational achievement were lower for the young onset ADEM group (average IQ 90) compared to the late onset (average IQ 100) and control groups (average IQ 106), while the late onset ADEM children scored lower on verbal processing speed. Again, all groups means were within one standard deviation of the controls, meaning that while effects were statistically reliable, the children were as a whole, still within the normal range. There were also more behavioural problems in the early onset group, although there is some suggestion that this may be due, at least in part, to the stress of hospitalization at a young age [cite journal |author=Douglas JW |title=Early hospital admissions and later disturbances of behaviour and learning |journal=Dev Med Child Neurol |volume=17 |issue=4 |pages=456–80 |year=1975 |month=Aug |pmid=1158052 |doi= |url=] [cite journal |author=Daviss WB, Racusin R, Fleischer A, Mooney D, Ford JD, McHugo GJ |title=Acute stress disorder symptomatology during hospitalization for pediatric injury |journal=J Am Acad Child Adolesc Psychiatry |volume=39 |issue=5 |pages=569–75 |year=2000 |month=May |pmid=10802974 |doi= |url=http://meta.wkhealth.com/pt/pt-core/template-journal/lwwgateway/media/landingpage.htm?issn=0890-8567&volume=39&issue=5&spage=569]

ADEM & MS

While ADEM and MS both involve autoimmune demylenation, they differ in many clinical, genetic, imaging, and histopathological differences [cite journal |author=Wingerchuk DM, Lucchinetti CF |title=Comparative immunopathogenesis of acute disseminated encephalomyelitis, neuromyelitis optica, and multiple sclerosis |journal=Curr. Opin. Neurol. |volume=20 |issue=3 |pages=343–50 |year=2007 |month=Jun |pmid=17495631 |doi=10.1097/WCO.0b013e3280be58d8 |url=] . Some authors consider MS and its borderline forms to constitute a spectrum, differing only in chronocity, severity, and clinical course [Weinshenker B, Miller D. (1999). Multiple sclerosis: one disease or many? In: Siva A, Kesselring J, Thompson A, eds. Frontiers in multiple sclerosis. London: Dunitz, p37-46.] [cite journal |author=Hartung HP, Grossman RI |title=ADEM: distinct disease or part of the MS spectrum? |journal=Neurology |volume=56 |issue=10 |pages=1257–60 |year=2001 |month=May |pmid=11376169 |doi= |url=http://www.neurology.org/cgi/pmidlookup?view=long&pmid=11376169] , while others consider them discretely different diseases.

Acute Hemorrhagic Leukoencephalitis

Acute hemorrhagic leukoencephalitis (AHL, or AHLE) or Acute necrotizing encephalopathy (ANE), Acute hemorrhagic encephalomyelitis (AHEM), Acute necrotizing hemorrhagic leukoencephalitis (ANHLE), Weston-Hurst syndrome, or Hurst's disease, is a hyperacute and frequently fatal form of ADEM. AHL is relatively rare (less than 100 cases have been reported in the medical literature as of 2006cite journal |author=Davies NW, Sharief MK, Howard RS |title=Infection-associated encephalopathies: their investigation, diagnosis, and treatment |journal=J. Neurol. |volume=253 |issue=7 |pages=833–45 |year=2006 |month=Jul |pmid=16715200 |doi=10.1007/s00415-006-0092-4 |url=] ), it is seen in about 2% of ADEM cases, and is characterized by necrotizing vasculitis of venules and hemorrhage, and edemacite journal |author=Stone MJ, Hawkins CP |title=A medical overview of encephalitis |journal=Neuropsychol Rehabil |volume=17 |issue=4-5 |pages=429–49 |year=2007 |pmid=17676529 |doi=10.1080/09602010601069430 |url=] , death is common in the first weekcite journal |author=Archer H, Wall R |title=Acute haemorrhagic leukoencephalopathy: two case reports and review of the literature |journal=J. Infect. |volume=46 |issue=2 |pages=133–7 |year=2003 |month=Feb |pmid=12634076 |doi= |url=http://linkinghub.elsevier.com/retrieve/pii/S0163445302910960] with mortality rate of about 70%, but increasing evidence points to favorable outcomes after aggressive treatment with corticosteroids, immunoglobulins, cyclophosphamide, and plasma exchange. About 70% of survivors show residual neurological deficits, but some survivors have shown surprisingly little deficit considering the magnitude of the white matter affected.

Experimental Allergic Encephalomyelitis

ee also

* Optic neuritis
* Transverse myelitis

References

External links

* [http://www.myelitis.org/adem.htm Acute Disseminated Encephalomyelitis (ADEM)] at myelitis.org
*RareDiseases|8639|Acute disseminated encephalomyelitis
*NINDS|acute_encephalomyelitis|Acute Disseminated Encephalomyelitis Information Page

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