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==Overview==
The analogy between the [[pathogenesis]] of [[ADEM]] and [[MS]] forms the basis of the use of [[high-dose]] [[steroids]], [[plasma exchange]] and [[intravenous]] [[immunoglobulin]] for the [[treatment]] of [[ADEM]].


== Medical Therapy ==
==Medical Therapy==
* There are case reports of improvement with treatment with plasma exhange and intravenous immunoglobulin in patients who have not responded to steroids.
===[[Supportive]] Care<ref name="pmid21847331">{{cite journal| author=Alexander M, Murthy JM| title=Acute disseminated encephalomyelitis: Treatment guidelines. | journal=Ann Indian Acad Neurol | year= 2011 | volume= 14 | issue= Suppl 1 | pages= S60-4 | pmid=21847331 | doi=10.4103/0972-2327.83095 | pmc=3152158 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=21847331  }} </ref>===
* The first treatment is usually [[steroid]]s and [[intensive care]] is often required.
* [[Airway]] protection in [[patients]] with altered [[mental]] status
*[[Mechanical ventilation]] in [[cervical]] [[myelitis]]
*[[Anti-seizure]] [[medications]]
*Correction of [[fluid]] and [[electrolyte]] disturbances
*[[Prophylactic]] [[anticoagulation]] for [[prevention]] of [[deep vein thrombosis]] in [[high-risk]] [[patients]]
*Early initiation of physical, occupational and speech [[therapy]], when applicable, can aid earlier and more complete [[recovery]] in [[pediatric]] [[ADEM]]<ref name="pmid24937355">{{cite journal| author=Carlisi E, Pavese C, Mandrini S, Carenzio G, Dalla Toffola E| title=Early rehabilitative treatment for pediatric acute disseminated encephalomyelitis: case report. | journal=Eur J Phys Rehabil Med | year= 2015 | volume= 51 | issue= 3 | pages= 341-3 | pmid=24937355 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=24937355  }} </ref>.
 
===[[Immunomodulation]]===
====Steroids<ref name="pmid21847331">{{cite journal| author=Alexander M, Murthy JM| title=Acute disseminated encephalomyelitis: Treatment guidelines. | journal=Ann Indian Acad Neurol | year= 2011 | volume= 14 | issue= Suppl 1 | pages= S60-4 | pmid=21847331 | doi=10.4103/0972-2327.83095 | pmc=3152158 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=21847331  }} </ref>====
*[[Intravenous]] [[methylprednisolone]] is the [[first-line]] [[drug]], leading to full [[recovery]] in 50-80% of [[patients]]. Concerning [[disability]] status, this [[cohort]] showed significantly better outcomes than the one treated with [[dexamethasone]]<ref name="pmid8912271">{{cite journal| author=Sakakibara R, Hattori T, Yasuda K, Yamanishi T| title=Micturitional disturbance in acute disseminated encephalomyelitis (ADEM). | journal=J Auton Nerv Syst | year= 1996 | volume= 60 | issue= 3 | pages= 200-5 | pmid=8912271 | doi=10.1016/0165-1838(96)00054-9 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=8912271  }} </ref>.
*The [[dose]] is 10-30mg/kg/day up to a maximum of 1g/day for 3-5days (Class IV)<ref name="pmid9339706">{{cite journal| author=Straub J, Chofflon M, Delavelle J| title=Early high-dose intravenous methylprednisolone in acute disseminated encephalomyelitis: a successful recovery. | journal=Neurology | year= 1997 | volume= 49 | issue= 4 | pages= 1145-7 | pmid=9339706 | doi=10.1212/wnl.49.4.1145 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=9339706  }} </ref>.
*[[Oral]] [[corticosteroids]] are gradually tapered over six weeks to reduce the [[risk]] of [[relapses]].
* Their role in late presentation of the [[disease]] is still doubtful.
* Any type of [[vaccination]] should be avoided during the first six months following [[recovery]].
 
====[[Plasma exchange]] (PE)====
*There is Class Ib evidence for [[plasma exchange]] as the next step in management if [[high-dose]] [[corticosteroids]] fail<ref name="pmid10589540">{{cite journal| author=Weinshenker BG, O'Brien PC, Petterson TM, Noseworthy JH, Lucchinetti CF, Dodick DW | display-authors=etal| title=A randomized trial of plasma exchange in acute central nervous system inflammatory demyelinating disease. | journal=Ann Neurol | year= 1999 | volume= 46 | issue= 6 | pages= 878-86 | pmid=10589540 | doi=10.1002/1531-8249(199912)46:6<878::aid-ana10>3.0.co;2-q | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=10589540  }} </ref><ref name="pmid11781423">{{cite journal| author=Keegan M, Pineda AA, McClelland RL, Darby CH, Rodriguez M, Weinshenker BG| title=Plasma exchange for severe attacks of CNS demyelination: predictors of response. | journal=Neurology | year= 2002 | volume= 58 | issue= 1 | pages= 143-6 | pmid=11781423 | doi=10.1212/wnl.58.1.143 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=11781423  }} </ref><ref name="pmid11578855">{{cite journal| author=Miyazawa R, Hikima A, Takano Y, Arakawa H, Tomomasa T, Morikawa A| title=Plasmapheresis in fulminant acute disseminated encephalomyelitis. | journal=Brain Dev | year= 2001 | volume= 23 | issue= 6 | pages= 424-6 | pmid=11578855 | doi=10.1016/s0387-7604(01)00256-x | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=11578855  }} </ref>.
*A course of 4-6 PEs has been associated with moderate to marked and sustained improvement, with large volumes of [[plasma]] removable per exchange if there are no signs of [[autonomic]] [[dysfunction]]. Predictors include male sex, preserved [[reflexes]], and early initiation of [[treatment]]<ref name="pmid8912271">{{cite journal| author=Sakakibara R, Hattori T, Yasuda K, Yamanishi T| title=Micturitional disturbance in acute disseminated encephalomyelitis (ADEM). | journal=J Auton Nerv Syst | year= 1996 | volume= 60 | issue= 3 | pages= 200-5 | pmid=8912271 | doi=10.1016/0165-1838(96)00054-9 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=8912271  }} </ref><ref name="pmid10589540">{{cite journal| author=Weinshenker BG, O'Brien PC, Petterson TM, Noseworthy JH, Lucchinetti CF, Dodick DW | display-authors=etal| title=A randomized trial of plasma exchange in acute central nervous system inflammatory demyelinating disease. | journal=Ann Neurol | year= 1999 | volume= 46 | issue= 6 | pages= 878-86 | pmid=10589540 | doi=10.1002/1531-8249(199912)46:6<878::aid-ana10>3.0.co;2-q | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=10589540  }} </ref>.
*[[Autonomic dysfunction]] and [[hypotension]] may preclude the use of PE<ref name="pmid11578855">{{cite journal| author=Miyazawa R, Hikima A, Takano Y, Arakawa H, Tomomasa T, Morikawa A| title=Plasmapheresis in fulminant acute disseminated encephalomyelitis. | journal=Brain Dev | year= 2001 | volume= 23 | issue= 6 | pages= 424-6 | pmid=11578855 | doi=10.1016/s0387-7604(01)00256-x | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=11578855  }} </ref>.
* If conventional PE is unavailable, a small volume [[manual]] [[plasma exchange]] can be performed. A [[phlebotomy]] is done, the [[blood]] is centrifuged, 250-300ml of [[plasma]] is removed and the [[cells]] are returned.It can be done twice daily for 7-10 days<ref name="pmid28970675">{{cite journal| author=Batra A, Periyavan S| title=Role of low plasma volume treatment on clinical efficacy of plasmapheresis in neuromyelitis optica. | journal=Asian J Transfus Sci | year= 2017 | volume= 11 | issue= 2 | pages= 102-107 | pmid=28970675 | doi=10.4103/ajts.AJTS_111_16 | pmc=5613414 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=28970675  }} </ref>.
 
====Intravenous immunoglobulin (IVIg)====
* [[IVIg]] (0.4g/kg/day for five days) is another option, yet its administration is limited by high costs and Class IV level evidence for use as a [[therapeutic]] option in [[ADEM]]<ref name="pmid12509759">{{cite journal| author=Brekke OH, Sandlie I| title=Therapeutic antibodies for human diseases at the dawn of the twenty-first century. | journal=Nat Rev Drug Discov | year= 2003 | volume= 2 | issue= 1 | pages= 52-62 | pmid=12509759 | doi=10.1038/nrd984 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=12509759  }} </ref>.
*[[Clinical]] improvement is apparent within 2-3 days<ref name="pmid10746613">{{cite journal| author=Sahlas DJ, Miller SP, Guerin M, Veilleux M, Francis G| title=Treatment of acute disseminated encephalomyelitis with intravenous immunoglobulin. | journal=Neurology | year= 2000 | volume= 54 | issue= 6 | pages= 1370-2 | pmid=10746613 | doi=10.1212/wnl.54.6.1370 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=10746613  }} </ref><ref name="pmid8576561">{{cite journal| author=Kleiman M, Brunquell P| title=Acute disseminated encephalomyelitis: response to intravenous immunoglobulin. | journal=J Child Neurol | year= 1995 | volume= 10 | issue= 6 | pages= 481-3 | pmid=8576561 | doi=10.1177/088307389501000612 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=8576561  }} </ref><ref name="pmid11784362">{{cite journal| author=Pittock SJ, Keir G, Alexander M, Brennan P, Hardiman O| title=Rapid clinical and CSF response to intravenous gamma globulin in acute disseminated encephalomyelitis. | journal=Eur J Neurol | year= 2001 | volume= 8 | issue= 6 | pages= 725 | pmid=11784362 | doi=10.1046/j.1468-1331.2001.00195.x | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=11784362  }} </ref>.
*[[Methylprednisolone]] along with [[IVIg]] has been successfully used in [[patients]] with [[atypical]] features and could be beneficial for [[fulminant]] and aggressive cases as well<ref name="pmid11275464">{{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 | year= 2001 | volume= 24 | issue= 2 | pages= 139-43 | pmid=11275464 | doi=10.1016/s0887-8994(00)00229-0 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=11275464  }} </ref>.
 
===Others===
[[Cyclophosphamide]]<ref name="pmid11376180">{{cite journal| author=Schwarz S, Mohr A, Knauth M, Wildemann B, Storch-Hagenlocher B| title=Acute disseminated encephalomyelitis: a follow-up study of 40 adult patients. | journal=Neurology | year= 2001 | volume= 56 | issue= 10 | pages= 1313-8 | pmid=11376180 | doi=10.1212/wnl.56.10.1313 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=11376180  }} </ref>, [[rituximab]]<ref name="pmid30626794">{{cite journal| author=Makuuchi Y, Nishimoto M, Yamamoto K, Takahashi T, Kuno M, Nakashima Y | display-authors=etal| title=[Successful treatment with rituximab in acute disseminated encephalomyelitis with whole spinal cord involvement following HLA haploidentical transplantation]. | journal=Rinsho Ketsueki | year= 2018 | volume= 59 | issue= 12 | pages= 2588-2593 | pmid=30626794 | doi=10.11406/rinketsu.59.2588 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=30626794  }} </ref> and [[hypothermia]]<ref name="pmid10426155">{{cite journal| author=Takata T, Hirakawa M, Sakurai M, Kanazawa I| title=Fulminant form of acute disseminated encephalomyelitis: successful treatment with hypothermia. | journal=J Neurol Sci | year= 1999 | volume= 165 | issue= 1 | pages= 94-7 | pmid=10426155 | doi=10.1016/s0022-510x(99)00089-1 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=10426155  }} </ref> have been successfully used to treat [[patients]] with [[fulminant]] [[ADEM]].


==References==
==References==
{{Reflist|2}}


{{Reflist|2}}
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Latest revision as of 10:20, 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

The analogy between the pathogenesis of ADEM and MS forms the basis of the use of high-dose steroids, plasma exchange and intravenous immunoglobulin for the treatment of ADEM.

Medical Therapy

Supportive Care[1]

Immunomodulation

Steroids[1]

Plasma exchange (PE)

Intravenous immunoglobulin (IVIg)

Others

Cyclophosphamide[14], rituximab[15] and hypothermia[16] have been successfully used to treat patients with fulminant ADEM.

References

  1. ↑ 1.0 1.1 Alexander M, Murthy JM (2011). "Acute disseminated encephalomyelitis: Treatment guidelines". Ann Indian Acad Neurol. 14 (Suppl 1): S60–4. doi:10.4103/0972-2327.83095. PMC 3152158. PMID 21847331.
  2. ↑ Carlisi E, Pavese C, Mandrini S, Carenzio G, Dalla Toffola E (2015). "Early rehabilitative treatment for pediatric acute disseminated encephalomyelitis: case report". Eur J Phys Rehabil Med. 51 (3): 341–3. PMID 24937355.
  3. ↑ 3.0 3.1 Sakakibara R, Hattori T, Yasuda K, Yamanishi T (1996). "Micturitional disturbance in acute disseminated encephalomyelitis (ADEM)". J Auton Nerv Syst. 60 (3): 200–5. doi:10.1016/0165-1838(96)00054-9. PMID 8912271.
  4. ↑ Straub J, Chofflon M, Delavelle J (1997). "Early high-dose intravenous methylprednisolone in acute disseminated encephalomyelitis: a successful recovery". Neurology. 49 (4): 1145–7. doi:10.1212/wnl.49.4.1145. PMID 9339706.
  5. ↑ 5.0 5.1 Weinshenker BG, O'Brien PC, Petterson TM, Noseworthy JH, Lucchinetti CF, Dodick DW; et al. (1999). "A randomized trial of plasma exchange in acute central nervous system inflammatory demyelinating disease". Ann Neurol. 46 (6): 878–86. doi:10.1002/1531-8249(199912)46:6<878::aid-ana10>3.0.co;2-q. PMID 10589540.
  6. ↑ Keegan M, Pineda AA, McClelland RL, Darby CH, Rodriguez M, Weinshenker BG (2002). "Plasma exchange for severe attacks of CNS demyelination: predictors of response". Neurology. 58 (1): 143–6. doi:10.1212/wnl.58.1.143. PMID 11781423.
  7. ↑ 7.0 7.1 Miyazawa R, Hikima A, Takano Y, Arakawa H, Tomomasa T, Morikawa A (2001). "Plasmapheresis in fulminant acute disseminated encephalomyelitis". Brain Dev. 23 (6): 424–6. doi:10.1016/s0387-7604(01)00256-x. PMID 11578855.
  8. ↑ Batra A, Periyavan S (2017). "Role of low plasma volume treatment on clinical efficacy of plasmapheresis in neuromyelitis optica". Asian J Transfus Sci. 11 (2): 102–107. doi:10.4103/ajts.AJTS_111_16. PMC 5613414. PMID 28970675.
  9. ↑ Brekke OH, Sandlie I (2003). "Therapeutic antibodies for human diseases at the dawn of the twenty-first century". Nat Rev Drug Discov. 2 (1): 52–62. doi:10.1038/nrd984. PMID 12509759.
  10. ↑ Sahlas DJ, Miller SP, Guerin M, Veilleux M, Francis G (2000). "Treatment of acute disseminated encephalomyelitis with intravenous immunoglobulin". Neurology. 54 (6): 1370–2. doi:10.1212/wnl.54.6.1370. PMID 10746613.
  11. ↑ Kleiman M, Brunquell P (1995). "Acute disseminated encephalomyelitis: response to intravenous immunoglobulin". J Child Neurol. 10 (6): 481–3. doi:10.1177/088307389501000612. PMID 8576561.
  12. ↑ Pittock SJ, Keir G, Alexander M, Brennan P, Hardiman O (2001). "Rapid clinical and CSF response to intravenous gamma globulin in acute disseminated encephalomyelitis". Eur J Neurol. 8 (6): 725. doi:10.1046/j.1468-1331.2001.00195.x. PMID 11784362.
  13. ↑ Straussberg R, Schonfeld T, Weitz R, Karmazyn B, Harel L (2001). "Improvement of atypical acute disseminated encephalomyelitis with steroids and intravenous immunoglobulins". Pediatr Neurol. 24 (2): 139–43. doi:10.1016/s0887-8994(00)00229-0. PMID 11275464.
  14. ↑ Schwarz S, Mohr A, Knauth M, Wildemann B, Storch-Hagenlocher B (2001). "Acute disseminated encephalomyelitis: a follow-up study of 40 adult patients". Neurology. 56 (10): 1313–8. doi:10.1212/wnl.56.10.1313. PMID 11376180.
  15. ↑ Makuuchi Y, Nishimoto M, Yamamoto K, Takahashi T, Kuno M, Nakashima Y; et al. (2018). "[Successful treatment with rituximab in acute disseminated encephalomyelitis with whole spinal cord involvement following HLA haploidentical transplantation]". Rinsho Ketsueki. 59 (12): 2588–2593. doi:10.11406/rinketsu.59.2588. PMID 30626794.
  16. ↑ Takata T, Hirakawa M, Sakurai M, Kanazawa I (1999). "Fulminant form of acute disseminated encephalomyelitis: successful treatment with hypothermia". J Neurol Sci. 165 (1): 94–7. doi:10.1016/s0022-510x(99)00089-1. PMID 10426155.

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