Acute disseminated encephalomyelitis medical therapy: Difference between revisions
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*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>. | *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>. | ||
* 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>. | * 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]]==== | ====[[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>. | |||
==References== | ==References== | ||
Revision as of 07:49, 21 November 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]
- 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
Immunomodulation
Steroids[1]
- 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[2].
- The dose is 10-30mg/kg/day up to a maximum of 1g/day for 3-5days (Class IV)[3].
- 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[4][5][6].
- 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[2][4].
- 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[7].
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[8].
- Clinical improvement is apparent within 2-3 days[9][10][11].
- Methylprednisolone along with IVIg has been successfully used in patients with atypical features and could be beneficial for fulminant and aggressive cases as well[12].
References
- ↑ 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.
- ↑ 2.0 2.1 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.
- ↑ 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.
- ↑ 4.0 4.1 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.
- ↑ 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.
- ↑ 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.
- ↑ 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.
- ↑ 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.
- ↑ 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.
- ↑ 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.
- ↑ 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.
- ↑ 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.