Acute disseminated encephalomyelitis medical therapy
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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
- Early initiation of physical, occupational and speech therapy, when applicable, can aid earlier and more complete recovery in pediatric ADEM[2].
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[3].
- The dose is 10-30mg/kg/day up to a maximum of 1g/day for 3-5days (Class IV)[4].
- 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[5][6][7].
- 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[3][5].
- Autonomic dysfunction and hypotension may preclude the use of PE[7].
- 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[8].
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[9].
- Clinical improvement is apparent within 2-3 days[10][11][12].
- Methylprednisolone along with IVIg has been successfully used in patients with atypical features and could be beneficial for fulminant and aggressive cases as well[13].
Others
Cyclophosphamide[14], rituximab[15] and hypothermia[16] have been successfully used to treat patients with fulminant ADEM.
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.
- ↑ 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.
- ↑ 3.0 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.
- ↑ 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.
- ↑ 5.0 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.
- ↑ 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.
- ↑ 7.0 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.
- ↑ 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.
- ↑ 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.
- ↑ 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.
- ↑ 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.