Specialist Working Group for Neurology Proposed changes to the Criteria for the clinical use of intravenous immunoglobulin in Australia, Second Edition ITEM CRITERIA FOR THE CLINICAL USE OF INTRAVENOUS IMMUNOGLOBULIN IN AUSTRALIA, SECOND EDITION (CRITERIA) PROPOSED REVISIONS TO THE CRITERIA SWG RATIONALE FOR PROPOSED CHANGE (A) Administrative) (B) Progressive (C) Programmed Condition Name Acute disseminated encephalomyelitis (ADEM) Acute disseminated encephalomyelitis (ADEM) Specialty Neurology Neurology Chapter 6 6 Specific Conditions Monophasic ADEM Recurrent ADEM Multiphasic ADEM Level of Evidence Evidence of probable benefit (Category 2a). Evidence of probable benefit (Category 2a) Justification for Evidence Category On review of multiple case series of IVIg use for paediatric ADEM found that children with monophasic ADEM completely recovered after administration of IVIg or IVIg plus corticosteroids. In recurrent ADEM, children either completely recovered after IVIg, or showed improvement. Adults with monophasic or recurrent ADEM recovered after treatment with IVIg. On review of multiple case series of intravenous immunoglobulin (IVIg) use for paediatric ADEM found that children with monophasic ADEM completely recovered after administration of IVIg or IVIg plus corticosteroids. In recurrent ADEM, children either completely recovered after IVIg, or showed improvement. Adults with monophasic or recurrent ADEM recovered after treatment with IVIg. Data from the International Pediatric MS Study Group (IPMSSG) in 2014 confirms this view. Revised with addition from the IPMSSG (International Paediatric MS Study Group) in 2014 confirms category 2a. Description and Diagnostic Criteria High-dose corticosteroids are first-line treatment of ADEM. IVIg has been used for patients who fail to respond to steroid therapy or in patients where steroids are contraindicated. Most patients with ADEM recover completely over a period of six weeks from onset. ADEM is a monophasic inflammatory condition of the central nervous system that usually presents in children and young adults. It typically occurs following a viral prodrome with multifocal neurological disturbance and altered conscious state. ADEM usually follows a monophasic Revised to describe and require IPMSSG Criteria to be used.
Diagnosis of recurrent or multiphasic ADEM (as per IPMSSG criteria) (Krupp et al. 2013). AND Evidence of deterioration in symptoms. AND Relapse occurs within six months of the last immunoglobulin (Ig) dose. 1Krupp, LB, Tardieu, M, Amato, MP, 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’, Multiple Sclerosis Journal, vol. 19, pp. 1261–1267. Review Criteria Monophasic ADEM unresponsive to steroid therapy or where steroids are contraindicated. No review is required. Further doses of Ig can be requested as required for patients with extended monophasic ADEM. Recurrent or multiphasic ADEM unresponsive to steroid therapy or where steroid therapy has become intolerable or is contraindicated, with assessment by a neurologist mandatory. Review Preamble Objective evidence of improvement in relapse rate in comparison to pre-treatment levels is required for reauthorisation. Six-monthly review by a neurologist is required Response must be demonstrated after 6 months treatment for Trial off therapy must be undertaken after 12 months treatment. If patients with Recurrent or Multi-phasic ADEM relapse within 6 months of ceasing Ig treatment, is defined with requirement for further response at review.
neurologist is mandatory. Dosing above 1 g/kg per day is contraindicated for some IVIg products. Refer to the current product information sheet for further information. The aim should be to use the lowest dose possible that achieves the appropriate clinical outcome for each patient. an alternative diagnosis should be considered. Induction - Up to 2 g/kg in 2 to 5 divided doses. Maintenance - For extended monophasic ADEM: 0.4–2 g/kg, 4-–6 weekly. After three months, if symptoms persist, an alternative diagnosis should be considered. The aim should be to use the lowest dose possible that achieves the appropriate clinical outcome for each patient. Dosing above 1 g/kg per day is contraindicated for some IVIg products. Refer to the current product information sheet for further information. Recurrent or multiphasic ADEM unresponsive to steroid therapy or where steroid therapy has become intolerable or is contraindicated, with assessment by a neurologist mandatory. Induction - Up to 2 g/kg in 2 to 5 divided doses kg as a single dose. Maintenance - For recurrent or multiphasic ADEM: 0.4–2 g/kg, 4–6 weekly. The aim should be to use the lowest dose possible that achieves the appropriate clinical be requested. If further treatment is needed, then an alternate diagnosis and review is mandatory.
Mariotti, P, Batocchi, AP, Colosimo, C, et al 2003, ‘Multiphasic demyelinating disease involving central and peripheral nervous system in a child’, Neurology, vol. 60, no. 2, pp. 348–9. Nishikawa, M, Ichiyama, T, Hayashi, T, et al 1999, ‘Intravenous immunoglobulin therapy in acute disseminated encephalomyelitis’, Paediatric Neurology, vol. 21, no. 2, pp. 583–6. Pittock, SJ, Keir, G, Alexander, M, et al 2001, ‘Rapid clinical and CSF response to intravenous gamma globulin in acute disseminated encephalomyelitis’, European Journal of Neurology, vol. 8, no. 6, p. 725. Pradhan, S, Gupta, RP, Shashank, S, et al 1999, ‘Intravenous immunoglobulin therapy in acute disseminated encephalomyelitis’, Journal of the Neurological Sciences, vol. 165, no. 1, pp. 56–61. Revel-Vilk, S, Hurvitz, H, Klar, A, et al 2000, ‘Recurrent acute disseminated encephalomyelitis associated with acute cytomegalovirus and Epstein-Barr virus infection’, Journal of Child Neurology, vol. 15, no. 6, pp. 421–4. Sahlas, DJ, Miller, SP, Guerin, M, et al 2000, ‘Treatment of acute disseminated encephalomyelitis with intravenous immunoglobulin’, Neurology, vol. 54, no. 6, pp. 1370–2. END OF DOCUMENT