Progression of ADEM

Progression of ADEM My patient´s diagnosis fits with ADEM, but the condition has been progressing for 2 months. profAskTheExpert neurology/neurosurgery Rohit Bakshi, MD, <a href="http://www.drbakshi.com/" target="_blank">http://www.drbakshi.com/</a>, Founding Director, Buffalo Neuroimaging Analysis Center, <a href="http://www.bnac.net/" target="_blank">http://www.bnac.net</a>; neuroimager and Associate Professor of Neurology, University at Buffalo, State University of New York Medscape Medscape Neurology & Neurosurgery 05/09/2000 2 1 <h3>Question</h3> What is the maximum duration of progression of acute disseminated encephalomyelitis (ADEM)? My patient's diagnosis fits with ADEM, but the condition has been progressing for 2 months. <H3>Response</H3> from , 05/09/2000 ADEM is an inflammatory demyelinating disorder of the CNS that seems to be related to autoimmunity.[1,2] ADEM is usually monophasic with peak disability a few days or weeks after the onset of illness, but recurrent or protracted episodes may occur.[3] When ADEM is recurrent, the distinction from multiple sclerosis (MS) and its variants becomes difficult.[2] ADEM that progresses over several weeks or a few months is usually accompanied by worsening of involvement seen on MRI. Because progression beyond 2 or 3 weeks is unusual, other diseases should be considered, such as a malignant form of MS, mitochondrial diseases, infections, vasculopathies, and neoplasia.[2-4] Treatment options for ADEM include a variety of immunosuppressive agents such as high-dose corticosteroids,[5,6] intravenous immunoglobulin (IVIG),[1] and plasmapheresis.[7,8] Recovery from ADEM is variable, with a mortality of approximately 20% and permanent sequelae common, especially when progression is rapid and the spinal cord is affected. When progression is slower, a response to steroids and a better outcome is likely.[5] Inflammatory demyelination may occur in a solitary form in the brain (leukoencephalitis)[9] or spinal cord (myelitis).[10] These findings seem to lie on a spectrum of inflammatory diseases linking ADEM and MS for which the acute therapies are similar. In ADEM, MRI typically shows multifocal asymmetric white matter lesions involving the cerebral hemispheres, cerebellum, and brain stem that are usually larger than those seen in MS but also may resemble MS.[2,11] The majority of these lesions enhance after contrast administration. Large supratentorial solitary inflammatory-demyelinating lesions (leukoencephalitis) may resemble neoplasms on MRI studies.[9] <OL><LI>Hahn JS, Siegler DJ, Enzmann D. Intravenous gammaglobulin therapy in recurrent acute disseminated encephalomyelitis. Neurology. 1996;46:1173-1174.</LI><LI>Kesselring J, Miller DH, Robb SA, et al. Acute disseminated encephalomyelitis: MRI findings and the distinction from multiple sclerosis. Brain. 1990;113:291-302.</LI><LI>Durston JHJ, Milnes JN. Relapsing encephalomyelitis. Brain. 1970;93:715-730.</LI><LI>Bakshi R, Mazziotta JC, Mischel PS, Jahan R, Seligson DB, Vinters HV. Lymphomatosis cerebri presenting as a rapidly progressive dementia: clinical, neuroimaging and pathologic findings. Dement Geriatr Cogn Disord. 1999;10:152-157.</LI><LI>Pasternak JF, De Vivo DC, Prensky AL. Steroid-responsive encephalomyelitis in childhood. Neurology. 1980;30:481-486.</LI><LI>Straub J, Chofflon M, Delavelle J. Early high-dose intravenous methylprednisolone in acute disseminated encephalomyelitis: a successful recovery. Neurology. 1997;49:1145-1147.</LI><LI>Kanter DS, Horensky D, Sperling RA, Kaplan JD, Malachowski ME, Churchill WH Jr. Plasmapheresis in fulminant acute disseminated encephalomyelitis. Neurology. 1995;45:824-827.</LI><LI>Stricker RB, Miller RG, Kiprov DD. Role of plasmapheresis in acute disseminated (postinfectious) encephalomyelitis. J Clin Apheresis. 1992;7:173-179.</LI><LI>Bakshi R, Glass J, Louis DN, Hochberg FH. Magnetic resonance imaging features of solitary inflammatory brain masses. J Neuroimaging 1998;8:8-14.</LI><LI>Bakshi R, Kinkel PR, Mechtler LL, et al. Magnetic resonance imaging findings in 22 cases of myelitis: comparison between patients with and without multiple sclerosis. Eur J Neurol. 1998;5:35-48.</LI><LI>Epperson LW, Whitaker JN, Kapila AL. Cranial MRI in acute disseminated encephalomyelitis. Neurology. 1988;38:332-333.</LI></OL>