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Acute disseminated encephalomyelitis following allo-SCT: central nervous system manifestation of GVHD

Bone Marrow Transplantation volume 49, pages 854–856 (2014)Cite this article

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Acute disseminated encephalomyelitis (ADEM) is a rare demyelinating disease of the central nervous system (CNS). It characteristically occurs in children and is putatively autoimmune in nature, triggered by environmental stimuli in susceptible individuals. A monophasic clinical presentation with multifocal neurological symptoms and encephalopathy is typical. Diagnosis is on the basis of a constellation of clinical and radiological features (Table 1).1 The principal differential diagnoses include multiple sclerosis, characterized by recurrent episodes of CNS demyelination, optic neuritis, transverse myelitis and neuromyelitis optica. ADEM is often preceded by infection (occasionally immunisation), typically 1 month before neurological dysfunction.

Table 1 Diagnostic criteria for ADEM
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The pathogenesis is not fully understood, although the principal hypothesis implicates myelin proteins serving as autoantigens sharing antigenic determinants with an infectious agent.2 One study demonstrated a 10-fold greater reactivity of T lymphocytes directed towards myelin basic protein in those with ADEM, compared with normal controls and encephalitis patients.3 Anti-myelin-associated glycoprotein (anti-MAG) and anti-myelin basic protein (anti-MBP) antibodies have been detected in cerebrospinal fluid (CSF).4 Immunomodulation in the form of steroids, i.v. Ig and plasmapheresis is the mainstay of treatment.5

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Figure 1

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Authors and Affiliations

  1. Department of Haematology, Nottingham University Hospitals NHS Trust, Nottingham, UK

    C M Harvey, R Gottipati, N H Russell & C P Fox

  2. Department of Radiology, Nottingham University Hospitals NHS Trust, Nottingham, UK

    S Schwarz & D Auer

  3. Department of Neurology, Nottingham University Hospitals NHS Trust, Nottingham, UK

    M O'Donoghue

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  1. C M Harvey
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  2. R Gottipati
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Correspondence to C M Harvey.

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Harvey, C., Gottipati, R., Schwarz, S. et al. Acute disseminated encephalomyelitis following allo-SCT: central nervous system manifestation of GVHD. Bone Marrow Transplant 49, 854–856 (2014). https://doi.org/10.1038/bmt.2014.29

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