Antibodies to native myelin oligodendrocyte glycoprotein in children with inflammatory
demyelinating central nervous system disease.
Brilot F, Dale RC, Selter RC, Grummel V, Kalluri SR, Aslam M, Busch V, Zhou D, Cepok
S, Hemmer B.
Neuroimmunology Group, Institute for Neuroscience and Muscle Research, the Kids
Research Institute at the Children's Hospital at Westmead, University of Sydney,
Sydney, Australia.
OBJECTIVE: Myelin oligodendrocyte glycoprotein (MOG) is a candidate target antigen
in demyelinating diseases of the central nervous system (CNS). Although MOG is encephalitogenic
in different animal models, the relevance of this antigen in human autoimmune diseases
of the CNS is still controversial. METHODS: We investigated the occurrence and biological
activity of antibodies to native MOG (nMOG) in 47 children during a first episode
of CNS demyelination (acute disseminated encephalomyelitis [ADEM], n = 19 and clinical
isolated syndrome [CIS], n = 28) by a cell-based bioassay. RESULTS: High serum immunoglobulin
G (IgG) titers to nMOG were detected in 40% of children with CIS/ADEM but 0% of
the control children affected by other neurological diseases, healthy children,
or adults with inflammatory demyelinating diseases, respectively. By contrast, IgM
antibodies to nMOG occurred in only 3 children affected by ADEM. Children with high
anti-nMOG IgG titer were significantly younger than those with low IgG titer. Anti-nMOG
IgG titers did not differ between the ADEM and CIS group, and did not predict conversion
from CIS to MS during a mean 2-year follow-up. However, intrathecal IgG anti-MOG
antibody synthesis was only seen in CIS children. IgG antibodies to nMOG not only
bound to the extracellular domain of nMOG, but also induced natural killer cell-mediated
killing of nMOG-expressing cells in vitro. INTERPRETATION: Overall, these findings
suggest nMOG as a major target of the humoral immune response in a subgroup of children
affected by inflammatory demyelinating diseases of the CNS. Children may provide
valuable insight into the earliest immune mechanisms of CNS demyelination.
Ann Neurol. 2009 Dec;66(6):833-42.
N-Methyl-D-Aspartate Receptor Antibodies in Pediatric Dyskinetic Encephalitis Lethargica
Russell C. Dale, Sarosh R. Irani, Fabienne Brilot, Sekhar Pillai, Richard Webster,
Deepak Gill, Bethan Lang, and Angela Vincent
Encephalitis lethargica (EL) describes an encephalitis with psychiatric, sleep,
and extrapyramidal movement disorders. Dyskinetic and Parkinsonian forms have been
described. EL shares clinical features with the anti–N-methyl-D-aspartate receptor
(NMDAR-Ab) encephalitis. We studied 20 sera from pediatric patients with contemporary
EL. Ten sera (from 2 males and 8 females, aged 1.3–13 years) and 6/6 cerebrospinal
fluid samples were positive for NMDAR-Ab. NMDAR-Ab–positive patients had dyskinesias,
agitation, seizures, and insomnia, whereas Parkinsonism and somnolence dominated
in the NMDAR-Ab–negative children. We were unable to identify any tumors. The dyskinetic
form of EL is an NMDAR-Ab encephalitis and can affect very young children.
ANN NEUROL 2009;66:704–709
Isolation of vaccinia virus from children with post-vaccinal encephalitis at late
interval after vaccination (1975)
Gurvich E G, Mouseyants A A, Stephanenkov A
Acta Virol 19: 92
Neurology
Acute disseminated encephalomyelitis
A long-term follow-up study of 84 pediatric patients
Silvia Tenembaum, MD; Nestor Chamoles, MD; and Natalio Fejerman, MD
Abstract—Background: Acute disseminated encephalomyelitis (ADEM) is
an inflammatory demyelinating disease of the CNS. Few pediatric series have been
published, with retrospective and short-term follow-up studies. Objectives:
To describe a cohort of pediatric patients with ADEM and to determine whether clinical
and neuroimaging findings predict outcome. Methods: A prospective study was
conducted between March 1988 and July 2000 on 84 consecutive children with ADEM
at the National Pediatric Hospital “Dr. J. P. Garrahan.” Results: Mean
age at onset was 5.3 - 3.9 years, with a significant male predominance. Sixty-two
patients (74%) had a preceding viral illness or vaccination. Acute hemiparesis (76%),
unilateral or bilateral long tract signs (85%), and changes in mental state (69%)
were the most prominent presenting features. Four MRI groups were identified: ADEM
with small lesions (62%), with large lesions (24%), with additional bithalamic involvement
(12%), and acute hemorrhagic encephalomyelitis (2%). Of the 54 children whose CSF
samples were analyzed, none showed intrathecal oligoclonal bands. The use of high-dose
corticosteroid treatment, particularly IV methylprednisolone, was associated with
good recovery and resolution of MRI lesions. After a mean follow-up of 6.6 - 3.8
years, 90% of children showed a monophasic course, and 10% a biphasic disease. Eighty-nine
percent of patients show at present Expanded Disability Status Scale scores of 0
to 2.5. Eleven percent have disability scores of 3 to 6.5. Conclusions: Childhood
acute disseminated encephalomyelitis is a benign condition, affecting boys more
frequently. No association was found between MRI groups and disability. Disability
was related to optic nerve involvement at presentation. Even in relapsing cases,
the distinction between acute disseminated encephalomyelitis and MS was possible
on the basis of long-term clinical and neuroimaging follow-up and the absence of
oligoclonal bands in CSF.
NEUROLOGY 2002;59:1224–1231
This information is current as of August 22, 2007
The online version of this article, along with updated information and services,
is located on the World Wide Web at:www.neurology.org/cgi/content/full/59/8/1224
Last modified: January 2012