Clinical and radiological features of recurrent demyelination following acute disseminated encephalomyelitis (ADEM).
OBJECTIVE
To identify clinical and radiological features of children that relapse following an initial presentation of acute disseminated encephalomyelitis (ADEM).


METHODS
Clinical records and neuroimaging of children under the age of 16 years presenting with ADEM to a pediatric neurology referral center between 2006 and 2010 were evaluated.


RESULTS
Of the 32 children with ADEM, 24 (7 female) with a median age of 4.8 (range 3-15) had a monophasic course. Eight patients (25%; 4 female) with median age of 6.9 (range 3-16) had relapsing demyelination; 3 relapsing within the 3 month interval (still defined within a monophasic event); 4 with multiphasic disseminated encephalomyelitis (MDEM), and 1 with non-multiple sclerosis recurrent demyelination. Clinical features at presentation could not distinguish the monophasic from the relapsing group. Infratentorial imaging changes (brain stem and cerebellar) were seen more frequently in the relapsing group (8/8 vs. 20/24), although differences were not statistically significant. At relapse, seven of the eight patients had clinical and radiologic infratentorial syndromes involving brainstem and/or cerebellum. Only one patient had more than one relapse. After a median follow up of 27 months (range 0-96) across the whole group, no patients were diagnosed with multiple sclerosis.


CONCLUSIONS
ADEM patients with infratentorial demyelination are more likely to present with a second infratentorial demyelination, although clinical and radiological features at outset could not predict the relapsing cohort.
Children's Neurosciences, Evelina London Children's Hospital at Guys and St Thomas' NHS Foundation Trust, Kings Health Partners Academic Health Science Centre, London, UK.
Sanduni
S
Kariyawasam
Children's Neurosciences, Evelina London Children's Hospital at Guys and St Thomas' NHS Foundation Trust, Kings Health Partners Academic Health Science Centre, London, UK.
Rahul R
RR
Singh
Children's Neurosciences, Evelina London Children's Hospital at Guys and St Thomas' NHS Foundation Trust, Kings Health Partners Academic Health Science Centre, London, UK.
Jonathan
J
Gadian
Children's Neurosciences, Evelina London Children's Hospital at Guys and St Thomas' NHS Foundation Trust, Kings Health Partners Academic Health Science Centre, London, UK.
Daniel E
DE
Lumsden
Children's Neurosciences, Evelina London Children's Hospital at Guys and St Thomas' NHS Foundation Trust, Kings Health Partners Academic Health Science Centre, London, UK.
Jean-Pierre
JP
Lin
Department of Neuroradiology, Kings College Hospital, King's Health Partners Academic Health Science Centre, London, UK.
Ata
A
Siddiqui
Children's Neurosciences, Evelina London Children's Hospital at Guys and St Thomas' NHS Foundation Trust, Kings Health Partners Academic Health Science Centre, London, UK.
Yael
Y
Hacohen
Children's Neurosciences, Evelina London Children's Hospital at Guys and St Thomas' NHS Foundation Trust, Kings Health Partners Academic Health Science Centre, London, UK.
Michael
M
Absoud
Children's Neurosciences, Evelina London Children's Hospital at Guys and St Thomas' NHS Foundation Trust, Kings Health Partners Academic Health Science Centre, London, UK. Electronic address: ming.lim@gstt.nhs.uk.
Ming
M
Lim
D000293:Adolescent; D001921:Brain / Q000473:pathology; D002648:Child; D002675:Child, Preschool; D004673:Encephalomyelitis, Acute Disseminated / Q000473:pathology* / Q000503:physiopathology*; D005260:Female; D005500:Follow-Up Studies; D006776:Hospitals, Pediatric; D006801:Humans; D008131:London; D008279:Magnetic Resonance Imaging; D008297:Male; D012008:Recurrence; D012189:Retrospective Studies; D012307:Risk Factors; D013116:Spinal Cord / Q000473:pathology; D062606:Tertiary Care Centers
ADEM; Acquired demyelination syndrome; Demyelination; Multiple sclerosis; Relapsing