Affiliations: Department of Child Health (Neurology) Sultan Qaboos
University Hospital, Muscat, Oman
Note: [] Correspondence: Dr. Roshan Koul, M.D., Senior Consultant and
Child Neurologist, Sultan Qaboos University Hospital, Muscat, Oman, BW 1, P Box
38, 123, Oman. Tel.: +968 99210428; Fax: +968 24414470; E-mail:
[email protected]
Abstract: Acute disseminated encephalomyelitis is typically a monophasic
multifocal autoimmune demyelinating disease of the central nervous system,
which generally follows a febrile infection or a vaccination. It presents with
a variety of clinical features reflecting the multifocal nature of the disease.
The study was carried out at Sultan Qaboos University Hospital Oman, which is a
tertiary care hospital, to see the pattern in this country. A review of
inpatients files' records was done with the diagnosis of acute disseminated
encephalomyelitis. Inclusion criteria were acute onset, a preceding infection
or vaccination, hyperattenuated lesions seen on T2 weighted magnetic resonance
imaging, or computerized tomography findings consistent with white matter
changes and rapid response to corticosteroids upon therapy. Twenty-three
children met the clinical and imaging criteria. Fifteen (65%) were males and
eight (35%) were females giving a ratio of 2:1. Their ages ranged from 9
months to 11 years with median age of 5 years. Eleven (48%) patients had a
history of an acute respiratory tract infection two to 20 days before
presentation. Two (9%) patients had chickenpox, and another two (9%)
patients had fever with a rash. One (4%) patient had pneumonia with positive
IgM for Mycoplasma pneumonia. None had received a vaccine prior to
presentation. Pyramidal signs were seen in the majority of children (83%).
Most children had involvement of the subcortical white matter (74%) followed
by basal ganglia (24%) and thalamic lesions (24%). Corticosteroids were
administered to 21 (91%) children. Eight (35%) of these children had
received intravenous immunoglobulin after failure to respond adequately to
steroid therapy. Full recovery was seen in 16 (70%) of the patients. The
average period of recovery was around 3 weeks. Seven (30%) patients had
persistent neurological deficits upon discharge as well as on follow up two
years later. The clinical features, outcome and response to treatment were
similar to other reported studies. This is the first study to show a clear-cut
male predilection of 2:1.
