ACUTE DISSEMINATED ENCEPHALOMYELITIS (ADEM)

Objective:

To describe the epidemiologic, clinical, neuroimaging, laboratory features, treatment; and outcome in a cohort of children with acute disseminated encephalomyelitis (ADEM).

Introduction: Acute disseminated encephalomyelitis (ADEM) is an acute demyelinating disorder of the central nervous system characterised by multifocal white matter involvement due to an autoimmune pathology. Diffuse neurological signs along with multifocal lesions in brain and spinal cord characterise the disease. It usually follows an infection or vaccination.

ADEM is a monophasic disease, 80% have good recovery with no neurologic deficit, Current treatment with pulse steroid therapy Vs IVIG.

Methods:

A 10-years retrospective chart review of children with the diagnosis of ADEM was conducted at King Abdulaziz Medical City (KAMC).

Results: Thirteen cases were identified. Six patient (46%) presented in the fall. Girls affected more than boys (5.5:1). Age of presentation range between (2–17y). Six children (46%) had recent upper respiratory tract illness, five children (38%) had gastrointestinal disturbance. patients presented most often with motor deficits (84%)

. 30% of the patients presented with encephalopathy. Spinal fluid abnormalities occurred in 61%. Despite rigorous microbiologic testing a definite microbiologic diagnosis was established in two patients with Epstein-Barr virus disease, one patients with herpes simplex virus, one patient with mycoplasma pneumoniae.

Brain magnetic resonance imaging identified lesion in subcortical white matter in all patients (100%), thalami in 38%, basal ganglia 38%, brain stem 23%, and in spinal cord 61% of patients. Follow up MRI done in 61%,62% was normal, 37% showed significant improvement, CT SCAN done in 70%, Was abnormal in only 11% of the cases, Eleven patients (84%) were treated with corticosteroid, 2 were treated with intravenous immunoglobulins.

Result: All patients survived, 58%had excellent recovery, 33% had mild neuralgic sequelae with high functional level, 8% had moderate to severe neuralgic sequelae.

Conclusion:

Epidemiologic evidence from this study suggests an autoimmune cause of ADEM. Most of the time it is difficult to identify the underling causative agent. Magnetic resonance imaging was the neuroimaging study of choice for establishing the diagnosis and for following the course of the disease. Prognosis for survival and outcome was favorable. Involvement of spinal cord with brain favor the outcome as in our study and one report of a previous study.