ACQUIRED DEMYELINATING DISORDERS IN A THIRD WORLD PAEDIATRIC POPULATION

Objectives: To describe the epidemiology, presentation, neuro-imaging, management and outcome of acquired CNS demyelinating disorders in a resource poor setting.

Methods: A retrospective review between January 1999– June 2005 at the Red Cross Hospital, South Africa.

Results: Nineteen children (0.6%) with acquired demyelinating CNS pathology were identified from a database of 3159; 8- ADEM, 1- Schilder's Disease, 4– Multiple sclerosis (MS), 6– transverse myelitis (TM). The median age was 83months (range 21–132) (7M:12F). 52% had a precipitant. A seasonal distribution was identified. The commonest clinical presentation was hemiparesis (50%). CSF protein was elevated in 47%. 52% had CSF pleocytosis. An organism was identified in 8 patients. The commonest sites of MRI abnormalities were: cerebellum (48%), deep white matter (68%), cervical spine (66%) in the TM group. Two patients with ADEM and 2 with MS had spinal cord involvement. Eighteen patients received steroids. 8 patients required ICU admission. Two MS patients received beta-interferon. The median time to improvement was 5 days. All MS patients relapsed within one year. Six of the ADEM and 3 of the TM patients made a complete recovery within 1 year. The following trends were identified in the ADEM vs. MS group: precipitant (62% vs. 20%), encephalopathy (38% vs. 25%), polysymptomatic presentation (62% vs. 25%), CSF protein elevation (47% VS 100%), sites of involvement: basal ganglia (50% vs. 0), cerebellar (38% vs. 70%), brainstem (25%vs 50%).

Conclusion: These disorders should be considered and differentiated from other disorders presenting with multifocal neurology especially tuberculosis and HIV. Trends followed international studies except for the lower incidence (0.6% vs. 1–2%). There were differences between the ADEM vs. MS group which are useful in predicting evolution to MS.