Objective: To describe the clinical, therapeutic and outcome features of movement disorders (MD) observed in children with West Syndrome (WS) at the Neurology Department of the Fann National Teaching Hospital in Dakar.

Background: Movement disorders in children are common but rarely studied in our country.They are most often secondary origin than primary and they are associated with brain lesions (cerebral palsy, stroke, head trauma, sequelae of neonatal asphyxia). The particularity of studying pediatric MD in epileptic encephalopathy such as West syndrome is to differentiate them from other epileptic seizures that can mimic MD.

Method: This was a prospective study conducted from October 2015 to December 2016. All of our WS outpatient clinic and followed up regularly for at least 12 months were included. Age at consultation, electroclinical, neuroimaging, therapeutic and outcome data were collected.

Results: A total of 14 children with WS were enrolled and 4 of them were diagnosed with MD. The mean age at diagnosis was 11 months (range 6-13) with a sex ratio of 1. The type of MD observed were: chorea (1), athetosis (1), axial dystonia (2). No family history of MD was found. Brain computed tomography showed: diffuse cerebral atrophy (4), thalamic calcifications (1), multicysts encephalomalacia (2), mega cisterna magna (1). WS was symptomatic in all the children and MD were  secondary. Drugs used to control MD were diazepam and/or haloperidol. All of the children have received physiotherapy. Overall, the outcome of MD was poor in the 4 children.

Conclusion: Movement disorders were found in these children with WS and their outcome was poor.

References: Delgado, Mauricio R., and A. Leland Albright. “Movement disorders in children: definitions, classifications, and grading systems.” Journal of child neurology 18.1_suppl (2003): S1-S8.

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MDS Abstracts - https://www.mdsabstracts.org/abstract/movement-disorders-features-associated-with-west-syndrome/