TITLE:
Clinical, aetiological and evolutive aspects of West syndrome in Yaoundé (Cameroon)
AUTHORS:
Séraphin Nguefack, Boniface Moifo, Andréas Chiabi, Antoine Ledoux Defo, Evelyn Mah, Florence Fru, Paul Cédric Mbonda, Pierre Fernand Tchokoteu, Elie Mbonda
KEYWORDS:
West Syndrome; Epilepsy; Aetiology; Evolution; Cameroon
JOURNAL NAME:
Open Journal of Pediatrics,
Vol.3 No.4,
November
27,
2013
ABSTRACT: Background: West syndrome (WS) is an epileptic syndrome of the
infant occurring between the 3rd and 12th months of life
and characterized by the triad: infantile spasms in flexion, extension or
mixed; global developmental delay; and hypsarrythmia on the electroencephalogram
(EEG). Its incidence varies between 2.9 and 4.5 per 10,000 live births. West syndrome
is caused by a brain dysfunction whose origins can be prenatal, neonatal and
postnatal. Sometimes the aetiology is genetic or unknown. Purpose: To determine the main clinical, aetiological and major
evolutive aspects of West syndrome in child neurology unit in a
university-affiliated hospital in Yaoundé. Materials
and Methods: It was a retrospective descriptive
study conducted from September 2011 to January 2012 inthe child neurology unit of the Yaoundé gynaeco-obstetric
and paediatric hospital. The medical records of 68 children followed for West
syndrome (WS) in the service during the period from February 2008 to January
2012 (48 months) were used. All infants of 1- to 16-month-old with the diagnosis
of WS were included. The diagnosis of WS was based on clinical evidence of
spasm in flexion and/or in extension with global development delay, and EEG
evidence of hypsarythmia or focal/multifocal epileptic abnormalities when
hypsarythmia is absent. For each included infant, relevant medical history and
complete physical examination were performed. The following data were collected
and reported on a standardized questionnaire: prenatal, perinatal and postnatal
past histories, age at onset of spasms, age at diagnosis, semiology of
spasms, psychomotor development, the EEG and CT aspects and the evolutive modes
of WS under treatment. Psychomotor development was assessed using theDenverdevelopmental
screening test (DDST) which assesses the mental age compared to chronological
age. Results: The age of onset of spasms varied between 1 and 16 months
with a mean of 4.69 (±1.98) months. Males were highly represented with a sex
ratio of 1.72. Flexion spasms were the most common clinical presentation
(79.41%). 82.83% of the patients had a global developmental delay on the onset
of spasms. Structural causes or symptomatic West syndrome was the most frequent
presentation (77.94%). Perinatal aetiologies were highly represented (73.58%)
with the main cause being neonatal asphyxia (55.88%). A hypsarrythmic tracing was found on the electroencephalogram (EEG) in 73.53% of cases. The most frequent CT abnormality was
cortico-subcortical atrophy (38.24%). At the end of our study, global
developmental delay persisted in 89.72%. Conclusion: The main aetiologies of West syndrome in our context are the sequelae of
neonatal asphyxia and viral embryofoetopathies. There is a high
incidence of associated global developmental delay. More prevention methods on
risk factors for foetal distress and proper monitoring of deliveries to
minimize severe neonatal asphyxia are indispensable.