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West syndrome: the Philippine experience
A M Salonga1, M B Lukban, M H Ortiz
1Department of Pediatrics, University of the Philippines-Philippine General Hospital, Taft Avenue, Manila, Philippines. rms_md@pacific.net.ph
Insights
West syndrome (WS) in the Philippines is often symptomatic and challenging to treat, with limited access to ideal therapies like ACTH. Most patients experience poor neurodevelopmental outcomes despite available treatments.
Area of Science:
- Pediatric Neurology
- Epilepsy Research
- Clinical Epidemiology
Background:
- West syndrome (WS) is a severe form of epilepsy in infancy.
- Understanding the current status and management of WS in the Philippines is crucial for improving patient outcomes.
Purpose of the Study:
- To investigate the current status of West syndrome in the Philippines.
- To analyze the etiologies, treatments, and outcomes of WS cases.
- To survey anticonvulsant usage among Filipino child neurologists.
Main Methods:
- Retrospective review of 12 WS cases from 1997-1999 at two major referral hospitals.
- Questionnaire survey on anticonvulsant use among practicing child neurologists.
Main Results:
- WS accounted for 3.18% of epileptic children under 3. Etiologies were 33% idiopathic and 66% symptomatic.
- Phenobarbital was the most common first-line drug; however, only 3 of 12 patients became seizure-free with poor neurodevelopmental outcomes.
- Adrenocorticotrophic hormone (ACTH) was the preferred ideal drug, but its unavailability and cost limited its use.
Conclusions:
- Data from referral centers may not represent true national prevalence of WS.
- Limited access to preferred treatments like ACTH impacts seizure control and developmental outcomes.
- Further national statistics and accessible treatment options are needed for effective WS management in the Philippines.
Aim:
To provide information on the current status of West syndrome (WS) in the Philippines.
Methods:
This is a retrospective review of WS cases from January 1997 to December 1999 from two largest referral government institutions. A questionnaire interview survey on anticonvulsant usage was also conducted among practicing child neurologists.
Results:
Twelve patients diagnosed to have infantile spasms at 2-15 months were included, with a male:female ratio of 1:1. The proportion of WS cases among epileptic children under age 3 was 3.18%. The etiologies were idiopathic/cryptogenic in four (33%) and symptomatic in eight (66%). Symptomatic cases include hypoxic-ischemic encephalopathy, neonatal sepsis, bacterial meningitis, inborn error of metabolism, congenital brain anomaly and intracranial hemorrhage. Phenobarbital was the first line drug in 75% of cases. Other drugs used were valproic acid, clonazepam and pyridoxine. With a follow-up duration of 1-40 months, only three patients became seizure free and most had poor neurodevelopmental outcome. Among practicing child neurologists, the preferred ideal drug was adrenocorticotrophic hormone (ACTH) and valproic acid for idiopathic and symptomatic cases, respectively. However, in actual clinical practice valproic acid or prednisone was the initial drug used. Pyridoxine was usually added on.
Conclusions:
The proportion of WS in our patient population may not reflect the true prevalence in our country since our data came from a biased population, i.e. referral centers. A national statistics is currently not available. ACTH, which was perceived by most child neurologists as the ideal first line drug was not used primarily because it is unavailable and unaffordable. The poor seizure control and developmental outcome may be due to the treatment given or directly related to the etiology of WS.