[MS8-3D-4] Epidemiology and response to treatment of West syndrome: Report from the Sri Lanka Infantile Spasm Registry (SLISR)
In spite of its first description about 175 years ago, West syndrome is still inadequately understood and researched. Prospective studies are difficult due to its rare nature, hence clinical registries are useful to describe important data on response to therapy and outcome. Sri Lanka Infantile Spasm Registry was launched to obtain prospective details about demographical features and treatment outcomes of West Syndrome.
Total number of entries at present is 183. A male predominance of 63% is observed. Mean age of spasm onset is 6 months. Spasms were the first epilepsy 82.5%. They presented with single spasms only in 24%, single spasms and clusters in 36.5%
First line therapy in a majority was oral prednisolone in 56.28%. The prescribed dose ranged between moderate to high dose (4-6mg/kg/day). Second most common medication was intramuscular ACTH in 27%) administered as a low dose regime (20-30 iu/day). The third most frequently was Sodium Valproate 13 (7.1%)
The response to therapy was considered as cessation of spasms for more than 48 hours at end of 14 days. The response rate to oral prednisolone was 48.07% and to ACTH was 26%. Response to other anticonvulsants (valproate, clonazepam) was 19.23%. No one was offered pyridoxime as first line therapy. Relapse of spasms was reported by 29% after initial response to first line therapy.
These findings indicate that West syndrome presents as first presentation with epilepsy around 6 months of age. Satisfactory initial response is seen to hormonal therapies. Subsequent relapses occur in about 30 percent.
Total number of entries at present is 183. A male predominance of 63% is observed. Mean age of spasm onset is 6 months. Spasms were the first epilepsy 82.5%. They presented with single spasms only in 24%, single spasms and clusters in 36.5%
First line therapy in a majority was oral prednisolone in 56.28%. The prescribed dose ranged between moderate to high dose (4-6mg/kg/day). Second most common medication was intramuscular ACTH in 27%) administered as a low dose regime (20-30 iu/day). The third most frequently was Sodium Valproate 13 (7.1%)
The response to therapy was considered as cessation of spasms for more than 48 hours at end of 14 days. The response rate to oral prednisolone was 48.07% and to ACTH was 26%. Response to other anticonvulsants (valproate, clonazepam) was 19.23%. No one was offered pyridoxime as first line therapy. Relapse of spasms was reported by 29% after initial response to first line therapy.
These findings indicate that West syndrome presents as first presentation with epilepsy around 6 months of age. Satisfactory initial response is seen to hormonal therapies. Subsequent relapses occur in about 30 percent.