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Long-term outcomes of conventional therapy for infantile spasms
K R Holden1, S L Clarke, D A Griesemer
1Department of Neurology, Medical University of South Carolina, Charleston, USA.
Insights
Infantile spasms (IS) treatment often reduces seizures but rarely improves long-term development. Current therapies are inadequate, highlighting the need for innovative infantile spasms treatments.
Area of Science:
- Pediatrics
- Neurology
- Epileptology
Background:
- Infantile spasms (IS) is an age-specific epilepsy with a generally poor psychomotor development prognosis.
- Prompt treatment initiation and cryptogenic etiology are thought to improve outcomes.
Purpose of the Study:
- To retrospectively review infantile spasms (IS) cases treated with adrenocorticotropic hormone (ACTH) or valproic acid (VPA).
- To correlate therapeutic response with long-term psychomotor development outcomes.
Main Methods:
- Retrospective review of 28 infants with IS treated between 1990 and 1996.
- Treatment included adrenocorticotropic hormone (ACTH), valproic acid (VPA), or combination therapy.
- Outcomes assessed based on seizure reduction, remission, mortality, and long-term neurodevelopmental status.
Main Results:
- The majority of infants showed significant spasm reduction (>75%) with ACTH or VPA; 52% achieved total seizure remission.
- Despite initial treatment success, all subgroups experienced poor long-term outcomes, including residual epilepsy, cerebral palsy, and mental retardation.
- Eight patients died; mean follow-up for survivors was 55 months.
Conclusions:
- Conventional treatments for infantile spasms (IS), even when effective in reducing seizures, are insufficient for ensuring normal long-term psychomotor development.
- Novel and innovative therapeutic strategies are urgently needed for infantile spasms (IS) to improve developmental outcomes.
Abstract:
Infantile spasms (IS) is an age-specific epilepsy which responds to anticonvulsant therapy but has a generally poor prognosis for normal psychomotor development. The subgroup of infants with a cryptogenic aetiology or whose therapy is initiated promptly is thought to have a more favourable prognosis. We retrospectively reviewed 28 infants with IS treated between 1990 and 1996 with adrenocorticotropic hormone (ACTH), valproic acid (VPA), or both, in order to correlate therapeutic response with long-term outcome. Mean age at onset of treatment was 6.4 months, with 57% of patients started within 1 month of IS appearance. IS was considered cryptogenic in 39%. The majority of infants responded to ACTH or VPA with a reduction in spasms of 75% or more. Total remission of seizures occurred in 52%. Death occurred in eight patients; mean duration of follow-up for survivors was 55 months. All subgroups based on age, aetiology, or treatment had poor outcomes, commonly with residual epilepsy, cerebral palsy or mental retardation. Conventional treatment for IS, even when initially successful in reducing spasms, is inadequate when viewed from a long-term developmental perspective, suggesting the need for novel innovative approaches for treating IS.