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[Therapy of infantile spasms (West syndrome) with sodium dipropylacetate]
Insights
Sodium Dipropylacetic acid (DPK) effectively reduced infantile spasms in 40% of idiopathic cases and 11% of secondary cases. Long-term follow-up showed persistent seizures in some patients, with mental retardation noted in both groups.
Area of Science:
- Pediatric Neurology
- Clinical Pharmacology
Context:
- Infantile spasms syndrome (ISS) is a severe epilepsy syndrome in infants.
- Treatment options for ISS include anticonvulsants and hormonal therapy.
Purpose:
- To evaluate the efficacy and long-term outcomes of Sodium Dipropylacetic acid (DPK) in treating infantile spasms.
- To compare treatment responses based on etiology (idiopathic vs. secondary ISS).
Summary:
- 42 patients with infantile spasms were treated with DPK, divided into idiopathic and secondary groups.
- DPK showed varying efficacy: 40% seizure-free in idiopathic, 11.1% in secondary. Long-term follow-up revealed persistent seizures in 18.18% (idiopathic) and 28.57% (secondary).
- Mental retardation was observed in 36.36% of idiopathic and 57.14% of secondary cases.
Impact:
- DPK demonstrates significant efficacy in managing infantile spasms, particularly in idiopathic cases.
- The study highlights the importance of considering etiology for treatment strategies in infantile spasms.
- Findings support the use of anticonvulsants as a first-line treatment for ISS, with hormonal therapy (ACTH) reserved for refractory cases.
Abstract:
The authors report the results obtained in 42 patients affected by infantile spasms syndrome during treatment with Sodium Dipropylacetic acid. The subjects were divided into two groups according to the aetiology: idiopathic and secondary. In the first group the use of DPK as determined the disappearance of the seizures in 6 cases (40%), reduction of the crises beyond 50% in 7 cases (46.6%), while in 2 subjects (13.3%) the crises persisted. In the secondary group the crises ceased in 3 cases (11.1%), in 17 (62.9%) there were a reduction of the crises beyond 50%, no response to the drug was observed in 7 subjects (25.9%). In 10 patients the anticonvulsant treatment was progressively diminished and was substituted with hormonal treatment. The long term follow up (1-6 years) gives the following results: the seizures persisted in 2 cases (18.18%) among the idiopathic form and in 6 cases (28.57%) among the secondary group. Mental retardation was found in 4 subjects (36.36%) among the idiopathic group and in 12 patients (57.14%) among the secondary group. The authors shortly report the side effect of the hormonal treatment: they prefer the initiation of treatment of I.S. with anticonvulsant drug and suggest to resort to the ACTH when the initial treatment is unsuccessful.