Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UNIPROT:P01189 (beta-endorphin)
 21,003 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Nine infants with an underlying static encephalopathy (confirmed as cerebral palsy in a later follow-up examination) and newly diagnosed infantile spasms were entered in an open study with adrenocorticotropic hormone (ACTH) and vigabatrin as the initial therapy regimen. The ACTH was discontinued after 4-6 weeks and the infants were maintained on vigabatrin alone. Following an initial response with complete suppression of spasms in all patients, a long term response maintained for a mean of 19.2 months was confirmed in all but one child. Tolerability appeared excellent with 7 of 9 patients reporting no side effects; vigabatrin related hypotonia presented in all patients and turned out to be a 'positive' side-effect on the abnormally increased muscle tone of these infants. Given the very poor prognosis of infantile spasms especially in such conditions as cerebral palsy, the combination of ACTH and vigabatrin appears to be an interesting therapy advance with very few side effects.
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PMID:Adrenocorticotropic hormone and vigabatrin treatment of children with infantile spasms underlying cerebral palsy. 898 Aug 41

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.
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PMID:Long-term outcomes of conventional therapy for infantile spasms. 920 48