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ABSTRACT
The purpose of this report is to review the efficacy and safety of vigabatrin in the treatment of infantile spasms in infants suffering from tuberous sclerosis complex. We reviewed all studies published in the English-language literature investigating the use of vigabatrin in the treatment of infantile spasms. Ten studies gave results for the efficacy of vigabatrin in infantile spasms for infants both with and without underlying diagnoses of tuberous sclerosis. Of the 313 patients without tuberous sclerosis complex, 170 (54%) had complete cessation of their infantile spasms; of the 77 patients with tuberous sclerosis complex, 73 (95%) had complete cessation of their seizures. We conclude that vigabatrin should be considered as first-line monotherapy for the treatment of infantile spasms in infants with either a confirmed diagnosis of tuberous sclerosis or those at high risk, ie, those with a first-degree relative with tuberous sclerosis complex. Paradoxically, in those without tuberous sclerosis complex, vigabatrin might be less efficacious than suggested by studies including patients with tuberous sclerosis complex. (J Child Neurol 1999;14:71-74).
Tuberous sclerosis complex is an inherited condition with an incidence of about 1 in 10,000, characterized by skin lesions, epilepsy, and mental retardation.l The epilepsy is often refractory to treatment. Infantile spasms are seen in about half of those infants with tuberous sclerosis with seizure onset in the first year of life, but partial and other seizures also occur. Conversely, it is estimated that approximately 10% of unselected children with infantile spasms will have an underlying diagnosis of tuberous sclerosis.2
Infantile spasms are a clinical seizure type that includes a unique type of seizure and a high risk of psychomotor retardation, usually with a distinctive electroencephalogram (EEG) pattern known as hypsarrhythmia.
Although the improvement of diagnostic techniques has led to better recognition of the underlying causes and pathology of infantile spasms, treatment has remained problematic. Before the advent of vigabatrin, either adrenocorticotropic hormone or prednisone/prednisolone seems to have been the most effective treatment for infantile spasms. Nevertheless, abolition of seizures has been attained only in two thirds of patients at best and the long-term prognosis is appalling. Severe learning disability occurs in 80% to 90% of patients.3
Recently, a new generation of anticonvulsants has appeared: lamotrigine, gabapentin, and vigabatrin. Vigabatrin rapidly...