Article

Vigabatrin for tuberous sclerosis complex.

Department of Neurosciences, Pediatric Neurology, Tor Vergata University, Via di Tor Vergata 135, 00133 Rome, Italy.
Brain and Development (impact factor: 2.12). 12/2001; 23(7):649-53. DOI:10.1016/S0387-7604(01)00290-X pp.649-53
Source: PubMed

ABSTRACT Vigabatrin (VGB) was found to be an effective anti-epileptic drug to reduce infantile spasms in about 50% of patients and it has been found most effective in infantile spasms due to tuberous sclerosis (TSC) in which up to 95% of infants had complete cessation of their spasms. VGB was synthesized to enhance inhibitory gamma-aminobutyric acidergic (GABAergic) transmission by elevating GABA levels via irreversible inhibition of GABA transaminase. The mechanism underlying the particular efficacy of VGB in TSC is still unknown. However, its efficacy suggests that epileptogenesis in TSC may be related to an impairment of GABAergic transmission. VGB should be considered as the first line monotheraphy for the treatment of infantile spasms in infants with confirmed diagnosis of TSC. The efficacy of VGB treatment can be assessed in less than 10 days, but usually a few days treatment with a dose of about 100 mg/kg/day stops infantile spasms. The cessation of the spasms is associated with a marked improvement of behaviour and mental development. Unfortunately, it has become clear that the use of VGB is associated with a late appearance of visual-field defects in up to 50% of patients. Currently the minimum duration and doses of VGB treatment that can produce side effects are unknown. The feasibility of using short treatment periods (2-3 months) should be investigated.

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    Article: Efficacy of Vigabatrin for Treatment of a Case of Infantile Spasms with Tuberous Sclerosis Complex in Taiwan
    Ann-Ching Wang, Ming-Chih Tsai, Chia-Hung Liu, Shyi-Jou Chen
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    ABSTRACT: Tuberous sclerosis complex (TSC) is an autosomal dominant neurocutaneous syndrome affecting multiple organ systems and featuring highly variable clinical manifestations. Epilepsy occurs in more than two-thirds of TSC victims, and up to one-third of TSC sufferers will develop infantile spasms (IS), a condition that is often medically intractable. Vigabatrin (VGB) has proved to be an effective antiepileptic drug for the treatment of IS resulting from TSC. A number of previous studies have indicated that earlier control of epilepsy contributes to a better prognosis for TSC-related mental retardation. To our knowledge, however, reports of treatment with VGB for IS for TSC victims are limited in Taiwan. Herein, we report on a case of IS associated with TSC that was effectively treated with VGB; successful treatment was defined as the patient achieving a complete recovery of electroencephalogram (EEG) recordings and being seizure free for at least six months. Moreover, we followed up this case for a period of more than one year from diagnosis, during which time the patient was seizure free and their psychomotor development remained within normal milestones.
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    Elizabeth J Donner, O Carter Snead
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    ABSTRACT: In the last 12 years, 10 new anticonvulsants have been approved by the U.S. Food and Drug Administration and, as a result, the treatment options for children and adults with epilepsy have been expanded considerably. These new generation antiepileptic drugs offer equal efficacy with improved tolerability, pharmacokinetic properties, and side effect profiles compared with the traditional drugs. With many new medications available, the clinician treating children with epilepsy must be well versed in the application of these drugs to their patient population. This manuscript will review the indications, mechanism of action, pharmacokinetics, adverse effects, and dosing of the new generation of anticonvulsant medications.
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Keywords

2-3 months
 
effective anti-epileptic drug
 
elevating GABA levels
 
epileptogenesis
 
first line monotheraphy
 
GABAergic
 
GABAergic transmission
 
infantile spasms
 
inhibitory gamma-aminobutyric acidergic
 
irreversible inhibition
 
marked improvement
 
mental development
 
minimum duration
 
patients
 
short treatment periods
 
TSC
 
tuberous sclerosis
 
VGB
 
VGB treatment
 
visual-field defects
 

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