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.
Keywords
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References
- Vigabatrin.Epilepsia. 1999; 40: S11-S16
- Vigabatrin and GABA concentration inter-relationship in rat extracellular fluid (EDF) from frontal cortex and hippocampus.Epilepsia. 2000; 41: 95
- Vigabatrin for refractory partial seizures in children with tuberous sclerosis.Neuropediatrics. 1994; 25: 55-56
- MRI in Bourneville disease: relationship with EEG findings.Neurophysiol Clin. 1988; 18: 149-157
- Neurochemical profile of glioneuronal lesions from patients with pharmacoresistant focal epilepsies.J Neuropathol Exp Neurol. 1995; 54: 689-697
- New developments in the neurobiology of the tuberous sclerosis complex.Neurology. 1999; 53: 1384-1390
- GABA and epileptogenesis.Epilepsia. 1997; 38: 399-407
- Physiological modulation of GABA(A) receptor plasticity by progesterone metabolites.Eur J Pharmacol. 1999; 375: 225-235
- Modulation of GABA(A) receptor function by neuroactive steroids: evidence for heterogeneity of steroid sensitivity of recombinant GABA(A) receptor isoforms.Can J Physiol Pharmacol. 1998; 76: 909-920
- Selective alterations in glutamate and GABA receptor subunit mRNA expression in dysplastic neurons and giant cells of cortical tubers.Ann Neurol. 2001; 49: 67-78
- GABA(A) receptor alpha4 subunit suppression prevents withdrawal properties of an endogenous steroid.Nature. 1998; 392: 926-929
- Relationship between neurosteroid gamma-aminobutyric-acid A modulators and seizure frequency in epileptic children.Ann Neurol. 1998; 44: 565
- Therapeutic trial of Vigabatrin in refractory infantile spasms.J Child Neurol. 1991; 6: 2552-2559
- Vigabatrin as initial therapy for infantile spasms; a European retrospective survey.Epilepsia. 1996; 37: 638-642
- Mental and behavioural outcome of infantile epilepsy treated by vigabatrin in tuberous sclerosis patients.Epilepsy Res. 2000; 38: 151-160
- Vigabatrin in the treatment of infantile spasm in tuberous sclerosis: literature review.J Child Neurol. 1999; 14: 71-74
- Vigabatrin in infantile spasms.Lancet. 1990; 10: 363-364
- Vigabatrin in the management of generalized seizures in children.Seizure. 1995; 4: 45-48
- Vigabatrin in the treatment of infantile spasms.Neuropediatrics. 1993; 24: 230-231
- Vigabatrin in newly diagnosed infantile spasms.Neuropediatrics. 1994; 25: 54
- Vigabatrin versus ACTH in the treatment of infantile spasms.Epilepsia. 1995; 36: s265
- Randomized trial comparing vigabatrin and hydrocortisone in infantile spasms due to tuberous sclerosis.Epilepsy Res. 1997; 26: 389-395
- Vigabatrin (VGB) for infantile spasms: non-dose dependent response.Epilepsia. 2000; 41: 187
- Acute psychotic disorder associated with Vigabatrin.Acta Med Port. 2000; 13: 111-114
- Vigabatrin: a novel therapy for seizure disorders.Ann Pharmacother. 1999; 33: 1277-1286
- Severe persistent visual field constriction associated with vigabatrin.BMJ. 1997; 314: 180-181
- Concentric contraction of the visual field in patients with temporal lobe epilepsy using vigabatrin, long term results.Epilepsia. 2000; 41: 136
- Visual field changes secondary to Vigabatrin treatment.Rev Neurol. 2000; 31: 1104-1108
- Is visual impairment related to vigabatrin reversible?.Epilepsia. 1999; 40: 256
- Reversibility of vigabatrin-induced visual-field defect.Lancet. 1999; 354: 486
- Another case of reversibility of visual-field defect induced by vigabatrin monotherapy: is young age a favorable factor?.Neurol Sci. 2000; 21: 185-186
Article info
Publication history
Accepted:
May 24,
2001
Received:
May 21,
2001
Identification
Copyright
© 2001 Elsevier Science B.V. Published by Elsevier Inc. All rights reserved.
