Low Dose and Long-term Vigabatrin Treatment in Tuberous Sclerosis Complex Children with Infantile Epileptic Spasm Syndrome
Abstract number :
1.421
Submission category :
7. Anti-seizure Medications / 7D. Drug Side Effects
Year :
2024
Submission ID :
1275
Source :
www.aesnet.org
Presentation date :
12/7/2024 12:00:00 AM
Published date :
Authors :
Presenting Author: Yuki Ueda, MD, PhD – Hokkaido University Hoppital
Midori Nakajima, MD.PhD – Hokkaido University Hospital
Sachiko Nakakubo, MD, PhD – Hokkaido University Hospital
Takeru Goto, MD – Hokkaido University Hospital
Yasuyoshi Hiramatsu, MD – Hokkaido University Hospital
Satoshi Yamada, MD – Hokkaido University Hospital
Kiyoshi Egawa, PhD – Hokkaido University
Rationale:
While Vigabatrin (VGB) is recommended as the first line treatment for epileptic spasms (ES) occurring in tuberous sclerosis complex (TSC) patients, its long-term administration is not generally recommended because of concerns for irreversible visual impairment. Nevertheless, long-term administration is practically chosen in some cases due to recurrence of ES or other types of seizures.
Methods: TSC children who had been continuing VGB administration more than two years were recruited from medical records in Hokkaido University Hospital. Four individuals were included. Median current age was 3.3 years-old (2.7 -7.5). We analyzed their characteristics based on clinical data and video electroencephalogram recording. To monitor ophthalmological adverse effects of VGB, ophthalmological examination including electroretinogram (ERG) were conducted every 3 to 6 months.
Results: In all patients VGB induced cessation of ES. The initial doses of VGB ranged from 50 to 100 mg/kg/day. Over a period of 12 months, we gradually deceased VGB doses. While ES did not relapse in all four patients, they had focal onset seizure or electrographic seizure. One patient developed acute encephalopathy with status epilepticus, but fortunately recovered without severe neurological impairment. Another one patient underwent epilepsy surgery, which resulted in the cessation of seizure. Low dose VGB administration was continued while other antiepileptic medications were added (median current dose: 17.9 mg/kg/day; median cumulative dose: 580 g; median duration: 1029 days). Ophthalmological examination including ERG showed no apparent abnormalities. No other severe adverse events (AEs), including changes in the brain magnetic resonance imaging, occurred.
Conclusions:
VGB is assumed to have the potential to suppress focal seizure as well as ES. Some TSC patients with infantile epileptic spasm syndrome have benefit to continuing VGB to control disabling seizure. Low dose treatment decreases cumulative dose which may contribute to avoid severe AEs.
Funding: No funding was received in support of this abstract.
Anti-seizure Medications