Lennox-Gastaut sendromlu hastaların klinik özellikleri ve uzun süreli seyri

Abstract

Aim:The aim of this study was to retrospectively evaluate clinical features, treatment and prognosis of 20 patients followed up as Lennox-Gastaut Syndrome in ESOGU in the Division of Pediatric Neurology. Material and Method: Multiple different types of seizure, diffuse spike-wave pattern(<3 Hz) on electroencephalogram, psychomotor retardation were the diagnostic criteria for Lennox-Gastaut Syndrome. The patients who had no etiologic reason were described as the idiopathic type. Prenatal, natal and postnatal risk factors, seizure and clinical features, neurodevelopmental status, treatment and prognostic features were evaluated. Results: Thirteen patients were female, seven were male; male/female ratio was 1,9. Nineteen patients were described as the symptomatic type. Hypoxic ischemic encephalopthy was the most common risk factor. Sixty percent of the patients had a history of infantile spasms. Generalized tonic, generalized tonic clonic and atonic drop attacks were the most common seizure types. Speech impairment and microcephaly were the most frequently seen neurologic findings. Diffuse spike-wave activity (1-3 Hz/s) on EEG, bioelectrical status epilepticus and 9-14 Hz/s fast activity discharges were the most commonly seen discharges. The most common neuroimaging finding was cerebral atrophy. Sixty percent of the patients suffered from a large number of seizures per day despite multiple antiepileptic drugs. The most commonly used antiepileptic drugs were valproate sodium, clobazam, lamotrigine and topiramate. Conclusions: The prognosis of Lennox-Gestaut Syndrome is poor due to either recurrent seizures or mental deterioration. Seizure control can not be provided despite the use of multiple anti-epileptic drugs. New antiepileptic drugs are needed. (Turk Arch Ped 2012; 47: 49-54)

A comprehensive systematic literature review of the burden of illness of Lennox–Gastaut syndrome on patients, caregivers, and society

“…Seizure frequency ranged widely within and across studies, which may be partly owing to interindividual heterogeneity; in one study, baseline seizure frequency was 15–3000 seizures per month 46,51 . These findings aligned with those from studies in which individuals were categorized by seizure frequency; 50%–100% of individuals were reported to have daily seizures, and those without daily seizures were usually reported to have seizures at least once per week 18,20,23,26,28,34–36,52–58 …”

“…28,[37][38][39][40] These data were generally aligned with those from studies that reported the proportion of individuals experiencing different numbers of seizure types (Figure 3). 20,21,[26][27][28][29][30][31][32][33][34][35][36] One study reported a high prevalence of individuals with one seizure type (38%); however, this study used historical records of seizure types experienced over 14 years. 29 Four studies reported a decrease in the number of seizure types experienced per individual during the study, which may reflect changes with age.…”

“…46,51 These findings aligned with those from studies in which individuals were categorized by seizure frequency; 50%-100% of individuals were reported to have daily seizures, and those without daily seizures were usually reported to have seizures at least once per week. 18,20,23,26,28,[34][35][36][52][53][54][55][56][57][58] Several prospective and retrospective studies showed decreases in seizure frequency over time, reporting similar ranges of seizure freedom prevalence at last visit (9%-28% and 4%-28%, respectively). 17,20,34,38,43,47,[59][60][61][62][63][64][65][66][67] Although the duration of data collection varied considerably between studies (3-20 years), no correlation between the change in seizure frequency and the duration of data collection was observed.…”

Band Heterotopia and Lennox Gastaut Syndrome: A Case Report

“…İdiyopatik LGS olgularında seyir daha iyidir. [14] Lennox-Gastaut sendromu etiyolojisinde saptanan olgularda bant ne kadar kalın ise prognozun o kadar ağır olduğu bilinmektedir. [4] Hastamızda kraniyal MRG'de bant heterotopi ile uyumlu olarak korteks ile ventrikül arasında iyi sınırlı gri madde ile izointens sinyal değişikliği izlendi.…”

“…Erken çocukluk döneminde başlayan birden fazla değişik tip nöbet, bilişsel işlevlerde bozulma ve EEG de yaygın yavaş diken-dalga (1-3 Hz/sn) izlenir. [14] Bizim hastamızın EEG'sinde 2-2.5 hz diken dalga, multipl diken ve hızlı ritmler görüldü.…”