Landau-Kleffner Syndrome and Sulthiame

Wakai, Shuji; Ito, Nobumasa; Ueda, Denis Massatsugu; Chiba, Shunzo

doi:10.1055/s-2007-973689

Cited by 20 publications

(6 citation statements)

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“…In addition, CBZ may have the disadvantage of worsening EEG characteristics and precipitating of epilepsy with continuous spikes and waves during slow sleep (CSWS) in a considerable number of patients (3,4,(9)(10)(11)(12)(13), although this influence is controversial (14). Early observations showed positive effects of STM treatment on the interictal EEG in BECTS (2,11,15). Of 58 patients treated with STM, 51 showed initial normalization, which turned out to have been transient in 19 of them (2).…”

Section: Discussionmentioning

confidence: 99%

The Influence of Sulthiame on EEG in Children with Benign Childhood Epilepsy with Centrotemporal Spikes (BECTS)

Bast

Völp²,

Wolf

et al. 2003

Epilepsia

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Summary:Purpose: To evaluate the effects of sulthiame (Ospolot; STM) monotherapy compared with placebo on the EEG in children with benign childhood epilepsy with centrotemporal spikes (BECTS).Methods: Sixty-six patients (aged 3-11 years) entered a 6-month double-blind trial and were randomized to either STM (n ‫ס‬ 31) or placebo (n ‫ס‬ 35). Clinical data and general results have been reported elsewhere (1). One-hundred seventy-nine sleep EEGs were recorded at screening and after 4 weeks, 3 months, and 6 months. EEGs were analyzed by a blind reviewer using a standard protocol for each EEG. This standard protocol collected data on general changes, specific epileptiform, and nonspecific focal and generalized changes. A classification system was defined depending on rating of pathologic EEG changes. Because of the higher number of treatment-failure events (i.e., seizures) in the placebo group, there was an increasing imbalance between the two groups regarding the number of recorded sleep EEGs over time (STM, 104; placebo, 74). A Wilcoxon-Mann-Whitney U test was used to describe differences in the grade of pathology during individual follow-up between the two groups.Results: The sleep-EEG was found to be normalized in 21 patients treated with STM (12/21 transient) and in five patients treated with placebo (4/5 transient). In the STM group, the EEG showed a marked improvement during intraindividual course when comparing the classification of follow-up EEGs at each time point with the screening EEG. Comparable improvements were not observed in the placebo group (exact two-tailed p value at 4 weeks, p < 0.0001; at 3 months, p ‫ס‬ 0.0010; and at 6 months, p < 0.0001).Conclusions: STM had marked effects on the EEG in BECTS, which led to normalization in the majority of the patients. Most of those whose EEGs were not normalized showed improvement in the grade of EEG pathology. Normalization persisted in >50% of patients during the investigation. Spontaneous normalization in the placebo group reflects the wide spectrum of individual courses, which must be considered when analyzing drug effects on EEG in BECTS.

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Section: Discussionmentioning

confidence: 99%

The Influence of Sulthiame on EEG in Children with Benign Childhood Epilepsy with Centrotemporal Spikes (BECTS)

Bast

Völp²,

Wolf

et al. 2003

Epilepsia

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“…In a single case report, they showed that sulthiame after 1 month of therapy had a positive effect both on EEG and language difficulties (Wirrel et al., 2006). Another single case report showing a positive effect of sulthiame was already published in 1997 (Wakai et al., 1997). The more recent report of Bast furthermore showed that sulthiame had a positive effect on different EEG parameters (Bast et al., 2003).…”

Section: Old Versus Newer Antiepileptic Drugs In Landau‐kleffner Syndmentioning

confidence: 95%

Rational treatment options with AEDs and ketogenic diet in Landau‐Kleffner syndrome: Still waiting after all these years

Lagae

2009

Epilepsia

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Summary Antiepileptic drugs (AEDs) remain a first treatment approach in Landau‐Kleffner syndrome (LKS) and related syndromes. In the current literature, only class IV evidence is available. Inclusion criteria and outcome parameters are ill‐defined. Most commonly, valproate, ethosuximide, and/or benzodiazepines are used. More recent case series show that sulthiame and especially levetiracetam can be considered as effective drugs. Smaller studies also point to the ketogenic diet as a valuable treatment option in LKS.

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“…There could be seen various pharmacological treatments and surgical approaches in literature but still there is not any certain approach (20)(21)(22)(23)(24)(25)(26)(27). Although Bharni and colleagues reported total recovery of clinical and EEG findings in a 8-years old case who was diagnosed as LKS and treated with 30 mg/kg valproic acid; reports could be found in literature which state inefficacy of anti-epileptic drugs (20,23,24,28).…”

Section: Discussionmentioning

confidence: 99%

Landau-Kleffner Syndrome: Case Report

Baytunca¹,

Percinel²,

Tekin³

et al. 2015

jpr

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Landau-Kleffner syndrome (LKS) is acquired aphasia circumstance with loss of gained language ability and epileptiform electroencephalography or clinical seizure described first in 1957 by Landau and Kleffner. Expressive aphasia starts commonly among children of 3 to 8 age group and verbal auditor agnosia are other clinical features of LKS. Patients have difficulty in understanding what they are being told and respond to visual as well as auditory arousals. Seizure is seen in 70% of the cases. Patients with LKS usually develop normally, but it has been reported that about 13% of the cases had speech ability problem previously. Due to resemblance of symptomatology, causals such as audition loss, deafness, psychiatric disorders, progressive encephalopathy, "Childhood Rolandic Epilepsy" should be considered as differential diagnoses of LKS. We have briefly discussed the clinical progress of an 8-year-old male child who was followed with prediagnosis of major depression by child psychiatry department because of agression, relationship problems with his friends and regression in his speech. His behavior problems and absence of seizures had delayed obtaining an EEG in the early period. Seizures started two years after the behavior problems and loss of speech. Thus, we suggest that if a child with normal language development starts to regress, Landau-Kleffner syndrome must be considered in the differential diagnosis even in the absence of epileptic seizures.

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Landau-Kleffner Syndrome and Sulthiame

Cited by 20 publications

References 0 publications

The Influence of Sulthiame on EEG in Children with Benign Childhood Epilepsy with Centrotemporal Spikes (BECTS)

The Influence of Sulthiame on EEG in Children with Benign Childhood Epilepsy with Centrotemporal Spikes (BECTS)

Rational treatment options with AEDs and ketogenic diet in Landau‐Kleffner syndrome: Still waiting after all these years

Landau-Kleffner Syndrome: Case Report

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