Successful use of intravenous immunoglobulins in Landau-Kleffner syndrome

Lagae, Lieven; Silberstein, Jon; Gillis, Philippe; Casaer, Paul

doi:10.1016/s0887-8994(97)00157-4

Cited by 65 publications

(32 citation statements)

References 23 publications

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“…Based on rather limited evidence, corticosteroids are currently considered the most effective treatment modality for this disorder. Intavenous gamma-globulins have also been anecdotally reported to be of benefit [Fayad et al, 1997;Lagae et al, 1998]. More recently, surgery using subpial transections has been advocated by some [Morrell et al, 1995].…”

Section: Landau-kleffner Syndromementioning

confidence: 99%

Landau–Kleffner syndrome, electrical status epilepticus in slow wave sleep, and language regression in children

McVicar

Shinnar

2004

Ment. Retard. Dev. Disabil. Res. Rev.

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The Landau-Kleffner syndrome (LKS) and electrical status epilepticus in slow wave sleep (ESES) are rare childhood-onset epileptic encephalopathies in which loss of language skills occurs in the context of an epileptiform EEG activated in sleep. Although in LKS the loss of function is limited to language, in ESES there is a wider spectrum of cognitive impairment. The two syndromes are distinct but have some overlap. The relationship between the epileptiform EEG abnormalities and the loss of cognitive function remains controversial, even in LKS which is the most widely accepted as an acquired epileptic aphasia. Language regression also occurs in younger children, frequently in the context of a more global autistic regression. Many of these children have epileptiform EEGs. The term autistic regression with epileptiform EEG has been proposed for these children. Whether these children are part of an extended LKS spectrum is very controversial, because there are differences in age of onset, clinical phenotype, and EEG findings. An understanding of the available data on clinical characteristics, EEG findings, pathology, prognosis, and treatment of these syndromes is essential for further progress in this area.

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Section: Landau-kleffner Syndromementioning

confidence: 99%

Landau–Kleffner syndrome, electrical status epilepticus in slow wave sleep, and language regression in children

McVicar

Shinnar

2004

Ment. Retard. Dev. Disabil. Res. Rev.

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“…Once the diagnosis of LKS is made, medical treatment must be initiated as this appears to improve long-term prognosis [12][13][14]. Despite the number of patients reported in the past 10 years with this syndrome there is no consensus in the literature regarding medical treatment.…”

Section: Discussionmentioning

confidence: 99%

Landau–Kleffner syndrome: A rare Auditory Processing Disorder

Fandiño

Connolly

Usher

et al. 2011

International Journal of Pediatric Otorhinolaryngology

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“…High-dose diazepam was also reported to be effective in some studies (32,33). Intravenous immunoglobulin also has been found successful in arresting LKS (34,35). Taken together, it appears that controlled studies are needed for evidence-based pharmacotherapy alghoritms.…”

Section: Treatmentmentioning

confidence: 96%

“…Case reports have suggested that intravenous gammaglobulin may benefit some children with ESES (35,60,63). Surgery is also suggested for the resistant cases to medication options.…”

Section: Treatmentmentioning

confidence: 99%

Landau kleffner syndrome, electrical status epilepticus in sleep and autistic regression: an overview of literature / Landau kleffner sendromu, uykuda elektriksel status epileptikus ve otistik regresyon: Literatüre bir bakış

Ekinci¹,

Işık²,

Melek³

2012

Dusunen Adam

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Landau kleffner syndrome, electrical status epilepticus in sleep and autistic regression: an overview of literature Language regression in children is a challenging condition for clinicians since the diagnostic and therapeutic algorithms are not well established. Landau Kleffner syndrome (LKS), Electrical Status Epilepticus in Sleep (ESES) and Autistic Regression (AR) are three rare clinical entities in which langauage regression is present. LKS and ESES appear to be two points on the spectrum of epileptic syndromes characterized by severe paroxysmal EEG disturbance in sleep and regression. However, the available literature indicates that AR has many distinct features than LKS and ESES. For all of these three conditions, early recognition and treatment is crucial for favorable outcomes. Overnight EEG has an important diagnostic role for LKS and ESES. In this review, the clinical features, prognosis and treatment options of LKS, ESES and AR will be discussed.

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Successful use of intravenous immunoglobulins in Landau-Kleffner syndrome

Cited by 65 publications

References 23 publications

Landau–Kleffner syndrome, electrical status epilepticus in slow wave sleep, and language regression in children

Landau–Kleffner syndrome, electrical status epilepticus in slow wave sleep, and language regression in children

Landau–Kleffner syndrome: A rare Auditory Processing Disorder

Landau kleffner syndrome, electrical status epilepticus in sleep and autistic regression: an overview of literature / Landau kleffner sendromu, uykuda elektriksel status epileptikus ve otistik regresyon: Literatüre bir bakış

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