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2.1
Acquired Epileptic Aphasia
Epileptic Encephalopathy with Continuous Spike-and-Wave during Slow-Wave Sleep

Presentation

The goal of this study was to verify if any cognitive and learning ability differences are present between patients affected by learning disorders with and without epilepsy and CSWS. [moh-it.pure.elsevier.com]

The survey presented a clinical vignette of CSWS. The questions addressed treatment choices for that clinical scenario. Surveys were self-administered and collected using an online survey website (www.surveymonkey.com). [scholars.northwestern.edu]

Children may present with focal clonic, generalized tonic-clonic, and absence seizures, though as many as 80% of children present with only one seizure type. [genome.jp]

It also presents a wide-ranging collection of cases presented by the participants in the meeting, and analysed in its various clinical, electrophysiological and psycho-intellectual aspects. [booktopia.com.au]

Forty-two of the 137 (31%) patients presented epilepsy.... Epileptic Encephalopathies: An Overview. [connection.ebscohost.com]

Neurologic

  • Aphasia

    However, these have typically had little to no effect on the long-term development of aphasia. [docneuro.com]

    […] outcome of CSWS syndrome p. 27 'Acquired epileptic aphasia' (AEA): definition of the syndrome and current problems p. 37 Evolution of acquired epileptic aphasia with or without continuous spikes and waves during slow sleep p. 47 The eponym 'Landau-Kleffner [booktopia.com.au]

    As the rolandic region is part of the brain regions involved in speech production, acquired aphasia, i.e. loss of speech, can be a prominent feature in some patients. [euroepinomics.wordpress.com]

    Symptoms - Continuous spike-wave during slow sleep syndrome * Impaired cognitive function * Behavioural problems * Language problems * Impaired attention span * Impaired constructive ability * Aphasia * Epilepsy * Unilateral seizures * Generalized motor [checkorphan.org]

  • Expressive Aphasia

    At age 7 years 3 months, he developed difficulties with verbal com- prehension followed by an expressive aphasia. At age 8, he was nearly mute, did not react to sound, but did understand instructions given by gestures. [docslide.com.br]

Treatment

Treatment for continuous spikes and waves during sleep (CSWS): Survey on treatment choices in North America. [scholars.northwestern.edu]

Treatment modalities Until recently treatment focused on seizure control using anti-epileptic drugs. However, these have typically had little to no effect on the long-term development of aphasia. [docneuro.com]

Treatment There is no international consensus regarding treatment of Landau-Kleffner syndrome. Rapid initiation of drug therapy has proven to be important for the prognosis ( 9 ). [tidsskriftet.no]

Treatment of CSWS There are no controlled trials for the treatment of CSWS, only large cohort studies. [acnr.co.uk]

ESES disappeared after OXC withdrawal and treatment with valproate. [functionalneurology.com]

Prognosis

The prognosis for the aphasia varies. [tidsskriftet.no]

Management options* Seizures are not a major problem as their final prognosis is good. Valproate, lamotrigine, levetiracetam and sulthiame# may be the most appropriate treatments. [epilepsy.com]

Prognosis The prognosis is related to the underlying disorder. The severity of developmental impairment varies with the type of epilepsy. Early infantile epileptic encephalopathy (Ohtahara syndrome) The prognosis is very poor. [emedicine.medscape.com]

The long-term prognosis of this condition is overall poor owing to the persistence of neuropsychological impairment. Therefore, early recognition and effective therapy are necessary to improve long-term prognosis. [link.springer.com]

A long duration of continuous spike and waves during slow wave sleep is the major factor of a poor prognosis. The benign atypical partial epilepsy syndrome bears a close relation to continuous spike and waves during slow wave sleep. [neuroscienceandgenetics.it]

Etiology

Rett syndrome Metabolic etiologies (e.g. mitochondrial disorders) [epilepsydiagnosis.org]

The developmental prognosis partially depends on the etiology. When classified by etiology, normal development was described in 51% of cryptogenic cases versus only 6% of symptomatic cases. [emedicine.medscape.com]

The author describes the aspects of classification, pathogenesis and etiology, prevalence, clinical picture and diagnostics of this disorder, including the peculiar anomalies on EEG. [ingentaconnect.com]

ESES/CSWS phenomena can be held as a common final pathway originating from different etiologies, including patients with early brain damage (probably involving thalamic structures), but also patients without structural pathology as in atypical evolution [psjd.icm.edu.pl]

A delineation of epilepsies based on physiopathologic mechanisms and on etiology remains the ultimate goal. © 1994 S. Karger AG, Basel Article / Publication Details First-Page Preview [karger.com]

Epidemiology

Epidemiology of epilepsy in childhood: a cohort of 440 consecutive patients. Pediatr Neurol. 1998 Jan. 18(1):46-50. [Medline]. Aicardi J, Ohtahara S. Severe neonatal epilepsies with suppression-burst pattern. [emedicine.medscape.com]

Epidemiology Landau & Kleffner described six children with the condition in 1957 ( 3 ). Since then, more than 350 children with the syndrome have been described worldwide. [tidsskriftet.no]

Epidemiological study of Landau-Kleffner syndrome (LKS) in Japan. Brain Dev. 2014;36:284–6. 50. Turner SJ, Morgan AT, Perez ER, Scheffer IE. New genes for focal epilepsies with speech and language disorders. Curr Neurosci Rep. 2015;15:35 51. [j-epilepsy.org]

Pathophysiology

This pathophysiological approach to monogenic epilepsies provides diagnostic clues and helps to guide treatment strategy. [ncbi.nlm.nih.gov]

Some considerations on the pathophysiology of generalized bilaterally synchronous spike and wave discharge. Epilepsia 9, 249-263 (1968). ‏ [books.google.com]

The pathophysiological mechanisms of cognitive and behavioral disturbances in children with Landau-Kleffner syndrome or epilepsy with continuous spike-and-waves during slow-wave sleep. Seizure 2006;15(4):249-58. 15. [acnr.co.uk]

Insights into the pathophysiology of psychomotor regression in CSWS syndromes from FDG-PET and EEG-fMRI // Epilepsia. — 2009. — V. 50 (7). — P.47-50. 8. Engel J.Jr. [rjdn.abvpress.ru]

The pathophysiology is not completely understood, but the corticothalamic neuronal network involved in sleep patterns is thought to be involved. Genetic predisposition and injury in early development are thought to play etiological roles. [genome.jp]

Prevention

Prevention - Continuous spike-wave during slow sleep syndrome Not supplied. [checkorphan.org]

LEV has the advantage of preventing any worsening in CSWS. [omicsonline.org]

[…] knowledge about the syndromes characterised by the presence of CSWS on the EEG, to bring out the many, still unanswered -- questions, and to stimulate further interdisciplinary research to verify the validity of present hypotheses, in order to clarify which preventive [booktopia.com.au]

The aim is to prevent the spread of epileptiform activity ( 15 ). In children with Landau-Kleffner syndrome who also have ADHD, central stimulating drugs may be appropriate. [tidsskriftet.no]

Of course, if intervention at some stage can prevent or ameliorate a life-long disorder, even a low yield may be well justified not only on economic bases but also for the suffering of patients and their families that may be avoided. [npepjournal.biomedcentral.com]

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