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]

[…] the 1st illustrated, transportable instruction manual to debate all features of scientific neurophysiology, is an important technique of speedy reference for an individual fascinated about EEG interpretation. instruction manual of EEG Interpretation presents [electricsexclothing.com]

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]

• Encephalopathy

  EPILEPTIC ENCEPHALOPATHY WITH CONTINUOUS SPIKE-AND-WAVE DURING SLEEP OVERVIEW Epileptic encephalopathy with continuous spike-and-wave during sleep is a syndrome characterized by continuous spike-and-wave during sleep, seizures and progressive decline [epilepsydiagnosis.org]

  What is Epileptic Encephalopathy with Continuous Spike and Wave During Sleep? The syndrome of Epileptic Encephalopathy with Continuous Spike and Wave During Sleep (CSWS) is a very rare form of epilepsy. [epilepsy.com]

  The more severe epileptic encephalopathies fall into this category. [emedicine.medscape.com]

  Entry H01827 Disease Name Epileptic encephalopathy with continuous spike-waves during slow-wave sleep; Rolandic epilepsy, mental retardation, and speech dyspraxia Description Epileptic encephalopathy with continuous spike-waves during slow-wave sleep [genome.jp]

  The author represents the review and discussion of current scientific literature devoted to epileptic encephalopathy with continuous spikes-waves activity during sleep — the special form of partly reversible age-dependent epileptic encephalopathy, characterized [ingentaconnect.com]

• 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]

  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]

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

• Hyperactivity

  Hyperactivity and oppositional behaviors are highly associated. [intechopen.com]

  There is marked decrease in intelligence quotient scores with attention deficit and hyperactivity, sometimes with language disturbances and autistic features. [neuroscienceandgenetics.it]

  […] acquired epileptic aphasia p. 143 CSWS: SW index value according to the slow sleep stages p. 149 Continuous focal discharges during REM sleep in a case of Landau-Kleffner syndrome: A 3-year follow-up p. 155 Methylphenidate treatment of attention deficit-hyperactivity [booktopia.com.au]

  There is often a history of loss of language and temporo-spatial skills, hyperactivity, short-term memory deficits and behavioural problems. [acnr.co.uk]

  There is an intense increase in epileptic activity, with left hemispheric dominance (red) Attention and inhibition difficulties, hyperactivity, aggression, social withdrawal, emotional lability, anxiety and depression are relatively common comorbid traits [tidsskriftet.no]

• Behavior Problem

  Pressler RM, Robinson RO, Wilson GA, Binnie CD (2005) Treatment of interictal epileptiform discharges can improve behavior in children with behavioral problems and epilepsy. J Pediatr 146: 112-117. [omicsonline.org]

  However, one month later, an abrupt cognitive deterioration appeared, consisting of severe language problems, difficulties in remembering words and numbers, and behavioral and attention disorders. [functionalneurology.com]

  This conclusion is supported by the work of Pressler et al. ( 2005 ) in which they show that treatment of interictal epileptiform discharges can improve behavior in epileptic children with behavioral problems. [npepjournal.biomedcentral.com]

  Nearly all investigators have reported a high prevalence of behavioral problems in children with epilepsy. [intechopen.com]

• Generalized Tonic-Clonic Seizure

  Second stage with CSWS: seizures more frequent and complicated with typical or more frequent atypical absences, myoclonic absences, absence status epilepticus, atonic or clonic seizures, and generalized tonic-clonic seizures. [epilepsy.com]

  When they occur, seizures have variable semiology (focal seizures without consciousness impairment, secondary generalized tonic–clonic seizures, focal negative myoclonia, atypical absences) but, unlike in Lennox–Gastaut syndrome, tonic seizures are usually [neupsykey.com]

  Generalized tonic-clonic noctur- nal seizures first appeared at age 8 accompanied by rare daytime absence seizures. With treatment with valproate (VPA) and then phenobarbital (PB) the TABLE 1. [docslide.com.br]

  At age 5 years, he started with sporadic, generalized tonic-clonic seizures, which were controlled by VPA. A sleep EEG recording showed focal spike and wave discharges over the right central and temporal leads (C4-T4). [intechopen.com]

• Epileptiform Activity

  Electrical status epilepticus of sleep (ESES) is characterized by marked sleep potentiation of epileptiform activity during SWS. Specific cutoff thresholds or measurement methods for epileptiform activity frequency have yet to be defined. [jcsm.aasm.org]

  EEG epileptiform activity is more prominent than clinical seizures. [emedicine.medscape.com]

  activity (> 85%) during NREM sleep. [functionalneurology.com]

  Melissa Filippini, Antonella Boni, Melania Giannotta and Giuseppe Gobbi, Neuropsychological development in children belonging to BECTS spectrum: Long-term effect of epileptiform activity, Epilepsy & Behavior, 28, 3, (504), (2013). [doi.org]

  Abnormalities of background cerebral rhythms, focal slow activity or regional attenuation are much less specific than epileptiform activity, although they can indicate localised structural pathology underlying the seizure disorder, or diffuse cortical [jnnp.bmj.com]

• Electroencephalogram Abnormal

  J Child Neurol 10(4):267–270 PubMed View Article Google Scholar Reinhold JA, Molloy CA, Manning-Courtney P (2005) Electroencephalogram abnormalities in children with autism spectrum disorders. [npepjournal.biomedcentral.com]

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

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 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 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]

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]

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]

Diagnosis - Continuous spike-wave during slow sleep syndrome * Sleep Disorders: Home Testing * Home Sleep Tests * Sleep Apnea Tests Prognosis - Continuous spike-wave during slow sleep syndrome Not supplied. [checkorphan.org]

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]

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]

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]

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]

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 - 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]