Edit concept Question Editor Create issue ticket

Epilepsy with Continuous Spike-and-Slow-Waves during Sleep

CSWS


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]
  • 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]
  • However, he presented a progressive cognitive and behavioral deterioration up to the present, and IQ dropped from 85 to 67 of total score. The duration of the CSWS period was 14 months. Figure 1.[intechopen.com]
  • Antecedent and birth history are usually normal, or previous structural brain abnormalities may be present.[epilepsydiagnosis.org]
Italian
  • Parmeggiani", year "2013", month "4", language "Italian", pages "154--161", journal "Bollettino - Lega Italiana contro l'Epilessia", issn "0394-560X", publisher "Lega Italiana contro l'Epilessia", number "145", } TY - JOUR T1 - Epilessia, punte-onda continue[moh-it.pure.elsevier.com]
Short Stature
  • Clinical indications for MR imaging were suspected speech delay, brain trauma, brain tumor, short stature and migraine, which turned out to be neurologically and / or psychologically insignificant during a 2-4 year follow-up period after this study.[intechopen.com]
Cerebral Palsy
  • These cases have usually preexisting signs of cerebral palsy.[neupsykey.com]
Impulsivity
  • The onset of paroxysmal electrical activity was closely related in time with the development of a neuropsychological and behavioral pattern characterized by inattention, impulsiveness, mood swings and perseveration, with deficits in time orientation,[omicsonline.org]
  • However, he exhibited a cognitive and behavioral deterioration, including poor school performance, hyperactivity, and impulsiveness. The duration of the CSWS period was 21 months. 2.1.4. Case 4 This patient was a 9-year-old male.[intechopen.com]
  • General senses are widespread Only those impulses that reach the cerebral cortex of the through the body and are structurally simple.[woodsholemuseum.org]
Emotional Lability
  • 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]
  • Aggressiveness, deficits in relatedness and inhibition, bizarre behavior, emotional lability and psychotic behavior have also been described ( Roulet-Perez et al., 1993 ; Kyllerman et al., 1996 ).[intechopen.com]
Psychomotor Retardation
  • Subsequently she developed CPS with secondary generalization and aphasia and psychomotor retardation. The child was initially started on Carbamazepine and later on Sodium Valproate and Clobazam was added to control the seizures.[indianpediatrics.net]
Aphasia
  • […] 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]
  • LKS is a particular presentation in which acquired aphasia is the core symptom. Seizures are not a mandatory feature.[neupsykey.com]
  • الصفحة 90 - Acquired Aphasia with Convulsive Disorder": long-term follow-up of six children and a review of the recent literature. ‏ الصفحة 35 - GLOOR, P.: Generalized cortico-reticular epilepsies.[books.google.com]
  • However, these have typically had little to no effect on the long-term development of aphasia.[docneuro.com]
  • 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]
Convulsions
  • الصفحة 90 - Acquired Aphasia with Convulsive Disorder": long-term follow-up of six children and a review of the recent literature. ‏ الصفحة 35 - GLOOR, P.: Generalized cortico-reticular epilepsies.[books.google.com]
  • When this happens, the seizure is called a bilateral seizure, usually tonic-clonic seizures or convulsions. Other seizure types include absence, atypical absence, and atonic seizures. Seizures usually happen during sleep.[epilepsy.com]
  • The authors suspected that persistent convulsive discharges resulted in the functional ablation of brain areas concerned with linguistic communication.[neupsykey.com]
  • Seizures are typically nocturnal, partial motor or generalized convulsions ( Tassinari et al., 2005 ).[intechopen.com]
  • EEG confirmation is also usually straightforward in persistent electrographic status after control of convulsive status, and in children with ESES.[jnnp.bmj.com]
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 tonicclonic seizures, focal negative myoclonia, atypical absences) but, unlike in Lennox–Gastaut syndrome, tonic seizures are usually[neupsykey.com]
  • In the immediate post-natal period she had 4-5 tonic-clonic seizures in a day which did not recur.[indianpediatrics.net]
  • In 70–80 % of patients, seizures of different types occur: absence seizures, focal seizures or generalised tonic-clonic seizures ( 8 ). Seizure frequency is generally low.[tidsskriftet.no]
Generalized Seizure
  • It differentiates between focal epilepsy and generalized seizures. The causes of secondary epilepsy, atomic seizures, childhood absence epilepsy, continuous spike and wave during slow sleep...[connection.ebscohost.com]
  • Nowadays, it is appreciated that CSWS often accompanies epileptic syndromes associated with partial or generalized seizures, occurring during sleep, as well as atypical absences when awake.[intechopen.com]
  • .: Thalamic lesion and epilepsy with generalized seizures, ESES and spike-wave paroxysms – report of three cases. Seizure, 2006, 15: 454–458.[degruyter.com]
  • Epilepsia 47: 1094–1120 PubMed Google Scholar Glauser T, Kluger G, Sachdeo R et al (2008) Rufinamide for generalized seizures associated with Lennox-Gastaut-syndrome.[link.springer.com]
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]
  • problems were still present (RIAS: verbal IQ 96, 40 th percentile; performance IQ 88, 21 st percentile; overall IQ 92, 30 th percentile).[functionalneurology.com]
  • Nearly all investigators have reported a high prevalence of behavioral problems in children with epilepsy.[intechopen.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]

Workup

Temporal Epileptiform Discharges
  • The inter-ictal right fronto-temporal epileptiform discharges support a diagnosis of localization related epilepsy. The history of left upper extremity tonic posturing during sleep is also suggestive of a right hemispheric focus.[jcsm.aasm.org]

Treatment

  • Treatment for continuous spikes and waves during sleep (CSWS): Survey on treatment choices in North America. Epilepsia, 55 (7), 1099-1108.[scholars.northwestern.edu]
  • Valproate, lamotrigine, levetiracetam and sulthiame# may be the most appropriate treatments. The treatment of CSWS, which is responsible for the neuropsychological impairment, is entirely empirical and usually of transient efficacy.[epilepsy.com]
  • Aggressive treatment approaches to abolish the paroxysmal disturbance should be seriously considered in the high-risk group. LEV may represent an important addition to the treatments available for CSWS.[omicsonline.org]
  • Treatment was changed to using VPA, but improvements to his clinical state were not recognized. Treatment was changed to using VPA with CLB, which led to improvement. The ESES pattern resolved with no further seizures.[intechopen.com]
  • […] during sleep p. 137 Late onset 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[booktopia.com.au]

Prognosis

  • 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]
  • Despite the usually benign evolution of seizures, prognosis is guarded because of the appearance of neuropsychologic disorders. ‏ الصفحة 26 - Ch. (1992): The Lennox-Gastaut syndrome.[books.google.com]
  • However, the long-term prognosis for neuropsychological consequences is not nearly as good as was once thought.[intechopen.com]
  • Based on these previous studies, the best treatment options may be required to remit seizures and EEG abnormalities as soon as possible to achieve the most favorable prognosis for CSWS patients.[omicsonline.org]
  • The prognosis for the aphasia varies.[tidsskriftet.no]

Etiology

  • Rett syndrome Metabolic etiologies (e.g. mitochondrial disorders)[epilepsydiagnosis.org]
  • Etiology Symptomatic, probably symptomatic and idiopathic.[epilepsy.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]
  • 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]

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]
Sex distribution
Age distribution

Pathophysiology

  • Some considerations on the pathophysiology of generalized bilaterally synchronous spike and wave discharge. Epilepsia 9, 249-263 (1968). ‏[books.google.com]
  • Pathophysiology of cognitive regression It should be noted that complete normalization of the sleep EEG is not always mandatory to obtain clinical improvement.[neupsykey.com]
  • The pathophysiology of CSWS is complex and far from being elucidated.[intechopen.com]
  • This pathophysiological approach to monogenic epilepsies provides diagnostic clues and helps to guide treatment strategy.[ncbi.nlm.nih.gov]
  • 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]

Prevention

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

Ask Question

5000 Characters left Format the text using: # Heading, **bold**, _italic_. HTML code is not allowed.
By publishing this question you agree to the TOS and Privacy policy.
• Use a precise title for your question.
• Ask a specific question and provide age, sex, symptoms, type and duration of treatment.
• Respect your own and other people's privacy, never post full names or contact information.
• Inappropriate questions will be deleted.
• In urgent cases contact a physician, visit a hospital or call an emergency service!