In the context of childhood epilepsy, the concept of continuous spike–waves during slow sleep (CSWS) includes several childhood-onset heterogeneous conditions that share electroencephalograms (EEGs) characterized by a high frequency of paroxysmal abnormalities during sleep, which have negative effects on the cognitive development and behavior of the child. These negative effects may have the characteristics of a clear regression or of a slowdown in development. Seizures are very often present, but not constantly. The above makes it clear why CSWS have been included in epileptic encephalopathies, in which, by definition, frequent EEG paroxysmal abnormalities have an unfavorable impact on cognitive functions, including socio-communicative skills, causing autistic features, even regardless of the presence of clinically overt seizures. Although several decades have passed since the original descriptions of the electroclinical condition of CSWS, there are still many areas that are little-known and deserve to be further studied, including the EEG diagnostic criteria, the most effective electrophysiological parameter for monitoring the role of the thalamus in CSWS pathogenesis, its long-term evolution, the nosographic location of Landau–Kleffner syndrome, standardized neuropsychological and behavioral assessments, and pharmacological and non-pharmacological therapies.

Continuous Spike–Waves during Slow Sleep Today: An Update

Akute und chronische organische Psychosyndrome im Kindes- und Jugendalter

Electro-clinical Syndromes and Epilepsies in the Neonatal Period, Infancy, and Childhood

Epileptic seizures account for common neurologic presentations in paediatric neurology units in developing countries. Atypical presentation of syndromic epilepsy may be missed especially sensory, psychic or autonomic manifestations. An 8 year old male Nigerian child presented with recurrent vomiting and delayed motor milestones, regression of speech (acquired aphasia) with unremarkable birth history or any underlying medical or surgical condition. He had delayed gross motor development and continuous bilateral polyspike waves of slow sleep on EEG. Self-limited autonomic epileptic seizure syndrome which was a strong differential of autonomic seizures in early childhood was not suggestive as his EEG findings failed to demonstrate occipital spike wave pattern nor did photic stimulation evoke any epileptiform waves. Child made significant clinical improvement with anticonvulsants, neuro vitamins, and behavioural therapy, evidenced by regain of non-verbal communication, resolution of autonomic symptoms and temper tantrums. A high index of suspicion should be applied in patients with acquired aphasia with subclinical or recurrent autonomic symptoms.

Atypical electroclinical presentation of Landau Kleffner syndrome: a case report of an 8 year old Nigerian child

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Landau-Kleffner syndrome.

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Landau-Kleffner syndrome.