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Epileptic encephalopathy with continuous spikes and waves during sleep in children with shunted hydrocephalus: a
Roberto Horacio Caraballo1, Lucas Bongiorni, Ricardo Cersósimo
1Hospital de Pediatría Prof Dr Juan P Garrahan, Buenos Aires, Argentina. rhcaraballo@arnet.com.ar
Insights
Early-onset hydrocephalus in children can present with seizures and continuous spikes and waves during slow sleep (CSWS), leading to cognitive decline. Prompt diagnosis and treatment are vital to prevent progressive neurodevelopmental issues.
Area of Science:
- Pediatric Neurology
- Epileptology
- Neurodevelopmental Disorders
Background:
- Hydrocephalus in early childhood can be associated with complex neurological symptoms.
- Continuous spikes and waves during slow sleep (CSWS) is an electroencephalographic pattern linked to cognitive regression.
Observation:
- Nine pediatric patients with early-onset hydrocephalus presented with seizures and CSWS.
- Symptoms included behavioral changes (hyperkinesia, aggressiveness), motor deterioration, and cognitive deficits like language impairment.
Findings:
- Epilepsy onset ranged from infancy to early childhood.
- CSWS was confirmed via EEG, and patients showed good response to antiepileptic drug adjustments.
- Most patients achieved seizure freedom or sporadic seizures after treatment.
Implications:
- CSWS should be suspected in children with hydrocephalus exhibiting behavioral, language, or motor regression.
- Regular EEG monitoring during sleep is recommended for early detection.
- Timely intervention for this electroclinical picture can mitigate progressive cognitive deterioration.
Purpose:
We present a series of nine patients with early-onset hydrocephalus who had seizures and continuous spikes and waves during slow sleep (CSWS) associated with neurocognitive and motor deterioration.
Methods:
Six boys and three girls aged 9-16 years (mean 11.3 years) were studied. [Correction added after online publication 12-Apr-2008: Number of girls and boys has been updated.] All patients underwent clinical examinations, electroencephalographic evaluations, neuroradiological imaging and neuropsychological assessment at first examination. Antiepileptic drugs (AEDs) were given in all cases and changed according to clinical and EEG evolution.
Results:
Onset of epilepsy occurred from age 8 to 60 months (mean 19.6 months and median 14 months) with focal seizures with or without secondary generalized tonic-clonic seizures. Between ages 6 and 13 years (mean 10.4 years and median 8 years), hyperkinesia, aggressiveness, and poor socialization appeared in all nine cases. Reduced attention span, deterioration of language, and temporospatial disorientation were found in three of them. Negative myoclonus was found in two patients. The EEG showed CSWS. Response to change in treatment was good in all patients. None of the patients had relapses, seven of them have remained seizure free, and two continued having sporadic focal motor seizures during 2-5 years (mean 3 years) of follow-up.
Conclusion:
In children with early-onset hydrocephalus, particularly with behavioral and language disturbances and/or motor deterioration, CSWS should be considered. Periodic EEG recordings during sleep should be done in these children. The early identification of this particular electroclinical picture is crucial to start adequate treatment to avoid progressive cognitive deterioration.
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