Epileptic encephalopathy with continuous spikes and waves during sleep in children with shunted hydrocephalus: a study of nine cases.
OBJECTIVE
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.


CONCLUSIONS
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.
Caraballo
Roberto Horacio
RH
Hospital de Pediatría Prof Dr Juan P Garrahan, Buenos Aires, Argentina. rhcaraballo@arnet.com.ar
Bongiorni
Lucas
L
Cersósimo
Ricardo
R
Semprino
Marcos
M
Espeche
Alberto
A
Fejerman
Natalio
N
D000293:Adolescent; D000927:Anticonvulsants / Q000627:therapeutic use; D002648:Child; D003072:Cognition Disorders / Q000175:diagnosis / Q000209:etiology; D004569:Electroencephalography; D004828:Epilepsies, Partial / Q000188:drug therapy / Q000209:etiology* / Q000503:physiopathology; D005260:Female; D006801:Humans; D006849:Hydrocephalus / Q000150:complications* / Q000601:surgery*; D007806:Language Disorders / Q000175:diagnosis / Q000209:etiology; D008297:Male; D019957:Motor Skills Disorders / Q000175:diagnosis / Q000209:etiology; D009483:Neuropsychological Tests; D010468:Perceptual Disorders / Q000175:diagnosis / Q000209:etiology; D011596:Psychomotor Disorders / Q000175:diagnosis / Q000209:etiology; D012720:Severity of Illness Index; D012894:Sleep Stages / Q000502:physiology*; D013028:Space Perception; D013998:Time Perception / Q000502:physiology; D017287:Ventriculoperitoneal Shunt
D000927:Anticonvulsants