Neuropsychological impairment in early‑onset hydrocephalus and epilepsy with continuous spike‑waves during slow‑wave sleep: A case report and literature review

Epilepsy with continuous spike‑waves during slow‑wave sleep (CSWS) is often characterized by a severe cognitive and behavioral impairment. Symptomatic cases also include patients with an early‑onset hydrocephalus, but in literature detailed neuropsychological data on these subjects are not available. We describe the results of serial cognitive assessments in a girl with shunted early‑onset hydrocephalus, followed by partial epilepsy complicated with CSWS at 4 years 10 months, in which a dramatic cognitive and behavioral deterioration occurred few months after CSWS onset. Adrenocorticotropic hormone treatment improved both clinical and electroencephalogram picture, but an impairment of visual perception, visual‑motor coordination and executive functions persisted after CSWS disappearance. We hypothesize, in this case, an involvement of right occipital‑parietal lobe and prefrontal lobe.