title: [Three cases with severe motor and intellectual disabilities presenting the severest condition caused by prolonged non-convulsive status epilepticus].
abstract: Three patients with severe motor and intellectual disabilities presented deterioration of the activities of daily living, which was revealed to be caused by prolonged non-convulsive status epilepticus (NCSE). Their condition improved by the treatment with antiepileptics. Case 1, a 4-year-old girl with profound psychomotor retardation and past history of West syndrome of unknown etiology, became unable to sit and eat orally above age of two years. EEG showed continuous generalized slow spike and wave bursts indicating NCSE. Continuous intravenous infusion of midazolam abolished EEG abnormalities of NCSE, and she regained the ability of oral feeding. Case 2, a 3-year-old boy with Angelman syndrome and past history of West syndrome, presented decreased mental response, poor oral intake and somnolence. EEG showed continuous slow spike and wave bursts, indicating NCSE. High-dose phenobarbital therapy and continuous intravenous injection of vitamin B6 were effective, and remarkably improved his psychomotor activities. Case 3, a 3-year-old boy with Lennox-Gastaut syndrome, developed decreased psychomotor activity and loss of vocalization and walking. He could not sit by himself and became nearly bed-ridden. EEG showed very frequent generalized spike and wave bursts, showing NCSE. Continuous infusion of thiopental diminished NCSE, and he could walk again. Psychomotor deterioration in patients with severe motor and intellectual disabilities may be caused by NCSE, which should not be overlooked.
authors.affiliation: Second Department of Pediatrics, Ohashi Hospital, Toho University School of Medicine, Tokyo.
authors.forename: Yoshinao
authors.initials: Y
authors.lastname: Fujikawa
authors.forename: Kenji
authors.initials: K
authors.lastname: Sugai
authors.forename: Shigeru
authors.initials: S
authors.lastname: Hanaoka
authors.forename: Michio
authors.initials: M
authors.lastname: Fukumizu
authors.forename: Masayuki
authors.initials: M
authors.lastname: Sasaki
authors.forename: Makiko
authors.initials: M
authors.lastname: Kaga
mesh_terms: D002675:Child, Preschool; D019988:Children with Disabilities; D018450:Disease Progression; D004569:Electroencephalography; D005260:Female; D006801:Humans; D007262:Infusions, Intravenous; D008297:Male; D008874:Midazolam / Q000008:administration & dosage; D010634:Phenobarbital / Q000008:administration & dosage; D011596:Psychomotor Disorders / Q000188:drug therapy / Q000209:etiology*; D012720:Severity of Illness Index; D013226:Status Epilepticus / Q000150:complications* / Q000175:diagnosis / Q000188:drug therapy; D013874:Thiopental / Q000008:administration & dosage; D025101:Vitamin B 6 / Q000008:administration & dosage
chemical_list: D025101:Vitamin B 6; D013874:Thiopental; D008874:Midazolam; D010634:Phenobarbital