title: [Epilepsy with myoclonic-astatic attacks (Lennox-Gastaut syndrome) with particularly unfavorable course].
abstract: Out of over 100 children with the Lennox-Gastaut syndrome observed in the Outpatient Clinic and Department of Paediatric Neurology, Children's Health Center the authors present 6 cases in which the course of the disease was progressive and devastating. The main signs were astatic-myoclonic and generalized seizures, regression of psychomotor development, followed by mental regression, pareses of extremities, decortication symptoms and somatic cachexia. The authors suggest that the clinical course, the character of epileptic seizures very poor prognosis in certain children with the Lennox-Gastaut syndrome make the syndrome similar in its clinical aspects to subacute sclerosing panencephalitis and require careful differential diagnosis for excluding the latter disease.
mesh_terms: D002100:Cachexia / Q000209:etiology; D002648:Child; D002675:Child, Preschool; D004831:Epilepsies, Myoclonic / Q000150:complications*; D004832:Epilepsy, Absence / Q000150:complications*; D005260:Female; D006801:Humans; D008607:Intellectual Disability / Q000209:etiology; D008297:Male; D011379:Prognosis; D011596:Psychomotor Disorders / Q000209:etiology; D011782:Quadriplegia / Q000209:etiology; D013577:Syndrome
vernacular_title: Padaczka z napadami miokloniczno-astatycznymi (zespól Lennoxa-Gastaut) o szczególnie niekorzystnym przebiegu.