Biomedical subjects
Publications and source records attributed to R Michałowicz.
The authors discuss the etiology and pathogenesis of SSPE in the light of own observations of two boys aged 8 and 9 years whose one grandfather and one grandmother were siblings. The boys lived in different parts of Poland and never been in contact with each other. In the light of this observation the authors discuss the possibility of a genetic background in SSPE.
The authors discussed ocular changes observed in 100 children with tuberous sclerosis. Retinal tumors, typical of this disease, were found in 19 patients (19%). This incidence was higher in older children. Three types of tumors were observed: a) flat or slightly elevated, indistinct, salmon-grey colour, b) whitish-yellow, mulberry-like, located in disc area, c) mixed or intermediate. The authors observed 4 stages of tumors' development: changes in distribution of pigment in retina, arising of semitranslucent, salmon-grey tumors, mineralization of tumors-so called intermediate type, mulberry-like type. Progress from one stage to the next one is slow.
West syndrome is a from of epileptic attacks of infants. Diagnosis of West syndrome includes: presence of the sudden violent flexion of the trunk and limbs, psychomotoric development retardation, especially after the onset of attacks, abnormal EEG records, and therapeutical problems. Clinical course and results of therapy were analysed in 66 children with West syndrome (39 boys and 27 girls). Children were divided into four groups, depending on etiology of the disease. Group 1 included 39 children with lesions to CNS during pregnancy; group 2-8 children with developmental CNS disorders, group 3-6 children with a history of encephalitis or meningitis, and group 4-13 children in whom etiology of West syndrome was unclear. Patients were treated with Synacthen-Depot in a daily dose of 0.03 mg/kg combined with other anti-epileptic agents. The most difficult to treat were those children in whom West syndrome occurred below 6 months of life, were psychomotor retarded before the onset of symptoms, hormonal treatment was introduced with delay, there were additional seizures of different etiology, and there were frequently recurrent infections.
Clinical course and results of therapy were analysed in the group of 92 children, aged between 3 and 9 years, with diagnosed Lennox-Gastaut syndrome. The obtained results of an analysis have shown that Lennox-Gastaut syndrome origin is not clear--causative factor can not be established in 1/3 of patients whereas in 1/2 of them abnormal course of pregnancy and perinatal period is noted. Together with seizures of various origin, other focal neurological symptoms, mental retardation and abnormalities in CT scans of the brain are frequently seen in patients with Lennox-Gastaut syndrome. Clinical course, prognosis and results of therapy are largely dependent on the degree of mental development before the onset of epileptic seizures, course of pregnancy and perinatal period, and the time of therapy. Children with Lennox-Gastaut syndrome require relative polytherapy in which valproic acid derivatives are predominating together with benzodiazepines, and temporary corticosteroids. An improvement was achieved in about 30% of the treated children. Prognosis in the remaining 70% of children is rather poor. Irregular administration of drugs, frequent changes of anti-epileptic agents, too low doses and abnormal environmental effects (abnormal parental attitudes) affect the results of therapy. An emphasis is on the poor prognosis in Lennox-Gastaut syndrome proceeded with West syndrome.
The authors describe clinical course of tuberous sclerosis in seven family members from two pedigrees. They stress the importance of careful medical anamnesis and skin examination in family members of persons affected with tuberous sclerosis. It is pointed out that in contrast to widespread stereotype of a patient with tuberous sclerosis-a person with epilepsy, mental retardation and multiple skin and organ involvement, much more common than it was previously thought, the course of the disease may be oligosymptomatic and the affected person may properly function in society. All 7 affected persons presented in the paper were mentally normal and only two of them suffered from epilepsy.