[Craniofacial dysostosis of the Crouzon type].
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Biomedical subjects
Publications and source records attributed to R Ignatowicz.
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Assessment was conducted on 50 children with multiple sclerosis ranging in age from 3 to 16 years. The aim of the study was to the discover how they deviate from the norm in their mental status. It was concluded that the most frequent symptoms in this group of children, were various disorders in the impulsive--emotional sphere. It was noted that with time the chance of organic pathology increases.
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The authors present their observations on treatment with Noveril of nocturnal enuresis in 49 children aged from 6 to 16 years. The drug is a derivative of dibenzodiazepine belonging to the group of thymoleptic agents with an action similar to that of imipramine. Noveril was given in doses from 20 to 100 mg daily during 3 to 9 weeks. In the final evaluation of the drug the frequency of enuresis before, during and after treatment was taken into account. In 25 cases an improvement was observed, usually in psychogenic nocturnal enuresis. Side effects included oral dryness, headaches and dizziness, and sleep disturbances observed in 4 cases. The tolerance of the drug was good. Noveril has a favourable effect in children with nocturnal enuresis, particularly of psychogenic origin.
The association of unusual types of aphasia and epilepsy (Landau-Kleffner syndrome) has been described in three children. The children were 5 to 11 years of age and all have EEG abnormalities. Speech disorders appeared after epileptic seizures. No organic causes of the disease have been found in axial computed tomography. In all described cases the increased titer of IgG antibodies against Toxoplasma gondii has been observed. The authors suggest that the Toxoplasma gondii infection should be taken into account as a possible cause of Landau-Kleffner syndrome.
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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.
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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 observed group included 91 children aged from 2 to 15 years divided into two groups: I control--25 children, and II--66 children with severe infantile cerebral palsy. The following biochemical investigations were done in the cerebrospinal fluid: total LDH activity and total protein level before and after fluid condensation, and determination of LDH isoenzymes after condensation of fluid. In the group of children with absence of organic lesions of the central nervous system the mean total LDH activity was 17.6 mU/ml, this value was similar to that found by most authors. The isoenzyme pattern in the control group contained 3--4 fractions, the LDH4 fraction was, however, found in 2 cases only. In the group with infantile cerebral palsy the total LDH activity in the cerebrospinal fluid showed a considerable range of variations, its mean value was 32.7 mU/ml and was highly significantly above the mean value in controls. The LDH isoenzyme pattern in this group consisted of 4--5 LDH fractions with a fairly high proportion of cases with LDH4 and LDH5 fractions and with a high absolute activity of these fractions.