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2.
Nervenarzt ; 78(10): 1188-94, 2007 Oct.
Article in German | MEDLINE | ID: mdl-17530210

ABSTRACT

Congenital hemiplegia, defined as unilateral motor disability, is the hemiplegic type of cerebral palsy. The prevalence of congenital hemiplegia is estimated to be about 0.41-0.79/1000 live births. We examined 223 children (122 boys and 101 girls) suffering from congenital hemiplegia at the age of 3 months to 12 years. Mild hemiplegia was found in 31%, a moderate form in 48%, and a severe form in 21%. The upper limb was affected in more than half of the patients, only the lower extremity in one third, and both upper and lower limbs in 20%. Electroencephalographic abnormalities were found in 75.8% of the patients. The most frequent type of epilepsy was complex partial seizures (33%). Severity of the motor handicap, grade of EEG abnormalities, and the prevalence of epilepsy showed a significant correlation. The magnitude of the lesions in neuroimaging directly correlated with these three clinical variables, particularly in children with cortical and subcortical defects (84.2%). Strabismus was the most common visual impairment (17%), while hearing impairment was found in 8% of the patients. Of them, 38.3% showed no cognitive deficits, while those with severe congenital hemiplegia were found to have a lower intelligence quotient.


Subject(s)
Cerebral Palsy/congenital , Brain/abnormalities , Brain/pathology , Cerebral Palsy/diagnosis , Child , Child, Preschool , Electroencephalography , Epilepsy, Complex Partial/congenital , Epilepsy, Complex Partial/diagnosis , Female , Humans , Infant , Magnetic Resonance Imaging , Male , Mobility Limitation , Muscular Atrophy/congenital , Muscular Atrophy/diagnosis , Neurologic Examination , Tomography, X-Ray Computed
3.
Klin Padiatr ; 218(5): 264-9, 2006.
Article in German | MEDLINE | ID: mdl-16947094

ABSTRACT

BACKGROUND: In a prospective controlled study we evaluated the efficacy of intermittent diazepam prophylaxis in the recurrence rate of febrile seizures (FS). PATIENTS: A total of 139 children aged between 6 and 36 months, who had a first FS, were enrolled in the study and were randomly allocated to two groups: group (A) that received diazepam prophylaxis and group (B) without prophylaxis. METHODS: All children were followed up for at least 3 years after their first FS. The prophylaxis group (n = 68) received rectal diazepam the first two days of a febrile illness, whenever the temperature was > 38 degrees C (0.33 mg/kg every 8 h on the first day, and 0.33 mg/kg every 12 h on the second day of fever, max. dosage 7.5 mg). The no-prophylaxis group (n = 71) did not receive any prophylaxis at all. Each group was stratified to low, intermediate and high risk subgroups according to the following clinical data: age at the first febrile seizure

Subject(s)
Anticonvulsants/administration & dosage , Diazepam/administration & dosage , Seizures, Febrile/prevention & control , Administration, Rectal , Child, Preschool , Female , Follow-Up Studies , Humans , Infant , Male , Prospective Studies , Risk Factors , Secondary Prevention
4.
Klin Padiatr ; 217(5): 281-5, 2005.
Article in German | MEDLINE | ID: mdl-16167276

ABSTRACT

Congenital muscular dystrophy (CMD) is a heterogeneous group of neuromuscular disorders characterized by muscle weakness and hypotonia at birth or within the first few months of life. It is inherited in an autosomal recessive pattern. About half of the patients have a deficiency of the alpha-2-chain of laminin (merosin). We describe a case of congenital muscular dystrophy in an infant with laminin-a2-chain deficiency, which appeared hypotonia in early infancy. Diagnosis was made by clinical features and the histological and immunohistochemical studies on muscle biopsy.


Subject(s)
Laminin/deficiency , Muscular Dystrophies/congenital , Biopsy , Female , Humans , Immunohistochemistry , Infant , Muscles/pathology , Muscular Dystrophies/diagnosis , Muscular Dystrophies/pathology
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