Early prediction of the development of microcephaly after hypoxic-ischemic encephalopathy in the full-term newborn.

The development of microcephaly after significant hypoxic-ischemic cerebral injury in the full-term newborn has major prognostic significance. However, the onset of microcephaly in this context may be delayed more than 12 months. OBJECTIVES. To determine whether serial head circumference measurements and decreased rate of head growth in asphyxiated full-term newborns during the first few months of life may predict the development of eventual microcephaly. METHODOLOGY. Serial head circumference measurements at 4, 8, and 18 months of age were obtained in 54 full-term newborns who had acute, hypoxic-ischemic encephalopathy. The rate of head growth was determined on the basis of changes in head circumference ratios which are calculated as follows: actual head circumference/mean head circumference for age x 100%. Head circumference ratios were correlated with severity of newborn encephalopathy and outcome at 18 months. RESULTS. A decrease in head circumference ratios of > 3.1% between birth and 4 months of age was highly predictive of the eventual development of microcephaly before 18 months (sensitivity 90%, specificity 85%). CONCLUSIONS. These data demonstrate that serial head circumference measurements during the first 4 months of life and calculation of decreased rate of head growth in full-term newborns with hypoxic-ischemic encephalopathy may predict microcephaly before its actual occurrence.

Lissencephaly: diagnosis by computed tomography and magnetic resonance imaging.

A patient with Miller-Dieker-Syndrome, which is associated with lissencephaly, was examined by Computed Tomography (CT) and Magnetic Resonance Imaging (MRI). While CT demonstrated the main features of lissencephaly, MRI detected disturbed myelination and cell migration in the cerebral hemispheres and a normal appearance of the cerebellum. MRI provides more accurate information of the pathomorphologic changes of lissencephaly and is thus superior to CT.

[Vitamin K deficiency with erythromycin. Observation of a boy treated with valproate]. / Vitamin K-Mangel unter Erythromycin. Beobachtung bei einem mit Valproat behandelten Jungen.

A nine year old boy had been treated with valproic acid during one and a half years because of a grand mal epilepsy. Under an additional medication with erythromycin succinate syrup this patient developed a deficiency of prothrombin-complex, which was reversible immediately after oral intake of vitamin K. In this case it is assumed that the simultaneous application of both valproic acid and erythromycin succinate seems to suppress the vitamin K producing intestinal tract bacteria, which has not been reported in the literature so far.

Dystonia in ataxia telangiectasia: report of a case with putaminal lesions and decreased striatal [123I]iodobenzamide binding.

A 6-year-old girl with ataxia telangiectasia and severe progressive dystonic posturing is presented. Magnetic resonance imaging showed cerebellar atrophy and a right-sided putaminal lesion. A single-photon emission computed tomography study of cerebral dopamine-(D2)-receptor binding with [123I]iodobenzamide showed a decreased tracer uptake in the striatum bilaterally. Dystonia deteriorated with levodopa treatment, whereas trihexyphenidyl led to significant improvement. Although dystonic symptoms have been repeatedly described in ataxia telangiectasia, this is the first report demonstrating structural and functional basal ganglia abnormalities in this disorder.

Daytime acid secretion after a single dose of ranitidine and cimetidine--a double blind crossover study.

The purpose of the study was to evaluate the duration of inhibition of acid secretion by single oral doses of cimetidine and ranitidine. Basal and postprandial acid secretion in 6 healthy volunteers were measured for 14h by intermittent aspiration and prolonged intragastric titration. 400 mg cimetidine reduced daytime acid secretion by 22% and 150 mg ranitidine produced 38% inhibition. Although the elimination half lives of the drugs were similar, ranitidine led to more pronounced inhibition of acid secretion during the later part of the day. The longer duration of pronounced acid inhibition by ranitidine appears to be due solely to its greater potency.

[Hereditary Parkinson disease: report of 3 families with dominant autosomal inheritance]. / Hereditärer Morbus Parkinson: Bericht über drei Familien mit autosomal-dominantem Erbgang.

We have studied three families who ancestors immigrated to North America from contiguous regions of northern Germany and southern Denmark. The pedigrees contain 77,206 and 376 individuals spanning 6, 7 and 8 generations with 7,7 and 11 affected members, respectively. Autosomal dominant inheritance pattern is present in two families and probable in the third. Typical L-dopa-responsive parkinsonism with bradykinesia, rigidity, resting tremor, and impaired postural reflexes uniformly develop in affected individuals from all three families. Further research on these three families, including genetic, pathologic and clinical examinations is planned.