SUMMARY A longitudinal study of 14 carefully selected low‐risk pre‐term infants was carried out by observations over a two‐hour observation period at weekly intervals. Observation was concentrated on their body postures, various types of motility, respiratory patterns and various aspects of behavioural states. Notes were made per minute of observation; only respiration (thermistor), EGG and actogram were recorded polygraphically. The gestational age of the infants ranged from 28 to 36 weeks and they were observed until they reached about term age. The age‐specific preference postures of the limbs described in the literature could not be registered. There was a clear‐cut dominant head position to the right side from 28 weeks onwards. Body postures lasted only briefly, but increased in duration with age. Related to this was the decline in gross motor activity, which less frequently led to a displacement of limb and head positions after about 36 weeks gestational age. There was no correlation with postnatal age. The amount of motility and of crying showed marked and consistent individual differences. Regular and irregular respiration can be divided into both continuous and periodic: there was a gradual increase in the percentage of regular respiration from 28 weeks to term age. The average duration of episodes of regular breathing reached the length found in full‐term infants at about 32 weeks. Respiratory pauses of 10 seconds or more without simultaneous gross movements were rare in these low‐risk pre‐term infants. Cycles of regular/irregular breathing, episodes of rapid/non‐rapid eye‐movements, and of motility/quiescence are seemingly independent of each other until about 36 weeks. Before that age, it is hazardous to apply the usual state concept, originally designed for full‐term infants, to pre‐term babies. RÉSUMÉ Postures, motricité et respiration chez les nourrissons prématurés a faible risque. Une étude longitudinale de 14 nourrissons prematurds, spécialement choisis à faible risque, a été entreprise par des observations de deux heures à intervalles d'une semaine. L'observation portait sur la posture corporelle, les types variés d'activité motrice, les schémas respiratoires et les aspects variés d'états comportementaux. Des notes étaient prises toutes les minutes d'observation, seule la respiration (thermistor) 1'ECG et l'actogramme étaient enregistrés polygraphiquement. L'âge de gestation des nourrissons allait de 28 à 36 semaines et ils furent observés jusquà atteindre l'âge du terme. Les postures préférentielles des membres, spécifiques d'un âge et décrites dans la littérature n'ont pas pu être observées. II apparut une position très nette dominante de la tête vers le côté droit depuis le début des 28 semaines. Les postures corporelles durèrent peu mais avec augmentation de durée en fonction de lâge; en relation avec cela, il fut observé une diminution de l'activité motrice globale qui, moins fréquemment, conduisit à un déplacement de la position des membres et de la tête après environ 36 semaines d'âge ge...

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Fibromatosis hyalinica multiplex juvenilis (juvenile hyalin fibromatosis) is a very rare mesenchymal dysplasia, probably inherited as an autosomal-recessive trait. Two nonrelated cases are reported. Among the clinical features, the most impressive lesions are multiple slowly growing subcutaneous nodules, hypertrophic gingiva, flexural contractures with joint stiffness and radiolucent bone destructions. Light microscopic examination of the nodules reveals tumor-like deposits of an amorphous hyaline ground substance with delicate staining properties situated partly between cellular and vascular areas. Ultrastructural characteristics are cystic, dilated rough endoplasmatic reticulum and cystic Golgi vesicles which contain a fine fibrillar material that is also found in the ground substance. Immunohistochemical examination shows collagen type I and type III in the hyaline material, but not type II and type IV. Quantitative biochemical investigation reveals a normal ratio of collagen types I and III.

777 cerebral MRI examinations of children aged 3 days to 14 years were staged for myelination to establish an age standardization. Staging was performed using a system proposed in a previous paper, separately ranking 10 different regions of the brain. Interpretation of the results led to the identification of four clinical diagnoses that are frequently associated with delays in myelination: West syndrome, cerebral palsy, developmental retardation, and congenital anomalies. In addition, it was found that assessment of myelination in children with head injuries was not practical as alterations in MRI signal can simulate earlier stages of myelination. Age limits were therefore calculated from the case material after excluding all children with these conditions. When simplifications of the definition of the stages are applied, these age limits for the various stages of myelination of each of the 10 regions of the brain make the staging system applicable for routine assessment of myelination.

Eighteen children with West syndrome underwent MRI-examination of the brain. Five children were examined twice. Myelination of ten regions of the brain was retrospectively assessed on the MRI-examinations. The children could be divided in three groups of main diagnosis (cryptogenic West syndrome, tuberous sclerosis, pre-/perinatally acquired brain lesions). Delays in myelination were found in 17 out of a total of 23 examinations. In children with cryptogenic West syndrome and with tuberous sclerosis myelination was normal or only mildly delayed in the first month after onset of the spasms, but delays seemed to increase in the course of the West syndrome in these groups. In children with pre- or perinatally acquired brain lesions severe delays of myelination could be found already in the first weeks after the onset of the West syndrome. The assessment of myelination can therefore contribute to the diagnosis and understanding of the underlying disease in children with West syndrome.