A term neonate with a transient form of Behcet's disease is described. The clinical and immunological features are detailed. As healing of the severe ulceration in this condition gives rise to scarring and cosmetic deformity recognition is important. Therapeutic intervention with corticosteroids is recommended after diagnosis.

Behcet Syndrome↗

PubMed1981

Ventricular size and intelligence in achondroplasia.

Of 10 children with macrocephaly in association with achondroplasia only two children had progressive hydrocephalus. They were treated with a shunt procedure. One of these is intellectually and neurologically normal, and one is wheelchair bound and mentally retarded, with a spastic quadraplegia. Three children showed mild dilatation of the ventricles and one of these died from severe constriction at the foramen magnum. The other two are neurologically normal; one is intellectually normal but below average (WISC 88) and the other is slow normal at 18 months of age. Four children with demonstrated megalencephaly and a fifth with probable megalencephaly are all intellectually and neurologically normal. Intelligence in achondroplasia appears to be mainly in the normal range.

Achondroplasia↗

PubMed1981

Children with large heads: a practical approach to diagnosis in 557 children, with special reference to 109 children with megalencephaly.

Among 557 children who presented a diagnostic problem of a large head, 109 had megalencephaly as the primary diagnosis. A clinical approach to the differentiation of this numerically important group from the various other causes of large head is outlined. The group is characterised by a familial incidence of large head in at least 50 per cent of cases; a male to female preponderance of four to one; an above-normal rate of head growth in 80 per cent of the children in the first four months after birth, and in a further 12 per cent in late infancy. The vast majority of these children were normal. Only seven children were retarded, and they also had a variety of neurological and other somatic abnormalities.

Birth Weight↗

PubMed1979