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Progressive cerebellocerebral atrophy is the name given to an apparently new syndrome by workers in Israel (
). They describe seven children from six families, all Sephardi Jews from Iraq or Morocco. The seven patients (five girls) had profound mental retardation, progressive spastic quadriplegia with joint contractures, and progressive microcephaly. Five developed generalised seizures in the second year of life but all seven had severe and widespread clonus. Irritability was a feature in six. Repeat MRI scans showed progressive cerebellar atrophy followed by cerebral atrophy involving both white and grey matter. No metabolic defect has been found. It is suggested that this is a new autosomal recessive syndrome affecting Sephardi Jews.
The transient gross ocular deviations, usually convergence, seen in many normal newborn babies are often regarded with some amusement by parents and others but do they have any significance? A group of 214 orthoptists have recorded observations on their own babies (
). They found that babies who squinted occasionally (<15% of waking time) were less likely to develop later strabismus or refractive error than those who never squinted or had frequent squinting (>15% of waking time). It is suggested that neonatal squinting usually reflects an emerging and normally developing vergence system.
Some people …
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