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A novel form of severe, X linked mental retardation associated with alpha thalassaemia (ATR-X syndrome) has recently been described. Two affected cousins are described, one of whom has an unusually mild haematological phenotype. HbH inclusions, which are the hallmark of this disease, were only detected in the peripheral red blood cells after repeated observations.

Disproportionate enlargement or isolation of the fourth ventricle are rare complications of ventriculo-peritoneal shunt for post-haemorrhagic hydrocephalus. Obvious features such as ataxia may indicate raised intracranial pressure in the posterior fossa. The child may, however, present with less specific signs of vomiting, anorexia, and lethargy and these symptoms may be misinterpreted as secondary to dilation of the lateral ventricular system due to malfunction of the ventriculo-peritoneal shunt. Two children with disproportionate enlargement of the fourth ventricle and two children with isolation of the fourth ventricle are described to illustrate the wide variations in clinical presentation. These rare complications can be diagnosed by real time ultrasound examination of the brain or computed tomography of the head. They should be considered in the differential diagnosis of a child who deteriorates after lateral ventriculo-peritoneal shunting for post-haemorrhagic hydrocephalus, and dealt with specifically to avoid the risk of upward herniation of the enlarged fourth ventricle.

We describe the failure of an intravenous benzodiazepine to control non-convulsive status epilepticus occurring in six patients with the Lennox-Gastaut syndrome. In one patient the benzodiazepine induced a paradoxical response with clinical and electroencephalographic seizures.

Erb's palsy has been observed in 2 infants who had been treated with constant positive airways pressure for idiopathic respiratory distress syndrome. Both infants made complete neurological recovery from what was thought to be an acquired injury from the neck seal.

Intracerebral haemorrhage is rare in childhood. We have reviewed the last 10 years' experience, in our referral area, of parenchymatous intracerebral haemorrhage in children from 1 month to 16 years of age. There were 27 cases, five of which were intracerebellar and two predominantly intraventricular. The commonest aetiology was vascular malformation (10), followed by haemorrhage into tumour (four), and coagulopathies (five). Clinical features were non-specific, but altered consciousness, headache, vomiting, and focal signs were the most common. Focal signs were, however, rare in the patients with intracerebellar haemorrhage. There was an overall mortality of 54% (14 out of 27). Nine patients were handicapped on follow up, but none severely so. For the diagnosis of intracerebral haemorrhage a high level of clinical suspicion is needed with early use of computed tomography. Maintenance of homeostasis, relief of raised intracranial pressure, and evacuation of haematoma are the aims of management.