(anteroposterior view) showing small uterine arteries (arrowed).escape of about 2 litres of infected liquor amnii and a volume of gas. A subtotal hysterectomy and excision of the sac was performed. The patient was maintained on intravenous fluids and antibiotics and she made a good recovery. She was discharged frI hospital after six weeks, and six weeks later she was welL Cases 3, 4, and 5 These three cases were identical in presentation and were all extrauterine pregnancies complicated by a pyogaseous infec The pregnancies were all postmature, with subsequent fetal death and failed induction of labour. The radiological findings (Fig. 3) confirmed pyogaseous infections similar to that in Case 2. CommentIn early cases of gas gangrene or pyogaseous infection of the uterus the features are those of septicaemia, which may lead to circulatory failure and death, as in Case 1. Extension of gas gangrene from the uterus to the peritoneum leads to peritonitis. Other complications are emphysematous vaginitis, thrombophlebitis, lymphangitis, and renal failure (Adams and Adams, 1931; Holly et al., 1960). The principles of treatment are early diagnosis, prompt prophylaxis, early elimination of the focus of infection, massive antisera administration, and systemic antibiotic therapy.The prognosis depends mainly on the extent and duration of the infection, early diagnosis and treatment, and the degree of kidney damage. Hill (1936) reported a mortality of 63% in 30 cases of postabortal and puerperal gas gangrene. Russel and Roach (1939) were the first to report the use of x-ray examination in the diagnosis of gas gangrene of the uterus. The presence of air and fluid in large amounts, as in the present cases, indicates sepsis by gas-forming organisms, since the gas is more than would be seen in cases of uncomplicated fetal death. Radiography is the diagnostic method of choice. It 1S quick and reliable, and thus ensures early and prompt surgical intervention to eliminate the focus of infection. Bacteriological examination of vaginal discharge or of a vaginal swab and blood cultures, though useful, may prove negative. Moreover, the results are usually delayed.We are grateful to Professors J. P. Hendrickse and 0. A. Ojo for permission to report these cases, and to Professor S. P. Bohrer for the use of the radiological museum and for his helpful criticisms.

A clinicopathological study of 10 cases of progressive neuronal degeneration of childhood is reported. In the typical clinical course early developmental delay is followed by intractable epilepsy leading rapidly to death, in some cases in liver failure. Diagnostically useful investigations include characteristic EEG changes, evidence of progressive atrophy (particularly occipital) on CT scan, absent or reduced visual evoked responses, and biochemical evidence of abnormal liver function in many cases before commencement of anticonvulsant therapy. Siblings of 4 of the reported cases suffered a similar clinical disorder. Macroscopic appearances of the brain varied from virtual normality to severe atrophy. The cortical ribbon showed patchy lesions, but the calcarine cortex was characteristically involved, narrowed, granular and discoloured. Histological damage to the cerebral cortex was widespread but patchily accentuated. In milder lesions status spongiosus, astrocytosis and neuronal loss occurred only in the superficial cortex, in moderately affected areas deeper laminae were involved, and in the most severe lesions the entire cortex was reduced to a thin densely gliotic remnant. There was a pronounced tendency for the striate cortex to be the worst affected area. Of subcortical structures the thalamus, hippocampus and cerebellum were particularly severely involved. There was usually accompanying liver disease, particularly a subacute hepatitis comprising massive fatty degeneration, hepatocyte loss, bile duct proliferation and fibrous scarring, with or without cirrhosis. These pathological features are distinct from other combined degenerations of liver and brain and the cortical lesions differ significantly from the neuropathological sequelae of birth injury or severe epilepsy. Hepatic pathology is distinctive and does not appear to be related to drug therapy. It is concluded that these 10 cases of progressive neuronal degeneration of childhood with concomitant liver disease, together with a small number of previously reported cases, are a nosological entity which may result from an autosomal recessive inherited metabolic defect, the nature of which is at present obscure.

SUMMARY Clinical and pathological findings are reported in two siblings who presented in the neonatal period with failure to thrive, hypotonia, pericardial effusions, limitation of joint movement, retinal dystrophy and loss of visual function. Additional features were biochemical evidence of purine overproduction and liver dysfunction. Post mortem, the neuropathological findings in both children were typical of olivopontocerebellar atrophy. It is suggested that the cases represent a recessively inherited inborn error of metabolism. OPCA is combined with systemic and biochemical abnormalities. Case reportsCase I was the first child of unrelated Caucasian parents. She was born at term, by normal delivery, weighing 2-8 kg. Apgar scores were satisfactory but after 48 hours she was transferred to the special care baby unit with lethargy, hypothermia and feeding problems. Clinical suspicions of septicaemia were not confirmed by screening but she continued to show failure to thrive. There was obvious oedema of the lower limbs and hepatomegaly, and a pericardial effusion was detected by 2D-Echo when she was 5 weeks old. Weight gain remained poor, diarrhoea was an additional problem, and protein losing enteropathy was suspected. She required several plasma transfusions because of oedema. There was also concern about her vision, when roving eye movements were observed. At the age of 6 months, she was transferred to the Hospital for Sick Children, for investigation of her failure to thrive. Her weight was 3-3 kg, length 55 cm, head circumference 38-5 cm; all measurements were well below the 3rd centile. She was slow to feed, wasted, lethargic, generally hypotonic and developmentally retarded. There were no dysmorphic features. She had occasional roving nystagmoid eye movements, but fixed well and could follow light. Ophthalmoscopic examination showed pale but not hypoplastic discs and pale fundi, diagnosed as retinal dystrophy. The liver edge was palpable 2-3 cm below the costal margin. Limitation of hip abduction was also noted.Chest radiographs confirmed the presence of a small pericardial effusion, and abdominal ultrasound demonstrated hepatomegaly and ascites. The EEG was normal. The ERGs had abnormally small amplitude and unusual waveforms, 385 by copyright.

A detailed autopsy study of three children with ornithine carbamoyl transferase (OCT) deficiency is presented. Although variable in extent, a basic pattern of neuropathological lesions is discernible. Case 1 shows gross cerebral atrophy, cases 2 and 3 milder lesions in the basal nuclei but also multiple cerebellar heterotopias and delayed myelination. We suggest that the findings may provide evidence that OCT deficiency can have a teratogenic effect in utero and suggest that there is a need to monitor the pregnancies of carriers of this disorder.