[A boy with acute cerebellar ataxia without opsoclonus caused by neuroblastoma]. / Een jongen met acute cerebellaire ataxie zonder opsoclonus, door een neuroblastoom.

A 2-year-old boy presented with acute cerebellar ataxia without opsoclonus. The ataxia was assumed to be post-viral. After a period of years a neuroblastoma was detected. Treatment with a curative intent was successful and consisted of metaiodobenzylguanidine I 131, chemotherapy, tumour resection, chemotherapy again and follow-up treatment with isotretinoin after irradiation. In the literature, 5 other children have been described with acute cerebellar ataxia without opsoclonus in whom neuroblastoma was detected eventually. The mean age of these children at initial presentation was 26 months. The mean time between initial presentation and diagnosis ofneuroblastoma or ganglioneuroblastoma was 12 months. Urine concentrations of catecholamine metabolites were normal in 5 of the 6 total children; concentrations were elevated in 1 child. The tumour was located paravertebrally in 5 of the 6 children. Ataxia resolved following resection of the neuroblastoma in all patients. Each child with prolonged or recurrent acute cerebellar ataxia should be extensively investigated for the presence of neuroblastoma, even in the absence of opsoclonus.

[Three infants with constipation and muscular weakness: infantile botulism]. / 3 zuigelingen met obstipatie en hypotonie: infantiel botulisme.

Two previously healthy infants, a boy of 10 weeks and a girl of 4 months presented with apathy and muscle weakness. A third previously healthy child, a girl of 6 weeks old was admitted with respiratory insufficiency. None of the three had had a bowel movement for a number of days. After extensive investigations which revealed few abnormalities Clostridium botulinum toxin was obtained in serum from all three children. Type-B-toxin was shown in the faeces of the older girl and boy; both recovered quickly. The other girl had type-A toxin; she died. Two of the three children were given honey to comfort them. Infantile botulism must be considered in every infant with symptoms of constipation and hypotonia. The diagnosis can quickly be confirmed by electromyography with repetitive 50-Hz-stimulation. Honey is a well-known source of the C. botulinum spore and should not be given to children under the age of 12 months. These three children are the first cases to be described in the Netherlands.

[The attending physician and the certificate of natural death in children]. / De behandelend arts en de verklaring van natuurlijke dood bij kinderen.

Three children died in the paediatric intensive-care unit or emergency department. The case of a 3.5-year-old girl who died after a pneumococcal infection was considered a natural death. An 8-year-old boy suddenly collapsed and died despite resuscitation. The attending physician considered this an unnatural death. The district medical examiner, after consulting the district attorney, required an autopsy, but the cause of death could not be ascertained and the next of kin were to be examined for cardiac rhythm disorders. A 2-year-old boy died after a fall out of a window. The district medical examiner and the district attorney concluded that there was sufficient explanation for this unnatural death and that autopsy was not necessary. The attending physician has several important tasks around the time of and after death in which he or she is subject to a number of legal regulations. Knowledge of these regulations is mandatory for good medical practice.

Craniopagus: the Suriname-Amsterdam conjunction.

OBJECTS: A case of a Suriname female occipito-parietal to occipito-parieto-temporal craniopagus twins is described. The girls were transferred to the VU University Medical Center (VUmc) in Amsterdam, the Netherlands, for further diagnostics and to analyze whether surgical separation was feasible and ethically justifiable. The multifactorial aspects of different treatment options are discussed. METHODS: The twins underwent multiple investigations by a multidisciplinary team. Advanced imaging techniques with 3D-CT scan, MRI and MRA scans, image fusion techniques and, most importantly, cerebral angiography with balloon occlusion tests were performed. CONCLUSIONS: Because of a shared venous ring, with preferential drainage to the left child, and which endovascular balloon occlusion showed could not be separated, surgical separation of the twins with a fair chance of survival without additional neurological damage and with prospects of a good quality of life was regarded as impossible. In accordance with the parents' wishes, the twins were not separated and offered optimal integral conservative treatment.