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Background: Acute flaccid myelitis (AFM) is a relatively rare disorder affecting the anterior horn of the spinal cord and brain stem. It is characterised by rapid progressive weakness of the limbs and respiratory muscles, often combined with cranial nerve dysfunction. This used to be seen in infections with the polio virus, but in recent years, AFM has been mainly associated with enterovirus D68 infection., Case Description: A boy of nearly 4 years-old developed rapidly progressive weakness and respiratory failure after an upper airway infection. Initially, Guillain-Barré syndrome was suspected, but after further investigations enterovirus D68 was detected in the nasopharyngeal aspirate and the diagnosis of AFM was made., Conclusion: Progressive weakness after a respiratory tract infection should raise the suspicion of enterovirus-associated AFM. This syndrome can be distinguished from Guillain-Barré syndrome by its more rapid progression, asymmetrical weakness and greater involvement of the upper limbs. The diagnosis can be confirmed by typical findings on MRI and electromyography of the spinal cord and brain stem, combined with the detection of enterovirus D68 in nasopharyngeal specimens.

A 9-year-old boy who since 3 years had been treated with a brace for presumed idiopathic thoracic scoliosis was referred because of severe syringomyelia on MRI of the spinal cord. Neuroimaging of the spinal cord had been performed because of back pain, although the patient had no neurological signs or symptoms. Besides extensive syringomyelia, the MRI revealed a type I Chiari's malformation which we assumed to be the primary cause of the syringomyelia and the subsequent scoliosis. Surgical decompression of the Chiari's malformation led to a remarkable reduction of the syringomyelia and to stabilisation of the scoliosis. In children with structural scoliosis, ancillary investigation in the way of neuroimaging of the spinal cord should be considered to exclude underlying spinal cord pathology, even in the absence of neurological abnormalities.

Objective: To gain insight into the reporting and assessment of active termination of life in newborns in The Netherlands. DESIGN; Retrospective., Method: Data on all cases of deliberate termination of life in newborns up to the age of 6 months, reported to the public prosecutor as from 1997 onward, were collected and compared., Results: In January 1997-June 2004, 22 cases of deliberate termination of life in newborns were reported. All cases concerned newborns with spina bifida and hydrocephalus. Deliberate termination of life was acceptable to the physicians because of the presence of hopeless suffering, with no means of alleviating the suffering. In all cases, at least 2 doctors were consulted outside the medical team. In 17 of 22 cases, a multidisciplinary spina bifida team was consulted. All parents consented to the termination of life; in 4 cases they explicitly requested it. In 14 cases a combination of analgesics, sedatives and paralyzing drugs were used, in 8 cases only analgesics and sedatives were administered. For the public prosecutor the termination of life was acceptable if 4 requirements were properly fulfilled: the presence of hopeless and unbearable suffering, consent of the parents to termination of life, consultation having taken place and a careful execution ofthe termination. The mean time between reporting of the case and the decision concerning prosecution was 5.3 months. None of the cases led to prosecution., Conclusion: Three cases of deliberate termination of life in newborns were reported in The Netherlands annually. The public prosecutor used fixed criteria to assess the case and all cases of active termination of life reported were found to be in accordance with good practice.

Four previously healthy children, two boys aged 5 and one boy and one girl aged 4 more or less acutely developed a stumbling gait. The causes varied from benign such as postviral acute cerebellar ataxia and benign paroxysmal vertigo to potentially life-threatening such as intoxication with benzodiazepines and medulloblastoma. Treatment led to complete or partial recovery. (Sub)acute balance disorders in previously healthy children can be due to cerebellar ataxia, vestibular disorders and abnormal proprioception. Ancillary investigations are warranted in case of gradually developing ataxia, accompanying neurological deficits, suspicion of intoxication, recurrent or familial ataxia, no spontaneous remission or even progression. In children with an isolated cerebellar ataxia without these features, ancillary investigations may be avoided, although in such cases careful follow-up remains necessary.

Three neonates had diverse kinds of central apnoea. The first child, a girl aged 3 weeks, had an upper respiratory tract infection caused by the respiratory syncytial virus; she was intubated and needed ventilatory support for three days. The second patient, a boy of 17 days, had an Arnold-Chiari-malformation with apnoeas treated with a carbonic anhydrase inhibitor (acetazolamide). The third patient, a boy of 5 days, had central apnoeas of epileptic origin and was treated with phenobarbital. All three recovered well. If in an infant with apnoeas no paediatric explanation is found, and the child is neurologically at risk, it is advisable to make an EEG to determine if an epileptic substrate is present, even in the absence of motor phenomena.

There exist various types of epilepsy in children, which can be classified on the basis of nature and severity of the impairment of consciousness and the motor phenomena. The anamnesis is the main aid in distinguishing a seizure from a non-epileptic convulsion. Convulsions may have a number of causes, which may or may not be acute, while several factors may provoke attacks. The extent and necessity of supplementary investigation depend mostly on the child's clinical condition at presentation and on the nature of the seizure(s). The electro-encephalogram is important for the classification of the type of seizure. Anti-epileptic medication should not be started after a single epileptic seizure; whether it will be started after several attacks depends on the frequency and severity of the seizures, and on the syndrome diagnosis made. The long-term prognosis depends mostly on the syndrome diagnosis.

The first report of a positive effect of allogeneic bone marrow transplantation (BMT) on the clinical course in a patient with a lysosomal storage disease was described in 1981. Since then, over 200 patients have been treated in this way but data are scarce and fragmentary. Allogeneic BMT involves replacement of the patient's haemopoietic system by that of a donor. The new cells that repopulate the body can correct the metabolic disturbance. Most experience with allogeneic BMT was gained in patients with mucopolysaccharidosis type I, metachromatic leukodystrophy and adrenoleukodystrophy. Allogeneic BMT reduces the amount of storage material in internal organs: skeletal abnormalities and neurological symptoms are at best stabilized. Transplantation-related mortality and morbidity are high. The applicability of allogeneic BMT is limited.

Background: Conversion symptoms with a neurological character are not unusual in children. Little is known about prognosis. This study aimed to determine the outcome in a group of children with conversion., Patients and Methods: Over the period 1979-1989 35 children from 6 to 16 who met DSM-III-R criteria for conversion were selected from the case-records of the Child Neurology department of the University Hospital in Leiden. Case histories were analysed and 20 patients were interviewed at home and a standard questionnaire was used additionally., Results: Conversion symptoms disappeared with time in 15 patients. In comparison with the control group, patients with conversion scored higher on nervousness., Conclusion: Conversion disorder runs a favourable course. The place of child psychiatric treatment should be analysed prospectively.

A girl with periodic weakness since the age of two, is presented. Not until four years after the first symptoms appeared hypokalaemic periodic paralysis was diagnosed. Some aspects of this disease are discussed.