How, as medical professionals, do we improve our skills so that we can enhance the life of our patients with developmental concerns? What is the best use of our learning time? Is our knowledge going to be as helpful to our patients as that which they acquire themselves? Do we learn more from observation and reading, or attending meetings? These are questions you may ask yourself when you pick up each issue of this journal.

This study investigated the accuracy of prediction of neurodevelopmental outcome at 1 year using cerebral proton magnetic resonance spectroscopy (MRS) and structured neonatal neurological assessment in term infants after presumed hypoxic–ischaemic brain injury. Eighteen control infants and 28 infants with presumed hypoxic–ischaemic brain injury underwent proton MRS investigation. Studies were carried out as soon as possible after the cerebral insult, most within 48 hours. Infants had an early structured neurological assessment at a median of 19 hours (range 0 hours to 9 days) from the presumed hypoxic–ischaemic insult and a late assessment at a median of 7 days (range 3 to 25 days) during recovery. The maximum cerebral peak–area ratio lactate:N-acetylaspartate measured by proton MRS accurately predicted adverse outcome at 1 year with a specificity of 93% and positive predictive value of 92%. Neurological assessment had a tendency for false-positive predictions. However, both early and late neurological examination can be used as a reliable indicator for a favourable outcome at 1 year having negative predictive values of 100% and 91% respectively.

The aim of this study was to examine children with myelomeningocele (spina bifida) who were investigated according to the level of neurosegmental lesion and to determine the number who walked and their average age at commencement/cessation of walking. The data have been collected since 1978 at the Royal Children's Hospital, Melbourne during annual evaluations of patients with spina bifida and high-level paralysis. After screening for established criteria and assigning to a neurosegmental lesion level, 173 children were examined for their ability to walk, with or without aids, in a community setting. Thirty-five children had lesions at the thoracic level, and seven walked at a mean age of 4 years 6 months. Three children with a lesion at the thoracic level ceased walking at a mean age of 7 years 6 months. Ten children had lesions at the high-lumbar level (L1/2), and five walked at a mean age of 5 years 2 months. Three children who had lesions at the high-lumbar level ceased walking at a mean age of 6 years 11 months. Fifteen children had lesions at the mid-lumbar level (L3) and nine walked at a mean age of 5 years. Three ceased walking at a mean age of 7 years. Forty-five children had lesions at the low-lumbar level (L4/5) and 38 walked at a mean age of 3 years 10 months. Five ceased walking at a mean age of 9 years 1 month. Sixty-eight children had lesions at the sacral level; all of whom walked at an average age of 2 years 2 months. None had ceased walking by the end of the study period. Delay in achieving ambulation can be expected in all children with spina bifida, including those with low neurosegmental level lesions. Furthermore, some children with high lesion level, given the opportunity, might be expected to ambulate effectively in the community but may cease walking after 3 to 4 years of this activity, which is earlier than previously recorded.

Secondary tethering of the spinal cord in people with myelomeningocele (MMC) is almost universal but there is a relatively low incidence of the secondary tethered cord syndrome (S-TCS). In view of this, we wish to explore the notion that cord pulsation, as demonstrated by ultrasound of the spinal cord, can become an independent measure for increased tension of the spinal cord in patients with MMC. One-hundred and six patients with MMC underwent ultrasonographic examination of their back, a thorough physical examination, and a careful review of their clinical history. Of these patients, 53 had MRI of their back. Cord pulsation was graded and the statistical relation between pulsation and various clinical and MRI findings was analysed. Age and arm span were not correlated with grades of cord pulsation. The absence of cord pulsation correlated with the presence of symptoms but this relation may be explained by MRI findings of certain structural cord malformations and not by tethering per se. Assuming that cord pulsation is a reliable marker for the tension within the spinal cord, the results of our study suggest that overstretching of the cord is not the main cause for the development of the S-TCS.

Cutaneomuscular reflex (CMR) responses were recorded from lower-limb and trunk muscles in 27 subjects with cerebral palsy (CP) (spastic, 21; athetoid, six) and in neurologically healthy (control) subjects, aged 3 to 15 years, while standing. In the 21 subjects with spastic CP, but not in the six subjects with athetoid CP, CMR responses were more widely distributed between ipsilateral lower-limb and trunk muscles compared with age-matched control children. CMR responses in older subjects with CP were similar to younger control subjects, lacking supraspinally mediated, long-latency components. Short-latency, spinally-mediated, excitatory CMR components were seen simultaneously in pairs of distal, antagonistic lower-limb muscles in half of the subjects with spastic CP, but in none of the control children. In subjects with spastic-type CP, the abnormal reflex responses indicate disordered spinal and supraspinal inputs to motor neurones, although there was no convincing correlation between these responses and the severity of spasticity.

The common synaptic drive shared between two groups of motor neurones synchronizes the timing of discharges between the motor-neurone groups. Recordings were made of motor-unit discharges during cocontraction of ipsilateral pairs of thumb muscles in eight subjects with cerebral palsy (CP) aged 4 to 13 years and eight neurologically healthy subjects aged 4 to 12 years, and in pairs of lower-limb muscles in 21 subjects with CP and 21 control subjects, both aged 3 to 15 years. Common synaptic drive, likely to be derived at least partly from activity in branched corticospinal-tract neurones, produced motor-unit synchronization between pairs of thumb muscles in control subjects but was absent in all subjects with CP. Motor unit synchronization was not found between lower-limb antagonist muscles that cocontract abnormally in CP, nor was synchronization present in more widely separated muscle pairs. Therefore, abnormal patterns of muscle activation and more widespread muscle reflex responses do not result from an abnormal distribution of common synaptic drive in CP.

A 4-year follow-up study of academic achievement in children aged between 11 and 17 years with epilepsy or asthma was carried out to identify differences between the two samples and to identify change in achievement over time. Differences based on sex and seizure severity also were explored. There were 98 subjects in the group with epilepsy and 96 subjects in the group with asthma. Academic achievement in five areas (Composite, Reading, Mathematics, Language, and Vocabulary) was measured using school-administered group test scores. To explore change over time in condition severity, each child was categorized as having a low or high condition severity at baseline (time I) and again 4 years later, resulting in four groups: low–low, low–high, high–low, and high–high. There were too few cases in the low–high group to be included in the analyses. Data were processed using analysis of covariance (ANCOVA), intraclass correlation coefficients, and paired t tests. At follow-up the children with epilepsy continued to perform significantly worse in all five achievement areas than the children with asthma. Children with either inactive or low-severity epilepsy had mean scores comparable to national norms; those with high seizure severity had mean scores ranging from 3 to 5 points below national norms. No changes were found in academic achievement over time for either sample, even among those whose conditions improved. Although boys with high-severity epilepsy continued to have the lowest achievement scores, there was no trend for them to decline in achievement over time.

The aim of this study was to examine the haemodynamic response to seizures in three infants with Sturge–Weber syndrome by measuring regional cerebral blood flow using transcranial Doppler sonography and 99mTc HMPAO SPECT. Time-locked video/digital EEG recording was carried out for ictal studies. MRI was performed in all subjects. SPECT showed hemispheric hypoperfusion interictally in all three patients and also ictally in one of the three; a small region of hyperperfusion was seen on the same ictal scan in the latter, ie. the patient with interictal and ictal hypoperfusion. In the two older children middle cerebral artery velocity (MCAV) was reduced by between 29 and 62% in the middle cerebral artery of the predominantly affected hemisphere compared with the contralateral side. During seizures, increases of 6 to 30% in MCAV were recorded for the clinically seizing hemisphere compared with 24 to 170% for the contralateral side in four of the seizures recorded. In one infant, MCAV fell bilaterally during a seizure that generalized (−18 and −43% in the predominantly affected and contralateral side respectively). Sequential recordings in one infant suggested that, with time, the haemodynamic response to seizures of the unaffected hemisphere may decrease. These findings suggest that the venous malformation in SWS is associated with an impairment of the cerebral haemodynamic response to seizure activity.

Bilateral periventricular nodular heterotopia (BPNH) is a neuronal migration disorder, characterized by grey-matter cellular rests in the periventricular regions. Patients frequently present with seizures during childhood or later in life. Monozygotic twin sisters were found to have enlarged head circumferences (>95th centile) during a routine medical visit at 6 months of age. A brain CT scan revealed grey-matter cellular rests in the subependymal regions extending to the subcortical white matter. In some areas these cell aggregates had nodular margins, consistent with the diagnosis of BPNH. At 6 years of age they are cognitively and neurologically normal. They have not had any seizures and their heads continue to grow along the 95th centile.

We describe an 18-month-old boy with insulin-dependent diabetes mellitus who developed idiopathic myoclonic encephalopathy (dancing eye syndrome) at 26 months of age. The neurological symptomatology (multifocal myoclonus, opsoclonus, ataxia, behavioural disturbance) developed within 10 to 14 days after presentation. Biological, neuroradiological, and scintigraphic examination excluded CNS infectious diseases, intoxication, or tumours. At onset of diabetes mellitus, anti-glutamic-acid decarboxylase (GAD) antibodies were observed, and markedly increased in titre when myoclonic encephalopathy occurred. Corticosteroid treatment resulted in a decrease in anti-GAD autoantibody titres and the disappearance of neurological disturbances. As GAD is expressed both in pancreatic β-cells and cerebellar Purkinje cells, it is possible that a common autoimmune disorder in this patient may account for both the diabetes and myoclonic encephalopathy.

Lerner, a dermatologist who isolated melatonin (MLT) in 1959, was the first to report on its hypnotic properties, and was also the first to self-administer this hormone. Later, in 1971, Anton-Tay and coworkers injected MLT into volunteers who quickly fell asleep. In adults, MLT treatment appears to have beneficial effects in those who have sleep disorders due to delayed sleep onset, shift work, and jet lag. MLT can enhance adaptation to forced phase shift in simulated shift-work and jet-lag conditions. However, better controlled, long-term studies are still required. MLT may also benefit certain types of adult insomnia.

Although no references can be given, the following experience is firmly established in the author's memory as a true account, and has certainly always focused his interest on the possible diagnosis of familial temporal epilepsy. Many years ago on a tour of Longleat House, Wiltshire, a guide pointed out a painting of a woman in an advanced stage of pregnancy. Apparently she had asked to be painted at that time because she was sure that she would die in childbirth (which, in fact, she did). The guide added that this woman was also well known for meeting herself in the passages of Longleat House. A few years later, when working in London as a neurological registrar, the author was reading a newspaper when he noted a paragraph describing the conversion of Holland House, London. A number of paintings from the house were described, including one of a woman, again well known for seeing an image of herself in the passages of this building. It eventually transpired that these women were sisters; and there seemed to be a possibility that they both suffered from heautoscopic hallucinations, in which sufferers see themselves carrying out some kind of action, and which are known to occur during complex partial seizures arising in the temporal lobes.