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The aim of this study was to observe any changes in cardiac and respiratory function that occur during seizures. Thirty-seven children (20 males, 17 females; median age 7y 6mo, range 1y 6mo to 15y 6mo) were studied. We recorded electroencephalograms, respiratory rate, heart rate, electrocardiograms, blood pressure, oxygen saturation, heart rate variability (time domain analysis), and cardiac vagal tone. A respiratory pause was defined as an interruption in respiration lasting more than 3s but less than 15s. Apnoea was defined as absence of respiration for more than 15s. Tachypnoea was defined as a 10% increase in respiratory rate from the pre-ictal baseline. Bradypnoea was defined as a 10% decrease in respiratory rate from the pre-ictal baseline. Significant hypoxia was defined as a saturation of less than 85%. A significant change in heart rate was taken as a 10% increase or decrease below the baseline rate. Data were obtained from 101 seizures: 40 focal seizures, 21 generalized seizures, and 40 absences. Focal seizures were frequently associated with significant respiratory abnormalities, tachypnoea in 56%, apnoea in 30%, frequent respiratory pauses in 70%, and significant hypoxaemia in 40%. The changes seen in respiratory rate were statistically significant. Changes in cardiac parameters, an increase or decrease in heart rate, were observed in only 26% of focal seizures and 48% of generalized seizures. We conclude that seizure activity can disrupt normal physiological regulation and control of respiratory and cardiac activity.

Somatosensory evoked potentials (SSEPs) are a very sensitive measure of the functional integrity of the neuroaxis, including peripheral and central structures. When used in diagnostic mode they can provide additional information regarding the probable areas of dysfunction. SSEPs were recorded from 44 children (64 feet with congenital talipes equinovarus, CTEV), between the ages of 2 to 15 years, who had structural CTEV deformity previously treated by surgery, with no clinical evidence of neurological deficit. SSEPs were elicited after sequential and bilateral stimulation (0.1 ms/5 Hz/10 to 20 mA) of the posterior tibial nerve and the common peroneal nerve and were recorded cortically (P40). In half the children, additional recordings were conducted at the knee (N5), the first lumbar spinous process (N14), and the seventh cervical spinous process (N20). Eighteen children had abnormal responses, four children had non-reproducible responses, and 22 children had normal responses. Analysis of the data at different levels of the nervous system showed that eight children had abnormality at the spinal level. The surgical outcome was influenced by the neurological abnormality, with an excellent or good outcome in 34 of 36 feet with normal neurology and 19 of 28 feet where a deficit was present (p<0.05). These findings support the neurological theory as an etiological factor in CTEV deformity.

The electromyographic (EMG) responses to tendon taps at the ankle and ensuing muscular twitch forces and temporal parameters were studied at varying angles across the joint range in 18 children, aged 3 to 14.9 years, with congenital hemiparetic cerebral palsy and 22 healthy (control) children, aged 3 to 13.6 years. Those subjects with hemiparesis were community ambulators without assistance. In all subjects, passive muscle stretch caused a waxing of the reflex EMG and twitch force near neutral (with the sole of the foot at right angles to the tibia) and a diminution of these with further dorsiflexion. Twitch times increased with each dorsiflexing increment, being slowest at maximum dorsiflexion and fastest at the resting plantarflexion angle. Heterogeneity of the hemiparetic-limb data is evident when compared with data of non-paretic and unaffected limbs, with clear differences in the clonic (fast twitch) as opposed to non-clonic (slow twitch) muscles. In four cases with clinical clonus, clonus frequency was reduced by passive dorsiflexion. Plaster immobilization for 1 month produced clonus which was previously absent in one subject, and caused a fast-twitch phenotype to emerge in two subjects. Follow-up after heel-cord lengthening in one subject showed that clonus frequency diminished from 9 to 3 Hz with slowing and strengthening of muscle-twitch phenotype. Short- and long-term peripheral manipulations appear to regulate neuromuscular excitability according to whether muscles are loaded or unloaded. Although damage to the nervous system provides the setting for reflex excitability, the data suggest that the muscle length (which specifies the joint angle) and the muscle-twitch phenotype of any given limb for any given case appear to dictate the actual speed and strength of reflex muscle-twitch and clonus profiles. This study illustrates how peripheral manipulations of muscles and tendons may alter the expression of what have hitherto been considered as exclusively central phenomena.

A series of 25 children, 13 females and 12 males, who had an acquired communication disorder together with epilepsy, but did not fulfil the strict criteria of the Landau-Kleffner syndrome, was studied. All children had a clinical neurological evaluation, speech and language assessment, an awake and sleep EEG, cranial MRI, SPET scan, and audiometry. Clinical seizures were most often polymorphic in type (17 of 25). Atypical absences were the commonest individual seizure type occurring in 15 cases. All patients had an unequivocal epileptiform EEG. Normal sleep phenomena were only observed in 10 cases, enhancement of epileptiform activity in sleep was seen in 16. Cranial MRI was abnormal in six and normal in 19 cases. The SPET scans were abnormal in 22 of 25 children. The language deficits were classified neurologically as receptive aphasia, 24 of 25; expressive aphasia, 20 of 25; nominal aphasia, eight of 25; articulatory dyspraxia, 10 of 25; and auditory agnosia, nine of 25. It is hypothesized that the loss of communication skills is due to an encephalopathy secondary to the persistent epileptic discharge and manifests as a hypometabolic area on the SPET scan.

Adrenocorticotrophin hormone (ACTH) has been used as an anticonvulsant for many years. In this paper, the use of ACTH in 23 children with intractable epilepsies is described. It was found that ACTH worked most effectively when the EEG showed benzodiazepine sensitivity. A mechanism of action of ACTH is proposed.

The influence of the joint angle on stretch reflex excitability of the soleus muscle at the ankle has been studied in 22 children aged 3.9 to 13.6 years and 9 adults aged 19 to 70 years. For all subjects, reflex EMG and mechanical twitch torque gain were trivial at resting plantar flexion. The reflex EMG gain reached a maximum between -15 degrees and -10 degrees of plantar flexion beyond the neutral angle, 0 degrees, defined as the foot at right angle to the tibia, diminishing steeply with further dorsiflexion. The reflex mechanical gain rose to a peak between 0 degrees and +10 degrees of dorsiflexion beyond neutral, declining steeply thereafter. By contrast, axonally stimulated muscle twitch torque increased serially up to +30 degrees dorsiflexion beyond neutral. For the soleus muscle, the optimal reflex neuromechanical angle lies approximately midway between the angle for optimal reflex EMG gain (in mild plantar flexion, at which the largest and strongest motor units can be activated) and the optimal muscle mechanical angle (at the extreme of soleus muscle dorsiflexion). These studies confirm that the excitability of the spinal alpha motor neuron pool in vivo is strongly influenced by muscle length and explain the variability in reflex excitability within and between subjects, if the joint angle is not controlled. They also indicate how posture influences movement, agreeing with the known function of the soleus muscle in the stance phase of gait and the modulation of motor unit recruitment during voluntary alternating movements at the ankle. Soleus muscle twitch characteristics show a fivefold to eightfold increase in peak force associated with a tenfold reduction in compliance in the first two decades of life and an apparent speeding up of twitch time in the first decade.

Benzodiazepines are routinely used by the rectal route for the treatment of acute epileptic seizures: if a benzodiazepine was absorbed from nasal administration this could provide a more acceptable alternative to rectal administration. Nineteen children (age range 7 months to 14 years) with intractable epilepsy were chosen. The EEG's showed unequivocal epileptic activity persisting during the recording. The midazolam was dripped slowly into the anterior nares. Fifteen had a positive response, a dramatic improvement in their EEG or cessation of fits. Drug induced beta activity occurred in 14 children. The mean time to appearance of beta activity was 111.5 secs (SD = 95.3 secs). The reduction in spike count pre and post midazolam was statistically significant (p < 0.01). The improvement in EEG background was also statistically significant. Midazolam is absorbed via the i.n. route. With the dosages used it suppressed epileptic activity and produced an improvement in EEG background. The children and parents found the method acceptable. This is the first study to use the i.n. route for anti-convulsant drugs.

The speed of alternating movements at the ankle, metacarpophalangeal and wrist joints in 11 healthy children and 13 adults doubled between age 3 and 11 years, despite a 32-fold increase in limb-segment inertia produced by the doubling in limb length over the same period. The data for the children showed little or no practice effect. The speeds for the adults, though faster than those for the children, were more widely dispersed, indicating the possibility that training might increase the speed of the slowest adult. The findings are consistent with a previous report demonstrating a parallel increase in the speed of calf muscles over the first 10 years of life and it is inferred that the increase in dexterity at the wrist and metacarpophalangeal joints also depends on an increase in muscle speed with age. Muscle maturation may impose a rate-limiting envelope for all motor tasks which is particularly evident in rapidly alternating movements. These findings have implications for training in sport and music and for the understanding of motor delay, clumsiness and speech difficulties.

Disability rates among low-birthweight infants, particularly those related to congenital abnormality and cerebral palsy, are high. Both prenatal and perinatal factors are likely to be involved in the aetiology of most types of disability. IQ tends to be lower among low-birthweight infants, but does not appear to be closely related to birthweight alone. The confounding effect of social class should be considered when assessing aetiology and outcome. The long-term outcome for the increasing number of low-birthweight infants who survive and receive intensive neonatal care requires to be continually assessed; however, studies should not be confined to the very- and extremely-low-birthweight infant requiring prolonged intensive care, but should include abortions, stillbirths and neonatal deaths. As disability in survivors can relate to preterm birth but not perinatal complications, all low-birthweight infants require to be studied if selective bias is to be solved.

The effect of electrical stimulation of the anterior tibial muscles of children with hemiplegic cerebral palsy was studied. 10 children received electrical stimulation, applied by their parents daily for an hour for 35 days; they were compared with 10 matched controls. Active and passive ranges of movement of the ankle, and knee and ankle motion during walking were measured before and after therapy using electrogoniometers. The results showed a significant increase in passive range of movement among children receiving electrical stimulation. Gait analysis of knee and ankle motion showed little change.

A clinical method for measuring the stretch reflex threshold and gain of muscles acting across the ankle joint in children with congenital hemiplegia is described. The stretch reflexes of all limbs were velocity-dependent. Hemiparetic limbs were not necessarily spastic compared with non-paretic limbs, suggesting that the term 'spastic hemiplegia' should be used more selectively and emphasis be placed on the heterogeneity of the hemisyndromes of childhood. Abnormal motor control, planning and dexterity, the hemipostures and plastic (non-electrical) muscle changes may be more important measures of impairment than reflex excitability. A general scheme for assessing reflex excitability and available treatments applicable to all types of cerebral palsy is proposed.

The quadriceps and hamstring muscles of 13 children with hemiplegic cerebral palsy were studied using ramp and sinusoidal stretches at three different muscle lengths. Overall, the hamstring muscles showed greater velocity sensitivity than the quadriceps, with the hamstrings having the lowest reflex velocity and frequency thresholds close to maximum knee extension. At this position, the hemiparetic hamstrings alone showed a weakly significant reduced reflex frequency threshold compared with non-paretic muscles. For all other muscle lengths, non-paretic and hemiparetic muscles displayed similar reflex thresholds when subjected to sudden, discontinuous or repetitive rhythmical, sinusoidal stretches. The number of muscles for which a reflex threshold could be established declined progressively as the angle at the knee joint approached 90 degrees of flexion. Muscles for which no reflex threshold could be demonstrated had similar velocities and frequencies of stretch as those in which a reflex was obtained.

A detailed clinical examination of 24 hemiplegic children at a mean age of 9.9 years showed that nine had an obligate toe-strike when walking at a self-selected speed and this was maintained throughout stance. None of the 24 children had a fixed equinus. No association could be found between this pattern of gait equinus and lower-limb atrophy, reduced ankle-joint range, muscle extensibilite, power of dorsiflexors and plantarflexors or actual muscle imbalance at the ankle joint. Gait equinus was independent of reduced compliance of the calf muscles, of a clinical diagnosis of tonic spasticity, of fine-motor dexterity of the toes, and of the side of the hemiplegia. Gait equinus cannot be explained merely in terms of a central paralytic foot-drop. A developmental model of equinus is advanced.

Equinus in hemiplegic children is multifactorial. In some cases it is due to a short muscle, in others to simple foot-drop, tonic spasticity, rigidity, compensation for a short limb, fixed flexion contracture at the hip, dominantly inherited forefoot deformity, forefoot equinus secondary to chronic toe-walking, or abnormalities of the visco-elastic properties of the muscle, with true intramuscular contracture. This neurophysiological study confirms that hemiplegia in children is not a homogeneous condition. Some have tonic spasticity; some, although stiff, show electrical silence on stretching; some appear to have a short muscle, with no hypertonicity; and others have hypertonicity in relation to position (i.e. rigidity). A short muscle is not always associated with tonic spasticity with reciprocal inhibition. Weakness can occur without spasticity. Speed of movement of toes, ankle and hip is also significantly reduced.

Problems in the diagnosis and management of children who present with an acute encephalopathic illness are examined. The aetiology, pathogenesis, pathology and clinical features of acute toxic encephalopathies are discussed, together with the differential diagnosis of those encephalopathies which are not induced by toxicity. Guidance is given on measures for the reduction of raised intracranial pressure and the management of defects of homeostasis. It is essential that facilities for intensive monitoring of these children be available during the acute phase of illness in order to reduce the number surviving with sequelae of mental and physical handicap.

An infant with antenatal diagnosis of intracranial bleeding from the choroid plexus is reported; diagnosis was by ultrasound. Postnatally the site, extent of the bleed and hydrocephalus were verified by ultrasound, and Doppler flow studies showed that cerebral blood-flow was compromised. A ventriculostomy was performed and intracranial pressure was monitored and relieved; removal of cerebrospinal fluid pending insertion of a ventriculo-peritoneal shunt was monitored by daily ultrasound. Thus the management of every stage of this infant's treatment depended on ultrasound.

Migraine is one of the most common disorders seen in paediatric neurological practice. It may present with an obvious diagnosis of 'classical migraine' or it may mimic acute neurological disease. It may require extensive investigation and, before the more classical full-blown features of migraine appear, there can be a worrying period of follow-up for the physician, wondering whether he has missed some serious pathology--especially in cases of hemiplegic and ophthalmoplegic migraine. Differentiation from epilepsy, particularly of psychomotor type, may be impossible. Migraine frequently presents as a stress reaction to school failure. If this underlying cause is unrecognised and uncorrected, the response to medication will be poor.

Twenty-five children with hemiplegic cerebral palsy between the ages of five and 15 years were examined in more detail, using a torque generator motor. A significant reduction in distal power, fatiguability and speed of movement was found on the hemiplegic side. Proximal power was relatively well preserved. Muscle tone, as would be expected, was significantly increased on the affected side and this was demonstrated by a significant rise in measured resonant frequency. Phasic spasticity in response to stretch was found on EMG to be much more common than phasic and tonic combined. Hand function correlated well with the loss of distal power and speed of movement. There was wide individual variation in the relationship between increased muscle tone and hand function. Thixotropy was normal in spastic muscles. Use of the unaffected hand had a marked effect on muscle tone and EMG of the affected side.

Cerebrospinal fluid (CSF) markers of cerebral energy depletion were measured in 32 infants and children following short (less than 10 minutes) febrile convulsions, and in 19 controls. Specific and sensitive indices of high-energy phosphate compound depletion (hypoxanthine, xanthine and uridine) showed no marked changes. Values for patients and febrile controls were significantly higher than for afebrile controls, which is consistent with increased cerebral metabolism in febrile patients. There were no differences in pH, lactate or creatine kinase levels in the CSF of patients and controls. The results suggest that short febrile convulsions are benign and that in the absence of risk factors for the subsequent development of epilepsy, prophylactic anticonvulsant treatment is not indicated.

The authors studied specific and sensitive indicators of neuronal adenosine triphosphate (ATP) depletion--hypoxanthine, xanthine and uridine levels--in the cerebrospinal fluid (CSF) of nine children during non-convulsive status epilepticus. No evidence of ATP depletion was found and CSF pH and creatine kinase levels were similar to those of controls. Hypoxanthine, xanthine and uridine had a tendency to be low, but this was significant only for xanthine. The authors speculatively link this reduction to a reduction in neuronal protein synthesis. This might be a mechanism whereby non-convulsive status epilepticus could lead to intellectual deterioration and dementia.

Five children are described who developed hypertension in relation to acute neurological disease. Possible pathophysiological mechanisms for the hypertension are considered. It is thought that the hypertension may have been related to interruption of the ascending tracts in the brain stem, leading to failure of integration between, or independent action of, the baroreceptors and osmoreceptor system. Management and treatment are discussed. Diazoxide (5 mg/kg bodyweight, given intravenously) appears to be the most effective drug for use in the acute episodes.

The asymmetrical deformities in 20 children with various types of cerebral palsy are compared with 20 children without cerebral palsy who have the so-called 'squint' baby syndrome (asymmetrical deformities of plagiocephaly, unilateral bat ear, facial and thoracic asymmetry, pelvic obliquity and apparent shortening of one leg). It is suggested that the 'squint' baby syndrome and the 'windswept' child syndrome in children with cerebral palsy are stages of the same syndrome and that in both the deformities are caused by the effect of gravity on an immobile growing child, rather than spasticity or muscle imbalance. Asymmetrical deformity should therefore be amenable to physiotherapeutic intervention, rather than trying to modify maturation of the damaged brain. As the 'windswept' cerebralpalsied child can develop some of the most severe deformities seen in cerebral palsy, it is important that asymmetrical deformities should be prevented.

Cerebrospinal fluid (CSF) myelin basic protein (MBP) was measured blind by double antibody competitive inhibition radioimmunoassay (RIA) in 20 children who had seizures and 17 children with hydrocephalus. MBP values correlated with clinical outcome and mean maximum intracranial pressure (ICP) in the hydrocephalic group, and with type of convulsion in the epileptic group. A value of 20ng/ml or more was regarded as significantly raised. A significant rise in MBP levels could be demonstrated in those with ICP alone and in patients with additional problems, whose levels tended to be even higher. Hydrocephalic children with normal ICP and children with seizures had similar normal MBP levels, and in the latter group clinical outcome was not related to MBP levels. For individual patients CSF MBP is of little value as a prognostic indicator, or as a method of quantifying cerebral damage.