Reflections on the history of the Canadian Association for Child Neurology.

The Canadian Association for Child Neurology (CACN) was founded in June 1971 to combine neurologists interested in children and paediatricians interested in the nervous system into an organization which would promote the development of this subspecialty. Initially, the members of the Association mostly wished to have a training programme under the combined supervision of University Departments of Paediatrics and Neurology. However, under the influence of the Royal College of Physicians & Surgeons of Canada, and its Committee on Neurology, the training of child neurologists was organized in a manner analogous to that of neurologists for adults, though with an initial one or two years of paediatrics instead of medicine. By 1975, four years within a recognized neurological training programme could lead to the Certification Examination in Neurology, as modified for paediatric neurology. In 1981, the CACN also joined the Canadian Congress of Neurological Sciences. It has played an increasing part in child care and also in a academic studies. However, evidence will be presented to show that the present number of paediatric neurologists in Canada is insufficient. The number of trainees also appears inadequate, and increased funding for training positions is needed. Close cooperation between paediatric neurologists, rehabilitation experts, developmental paediatricians and related subspecialists is required.

Changes in serum anticonvulsant levels with febrile illness in children with epilepsy.

Changes in anticonvulsant serum levels during intercurrent illness may cause toxicity or decreased seizure control in children with epilepsy. We studied prospectively the effect of intercurrent illness and its treatment in 111 children being treated with AC monotherapy. Free fraction and total serum AC levels were determined when the child was well, on the fifth day of any illness with fever and one month after recovery. There were 55 episodes of febrile illness in 39 children during the study period. Twelve illnesses were associated with significant increases or decreases in serum AC levels; 7 children became clinically toxic; 1 child had increased seizures during illness. The mechanisms of AC level changes appeared to include interaction with antibiotics, with antipyretics or with viral illness. Amoxycillin and acetaminophen did not appear to interact with the AC's used. Physicians caring for children with epilepsy should be aware of the frequency and complexity of potential interactions between intercurrent febrile illness and anticonvulsant medication.

The Proteus syndrome: the Elephant Man diagnosed.

Sir Frederick Treves first showed Joseph Merrick, the famous Elephant Man, to the Pathological Society of London in 1884. A diagnosis of neurofibromatosis was suggested in 1909 and was widely accepted. There is no evidence, however, of café au lait spots or histological proof of neurofibromas. It is also clear that Joseph Merrick's manifestations were much more bizarre than those commonly seen in neurofibromatosis. Evidence indicates that Merrick suffered from the Proteus syndrome and had the following features compatible with this diagnosis: macrocephaly; hyperostosis of the skull; hypertrophy of long bones; and thickened skin and subcutaneous tissues, particularly of the hands and feet, including plantar hyperplasia, lipomas, and other unspecified subcutaneous masses.

Asymptomatic children with epilepsy: little benefit from screening for anticonvulsant-induced liver, blood, or renal damage.

In a prospective study of 199 asymptomatic children receiving anticonvulsant drugs, we evaluated routine screening for liver, renal, and hematologic toxicity. Urinalysis, CBC, differential, platelets, SGOT, bilirubin, and alkaline phosphatase (plus amylase and fibrinogen in patients taking valproate) were obtained between 0 and 24 months. Compliance was excellent. Urine was always normal. Six percent of patients had transient minor abnormalities of blood studies necessitating rechecks. All were normal on repeat. Two children had major blood abnormalities recognized, leading to drug changes that proved to have been unnecessary. Baseline determinations and prompt attention to symptoms of toxicity should replace routine screening of blood and urine.

Further delineation of Weaver syndrome.

Seven new cases of Weaver syndrome are described, including the first reported case in an adult. Overgrowth is usually but not always present. The combination of characteristic facies and developmental delay, with the peculiar radiographic findings of accelerated dysharmonic osseous maturation and splaying of the distal long bones, is diagnostic of Weaver syndrome.

Dominant recurrent ataxia and vertigo of childhood.

Four families are described with an autosomal dominant illness characterized by the childhood onset of recurrent attacks of prolonged ataxia, server vertigo, and vomiting. The attacks often begin in infancy. On the average, attacks occur monthly, and last between one hour to more than a week. Variations in severity occur within families. During an attack, consciousness is unaltered, but severe vertigo makes walking impossible and vomiting is frequent and severe. An attack is marked by horizontal and vertical jerk nystagmus, accompanied by vertigo which is sometimes worsened by position; however, there is no muscular weakness. During an attack, blood gases, ammonia, and amino acid studies are normal. Between attacks patients manifest combinations of slight horizontal or vertical jerk nystagmus or mild clumsiness. Cochlear and labyrinthine studies and neurologic investigations were noncontributory. Conventional therapies for vertigo, epilepsy, and migraine were ineffective, but acetazolamide (250-500 mg/day) stopped the attacks.

Epilepsy after a first unprovoked seizure in childhood.

One hundred sixty-eight children with an initial afebrile, unprovoked seizure were identified from a regional EEG laboratory. This case-finding method seemed justified because 86% of regional physicians indicated they order an EEG after a first seizure. Clinical information and recurrence rate were determined from records and telephone calls. Eighty-one percent had been seen by a pediatric neurologist. Overall, 51.8% recurred, and of those with a recurrence, 79% had additional seizures. Recurrence rates were highest in those with abnormal neurologic examination, focal spikes on EEG, and complex partial seizures. The lowest rates of recurrence followed a generalized tonic-clonic seizure, with normal EEG and normal neurologic examination. Prescription of anticonvulsants did not alter the recurrence rate.

Newly treated childhood epilepsy: a prospective study of recurrences and side effects.

Eighty-two newly diagnosed children were started on anticonvulsant therapy and followed prospectively for 12 to 36 months. Compliance was excellent. However, 41% had a recurrent seizure within 6 months of starting therapy despite "adequate" serum levels. Recurrences were least frequent in those with generalized tonic-clonic seizures or those with a single seizure plus epileptiform EEG before treatment. Toxicity screening led to unnecessary drug change in two children. Seven other children had behavioral side effects requiring drug change.

The changing picture of pseudotumor cerebri in children.

Among 38 children with pseudotumor cerebri only 3 cases were due to ear disease or its complications. The commonest cause was a refeeding syndrome either due to nutritional deprivation or as an early finding in the treatment of cystic fibrosis. It is uncommon to find the cause of pseudotumor in older children but in those under 6 years the cause was found in 85%. Because of the adverse effects of steroids we use this treatment in the more resistant cases.

Evoked potentials during recovery from blindness recorded serially from an infant and his normally sighted twin.

Visual evoked potentials (VEPs) elicited by diffuse field flashes wee recorded from a behaviourally blind infant with his twin as control. The patient was tested at ages 4, 5, 6, 8, 10 and 15 months. In spite of his behavioural blindness, clear VEPs were recorded from the patient at age 4 months, although the wave form was monophasic as contrasted with the multiphasic wave form recorded from his twin at the same age. Latency to first deflection and to first peak were considerably longer for the patient. The patient's VEP wave forM grew progressively more complex with age, paralleling recovery of useful vision. However, the VEP development anticipated behavioural recovery.

Dominant benign neonatal seizures.

Three families suffering from benign neonatal seizures are described, and the value of recognizing the condition in order to offer realistic reassurance to the family is stressed. Although it is uncommon, the rarity of its description in the literature probably does not reflect the true incidence of this dominantly inherited condition, in which there is an increased risk of the latter development of epilepsy.

The functions and the training of a paediatric neurologist.

A total of 2018 office referrals of children with neurological problems is compared with 635 adult neurological referrals in the same Canadian city. Data of children and adults admitted to hospital with neurological problems are also compared. It is suggested that the different types of caseload encountered justify changes in the training programs for paediatric neurologists.

Acute hemiplegia of childhood.

Acute hemiplegia of obscure cause occurred in 28 children: 13 had had prolonged seizures and a high temperature (considered to have been the direct cause of the brain damage); 5 had had brief seizures, a lower temperature and a depressed level of consciousness; and 10 had a nonfebrile onset of hemiplegia and were found to have vascular abnormalities. Most of the first group were retarded and epileptic at long-term follow-up, as were about half of the second group, whereas children in the third group were of normal intelligence and epilepsy was uncommon among them. Hemiplegia persisted at follow-up in most of the children in each group, the proportion being at least in the third group; if cerebral angiography had demonstrated carotid stenosis or occlusion there was usually poor recovery from the hemiplegia. Bilateral changes on plain skull films or pneumoencephalograms were associated with mental retardation. Failure to control prolonged seizures accompanied by a high temperature predisposes to brain damage; therefore, early and vigorous management is essential.