Mokken scaling and principal components analyses of the CORE-OM in a large clinical sample.

In a sample of 543 adult National Health Service (NHS) patients referred to a Psychological Therapies Service, the responses to the Clinical Outcomes in Routine Evaluation-Outcome Measure (CORE-OM) self-report questionnaire were examined using conventional principal components analysis (PCA) and a unique application of Mokken Scaling Procedure (MSP). Following the theoretical views of G. A. Foulds, it was suggested that some items more properly belong to the universe of attitudes and traits rather than that of symptoms and states. Accordingly, the analyses were carried out both with and without the CORE-OM Risk domain items. Both PCAs produced a very large first component of Psychological distress, while the small second component differs. With all items included, the second component was of Risk. With the risk items excluded, the second component was now Functioning. The MSP results, respectively, were of a five-item scale of Functioning (impaired by depression) and on the second analysis, a five-item Functioning scale (impaired by anxiety). There was discussion on the criteria for item selection, the time scale specified in questionnaire instructions and the optimum number of items required for a symptom scale. It was concluded that the CORE-OM item pool did not conform to its purported face validity domains and subdomains, but predominantly constitutes a large Psychological distress scale with considerable item redundancy.

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.

EEG results are rarely the same if repeated within six months in childhood epilepsy.

OBJECTIVE: To assess the reliability of interictal spike discharge in routine electroencephalography (EEG) testing in children. METHOD: EEG results of all children diagnosed in Nova Scotia with epilepsy onset between 1977-85 (excluding myoclonic, akinetic-atonic and absence) were reviewed. The results of the EEG at time of diagnosis (EEG1) were compared with those of a second EEG (EEG2) within 6 months. RESULTS: Of 504 children with epilepsy, 159 had both EEG1 and EEG2. EEG2 was more likely ordered if EEG1 was normal or showed focal slowing but less likely if EEG1 contained sleep (p < 0.05). EEG1 and EEG2 were both normal in 23%. If EEG1 was abnormal, there was a 40-70% discordance for the type of abnormality on EEG2. Abnormalities were present on both EEG1 and EEG2 in 67 cases. Of the 42/67 with major focal abnormalities on EEG1, 7 had only generalized spike wave on EEG2. Of the 17/67 with only generalized spike wave on EEG1, 7 showed only major focal abnormalities on EEG2. Statistical testing showed low Kappa scores indicating low reliability. CONCLUSIONS: The interictal EEG in childhood epilepsy appears to be an unstable test. A repeat EEG within 6 months of a first EEG may yield different and sometimes conflicting information.

In vitro T cell immune responses to the preS2 antigen of the hepatitis B virus envelope protein in preS2 + S vaccine recipients. Absence of cross-reactivity of subtypes at a major T cell recognition site.

The immune responses to hepatitis B virus envelope antigen were investigated in 16 vaccine recipients after immunization with a recombinant yeast-derived preS2 + S (adw) vaccine for hepatitis B virus. After the completion of the three-slot immunization series, all vaccine recipients developed antibody to the S domain and anti-preS2 antibody. In vitro proliferative responses to preS2 (120-174) peptide were demonstrated in 10 of 16 vaccine recipients. Although reactivity could be demonstrated through the length of the preS2 peptide, the principal site of proliferative activity was contained within the preS2 (146-165) region of the peptide. The principal T cell reactive site coincides with a region of significant amino acid variability of the different hepatitis B virus serotypes. Cross-reactivity with a serotype (ayw) not present in the preS2 + S vaccine could not be demonstrated at this widely recognized T cell epitope. The low level of cross-reactivity demonstrated in a limited subset of the vaccine recipients was mediated through nondominant T cell reactive sites contained in the relatively conserved preS2 (120-146) region of the molecule. The identification of widely recognized but serotype-specific T cell epitopes in the preS2 region of the hepatitis B virus envelope antigen may be an important consideration in future vaccine development.

Anti-idiotype modulation of the in vitro immune response to hepatitis B surface antigen (HBsAg) following remote infection by hepatitis B virus in man.

An antigen-inhibitable Ab-2 that exhibits internal image activity will selectively stimulate the in vitro production of anti-HBs in individuals with remotely established immunity to hepatitis B virus. This response is seen (1) in the absence of a polyconal increase in total IgG, (2) with the F(ab')2 component of the Ab-2, (3) in cultures depleted of T-cells, and (4) in the absence of stimulation by antigen. This observation demonstrates that the Ab-2-mediated stimulation of specific IgG production may be an important regulatory function in man.

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.

Benign epilepsy of childhood: a speculation and its ramifications.

Most children with generalized tonic-clonic seizures have a benign developmental disorder of seizure threshold that will be outgrown with or without treatment. Such children may have one or more infrequent seizures before adequate threshold is achieved. A small percentage of children will have frequent generalized seizures (epilepsy) due to brain damage or abnormality. Such patients require vigorous anticonvulsant therapy. However, for the children with "benign childhood epilepsy" treatment may be worse than the disease.

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.

Treatment of Fisher's variant of Guillain Barré syndrome by exchange transfusion.

A case of Fisher's variant of Guillain Barré Syndrome is presented. Treatment with exchange transfusion coincided with clinical improvement. This is the first case of Fisher's variant treated in this manner.