Quantitative electromyography in babies and young children with primary muscle disease and neurogenic lesions.

The technique of quantitative electromyography suitable for babies and young children is reviewed. The findings in 22 children with myopathic disease and 21 with neurogenic disease have been compared with the results from 25 children with no evidence of neuromuscular disease (the Apparent control group). Children with primary muscle disease had low Mean Ratios of mean amplitude/turns per second whereas those with neurogenic lesions had high Mean Ratios. The most abnormal results were found in children with muscular dystrophies and anterior horn cell disease.

Quantitative electromyography in babies and young children with no evidence of neuromuscular disease.

A technique of quantitative EMG suitable for use in babies and young children is described. No specific level of force is required and portions of EMG as short as 100 ms are suitable for analysis. A specially modified Medelec APA6 action potential analyser converted the EMG into two pulse trains representing amplitude pulses and turns pulses with a display on the CRT screen. The ratio of the mean amplitude/turns per second was determined for between 15 and 20 different portions of EMG to produce a Mean Ratio of mean amplitude/turns per second. Ninety-seven children were investigated for neuromuscular disease. Twenty-two had a primary muscle disease, 21 a neurogenic process and 25 no neuromuscular disease. This last group was called the Apparent control group. The problem of obtaining control values is discussed. A 5-year follow-up study found no change in the clinical diagnosis of the patients in the 3 groups. Numerical limits for the Mean Ratio of mean amplitude/turns per second for the Apparent control group are defined.

Paratuberculosis in farmed red deer (Cervus elaphus) in Ireland.

Paratuberculosis was diagnosed in one 18-month-old and two 30-month-old hinds in a herd of 70 red deer (Cervus elaphus) in Ireland. Loss of condition and intermittent diarrhoea were the main clinical findings. Clumps of acid-fast organisms were found in the faeces of the three deer. Post mortem examination of one deer showed a slight swelling and pallor of the intestinal tract and associated lymph nodes. Histopathology showed a severe, granulomatous enteritis and lymphadenitis, with extensive cellular infiltration, notably with epithelioid macrophages containing numerous acid-fast organisms. Mycobacterium paratuberculosis was isolated from intestinal and lymph node samples. Paratuberculosis was also confirmed in one of nine clinically normal, yearling stags, sampled at slaughter. Complement fixation tests and enzyme-linked immunosorbent assays gave higher readings for clinically affected deer than healthy ones. Acid soil on the farm was believed to be a contributory cause.

Juvenile Krabbe's Leukodystrophy precipitated by influenza A infection.

Juvenile Krabbe's disease, a neurometabolic disorder, presented as an acute demyelinating episode characterised by an abnormal gait. The neurological presentation was preceded by influenza A infection.

Health care of physically handicapped young adults.

Little is known about the health needs of physically handicapped young adults after they become too old for the paediatric service. To assess these needs 104 young people with physical handicaps were given a medical examination and interviewed. They had diverse physical handicaps, many of which were rare (41 subjects); 45 had cerebral palsy and 18 spina bifida. Mental handicap (42 subjects) and a need for special education (28) were also diagnosed. The state of health of all subjects was generally poor. Sixty one subjects had contractures of the lower joints and almost a quarter contractures of the upper joints; 26 had deformed feet. Consequently, many subjects (41) could not walk; only 13 could walk without difficulty. Urinary and bowel incontinence were prevalent (58 and 55 subjects, respectively) and with contractures and pressure sores resulted in skin problems (35 subjects). Kyphoscoliosis was present in 30 subjects, particularly those with spina bifida (10 out of 18), of whom one third had respiratory problems and almost half cardiac problems. Thirty one subjects had epilepsy. Many subjects (49) had difficulty communicating or some visual defect (27). Although over half the subjects had health problems that were severe enough to warrant intervention, less than a third were receiving any form of regular hospital care; regular contact with physiotherapists, speech therapists, and dentists was also poor. Moreover, the original diagnoses and prescriptions had not been reviewed regularly. Teams for handicapped adults should be set up in all district health authorities to provide a clinical and coordination service for all adults with physical and mental handicaps.

The health care of physically handicapped young adults.

It has been recognised for many years that the amount of health care received by physically handicapped young adults rapidly declines after they leave the paediatric services. However, little is known about their specific health needs at that stage of their lives, or how these needs may be best met by the statutory services. The results of this study indicate a considerable degree of unmet need and point to a failure by the Health Service to maintain a continuity of health care for young adults with physical handicaps. A policy is called for to set up multidisciplinary Handicapped Adult Teams, at District level, to provide a clinical service for all physically and multiply handicapped adults.

The social skill difficulties of young adults with physical disabilities.

Adolescence is generally considered to be a time of increased social activity and the rapid expansion of a teenager's social circle. By contrast, a young person with a physical disability is likely to lack social independence, become socially isolated and experience difficulties in maintaining social relationships. The present study indicates that while both able-bodied and physically disabled young people experience difficulties in social situations, those with physical disabilities experience difficulties that are more severe in nature. Overall, 75% of the disabled group and 70% of the comparison group experienced difficulties in social situations. However, 37% of the disabled group experienced difficulties that were severe in nature compared to only 3% of the comparison group. Whilst these problems are of sufficient magnitude to warrant intervention, programmes of social skills training for people with physical disabilities are not yet generally available. It is recommended that training programmes in social skills should be made available to teenagers with physical disabilities.

A comparison between electrical impedance and strain gauge plethysmography for the study of cerebral blood flow in the newborn.

This study investigates the possibility of using pulsatile transcephalic impedance changes, delta Z, for the continuous monitoring of cerebral blood flow, CBF, in the sick newborn infant. The performance of the impedance method is compared with the measurement of cranial flow, Fc, using a strain gauge for detecting the predicted changes in cerebral blood flow when the baby breathes 2% CO2 in air, or O2. In all five studies in which measurements were compared in air and CO2 the expected increases in Fc and delta Z were seen. In six of seven studies comparing measurements in air and O2 the expected fall in the variables was seen, indicating reduced cerebral blood flow. The problems of both these methods are discussed and we conclude that the impedance method is as good as the strain gauge method for detecting changes in cerebral blood flow.