Muscle disorders in children.

London : University of London Audio-Visual Centre, 1976.

1 encoded moving image (39 min.) : sound, black and white.

00:39:05

University of London

Restricted

In English

Presented by Professor Victor Dubowitz, Professor of Paediatrics, Institute of Child Health. Made by University of London Audio-Visual Centre for the British Postgraduate Medical Federation. Produced by David Sharp.

This video is one of around 310 titles, originally broadcast on Channel 7 of the ILEA closed-circuit television network, given to Wellcome Trust from the University of London Audio-Visual Centre shortly after it closed in the late 1980s. Although some of these programmes might now seem rather out-dated, they probably represent the largest and most diversified body of medical video produced in any British university at this time, and give a comprehensive and fascinating view of the state of medical and surgical research and practice in the 1970s and 1980s, thus constituting a contemporary medical-historical archive of great interest. The lectures mostly take place in a small and intimate studio setting and are often face-to-face. The lecturers use a wide variety of resources to illustrate their points, including film clips, slides, graphs, animated diagrams, charts and tables as well as 3-dimensional models and display boards with movable pieces. Some of the lecturers are telegenic while some are clearly less comfortable about being recorded; all are experts in their field and show great enthusiasm to share both the latest research and the historical context of their specialist areas.

Segment 1 Prof. Dubowitz begins the lecture by discussing Duchenne de Boulogne, the pioneering French neurologist who introduced electricity into medicine. A French text and illustration from Duchenne's book are seen - his first mention of muscular dystrophy. Dubowitz explains that pseudohypertrophy is now referred to as Duchenne dystrophy for the common and severe childhood form of the disease. Dubowitz continues to describe the history of research into childhood muscular dystrophy, including an 1851 British paper and an 1879 British monograph by the neurologist Gowers. Time start: 00:00:00:00 Time end: 00:04:59:00 Length: 00:04:59:00

Segment 2 Dubowitz reviews some clinical aspects of muscular dystrophy, particularly Duchenne dystrophy. Photographs of three brothers (naked) of increasing ages, all with the disease, are shown. Dubowitz comments on the progression of the disease with age. He then discusses diagnosis of the disease using the enzyme creatine phosphokinase (CPK) as a diagnostic criterion. CPK is elevated in the disease. A chart shows the normal range of the enzyme compared to that in muscle disorders. Time start: 00:04:59:00 Time end: 00:09:56:00 Length: 00:04:57:00

Segment 3 An image of a CPK screening test is seen and the process is described by Dubowitz. He also describes another type of test that uses luciferase obtained from American fireflies. He then discusses whether early screening of children to test for Duchenne dystrophy would benefit their families or not. Next, two photomicrographs of muscles with Duchenne dystrophy are seen, and Dubowitz talks about the clinical aspects of the disease. Photographs of a child who looks normal but who has the disease are seen. Time start: 00:09:56:00 Time end: 00:14:42:12 Length: 00:04:46:12

Segment 4 Two photomicrographs of a muscle biopsy from a child of just under 3 months with Duchenne dystrophy are seen, and Dubowitz discusses the effect on the muscle fibres. He also talks about whether feotuses display the effects of Duchenne dystrophy. Next, he discusses therapy for the dystrophy, including the importance of keeping the children ambulant. A photograph of a child wearing callipers on the legs is seen, one method that Dubowitz suggests so that the child can keep walking. Time start: 00:14:42:12 Time end: 00:19:44:19 Length: 00:05:02:07

Segment 5 Dubowitz discusses how to prevent and treat scoliosis in patients with muscular dystrophy. A photograph of a boy in a wheelchair is seen, and Dubowitz suggests fitting jackets and braces to ensure the child sits properly. Next, he discusses respiratory infection, the usual cause of death for young people with Duchenne dystrophy. He explains why these infections are so dangerous to patients with the disease. A series of ECG readouts is seen. Dubowitz goes on to talk about how about 40% of children with the disease show some intellectual impairment as well as muscle weakness. A graph of IQs is seen. Time start: 00:19:44:19 Time end: 00:24:54:24 Length: 00:05:10:05

Segment 6 Next, Dubowitz discusses genetic counselling and carrier detection (in the parents). A family tree shows carriers and children with the dystrophy. He explains how to test females to see if they are carriers. The test also measures CPK. He describes other approaches, including muscle biopsy. A photograph of a woman's calf is seen, the woman having had a biopsy that tested positive. Time start: 00:24:54:24 Time end: 00:30:39:00 Length: 00:05:44:01

Segment 7 Dubowitz continues to discuss testing for carriers of Duchenne dystrophy. Another method is to perform an EMG (electromyography) on potential carriers. Next, he talks about the procedure of muscle biopsy and the kind of information that can be obtained from one. Photographs of the surgical procedure are seen. He explains needle biopgy, open biopsy and how to analyse the muscle tissue. Time start: 00:30:39:00 Time end: 00:35:27:19 Length: 00:04:48:19

Segment 8 He explains advances in knowledge that have come as a result of histochemical techniques in muscle biopsies. Photomicrophs are seen of muscle fibres as Dubowitz explains how a new family of disorders has been discovered. Dubowitz seems to suddenly realise that he is out of time and ends the lecture. Time start: 00:35:27:19 Time end: 00:39:05:05 Length: 00:03:37:11