Background -The effect on subsequent respiratory function of spinal stabilisation for scoliosis in Duchenne muscular dystrophy is unclear. In order to clarify this clinical problem, changes in the forced vital capacity of a group of children with Duchenne muscular dystrophy who had undergone spinal surgery were measured and compared with a group of children with Duchenne muscular dystrophy who had not had surgery. Methods -In this retrospective study 17 boys with Duchenne muscular dystrophy who underwent spinal stabilisation at a mean age of 14-9 years (surgical group) were compared with 21 boys with Duchenne muscular dystrophy who had not had surgery (non-surgical group). The mean (SD) Cobb angle of the surgical group at 14-9 years was 57 (16.4)0, and of the non-surgical group at 15 years was 45 (29.9)°. Forced vital capacity expressed as percentage predicted (% FVC) was measured in total over a seven year period in the surgical group and over 6 5 years in the non-surgical group, and regression equations were calculated. Survival curves for both groups were also constructed. Results -No difference was found between spinal stabilisation (surgical group) and the non-surgical group in the rate of deterioration of % FVC which was 3-5% per year. There was no difference in survival in either group. Conclusions -Spinal stabilisation in Duchenne muscular dystrophy does not alter the decline in pulmonary function, nor does it improve survival.

Event-related potentials (ERPs) were recorded from midline (Fz, Cz, Pz) and lateral sites (F3, F4, P3, P4) in autistic children (n = 7) and age-matched controls (n = 9) on an auditory choice reaction time task. Subjects were asked to press a button to an infrequent target (500 Hz, P = 0.14) and to ignore higher pitched infrequent (2000 Hz, P = 0.14) and frequent (1000 Hz) non-targets. Autistic subjects made twice as many errors of omission as controls and showed a higher criterion (beta) for targets. Maximum ERP peak amplitudes showed a more varied scalp distribution in the autistic group. N1 latencies were consistently shorter in the autistic group and in 3 subjects the target P3 latencies were markedly longer than for the controls. Compared to controls, the N1 amplitude of the autistic response was larger to the rare stimuli (particularly to non-targets). The amplitude of the P3 component was smaller in the autistic group (particularly to the target). The stimuli were also presented in a passive condition requiring no response. After subtraction of the waveform obtained in the passive condition from that obtained in the active condition or subtraction of the waveform elicited by the rare non-target from that elicited by the target, N1 target amplitude was larger in control than in autistic children. Autistic subjects showed more early negativity to the rare non-target at left frontal and a larger P3 to the target at right parietal sites. ERPs of autistic children are more responsive to stimulus features (e.g. high/rare non-target tone) and less responsive to their associations or meaningfulness (e.g. target P3). Attention-related ERPs of autistic children show signs of precocious (right dominance for P3) and delayed development (P3 not maximal at parietal sites).

The terminal care for individuals with muscular dystrophy and spinal muscular atrophy and their families requires improvement. Although many individuals with these conditions will die following an acute event, palliative care services may be appropriate for those who require a period of terminal care at home.

SUMMARY Eighteen boys with Duchenne muscular dystrophy were entered into trials to assess the effects of specific ventilatory strength and endurance training programmes. The findings showed an improvement in ventilatory muscle endurance but not in strength as a result of specific respiratory muscle training. The clinical significance of these findings is uncertain, however, and needs further evaluation. RÉSUMÉ Entrainement des muscles respiratoires dans la myopathie de Duchenne Des examens ont été effectúes pour apprécier les effets de programmes spécifiques d'entrainement de la force et de l'endurance de la fonction ventilatoire, chez dix‐huit garçons présentant une myopathie de Duchenne. Les données recueillies ont montré une amélioration dans l'endurance des muscle respiratoires mais non dans la force, en réponse à cet entrainement spécifique de muscles respiratoires. La signification clinique de ces résultats demeure cependant incertaine et demanderait un complément d'étude. ZUSAMMENFASSUNG Atemmuskeltraining bei Duchenne'scher Muskeldystrophie Um den Erfolg eines speziellen Trainingsprogramms für Atemstärke und ‐ausdauer zu untersuchen, wurden 18 Jungen mit Duchenne'scher Muskeldystrophie getestet. Die Ergebnisse des speziellen Atemmuskeltrainings zeigten eine Besserung in der Ausdauer der Atemmuskulatur, nicht aber der Muskelstärke. Die klinische Signifikanz dieser Befunde ist jedoch noch unklar und es sind weitere Untersuchungen erforderlich. RESUMEN Entrenamiento de la musculatura respiratoria en la distrofia muscular de Duchenne Dieciocho muchachos con distrofia muscular de Duchenne fueron observados para determinar los efectos de programas específicos de entrenamiento de la fuerza y resistencia ventilatorias. Los resultados mostraron una mejoria en la resistencia muscular ventilatoria pero no en la fuerza, como resultado de un entrenamiento específico de la musculatura respiratoria. Sin embargo el significado clinico de estos hallazgos es incierto y necesita evaluaciones posteriores.

SUMMARY Computer games were modified to encourage respiratory effort by two groups of patients with Duchenne muscular dystrophy. One group trained during the first six months of the study, the other during the second six months. Inspiratory effort was increased by their having to breathe through a mask to both start and continue the games. Pulmonary function tests were done at the beginning of the trial and six, 12 and 18 months later, when forced vital capacity, respiratory muscle endurance and strength were measured. Although there were some individual improvements, over‐all the results were not statistically significant. RÉSUMÉ Entrainement á la resistance inspiratoire chez les myopathes de Duchenne, par jeux d'ordinateur adaptés Des jeux d'ordinateur ont été modifies pour encourager l'effort respiratoire dans deux groupes de myopathes de Duchenne. Un groupe fut entrainé durant les six premiers mois de l'étude, l'autre durant les seconds six mois. L'effort inspiratoire fut accru par une respiration à travers un masque, pour débuter et poursuivre les jeux. Les tests de fonction pulmonaire furent effectués au début de l'apprentissage et six, 12 et 18 mois plus tard, comprenant la capacité vitale maximale, 1'endurance des muscles respiratoires et leur force. Bien qu'il y ait eu quelques améliorations individuelles, les résultats globaux ne furent pas statistiquement significatifs. ZUSAMMENFASSUNG Training der inspiratorischen Resistenz bei Patienten mit Duchenne'scher Muskeldystrophie unter Verwendung von dafiir entwickelten Computerspielen Mit Hilfe von modifizierten Computerspielen wurde bei zwei Gruppen von Patienten mit Duchenne'scher Muskeldystrophie versucht, die Atemanstrengungen zu fördern. Eine Gruppe trainierte während der ersten sechs Monate der Studie und die andere während der zweiten sechs Monate. Die inspiratorischen Anstrengungen wurden dadurch erhöht, daß sie durch eine Maske atmen mußten, um die Spiele zu starten und zu spielen. Die Lungenfunktionstests wurden zu Beginn der Untersuchung und sechs, 12 und 18 Monate später, als auch die forcierte Vitalkapazität, die Atemmuskelausdauer und‐stärke gemessen wurden, durchgeführt. Obwohl einige individuelle Verbesserungen verzeichnet werden konnten, waren die Ergebnisse nicht statistisch signifikant. RESUMEN Entrenamiemto de la resistencia inspiratoria en pacientes con distrofia de Duchenne usando juegos de computadora adaptados Se modificaron juegos de computadora para facilitar el esfuerzo respiratorio en dos grupos de pacientes con distrofia muscular de Duchene. Un grupo se entrenó durante los primeros seis meses del estudio y el otro durante los siguientes seis meses. El esfuerzo inspiratorio aumentó al tener que respirar a través de una máscara para iniciar y continuar los juegos. Se hicieron pruebas de la función pulmonar al inicio del aprendizaje y a los seis, 12 y 18 meses después, midiendo la capacidad vital, la resistencia de los músculos respiratorios y su fuerza. Aunque hubo algunas mejorias individuates, en conjunto los resultados no fu...