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- W2002166489 abstract "Using a technique previously described by two of the authors of counting the total numbers of limb motor neurones in the lumbosacral enlargement of the spinal cord, 9 cases previously diagnosed as examples of muscular dystrophy have been studied at autopsy. In 6 established cases of muscular dystrophy of the Duchenne type the total numbers of motor neurones were normal, except in 1 case in which focal loss of neurones in several spinal cord segments was attributed to a previous attack of poliomyelitis. Variable chromatolysis of neurones and increase of glial cells were also noted in these cases but the latter change may in part have been due to “crowding” of cells resulting from shortening of the lumbosacral segments. In 1 other case previously diagnosed as an example of Duchenne muscular dystrophy, the findings at autopsy were those of spinal muscular atrophy but in fact investigation in life shortly before death had led to revision of the clinical diagnosis in this case to one of the Kugelberg-Welander syndrome. In 1 other case, diagnosed in life as an example of limb-girdle muscular dystrophy, the reduction of limb motor neurones found at autopsy indicated that the patient had been suffering from chronic spinal muscular atrophy. In the final case of this series in whom diagnoses of peroneal muscular atrophy, spinal muscular atrophy and distal muscular dystrophy had been entertained during life, the total numbers of limb motor neurones in the lumbo-sacral cord were only just below the lower limit of normal and it was concluded that the patient had been suffering from distal muscular dystrophy. Histological changes of widespread myocardial fibrosis indicative of cardiomyopathy were found in all 6 cases of Duchenne type muscular dystrophy but also in the case of spinal muscular atrophy of the Kugelberg-Welander type and in the patient with distal muscular dystrophy. The possibility that such a cardiomyopathy could be the result of an autoimmune process due to the production of circulating antibodies which attack cardiac muscle in patients suffering from any disease giving longstanding and progressive destruction of voluntary muscle is raised." @default.
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- W2002166489 title "Spinal cord limb motor neurones in muscular dystrophy" @default.
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- W2002166489 doi "https://doi.org/10.1016/0022-510x(74)90003-3" @default.
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