Regeneration in Duchenne Muscular Dystrophy

Desmedt, John E.

doi:10.1001/archneur.1976.00500090048010

Cited by 63 publications

(22 citation statements)

References 33 publications

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“…This may also result in the presence of satellite motor unit potentials. [73,74] Over time the motor units become very small and some areas become electrically silent. [2] Nerve conduction studies are normal in early DMD.…”

Section: Electromyographymentioning

confidence: 99%

Duchenne muscular dystrophy

Yiu

Kornberg

2015

J Paediatrics Child Health

272

242

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Duchenne muscular dystrophy (DMD), an X-linked disorder, is the most common muscular dystrophy in children, presenting in early childhood and characterized by proximal muscle weakness and calf hypertrophy in affected boys. Patients usually become wheelchair-bound by the age of 12 years, and die of cardiorespiratory complications in their late teens to early twenties. Advances in the management of DMD, including treatment with corticosteroids and the use of intermittent positive pressure ventilation have provided improvements in function, ambulation, quality of life and life expectancy, although novel therapies still aim to provide a cure for this devastating disorder. The clinical features, investigations, and management of DMD are reviewed, as well as the latest in some of the novel therapies.

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Section: Electromyographymentioning

confidence: 99%

Duchenne muscular dystrophy

Yiu

Kornberg

2015

J Paediatrics Child Health

272

242

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“…These abnormalities have been reported in patients with Duchenne dystrophy [25], acid maltase deficiency, limb girdle dystrophy [27], myotonic dystrophy [28].…”

Section: Discussionmentioning

confidence: 89%

“…Fiber density increase has been observed in myopathic muscles, reflecting a localized change in the distribution of muscle fibers within each motor unit following fiber loss, fiber regeneration and reinnervation or fiber splitting [25,26]. These abnormalities have been reported in patients with Duchenne dystrophy [25], acid maltase deficiency, limb girdle dystrophy [27], myotonic dystrophy [28].…”

Section: Discussionmentioning

confidence: 99%

Single-fiber electromyography in hyperCKemia: the value of fiber density

2011

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Although persistently raised serum creatine kinase (sCK), or hyperCKemia, is considered the biological hallmark of neuromuscular diseases, pauci- or asymptomatic- or isolated-hyperCKemia can often be found. Single-fiber electromyography (SFEMG) is an electrophysiological technique of great value in the assessment of neuromuscular, neuropathic and myopathic disorders. We hypothesize that SFEMG fiber density (FD) evaluation is able to detect subclinical electrophysiological abnormalities indicating a myopathic process in subjects with hyperCKemia. Nineteen subjects with hyperCKemia without evident clinical signs of muscle involvement and 15 healthy controls were studied. Electrophysiological investigations including nerve conduction studies (NCS), quantitative EMG (QEMG), SFEMG with focus on FD measurements, and muscle biopsy were performed. NCS, QEMG, SFEMG were normal in all controls. In subjects with hyperCKemia, NCS were normal; QEMG was abnormal in 5, while both SFEMG and muscle biopsy disclosed abnormalities in 12 subjects. The mean FD value was 2.6 ± 0.5 in the control and 4 ± 1.4 (p = 0.003) in the hyperCKemia group. SFEMG revealed subclinical changes in the majority of subjects with hyperCKemia. To the best of our knowledge, this is the first study demonstrating that SFEMG FD evaluation is able to detect the presence of muscle diseases, which are in a subclinical phase and would remain unidentified otherwise. SFEMG may be used to distinguish hyperCKemia associated to asymptomatic muscle disorders from idiopathic hyperCKemia. We believe that SFEMG FD evaluation should be added to the routine examinations in the screening of idiopathic hyperCKemia.

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“…'~ A third possible cause of the neurogenic changes in BMD may be secondary to reinnervation of regenerating fibers, where intramuscular peripheral nerves are involved, to some extent, during chronic degenerating and regenerating processes of muscle fibers. 6 The relationship of these neurogenic changes to specific dystrophin abnormalities in BMD muscle remains to be elucidated.…”

Section: Discussionmentioning

confidence: 99%

Muscle histology in becker muscular dystrophy

et al. 1991

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Twenty patients with Becker muscular dystrophy (BMD), confirmed by dystrophin tests, were studied histologically. There were several morphological differences between younger (less than or equal to 15-year-old) and older (greater than 15-year-old) patients. In the younger patients, active muscle fiber necrosis followed by a regenerating process was conspicuous. In the older patients, the active degenerative changes appeared less prominent and, instead, more chronic myopathic changes such as moth-eaten fibers, fiber splitting, and hypertrophic fibers were evident. These age-dependent differences in the pathology of BMD were irrespective of the duration of clinical symptoms, i.e., BMD patients of a similar age showed a similar morphological feature regardless of age at onset. Although the presence of mild fiber type grouping and some small angulated atrophic fibers suggested a certain degree of neurogenic involvement, none of biopsies showed significant grouped atrophy as seen in neuropathic disorders. There was no correlation between the histological changes and the specific dystrophin abnormality.

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Regeneration in Duchenne Muscular Dystrophy

Cited by 63 publications

References 33 publications

Duchenne muscular dystrophy

Duchenne muscular dystrophy

Single-fiber electromyography in hyperCKemia: the value of fiber density

Muscle histology in becker muscular dystrophy

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