Myocardial ultrastructural changes in Kugelberg-Welander syndrome.
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چکیده
منابع مشابه
Sensory neuron degeneration in familial Kugelberg-Welander disease.
A 53 year old man developed symptoms of motor neuron disease in childhood. There was a family history of a similar disorder and it was felt to represent a form of Kugelberg-Welander disease. In addition to the motor deficits, sensory abnormalities in his legs were documented during life. Autopsy revealed anterior horn cell loss throughout the length of the spinal cord, with preservation of the ...
متن کاملA case of the Kugelberg-Welander syndrome complicated with cardiac lesions.
There have been a few reports of cardiac involvement in the Kugelberg-Welander syndrome. We presented a case of this syndrome complicated with cardiomegaly and conduction disturbance. The patient, a 21-year-old woman, had atrial standstill and A-V junctional rhythm. The chest X-ray film showed marked cardiomegaly. The His bundle electrogram revealed that H-V interval was 40 msec. She was treate...
متن کاملCell adhesion molecule N-CAM is expressed by denervated myofibres in Werdnig-Hoffman and Kugelberg-Welander type spinal muscular atrophies.
Immunocytochemical analysis utilising antibody to neural cell adhesion molecule (N-CAM) was carried out on skeletal muscle biopsies from patients with childhood spinal muscular atrophy. Children with both Werdnig-Hoffmann and Kugelberg-Welander disease showed positive N-CAM reactivity. There were however differences in the N-CAM expression profiles in these two sets of patients. All myofibres w...
متن کاملgeneral anesthesia plus muscle relaxant in a patient with kugelberg welander disease: a case report
spinal muscular atrophies (smas) represent a rare group of inherited disorders that cause progressive degeneration of the anterior horn cells of the spinal cord. the exact cause of the degeneration is unknown. loss of these cells results in a progressive lower motor neuron disease that has no sensory involvement and that is manifested as hypotonia, weakness, and progressive paralysis. kugelberg...
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ژورنال
عنوان ژورنال: Heart
سال: 1977
ISSN: 1355-6037
DOI: 10.1136/hrt.39.12.1390