Kugelberg-Welander

Kugelberg-Welander disease (type III SMA) is characterized by juvenile onset and prolonged survival. In early stages of the disease the process of reinnervation... 

Generally a much more limited increase in serum creatine kinase is encountered in the adult form of muscular dystrophy and in myotonic dystrophy. It is normally raised in untreated cases of polymyositis, and the level may be very high. Typical data are provided by Pearce et al [113] and other workers. It was at one time considered that little or no rise in serum creatine kinase occurred in muscle diseases of recognized neurogenic origin, but modest increases have been recorded in Kugelberg-Welander disease and spastic spinal paralysis [115] and in motor neurone disease [116]. Elevated values may occur also in hyperkalaemic and hypokalaemic myopathies [117, 118]. 

Fig. 3. Phosphorus-31 spectra (81.0 MHz) from PCA extracts of left (top) and right (bottom) gastrocnemius muscles of a patient with Kugelberg-Welander disease.

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