Which of the following is not a limb girdle dystrophy-
Correct Answer: Dystrophin dystrophy
Description: *Autosomal recessive limb girdle muscle dystrophies: -LGMD2A disease associate with calpain3 -LGMDB associated with dysferin -LGMD2C-F associate with alpha,beta,gamma,delta sarcoglycans. Limb-girdle muscular dystrophy (LGMD) is a diverse group of disorders with many subtypes categorized by disease gene and inheritance. LGMD usually manifests in the proximal muscles around the hips and shoulders. (The proximal muscles are those closest to the center of the body; distal muscles are faher away from the center -- for example, in the hands and feet). The shoulder girdle is the bony structure that surrounds the shoulder area, and the pelvic girdle is the bony structure surrounding the hips. Collectively, these are called the limb girdles, and it is the observed weakness and atrophy (wasting) of the muscles connected to the limb girdles that has given this group of disorders its name. Weakness in the shoulder area may make reaching over the head, holding the arms outstretched, or carrying heavy objects difficult. It may become increasingly hard to keep the arms above the head for such activities as combing one's hair or arranging things on a high shelf. Some people find it harder to type on a computer or other keyboard and may even have trouble feeding themselves. Some of the LGMD subtypes also are characterized by additional symptoms. For example, the hea can be affected in some types of LGMD, with weakness of the hea muscle (cardiomyopathy) and/or abnormal transmission of signals that regulate the heabeat (conduction abnormalities or arrhythmias). Some disease subtypes also involve the muscles used for breathing, and for that reason, respiratory function, along with cardiac function, should be monitored regularly. Ref Harrison20th edition pg 2456
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