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The call of strncpy() copies the string "Dave " into the array name. The call of strncat() copies the string "Mark" onto the end of dest, leaving dest with the properly terminated string "Dave Mark". Again, the return value was ignored. As was the case with strcpy(), the Standard Library offers a matching function for strncat() called strcat(). It does not provide the safeguard against buffer overflow and should not be used.

Timeline If you are having trouble selecting the mcHammer movie clip, you can lock the buttons layer so that you won t have to worry about accidentally selecting one of the buttons

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With so many different types of muscular dystrophies and the variability of clinical phenotypes associated with speci c forms of dystrophy, even within individual families, the evaluation of patients presenting with weakness can be quite daunting However, rather than ordering every genetic test possible or doing a muscle biopsy initially on every patient, an approach to ordering tests based on clinical phenotype (inheritance pattern, age of onset, pattern of weakness, and associated manifestations early contractures and cardiac or respiratory involvement) can very useful (Fig 24 20) Unfortunately, there are limited medical treatments of bene ts, other than corticosteroids for children with DMD, that have been demonstrated to be bene cial Still with supportive treatments (physical and occupational therapy, bracing, respiratory, and cardiac) the quality of life can be improved in patients More work needs to be done to further understand the pathogenesis of these disorders and discover better treatments

1 Hoffman EP, Brown RH, Jr, et al Dystrophin: The protein product of the Duchenne muscular dystrophy locus Cell 1987;51(6):919 928

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bottom right of the hammer, as shown here This is the rotation point for this movie clip Because the registration point is there, the hammer will rotate around that point If the rotation point of the hammer were at the top left, the hammer would rotate around its top-left corner

CHAPTER 24 22 Vorgerd M, van der Ven PF, et al A mutation in the dimerization domain of lamin c causes a novel type of autosomal dominant myo brillar myopathy Am J Hum Genet 2005;77(2):297 304 23 Nagano A, Arahata K Nuclear envelope proteins and associated diseases Curr Opin Neurol 2000;13(5): 533 539 24 Nagano A, Koga R, et al Emerin de ciency at the nuclear membrane in patients with Emery Dreifuss muscular dystrophy Nat Genet 1996;12(3):254 259 25 Ognibene A, Sabatelli P, et al Nuclear changes in a case of X-linked Emery Dreifuss muscular dystrophy Muscle Nerve 1999;22(7):864 869 26 Sabatelli P, Lattanzi G, et al Nuclear alterations in autosomal-dominant Emery Dreifuss muscular dystrophy Muscle Nerve 2001;24(6):826 829 27 Bonne G, Mercuri E, et al Clinical and molecular genetic spectrum of autosomal dominant Emery Dreifuss muscular dystrophy due to mutations of the lamin A/C gene Ann Neurol 2000;48(2):170 180 28 Felice KJ, Schwartz RC, et al Autosomal dominant Emery Dreifuss dystrophy due to mutations in rod domain of the lamin A/C gene Neurology 2000;55(2):275 280 29 Frosk P, Weiler T, et al Limb-girdle muscular dystrophy type 2H associated with mutation in TRIM32, a putative E3-ubiquitin-ligase gene Am J Hum Genet 2002; 70(3):663 672 30 Brockington M, Blake DJ, et al Mutations in the fukutinrelated protein gene (FKRP) cause a form of congenital muscular dystrophy with secondary laminin alpha2 de ciency and abnormal glycosylation of alpha-dystroglycan Am J Hum Genet 2001;69(6):1198 1209 31 Brockington M, Yuva Y, et al Mutations in the fukutinrelated protein gene (FKRP) identify limb girdle muscular dystrophy 2I as a milder allelic variant of congenital muscular dystrophy MDC1C Hum Mol Genet 2001;10(25):2851 2859 32 Brown SC, Torelli S, et al Abnormalities in alphadystroglycan expression in MDC1C and LGMD2I muscular dystrophies Am J Pathol 2004;164(2):727 737 33 Beltran-Valero de Bernabe D, Voit T, et al Mutations in the FKRP gene can cause muscle eye brain disease and Walker Warburg syndrome J Med Genet 2004;41(5):e61 34 Brockington M, Torelli S, et al Localization and functional analysis of the LARGE family of glycosyltransferases: Signi cance for muscular dystrophy Hum Mol Genet 2005;14(5):657 665 35 Longman C, Brockington M, et al Mutations in the human LARGE gene cause MDC1D, a novel form of congenital muscular dystrophy with severe mental retardation and abnormal glycosylation of alpha-dystroglycan Hum Mol Genet 2003;12(21):2853 2861 36 Emery AE Population frequencies of inherited neuromuscular diseases a world survey Neuromuscul Disord 1991;1(1):19 29 37 Brooke MH, Fenichel GM, et al Duchenne muscular dystrophy: Patterns of clinical progression and effects of supportive therapy Neurology 1989;39(4):475 481 38 Brooke MH, Griggs RC, et al The natural history of Duchenne muscular dystrophy: A caveat for therapeutic trials Trans Am Neurol Assoc 1981;106:195 199.

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