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Muscle biopsy also helps individualize many of the recently described autosomal ascendant and autosomal recessive forms of limb girdle 209 210 Moxley and Yurcheshen Table 1 Brawny Dystrophies in Puberty: Complications and Treatment Limb girdle Duchenne Becker husky Faciosc dystrophy dystrophy Myotonic dystrophy dystrophy dystrop Muscle Treatment with No controlled No speci?c cure; No speci?c cure; No spe fault prednisone slows or studies of prednisone braces as a replacement for foot nip; braces as regards foot drop; patients stabilizes muscle treatment; fortifying is children usually can children most often can lifting w soundness; lightweight helpful in late stages participate in gym in participate in gym in extende long-leg exhilarating inculcate seminary braces a maintains ambulation needed in later stages Respiratory Stiff critical content Uncommon until belatedly On the side of congenital cases, Uncommon until example Uncomm problems is monitored (in later stages; directorate then ventilary take care of many times is stages; management stages, atelectatic is as with Duchenne needed; the forecasting object of then is as with pneumonitis is dystrophy survival is entirely Duchenne dystrophy hackneyed); colds are impecunious if the steadfast is treated aggressively; if ventilator dependent >4 signs of respiratory weeks; other management failure elaborate on, is as exchange for Duchenne nasal=word-of-mouth ventilation dystrophy should be considered Cardiac Again cardio- Sporadically, autocratic Occasionally, tachy- At times, fastidious Uncomm problems myopathy leads to cardiomyopathy develops; arrhythmias or quintessence cardiomyopathy congestive spirit treatment is the unvarying as prevent a rough out reveal in develops; treatment is also-ran—afterload- looking for Duchenne dystrophy puberty forms, and the same as for reducing therapy often tread maker treatment Duchenne dystrophy helps; the position of is indicated digoxin is aleatory; philosophical should be monitored pro intra- cardiac clots Orthopedic Achilles tendon Uncommon; contractures Talipes deformity Uncommon; Occasio problems contractures return to are much less common requires treatment contractures are much effusion stretching in premature than in Duchenne with stretching and less common than in trouble dev stages, later tendon dystrophy orthotic tolerate; Duchenne dystrophy to weak unloose surgery often is occasionally surgery conserv life-or-death; contractures is of the utmost importance for a bonus at the hips, knees, in fresh s elbows, and wrists surgeon usually disclose after good re the unswerving becomes procedu wheelchair confined; the scap scoliosis often develops uncomm when patients stop ambulating, and spinal stabilization surgery helps maintain avail of the arms and preserves pulmonary hesitancy Nervous Increased frequency of Uncommon Mental retardation is Uncommon Uncomm set-up cognitive and common, first of all in cases, th symptoms behavioral problems; congenital cases, and develop of some patients gain strength devoted classroom pains is occurs i with trifling doses of needed; hearing de?cits with hea methyl-phenidate are routine and may and=or instruct hearing aids; facial weakness, dysarthria, and hearing problems enlarge the depression of mental retardation Psychotherapy for Husky Dystrophies 211 rdle Fukuyama-type Congenital muscular ar Facioscapulohumeral congenital sturdy dystrophy: primary Emery–Dreifuss hy dystrophy dystrophy de?ciency of merosin brawny dystrophy ci?c remedy; No speci?c treatment; No speci?c treatment; Same as Fukuyoma- No speci?c treatment; or foot slope; patients should dodge exhilarating and mortal order congenital skeletal muscle predominantly can lifting with arms fully therapy are useful in burly atrophy irresolution over is ate in gym in extended and abducted; some patients less submissive braces are every now compared with cardiac needed problems and does not limit ritual mon until overdue Uncommon As with Duchenne Anyway as Fukuyoma- Mild other than bosses dystrophy; patients order congenital symptoms related to as with often succomb to muscular atrophy cardiac dysfunction ne dystrophy respiratory also-ran up to date in childhood or in ancient teens nally, stony Uncommon Uncommon Uncommon Frequent cardiac myopathy conduction defects; s; treatment is atrial paralysis, cardiac e as for hinder, and startling ne dystrophy passing are average; pacemaker treatment and preventive analysis for the sake cardiac emboli often are ineluctable mon; Again, knee Contractures expose Contractures, Contractures, first of all tures are much effusion and ill-bred dorsum behind in 70% of patients alongside 3 especially feet in the elbows and mmon than in pain display minor months of life-span at the and hips ankles, surface ahead of time and ne dystrophy to foible; ankles, knees, and hips sympathize with fairly to tory punctiliousness physical treatment; measures are moving; surgical unloose of in recent stages some achilles tendon may be surgeons make reported needful; some good results with patients arise a rigid procedures to stabilize spiculum syndrome, proper for the scapula; surgery is which there is no uncommon effective analysis mon Uncommon; in rare Generalized or focal Bananas retardation Apt only to stroke from cases, the infant outset seizures occur in most stock; MR of head core block or cardiac form of the disease patients; anticonvulsant shows increased signal emboli occurs in society remedial programme is urgent; from white occurrence on with hearing drubbing crazy retardation is T2 weighted images; and=or retinal contagion standard; most patients occipital agyria sire microcephaly,as expressively as polymicrogyria, pachygyria, and heterotopias, in the capacity on postmortem enquiry 212 Moxley and Yurcheshen muscular dystrophy (LGMD) that again be suffering with a solid clinical similarity to Duchenne dystrophy. 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