Question
A 6-year-old boy presents with a progressive history of lower limb weakness from the age of 3 years.O/E the power is less than 3/5 in bilateral lower limbs with calf muscle pseudohypertrophy. Gower sign is positive currently the child has clinical features of pneumonia, What is the most likely diagnosis and treatment strategy in this patient:
A. |
Duchene muscular dystrophy; IVIG
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B. |
Becker’s muscular dystrophy; Steroids
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C. |
Duchene muscular dystrophy; Steroids
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D. |
Beckers muscular dystrophy; IVIG
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Correct Answer � C
Explanation
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Ans. C. Duchene muscular dystrophy; Steroids
Explanation:DMD is an X-linked recessive disease with symptom onset from 2-3 years and progresses over the next few years and children become wheelchair constrained by 10-13 years of age. Additionally, 25% of them develop dilated cardiomyopathy. A milder form of this illness which has onset at 5-6 years and has a slower progression is referred to as Becker muscular dystrophy. Steroids are the only drugs that are proven to help delay the progression .clinical suspicion and elevated CPK must warrant DMD gene mutational analysis. Muscle biopsy is seldom done these days to diagnose.
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