Histochemical and Electron Microscopic Diagnosis of Mitochondrial Myopathy: The First Case Report From Iran

  • سال انتشار: 1387
  • محل انتشار: فصلنامه آسیب شناسی ایران، دوره: 3، شماره: 2
  • کد COI اختصاصی: JR_IJP-3-2_008
  • زبان مقاله: انگلیسی
  • تعداد مشاهده: 617
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نویسندگان

Farrokh Tirgari

Dept. of Pathology and Cancer Institute, Tehran University of Medical Sciences, Tehran, Iran

Fatemeh Mahjoub

Dept. of Pathology and Cancer Institute, Tehran University of Medical Sciences, Tehran, Iran

Zarrin Keyhani

Dept. of Pediatric Neurology, Tehran University of Medical Sciences, Tehran, Iran

Nargess Tabarzan

Tehran University of Medical Sciences, Tehran

چکیده

Muscle tissue, skeletal muscle as well as cardiac muscle, is commonly affected in mitochondrial disorders. One explanation for this observation is that muscle tissue has a high-energy demandand therefore is more sensitive to a deficiency of mitochondrial energy production than some othertissues. In mitochondrial disorders, skeletal muscle tissue may be affected primarily by defective respiratory chain function or secondarily to peripheral neuropathy with neurogenic muscle atrophy.The clinical manifestations of mitochondrial myopathies are variable and include muscle weakness,exercise induced cramps and myalgia. Also, ptosis and progressive external ophtalmoplegia are typical but not obligate finding. Hereby we wanted to report a case of mitochondrial myopathy, diagnosed by histochemical and electron microscopic studies for the first time in Iran.Our case was a 12 year old girl who referred due to muscle weakness to our center which started an 4years ago. Later, she also developed ptosis. EMG studies were inconclusive and muscle biopsy revealedtypical red ragged fibers with special staining. By electron microscopy, typical mitochondrial changes were detected.

کلیدواژه ها

Mitochondrial Myopathies, Electron microscopy, Histocytochemistry

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