The red ragged edge

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Myoclonic epilepsy with ragged red fibres.

MERRF is characterized by muscle twitches (myoclonus), weakness (myopathy), and progressive stiffness (spasticity). When the muscle cells of affected individuals are stained and viewed under a microscope, these cells usually appear abnormal. These abnormal muscle cells are called ragged-red fibers. Other features of MERRF include recurrent seizures (epilepsy), difficulty coordinating movements ...

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Molecular Diagnosis of Myoclonus Epilepsy Associated with Ragged-Red Fibers Syndrome in the Absence of Ragged Red Fibers

Myoclonus epilepsy with ragged-red fibers (MERRFs), an inherited mitochondrial disorder, has characteristic morphological changes of ragged-red fibers (RRFs) in muscle biopsy, in the absence of which mitochondrial etiology is usually not considered in patients with phenotypes suggestive of MERRF. In these circumstances, MERRF can only be diagnosed using genetic analyses. The symptoms, pathologi...

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Myoclonic epilepsy with ragged-red fibers: A case report

Myoclonic epilepsy with ragged-red fibers is a maternally inherited disease that is characterized by myoclonic epilepsy, cerebellar ataxia and progressive muscular weakness. The present study reports the case of a 25-year-old male who presented with paroxysmal left upper limb tics and weakness for two years. Neurological examination revealed intact cranial nerves, decreased deep tendon reflexes...

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The Red-Edge Effects in Laurdan Solutions

The luminescence spectrum of laurdan in glycerol consists of two bands with maxima at 425 and 500 nm. The strong dependence of this spectrum on the energy of the excitation quanta is demonstrated. When, excited on the red-edge slope of the absorption band, it is investigated at various temperatures. The biggest red shift (about 10 nm) of λmax is found for the long wavelength band at low tempera...

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Diagnosis of mitochondrial diseases: clinical and histological study of sixty patients with ragged red fibers.

BACKGROUND Mitochondrial diseases are caused by mutations in mitochondrial or nuclear genes, or both and most patients do not present with easily recognizable disorders. The characteristic morphologic change in muscle biopsy, ragged-red fibers (RRFs) provides an important clue to the diagnosis. MATERIALS AND METHODS Demographic data, presenting symptoms, neurological features, and investigati...

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ژورنال

عنوان ژورنال: Nature

سال: 2000

ISSN: 0028-0836,1476-4687

DOI: 10.1038/35039651