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The myopathic EMG demonstrates fibrillation potentials. The serum CK level will be normal or low normal. The muscle biopsy will demonstrate the nemaline rods, but as they are less than 1 μm in length they are easily overlooked. The sections must be trichromatically stained and sectioned at a thickness of 2 to 4 μm for effective visualization.
Proximal muscle weakness, exercise intolerance, lactic acidosis, high serum lactate/pyruvate ratio, normal to elevated serum CK, dyspnea, exaggerated cardiorespiratory response to exercise are common symptoms. It may be isolated to the muscle (pure myopathy) or may be systemic including not only myopathy, but also eye abnormalities, peripheral ...
Weakness comes on slowly (over months to years) in an asymmetric manner and progresses steadily, leading to severe weakness and wasting of arm and leg muscles. IBM is more common in men than women. [10] Patients may become unable to perform activities of daily living and most require assistive devices within 5 to 10 years of symptom onset.
Corticosteroids often cause muscle weakness to some degree in patients. Symptoms are usually weakness of the proximal muscles, neck flexor, and in extreme cases, respiratory muscle weakness can also occur. [1] Corticosteroids have not only been found to cause some degree of muscle atrophy, but also a local or diffuse cell death. These side ...
Brody disease (a disease of ion pump transport) has symptoms similar to myotonia congenita, however, the delayed muscle relaxation is pseudo-myotonia as the EMG is normal. [5] Other diseases that exhibit pseudo-myotonia are myositis , glycogen storage diseases , hyperkalemic periodic paralysis , root disease, anterior horn cell disorders ...
Electromyography is the measurement and analysis of the electrical activity in skeletal muscles. This technique is useful for diagnosing the health of the muscle tissue and the nerves that control them. [8] EMG measures action potentials, called Motor Unit Action Potentials (MUAPs), created during muscle contraction.
Electromyography (EMG) can be used in diagnosis to rule out myotonia, or muscle stiffness that is detected by EMG. Individuals with BD have stiff muscles but normal EMG results (pseudo-myotonia), where no myotonic discharges are detected. [5] [2] Genetic testing may also be used in the diagnosis of BD to look for mutations in ATP2A1. [11]
Brody disease (also known as Brody myopathy) includes symptoms similar to myotonia congenita, including muscle stiffness and cramping after initiating exercise (delayed muscle relaxation). However, it is pseudo- myotonia as those with Brody disease have normal EMG .