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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.
This leads to difficulties climbing stairs and rising from a sitting position. Weakness is often relieved temporarily after exertion or physical exercise. High temperatures can worsen the symptoms. Weakness of the bulbar muscles (muscles of the mouth and throat) is occasionally encountered. [4] Weakness of the eye muscles is uncommon.
Symptoms may include difficulty with balance, weakness and stiffness in the legs, and clumsiness. Other common symptoms are spasticity (involuntary muscle contraction due to the stretching of muscle, which depends on the velocity of the stretch) in the hands, feet, or legs, foot dragging , and speech and swallowing problems due to involvement ...
Various patterns of muscle weakness occur in different motor neuron diseases. [6] Weakness can be symmetric or asymmetric, and it can occur in body parts that are distal, proximal, or both. According to Statland et al., there are three main weakness patterns that are seen in motor neuron diseases, which are: [6] [9]
Tests used in the diagnostic process include MRI, clinical examination, and EMG. EMG tests in patients who do have PMA usually show denervation (neuron death) in most affected body parts, and in some unaffected parts too. [2] It typically takes longer to be diagnosed with PMA than ALS, an average of 20 months for PMA vs 15 months in ALS.
Multifocal motor neuropathy (MMN) is a progressively worsening condition where muscles in the extremities gradually weaken.The disorder, a pure motor neuropathy syndrome, is sometimes mistaken for amyotrophic lateral sclerosis (ALS) because of the similarity in the clinical picture, especially if muscle fasciculations are present.
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 ...
EMG can also be used for indicating the amount of fatigue in a muscle. The following changes in the EMG signal can signify muscle fatigue: an increase in the mean absolute value of the signal, increase in the amplitude and duration of the muscle action potential and an overall shift to lower frequencies. Monitoring the changes of different ...