Aran-Duchenne disease: Description, causes and risk factors:A fatal degenerative disease involving the corticobulbar, corticospinal, and spinal motor neurons, manifested by progressive weakness and wasting of muscles innervated by the affected neurons; fasciculations and cramps commonly occur. The disorder is 90-95% sporadic in nature. It affects adults and usually is fatal within 2-5 years of onset. It is the most common subgroup of motor neuron disease, and the only one manifested by a combination of upper and lower abnormalities. Variants include: 1) progressive bulbar palsy, in which isolated or predominant lower brainstem motor involvement occurs; 2) primary lateral sclerosis, in which only upper motor neuron abnormalities are seen; and 3) progressive spinal muscle atrophy, in which only lower motor neuron dysfunction is noted.Alternative Names: Progressive spinal amyotrophy, progressive muscular atrophy, Charcot disease, Duchenne-Aran disease, Lou Gehrig disease.Aran-Duchenne disease leads to progressive muscle weakness, wasting and paralysis due to degeneration of motor neurons in the spinal cord. Muscle weakness and wasting usually starts in the hands and may gradually spread to other muscle groups.Muscle atrophy and muscle tissue loss or wasting are synonymous. Lack of use, poor nutrition and nerve or muscular diseases are major “losing muscle” contributors. Weakness is your key symptom for wasted muscles.Your muscles are in a continual process of producing and breaking down protein. During a period of muscle wasting, this process is out of balance. Simply put, protein is broken down quicker than it's produced.In the absence of disease, healthy muscle fitness balance requires physical activity and nutritional building blocks. The deficiency of either advances to muscular atrophy.Aran-Duchenne caused by non-use bestows the greatest loss of muscle size and strength. A sedentary lifestyle is a primary factor leading to muscle tone lose and atrophy development. Additionally, inadequate nutrition can support muscle wasting in spite of any movement.Luckily, this sort of imbalance causing muscular atrophy is changeable via physical activity and consumption of a healthy diet.Diagnosis:In most cases a diagnosis can be made by the SMN (Survival of Motor Neuron) gene test, which determines whether there is at least one copy of the SMN1 gene by looking for its unique sequences (that distinguish it from the almost identical SMN2) in exons 7 and 8. In some cases, when the SMN gene test is not possible or does not show any abnormality, other tests such as an EMG electromyography (EMG) or muscle biopsy may be indicated.The course of SMA (Spinal Muscular Atrophy) is directly related to the severity of weakness. Infants with the severe form of SMA frequently succumb to respiratory disease due to weakness of the muscles that support breathing. Children with milder forms of SMA naturally live much longer although they may need extensive medical support, especially those at the more severe end of the spectrum.Treatment:Although gene replacement strategies are being tested in animals, current treatment for SMA consists of prevention and management of the secondary effect of chronic motor unit loss. It is likely that gene replacement for SMA will require many more years of investigation before it can be applied to humans. Due to molecular biology, there is a better understanding of SMA. The disease is caused by deficiency of SMN protein, and therefore approaches to developing treatment include searching for drugs that increase SMN levels, enhance residual SMN function, or compensate for its loss.Some Aran-Duchenne diseases do not cut off your ability to work and nutritionally support your muscles. Some form of exercise is often recommended. If you are experiencing a movement disability, you will likely need to discover creative methods for keeping healthy muscles moving to avert tissue loss. Using water or joint bracing are just two muscle assisting means. Neuromuscular electrical stimulation (NEMS) may help as well.DISCLAIMER: This information should not substitute for seeking responsible, professional medical care.
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