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What is Hereditary spastic paraplegia Explain Hereditary spastic paraplegia
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~~~ Hereditary spastic paraplegia ~~~ • Title: What is Hereditary spastic paraplegia?, Explain Hereditary spastic paraplegia • Created on: 2018-10-23 • Source Link: https://en.wikipedia.org/wiki/Heredit... • ------ • Description: Hereditary spastic paraplegia is a group of inherited diseases whose main feature is a progressive gait disorder. The disease presents with progressive stiffness and contraction in the lower limbs. HSP is also known as hereditary spastic paraparesis, familial spastic paraplegia, French settlement disease, or Strumpell-Lorrain disease. The symptoms are a result of dysfunction of long axons in the spinal cord. The affected cells are the primary motor neurons; therefore, the disease is an upper motor neuron disease. HSP is not a form of cerebral palsy even though it physically may appear and behave much the same as spastic diplegia. The origin of HSP is different from cerebral palsy. Despite this, some of the same anti-spasticity medications used in spastic cerebral palsy are sometimes used to treat HSP symptoms. HSP is caused by defects in transport of proteins, structural proteins, cell maintaining proteins, lipids, and other substances through the cell. Long nerve fibers are affected because long distances make nerve cells particularly sensitive to defects in these mentioned mechanisms.The disease was first mentioned in 1876 by Adolph Seeligmüller, a German neurologist, who described a family of four affected children with spasticity. Further cases were described in 1883 by Adolph Strümpell, a German neurologist. Those cases were described more extensively in 1888 by Maurice Lorrain, a French physician. Due to their contribution in describing the disease, it is still named Strümpell-Lorrain disease in French speaking countries. The term hereditary spastic paraplegia was coined by Anita Harding in 1983. • ------ • To see your favorite topic here, fill out this request form: https://docs.google.com/forms/d/e/1FA... • ------ • Source: Wikipedia.org articles, adapted under https://creativecommons.org/licenses/... license. • Support: Donations can be made from https://wikimediafoundation.org/wiki/... to support Wikimedia Foundation and knowledge sharing.
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