Hereditary spastic paralysis- infantile onset ascending


A rare inherited progressive condition where the muscles of the arms, legs and face become increasingly weak and stiff due to damage to nerve cells that control muscle movement. The legs are affected first and then the arms and face - the symptoms ascend up the body. This condition involves mutations in the same gene and overlapping symptoms with juvenile primary lateral sclerosis but the difference is that primary lateral sclerosis only involves degeneration of the upper motor neurons whereas infantile-onset spastic paralysis is more severe and involves degeneration of upper and lower motor neurons


* Progressive muscle weakness * Progressive muscle stiffness * Stiff leg muscles by age of 2 * Weak leg muscles by age of 2 * Arm weakness by age of 8 * Arm stiffness by age of 8 * Slow eye movements by early adolescence * Speech difficulty by early adolescence * Swallowing difficulty by early adolescence


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Prognosis of Hereditary spastic paralysis, infantile onset ascending: usually wheelchair bound by late childhood or early adolescence