Hereditary Spastic Paraplegia

The hereditary spastic paraparesis are a group of rare hereditary, which are characterized by a progressive, spinal, nichtsegmentale spastic leg paresis, sometimes with mental retardation, seizures, and other extraspinalen deficits. The diagnosis is made clinically and is sometimes done by genetic testing. Treatment is symptomatic, including drugs to relieve spasticity.

(See also overview of diseases of the spinal cord.)

The hereditary spastic paraparesis are a group of rare hereditary, which are characterized by a progressive, spinal, nichtsegmentale spastic leg paresis, sometimes with mental retardation, seizures, and other extraspinalen deficits. The diagnosis is made clinically and is sometimes done by genetic testing. Treatment is symptomatic, including drugs to relieve spasticity. (See also overview of diseases of the spinal cord.) Varies The genetic basis of hereditary spastic paraplegia and is unknown to many forms. In all forms the descending corticospinal tracts degenerate and, to a lesser extent, the background strands and spinocerebellar tracks, sometimes with loss of anterior horn cells. The disease can use depending on the specific genetic form at any age from 1 year up in the high age. Symptoms and signs The symptoms of spastic paresis heriditären includes spastic leg paralysis with increasing transitional difficulties, hyperreflexia, clonus, and positive Babinski sign. The sensitivity and Sphinkterfunktionen are usually spared. The arms can also be affected. The deficits are not limited to a spinal segment. Some patients have forms (spinocerebellar z. B. and ocular symptoms, extrapyramidal symptoms, optic atrophy, retinal degeneration, mental retardation, dementia, polyneuropathy) also extraspinal neurological deficits. Diagnosis Clinical assessment A hereditary spastic paraplegia may be considered for a positive family history and signs of spastic paraparesis into consideration. The diagnosis of hereditary spastic paraplegia is made by excluding other causes and sometimes by genetic studies. Symptomatic treatment, including medications for the relief of spasticity Treatment is symptomatic in all forms of hereditary spastic paraplegia. Against spasticity baclofen is 10 mg p.o. 2 times / day, po increase as needed up to 40 mg 2 times / day, used. Alternatives are diazepam, clonazepam, dantrolene, botulinum toxin (botulinum toxin Type A and Type B) and tizanidine. Physical therapy and exercise can help maintain mobility and muscle strength to improve the level of agility and endurance, reduce fatigue and prevent cramps. Some patients benefit from use of a splint or crutches.

Health Life Media Team

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