Everything about hereditary spastic paraplegia

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hereditary spastic paraplegia

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, Strumpell 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.

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paraplegia spastic cerebral palsy cerebral palsy myotonic dystrophy ataxia spastic quadriplegia parkinsonism locomotor ataxia congenital myasthenic syndrome huntington's disease

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Amelie Round's life with Hereditary Spastic Paraplegia

Amelie Round's life with Hereditary Spastic Paraplegia

Eight-year-old Amelie Round was diagnosed with Hereditary Spastic Paraplegia (HSP) four years ago, a lifelong and progressive condition which affects less than 0.1 per cent of the population.<br /><br />She is preparing to board a flight to America to undergo pioneering surgery which could change her life.

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What is hereditary spastic paraplegia?

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, Strumpell 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.

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