Hereditary spastic paraplegia [SEP] named after
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.
Hereditary spastic paraplegia
Hereditary spastic paraplegia (HSP) is a group of inherited diseases whose main feature is a progressive gait disorder. The disease presents with progressive stiffness (spasticity) 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