Strümpell-Lorrain Familial Spasmodic Paraplegia

Thursday, May 10, 2012

The Strümpell-Lorrain's familial spasmodic paraplegia is a hereditary disease characterized by spastic paraplegia (abnormal muscle constraction and stiffness of the lower limbs), difficulty in lifting the feet off the ground when walking, with the toe dragging, muscle spasm, and sometimes speech disorders. Beginning before the age of 18, the symptoms of the Strümpell-Lorrain familial spasmodic paraplegia are caused by a systematic degeneration of the pyramidal tracts, which are composed of motor axons descending from the cerebral cortex; this degeneration does not stretch beyond the spinal cervical portion. This degenerating process of pyramidal tracts is accompanied with alteration of cerebellar tracts. The prognosis is generally good, even though the disease advance and improvement is slow.