Hereditary spastic paraplegia

Historical note and nomenclature. Hereditary spastic paraplegia is the name given to a group of diseases that are heterogenous and inherited, in which the main clinical feature is progressive spasticity of the lower limbs. The original description of hereditary spastic paraplegia was made by Strümpell in 1880. He described “a pure spastic movement disorder of the legs” in 2 brothers who developed a spastic gait at the ages of 37 and 56 years. Their father was said to be “a little lame,” suggesting that the mode of inheritance might be autosomal dominant (Strumpell 1880). He later defined additional cases and described the pathological changes of the spinal cord, especially the degeneration of the pyramidal tracts. At the end of 19th century, Lorrain published 3 cases with similar clinical features (Lorrain 1898). The disease was also called Strümpell-Lorrain syndrome. Many cases with additional neurologic features were added to the literature, and many case reports seem to have given different names to possibly the same disease.

Wide-Open Eyes to the “Snake-Eye Sign”

Patient with progressive four-limb weakness, unsteadiness and falls. Examination revealed paretic-spastic gait, spastic tetraparesis, brisk tendon reflexes, reduced pain sensation at the right side and bilateral Hoffman and Babinski signs. Cervical magnetic resonance (MRI) revealed the “snake-eye sign” and spinal cord compression by protruded discs during cervical extension.