This is a complex form of spastic paraplegia in which primarily lower limb spasticity is associated with dysarthria, sensory disturbances, cognitive deficits, muscle wasting and mild ataxia. There is, however, considerable variability in age of onset and rate of symptom progression. The first motor symptoms are often evident when children start walking, which is often delayed and clumsy. However, evidence of spasticity may be present in children under 1 year of age. Some patients have normal mental functions while others are considered mentally retarded. The MRI reveals patchy leukodystrophy and degeneration of both corticospinal and spinocerebellar tracks was found in an autopsied individual. Progression is relentless with many individuals requiring assistive devices such as crutches or walkers by early adult life.
