Henry Ford Hospital Medical Journal


Hereditary spastic paraplegia was diagnosed in 19 patients belonging to four families. All had spastic gait and upper motor neuron signs in lower limbs. In family A, the disease was expressed in this manner, although one member had muscle wasting in an upper limb. There was additional involvement of the posterior columns in six of the eight affected members of family B and cerebellar ataxia was noted in upper limbs of three of them. Posterior columns were also involved in three of the four affected members of family C. The disease was transmitted in these three families by an autosomal dominant gene. With autosomal recessive inheritance in family C, spastic gait and mental retardation were present during early childhood in the three affected members. Abnormalities of feet, vertebrae and other anomalies were also encountered.