Abstract

A Korean family of hereditary 'pure' spastic paraplegia is presented with literature review.

There were 10 affected members involving 4 generations and the onsets were in the 1^st decade by history.

The symptoms were spastic paraplegia with hyperactive deep tendon reflexes and pathologic reflexes but there were no muscle atrophy, sensory changes, sphincter nor genitourinary abnormality.

There were prolongation in the P₄₀ potentials of bilateral tibial somatosensory evoked potential study and central motor conduction time of magnetic stimulation study.

The mode of inheritance were autosomal dominant and were consistant with those of TypeⅠ of AE Harding's classification.

• Keywords: Hereditary spastic paraplegia, Toe gait