Paraplegia and mongolism in twins

Abstract

Clinical record M. G., aged four years and six months, was an undoubted mongol, showing the typical signs of round head, slanting eyes, broad face and incurved little finger. She had an internal strabismus and chronically inflamed conjunctivae. She suffered from a paraplegia, a condition rarely seen amongst mongols. On examination of her nervous system the following data were obtained: There was marked spasticity of the legs, especially of the ankles. She walked unsteadily in the equino-varus position, the heels only occasionally reaching the ground. On attempting to rise off the floor she showed a marked ' cuisse- tronc' sign and then could only raise herself by getting on to all fours. Her arms were normal as to muscular tone, reflexes and voluntary movements, but showed a slight degree of choreic movement. Her legs were hypertonic, paretic, showed exaggerated tendon reflexes, the knee-jerks being obtained from well down the shins (i.e. Strumpell's sign was positive). The plantar reflexes were extensor, but the Oppenheim reflexes were negative. No clonus was obtained. There was no evidence of cerebellar dysfunction.