FRIEDREICH'S ATAXIA

Four cases of Friedreich’s ataxia(hereditary ataxia)are reported.Case1 was a Russian boy of 19 who was admitted for epileptic attacks,mentaldeficiency and progressive deformity of feet.He showed pes cavus,smallermuscles in the left leg,ankle clonus,Babinski sign on the right side,impair-ment of sense of recognition of figures drawn on the outer aspect of legs,Darwinian tubercles,lumbralization of the first sacral spine and congenitalurethral valve.Case 2 was a Chinese girl of 18 who came because of pro-gressive deformity of feet for six years.She showed bilateral pes cavus,diminution of deep sensations,loss of patellar and ankle reflexes and sugges-tive Chaddock sign.She improved after operative treatment.These twocases were isolated cases.Case 3 was a Chinese research associate,47 yearsof age,who was admitted for Guillane-Barré syndrome.He presented thehistory of difficulty in jumping and running from childhood.His mother andelder brother had the same trouble.His 7 year old son showed high insteps.The patient had Friedreieh’s foot,hypotonia,smaller muscles in the right leg,broad station,staggering gait,impairment of deep sensation,loss of deepreflexes,albumino-cytologic dissociation and diabetes mellitus.Case 4 was aChinese male of 19 who had unsteady gait and indistinct speech from earlychildhood,progressive weakness of both legs as from 9 years of age,and whohad been confined to bed for a year.He presented a small head,systolicmurmur,bilateral optic atrophy,slow and slurred speech,ataxia,scissors gait,rebound phenomenon,Romberg sign,absence of deep and abdominal reflexes,bilateral Babinski and Chaddoek signs,abnormal collection of air in the rightpariental region in the encephalogram,and electrocardiographic evidence ofleft heart strain.The first three cases appear to belong to Friedreich’s spinaltype and the last to Marie’s cerebellar type.It is surmised that Filatov’stissue therapy would be useful to halt the progress of this and other degenera-tive diseases of the nervous system.