VII. EFFECTS OF niOFICIENCY 559 



with iiuiscular dystrophy. There was a tainilial history of the disea.se, as 

 one older brother of our patient and a maternal uncle had already died after 

 runnino- a typical course. Within the next 2 years this youn{j;er brother de- 

 velopetl tlu> typical clinical picture of rapidly progressing muscular dys- 

 trophy. It is possible that disintegration of muscle cells was already in 

 progress before the appearance of detectable dysfunction. It is also con- 

 ceivai)le that some inborn anomaly of vitally important nucleotide me- 

 tabolism is responsible for the deterioration seen in clinical dystrophy." 

 .\ generalized aminoaciduria observed in muscular dystrophy--- may reflect 

 muscle l)reakdown and not a primary metabolic disturbance. 



It is beyond the scope of this chapter to review the numerous reports 

 affirming or denying the therapeutic usefulness of wheat germ products or 

 tocopherol in human muscular dystrophy. Over a period of more than 15 

 years the results have been predominantly negative. This is not surprising 

 in ^•iew of the normal levels of tocopherol found in the blood^^®- -^^ and 

 in ^'arious tissues and organs. ^^^' -^^'^ It is possible, however, that in indi- 

 viduals with muscular dystrophy tocopherols cannot exert their normal 

 functions in skeletal muscles, either because of a metabolic block such as 

 referred to by Minot or because of an inability to effectively convert to- 

 copherols to their hydroquinones or to other compounds which exert the 

 antidystrophy efTects of vitamin E. It is this general approach which has 

 been followed by ]\Iilhorat el al.~-^' "^''' -^^^ who report that (//-a-tocopheryl- 

 hj'drociuinone and related compounds are effective in reducing creatinuria 

 in dystrophic patients and also in curing dystrophy of vitamin E deficiency 

 in rabbits. Unquestionably, this constitutes an important area for continued 

 investigation. 



Other investigators"^""" are of the opinion that wheat germ oil and to- 

 copherol are effective in treating those diseases of muscle which they clas- 

 sify as collagen diseases; namely, dermatomyositis and menopausal dys- 

 trophy. The latter term has been proposed by Shy and McEachern--^ for 



2" S. R. Ames and H. A. Risley, Proc. Soc. ExpU. Biol. Mel. 68, 131 (1948). 



"3 A. S. Minot and H. E. Frank, .4m. ./. Diseases Children 67, 371 (1944). 



223* K. E. Mason, M. Y. Dju, and S. J. Chapin, Federation Pmc. 12, 422 (195.3); Proc. 



Isi & 2nd Med. Conf. (1951-52) Muscular Dystr. Ass'ns. Amer., Xew York p. 94. 

 "^ A. T. Milhorat, J. B. Mackenzie, S. Uiick, H. Rosenkrantz, and W. E. Bartels, 



Ann. N. Y. Acad. Sci. 52, 334 (1949). 

 ^z-" J. B. Mackenzie, H. Rosenkrantz, S. Ulick, and A. T. Milhorat, ./. Biol. Chem. 



183, 655 (1950). 

 ^^^ A. T. Milhorat. Proc. Ist & 2nd .Med. Conf. (1951-52) Muscular Dystr. Ass'ns. 



Amer., Xew York p. 78. 

 =25 D. McEachern, Bull. X. Y. Acad. Med. 27, 3 (1951). 

 '-^ R. Raliinovitch, W. C. Gibson, and D. McEachern, J. Xeurol. Xeurosury. Psychiai. 



14, 95 (1951). 

 --' C!. .M. Shy and D. McEachern, J. .Xeurol. Xeurosury. Psychial. 14, 101 (1951). 



