METABOLISM IN NEUROMUSCULAR DISEASES '725 



and acetone and diacetic acid are not present in the urine (McCrudden 

 and Sargent (a)). 



Hypocholesterinemia. The cholesterin of the blood in three cases of 

 progressive muscular dystrophy was : 



Case 1. 0.50-1.44 mg. per 100 grams blood, 

 Case 2. 1.18 mg. per 100 grams blood, 

 Case 3. 1.40 mg. per 100 grams blood. 



By the same method, the amount in normal blood runs from 1.60 to 2.40 

 mg. per 100 grams blood and averages about 1.90 mg. (McCrudden and 

 Sargent (&)). 



Hypocholesterinemia alone does not indicate any abnormality in the 

 carbohydrate metabolism; the amount of cholesterin in the blood does 

 not run parallel with the amount of glucose (McCrudden and Sargent (c) ). 

 But, taken together, with the fact that hypercholesterinemia accompanies 

 the hyperglycemia of diabetes (Bloor(c), Joslin, Bloor and Gray), 

 the association of hypocholesterinemia with the hypoglycemia of pro- 

 gressive muscular dystrophy is certainly suggestive. In this connection it 

 is of significance to note that in the case which showed such marked im- 

 provement, followed \)j McCrudden and Sargent (a), the cholesterin con- 

 tent of the blood increased with the improvement in strength and the rise in 

 blood sugar. The amount rose rapidly to 0.177 per cent and later to 0.211 

 per cent, both of these normal figures. 



Delayed Glucose Utilization. It has been observed by Janney that 

 the administration of 1.75 grams of glucose per kilogram body weight in 

 forty per cent solution causes a rise in the blood sugar in normal indi- 

 viduals of about twenty per cent, followed by a return to normal after one 

 and one-half to two hours ; glycosuria does not occur. This is the glucose 

 tolerance test, the details of which were first worked out by Hamman and 

 Hirschman. The results have since been confirmed by other observers 

 (Rohdenberg, Bernard, and Krehbiel). According to Janney, Goodhart, 

 and Isaacson, the result of this test in cases of progressive muscular 

 dystrophy is different in three respects : 3 



(1) The percentage rise is greater than in the normal. In nine 

 cases studied, the increase in blood sugar averaged 65 per cent as com- 

 pared with 20 per cent in normal cases. But on account of the lower base 

 level of blood sugar concentration from which the rise starts, the absolute 

 level attained is generally no higher than the level attained normally. 



(2) In five out of nine cases which they studied, there was a distinct 

 delay in the disappearance of carbohydrate from the blood. Instead of 

 a fall in the blood sugar to normal before the end of the second hour after 

 glucose ingestion, the normal value was not reached until the end of the 

 fourth or fifth hour after ingestion. 



3 Confirmed recently by Brock and Kay and by Gibson, Martin, and Buell. 



