SUMMARY A detailed study of four cases of MND-Madras has shown a significantly low plasma citrate level in these subjects. Comparison with other related groups of neurological disorders indicates the citrate/pyruvate ratio to be of diagnostic value in MND-Madras. This seems to suggest that the pathophysiology of MND-Madras may centre round altered citrate metabolism.

X simple colour reaction on aqueous extracts of urine from 10 alkaptonuric subjects has been developed for the detection of alkaptonuria. Such extracts invariably give a pinkish brown colour with trace amounts of copper in alkaline medium but aqueous extracts of urine from normal and other pathological cases failed to give this colour.Alkaptonuria is an inborn error associated with the excretion of homogentisic acid in the urine. The most commonly used tests for its detection are the darkening of urine on exposure to air, reduction of Benedict's solution, colour reactions with FeCIi. However, none of these tests are specific for alkaptonuria. While investigating some cases of alkaptonuria, a simple but specific colour reaction was developed for detecting such cases. MET HODSFreshly voided urine samiiples (10-15 ml.) fiom alkaptonuric subjects were concentrated on a boiling water bath.The concentrates were extracted with 3 ml. of n-butanol. The butanol extracts were then partitioned with 5 ml. of distilled water and the aqueous extracts separated for use in the subsequent work. This process removed most of the urinary pigments.To 2 drops of the aqueous extract in a test tube. 2 drops of CUS04 (0 01 a,) were added followed by 5 ml. of distilled water and 2 drops 01N NaOH. The tube was agitated immediately after the addition of alkali and left aside for 20 minutes when a pinkish brown colour developed.These aqueous extracts were usually subjected to paper chromatography in a butanol:acetic acid:water (4:1:5) system to confirm the presence of homogentisic acid.RESULTS AND i)ISC USSIONS fcesh urine samiiples ol alkaptonuric subjects give a reddish brown colour oni addition of alkali. In our experiimiental conditions, the addition of alkali has been shown to have no effect on the colour formation. Howcser, under the same set of experimental conditions.

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