Biochemical aspects of motor neurone disease--Madras pattern

Abstract

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.

Carbohydrate metabolism in motor neurone disease.

“…This finding is in contrast to that of Valmikirathan et al (1973) who found low citrate levels in patients with the Madras variety. This is the first study of blood citrate levels in classical motor neurone disease; and these findings would suggest that motor neurone disease-Madras is a distinct entity biochemically as well as clinically (Valmikirathan et al, 1973).…”

“…Our patients (with one exception) failed to demonstrate the abnormal pyruvate metabolism shown by Cumings (1962) in classical motor neurone disease, and by Valmikirathan et al (1973) in the Madras variety of the disease.…”

“…It would appear, however, that this mechanism is unlikely as the liver is the main source for glucose disposal. In addition, it has been shown that, in some forms of motor neurone disease, basal pyruvate concentrations are raised (Cumings, 1962), and that pyruvate concentrations are significantly higher at 60 minutes after a glucose load (Shahani et al, 1971 have significantly low fasting serum citrate levels, and the ratio of citrate to pyruvate was reduced (Valmikirathan et al, 1973).…”

Madras motor neuron disease (MMND): Clinical description and survival pattern of 116 patients from Southern India seen over 36 years (1971–2007)

“…A characteristic biochemical finding of persistently low citrate and elevated pyruvate level has been described in patients with MMND [39]. The fact that MMND is prevalent in southern India and with this geographic distribution and clustering it is worthy of studying the ethnic, genetic and environmental factors contributing to the causation of this rare and intriguing disorder.…”

A Madras Motor Neuron Disease Patient With Cerebellar Atrophy: A New Clinical Feature

“…Mitochondrial DNA variations may have an indirect role in the pathogenesis of MMND ( Govindaraj et al, 2013 ), but we did not check mitochondrial DNA in this case. Additionally, Valmikinathan et al (1973) suggested that the pathophysiology of MMND might be associated with altered citrate metabolism. The first biopsy proposed that the possibility of an inflammatory etiology for MMND needs to be considered ( Phanthumchinda et al, 1996 ).…”