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Correlation Between Serum Lipoamide Dehydrogenase Activity and Phosphatidylcholine Therapyin Friedreich's Ataxia

Published online by Cambridge University Press:  18 September 2015

S.B. Melançon ,
G. Fontaine ,
G. Geoffroy ,
M. Vanasse ,
L. Dallaire  and
M. Potier
S.B. Melançon
Affiliation:
Centre de Recherche Pédiatrique, Hôpital Ste-Justine; Département de Pédiatric et Neurologie, Université de Montréal
G. Fontaine
Affiliation:
Centre de Recherche Pédiatrique, Hôpital Ste-Justine; Département de Pédiatric et Neurologie, Université de Montréal
G. Geoffroy
Affiliation:
Centre de Recherche Pédiatrique, Hôpital Ste-Justine; Département de Pédiatric et Neurologie, Université de Montréal
M. Vanasse
Affiliation:
Centre de Recherche Pédiatrique, Hôpital Ste-Justine; Département de Pédiatric et Neurologie, Université de Montréal
L. Dallaire
Affiliation:
Centre de Recherche Pédiatrique, Hôpital Ste-Justine; Département de Pédiatric et Neurologie, Université de Montréal
M. Potier
Affiliation:
Centre de Recherche Pédiatrique, Hôpital Ste-Justine; Département de Pédiatric et Neurologie, Université de Montréal
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Serum lipoamide dehydrogenase activity and kinetics were studied in nine patients with Friedreich's ataxia before and three months after therapy with oral lecithin. Results disclosed a significant reduction in LAD inhibition by NADH in all patients after therapy. Three patients normalized their increased Km for lipoamide and one patient showed the opposite results after therapy. Two patients ceased lecithin after one month. All seven patients who remained in the trial group and one additional patient, showed subjective and objective signs of improvement in physical resistance. This study has offered some biochemical basis for the apparent clinical improvement in patients with Friedreich's ataxia who undergo lecithin therapy.

Type
Quebec Cooperative Study of Friedreich's Ataxia
Information
Canadian Journal of Neurological Sciences , Volume 7 , Issue 4 , November 1980 , pp. 413 - 416
Copyright
Copyright © Canadian Neurological Sciences Federation 1980

References

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