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Thiamin Monophosphate in the CSF of Patients With Amyotrophic Lateral Sclerosis
Marco Poloni, MD;
Cesare Patrini;
Bruno Rocchelli, MD;
Gianguido Rindi, MD
Arch Neurol. 1982;39(8):507-509.
Abstract
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• Free thiamin and thiamin monophosphate levels were determined by an electrophoretic fluorometric micromethod in plasma and CSF of patients with amyotrophic lateral sclerosis (ALS), alcoholics, and controls. In plasma of patients with ALS as well as in plasma and CSF of alcoholics, both thiamin and thiamin monophosphate concentrations were decreased so that the thiamin-thiamin monophosphate (T/TMP) ratio remained unchanged compared with that of controls. In CSF of patients with ALS, however, thiamin monophosphate values decreased much more than thiamin levels, so that the T/TMP ratio was significantly increased. The selective impairment of thiamin monophosphate production by nerve cells is likely to result from the reduction of the activity of thiamin pyrophosphatase, an enzyme synthetized and highly concentrated in the Golgi complex. Thiamin pyrophosphatase is known to diminish in ALS as well as in experimental motor neuronal degeneration or axotomy. Thus, the T/TMP ratio could be taken as an index of the impairment of neuronal protein synthesis in ALS.
Author Affiliations
From the Neurological Clinic (Drs Poloni and Rocchelli), and the Institute of Human Physiology (Dr Rindi and Mr Patrini), University of Pavia (Italy).
Footnotes
Accepted for publication Nov 1, 1981.
Reprint requests to Institute of Human Physiology, University of Pavia, Via Forlanini 6, 27100 Pavia, Italy (Dr Rindi).
THIS ARTICLE HAS BEEN CITED BY OTHER ARTICLES
Amyotrophic Lateral Sclerosis: Recent Advances in Pathogenesis and Therapeutic Trials
Mitsumoto et al.
Arch Neurol 1988;45:189-202.
ABSTRACT
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