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Elevated cerebrospinal fluid lactate/pyruvate ratio in Machado‐Joseph disease
Author(s) -
Matsuishi T.,
Sakai T.,
Naito E.,
Nagamitsu S.,
Kuroda Y.,
Iwashita H.,
Kato H.
Publication year - 1996
Publication title -
acta neurologica scandinavica
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.967
H-Index - 95
eISSN - 1600-0404
pISSN - 0001-6314
DOI - 10.1111/j.1600-0404.1996.tb00174.x
Subject(s) - citric acid cycle , cerebrospinal fluid , pyruvate dehydrogenase complex , endocrinology , medicine , lactate dehydrogenase , lactic acid , mitochondrion , pyruvate decarboxylation , chemistry , metabolism , biology , biochemistry , enzyme , genetics , bacteria
To identify the metabolic alterations related to mitochondrial functions in Machado‐Joseph disease (MJD), we analyzed the cerebrospinal fluid (CSF) levels of lactate, pyruvate, and citric acid cycle intermediates by high performance liquid chromatography (HPLC) in 7 Japanese patients with that disease and then measured some mitochondrial enzymes. Their mean age was 46 years. Diseased controls were matched by age to the patients studied. The CSF level of lactate was significantly elevated, pyruvate was significantly decreased, and the lactate/pyruvate (L/P) ratio was significantly elevated in the patients with MJD. There were no significant differences of citric acid cycle intermediates of the CSF between the patients and the controls. We measured the native and dichloroacetate (DCA)‐activated pyruvate dehydrogenase complex (PDHC) activities, and mitochondrial electron transport activities in 3 patients with MJD, and found these activities to be normal. Therefore, the increased CSF lactate, increased lactate/pyruvate ratio, and decreased pyruvate may reflect the decreased regional cerebral blood flow rather than metabolic derangement of the mitochondria.