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Brain levels of thiamine and its phosphate esters in Friedreich's ataxia and spinocerebellar ataxia type 1
Author(s) -
Bettendorff Lucien,
Mastrogiacomo Frank,
LaMarche Jacques,
Diožić Slobodan,
Kish Stephen J.
Publication year - 1996
Publication title -
movement disorders
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 3.352
H-Index - 198
eISSN - 1531-8257
pISSN - 0885-3185
DOI - 10.1002/mds.870110415
Subject(s) - thiamine , ataxia , spinocerebellar ataxia , cerebrospinal fluid , endocrinology , medicine , cerebellum , thiamine deficiency , chemistry , biochemistry , neuroscience , biology
Decreased blood and cerebrospinal fluid levels of thiamine have been reported in patients with spinocerebellar ataxia disorders. To determine whether a thiamine deficiency is present in the brain, we measured levels of thiamine and its phosphate esters thiamine monophosphate (TMP) and thiamine diphosphate (TDP), in postmortem cerebellar and cerebral cortices of patients with Friedreich's ataxia (FA) and spinocerebellar ataxia type 1 (SCA1). Brain levels of free (nonphosphorylated) thiamine, TMP, TDP, and total thiamine in FA and SCAI were, on average, not significantly different from control values. However, a nonsignificant trend was observed for slightly reduced levels of TDP and total thiamine in cerebellar cortex of the SCA1 patients, a finding that might be related to the severe neuronal damage in this brain area. We conclude that in FA, brain thiamine concentrations are normal, whereas in SCA1 the levels are, at most, only slightly reduced.