Brain levels of thiamine and its phosphate esters in Friedreich's ataxia and spinocerebellar ataxia type 1.

Abstract

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 SCA1 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.

• Authors: L Bettendorff, F Mastrogiacomo, J LaMarche, S Dozić, S J Kish
• Journal: Movement disorders : official journal of the Movement Disorder Society (Mov Disord) Vol. 11 Issue 4 Pg. 437-9 (Jul 1996) ISSN: 0885-3185 [Print] United States
• PMID: 8813226 (Publication Type: Journal Article, Research Support, Non-U.S. Gov't, Research Support, U.S. Gov't, P.H.S.)

Chemical References

• Thiamine Monophosphate
• Thiamine Pyrophosphate
• Thiamine

Topics

• Adolescent
• Adult
• Cerebellar Cortex (pathology)
• Cerebral Cortex (pathology)
• Child
• Child, Preschool
• Female
• Friedreich Ataxia (pathology)
• Humans
• Male
• Middle Aged
• Reference Values
• Spinocerebellar Degenerations (pathology)
• Thiamine (metabolism)
• Thiamine Monophosphate (metabolism)
• Thiamine Pyrophosphate (metabolism)