1043-3155 Pediatr Neurol Briefs pedneurbriefs Pediatr Neurol Briefs Pediatric Neurology Briefs Pediatr Neurol Briefs 2166-6482 1043-3155 2166-3155 Pediatric Neurology Briefs Publishers Chicago, IL, USA PNB-2-58-b 10.15844/pedneurbriefs-2-8-3 Heredo-Degenerative Diseases Neurology Pediatrics Nervous System Diseases Child Development Brain Diseases Neurosurgery Child Infant Friedreich's Ataxia and Glucose Metabolism http://orcid.org/0000-0002-0173-7931 Millichap J. Gordon MD 1 2 * Division of Neurology, Children's Memorial Hospital, Chicago, IL Departments of Pediatrics and Neurology, Northwestern University Feinberg School of Medicine, Chicago, IL Correspondence: Dr. J. Gordon Millichap, E-mail: jgmillichap@northwestern.edu 08 1988 01 08 2016 2 8 58 59 Copyright: © 1988 The Author(s) 1988 This work is licensed under the Creative Commons Attribution 4.0 International License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. Glucose metabolism alterations in Friedreich's ataxia

Glucose metabolism was investigated in 21 patients with FA at the Instituto Neurogico, Cattedra di Clinica Medica, Milan, Italy.

 Glucose Metabolism Glucose Tolerance Insulin Resistance

Glucose metabolism was investigated in 21 patients with FA at the Instituto Neurogico, Cattedra di Clinica Medica, Milan, Italy. Abnormalities of glucose tolerance occurred in 5 (23.8%) and 4 were diabetic (19%). By oral glucose tolerance tests, the plasma glucose levels of 5 patients were 140-200 mg/ml 2 hours after glucose ingestion. Plasma insulin levels of glucose-intolerant patients were significantly higher than controls after 180 minutes following glucose ingestion. Plasma glucagon levels of FA patients were higher than controls, and plasma lactate levels of glucose intolerant FA patients were higher than controls. By intravenous glucose load, glucose responses did not differ significantly between patients and controls. The results showed that FA is associated with insulin resistance, B-cell deficiency, and type I diabetes. The alterations might be genetically linked or metabolically related to the primary defect in FA. [1]

COMMENT. Friedreich's ataxia, the most common heredodegenerative ataxia, is recessively inherited. The incidence of diabetes in FA patients is high, glucose intolerance during oral glucose tests have been reported previously, but the abnormalities of glucose metabolism have not previously been studied in detail. The abnormalities of glycemic control in FA may be due to the interplay of insulin resistance and B-cell dysfunction or their independent effects.

 Finocchiaro G Baio G Micossi P Pozza G di Donato S Glucose metabolism alterations in Friedreich's ataxia Neurology 1988 Aug 38 8 1292 1296 10.1212/WNL.38.8.1292 3041313