Association of Diabetes Mellitus and Friedreich's Ataxia

Progressive hereditary cerebellar dysfunction with hypoactive knee and ankle jerks and with onset in preadolescence was first described by Nikolaus Friedreich of Heidelberg in 1863.1This disorder is uncommon and constitutes less than 0.5% of total admissions to the National Hospital of London.2The association of Friedreich's ataxia and diabetes mellitus is rare, and its occurrence in two sisters prompts this report. Report of Cases Case 1. —An 18-year-old girl was first seen in the New England Deaconess Hospital on Oct 19, 1963, with a history of polyuria, intense thirst, and malaise of two months' duration. Glycosuria had been found on a routine test by her mother; there had been no urine tests for the two previous years. The patient was known to have had ataxia since age 5. Prenatal course and delivery were reported to have been normal. The child sat up at 6 months and walked