Friedreich's ataxia is one of the best defined and most common forms o
f hereditary ataxia of unknown aetiology. It is transmitted in an auto
somal recessive manner, appearing sporadically, usually in childhood o
r adolescence. The case of an elderly patient with a possible diagnosi
s of late-onset Friedreich's ataxia is reported; this is thought to be
the only such case in the literature. The 91-year-old Angle female pr
esented with ataxia that had been progressive over the last 5 years. M
agnetic resonance imaging scans of the head revealed mild peripheral c
erebellar atrophy and moderate cerebral atrophy. The patient's parents
were unaffected but two of her six siblings had had Friedreich's atax
ia starting in childhood, and four of her grandfather's siblings had h
ad an undiagnosed illness that left them in wheelchairs early in life,
Friedreich's ataxia was diagnosed in view of the strong family histor
y and non-revealing magnetic resonance imaging of the brain.