Friedreich's Ataxia in the Elderly

Abstract

Friedreich's ataxia is one of the best defined and most common forms of hereditary ataxia of unknown aetiology. It is transmitted in an autosomal recessive manner, appearing sporadically, usually in childhood or adolescence. The case of an elderly patient with a possible diagnosis of late-onset Friedreich's ataxia is reported; this is thought to be the only such case in the literature. The 91-year-old Anglo female presented with ataxia that had been progressive over the last 5 years. Magnetic resonance imaging scans of the head revealed mild peripheral cerebellar atrophy and moderate cerebral atrophy. The patient's parents were unaffected but two of her six siblings had had Friedreich's ataxia starting in childhood, and four of her grandfather's siblings had had an undiagnosed illness that left them in wheelchairs early in life. Friedreich's ataxia was diagnosed in view of the strong family history and non-revealing magnetic resonance imaging of the brain.