Osteoporosis, osteopenia appear common in Friedreich’s ataxia

From Clinical Neurology News, 21 June 2013:

Friedreich’s ataxia patients may be at greater risk of osteopenia and osteoporotic fractures, according to a study showing significant decreases in bone mineral density at key sites such as the femoral neck.

Data presented in a poster at the international congress of Parkinson’s Disease and Movement Disorders also showed a correlation between Friedreich’s ataxia disease severity measures and bone mineral density measures.

Dr. Wolfgang Nachbauer of the department of neurology at Innsbruck (Austria) Medical University and his colleagues measured bone mineral density of the femur, lumbar vertebral column, radius, and ulna, using dual-energy x-ray absorptiometry in 20 patients with Friedreich’s ataxia. Mean age of the patients was 39.4 years.

They found 45% of patients (9 of 20) had osteoporosis at the femur, and 20% (4 of 20) had osteopenia at the same site, while bone mineral density measurements of the lumbar vertebral column showed osteopenia in 30% (6 of 20) and osteoporosis in 10% (2 of 20). Seven patients (35%) had osteopenia in the forearm.

Dr. Nachbauer said this was the first study exploring the incidence of osteopenia and osteoporosis in patients with this disorder.

“We observed clinically some pathological fractures within our Friedreich’s ataxia patients and together with the known action of the iron metabolism it made us think there could be an increased prevalence of osteoporosis and osteopenia with Friedreich’s ataxia,” Dr. Nachbauer said in an interview.

Friedreich’s ataxia is a neurodegenerative disorder caused by a mutation in the frataxin gene, which codes for a protein involved in mitochondrial oxidative metabolism and iron homeostasis. Nonneurologic manifestations of Friedreich’s ataxia include scoliosis and foot deformities. Read more.

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