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Superior Cerebellar Peduncle Atrophy in Friedreich’s Ataxia Correlates with Disease Symptoms
- Source :
- The Cerebellum. 10:81-87
- Publication Year :
- 2010
- Publisher :
- Springer Science and Business Media LLC, 2010.
-
Abstract
- Friedreich's ataxia (FRDA) is the most common early onset inherited ataxia with clinical manifestations, including gradual progression of unremitting cerebellar-sensory ataxia, peripheral sensory loss, loss of lower limb tendon reflexes and hypertrophic cardiomyopathy. Although atrophy of the superior cerebellar peduncle (SCP) has been reported in several magnetic resonance imaging (MRI) studies of FRDA, the relationship of SCP changes to genetic and clinical features of FRDA has not been investigated. We acquired T1-weighted MRI scans in 12 right-handed individuals with FRDA, homozygous for a GAA expansion in intron 1 of FXN, as well as 13 healthy age-matched controls. The corrected cross-sectional areas of the right (left) SCP in the individuals with FRDA (R, 20 ± 7.9 mm(2); L, 25 ± 5.6 mm(2)) were significantly smaller than for controls (R, 68 ± 16 mm(2); L, 78 ± 17 mm(2)) (p0.001). The SCP volumes of individuals with FRDA were negatively correlated with Friedreich's ataxia rating scale score (r = -0.553) and disease duration (r = -0.541), and positively correlated with the age of onset (r = 0.548) (p0.05). These findings suggest that structural MR imaging of the SCP can provide a surrogate marker of disease severity in FRDA and support the potential role of structural MRI as a biomarker in the evaluation of neurodegenerative diseases and therapies.
- Subjects :
- Adult
Male
Pathology
medicine.medical_specialty
Cerebellum
Ataxia
Gastroenterology
Atrophy
Internal medicine
Image Processing, Computer-Assisted
medicine
Humans
Anatomy, Cross-Sectional
medicine.diagnostic_test
Depression
Hypertrophic cardiomyopathy
Brain
Magnetic resonance imaging
medicine.disease
Magnetic Resonance Imaging
Diffusion Tensor Imaging
Dentate nucleus
Superior cerebellar peduncle
medicine.anatomical_structure
Neurology
Friedreich Ataxia
Female
Neurology (clinical)
Age of onset
medicine.symptom
Psychology
Biomarkers
Subjects
Details
- ISSN :
- 14734230 and 14734222
- Volume :
- 10
- Database :
- OpenAIRE
- Journal :
- The Cerebellum
- Accession number :
- edsair.doi.dedup.....5d718a09dad99636640559296fa23915
- Full Text :
- https://doi.org/10.1007/s12311-010-0232-3