Ophthalmic features of Friedreich ataxia.
Eye (Lond)
; 26(2): 315-20, 2012 Feb.
Article
em En
| MEDLINE
| ID: mdl-22094302
ABSTRACT
PURPOSE:
To describe ocular abnormalities in patients with Friedreich ataxia (FRDA).METHODS:
Patients diagnosed with FRDA by genetic analysis were invited to participate in a prospective cohort. The patients included underwent an extensive ophthalmologic examination, including low-contrast Sloan letter charts test and retinal nerve fiber layer (RNFL) thickness analysis by optical coherence tomography (OCT).RESULTS:
Twenty-three patients agreed to participate. In all, 19 patients (83%) had a visual acuity of at least 0.8 in both eyes. Fundus examination showed diffuse optic nerve pallor in four patients. However, OCT showed a decreased mean peripapillary RNFL thickness in all but three adult cases and one teenager. The RNFL thickness was found to have a positive correlation with visual acuity (P=0.001) and contrast sensitivity (P=0.001) and a negative correlation with time elapsed from diagnosis (P=0.001).CONCLUSIONS:
OCT and low contrast test sensitivity show that the visual pathway is affected in FRDA. However, in most patients there is no significant visual impairment. In a small proportion of patients visual acuity declines with disease progression. This study provides a better understanding of the ophthalmic features of FRDA.
Texto completo:
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Base de dados:
MEDLINE
Assunto principal:
Células Ganglionares da Retina
/
Ataxia de Friedreich
/
Sensibilidades de Contraste
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Acuidade Visual
/
Fibras Nervosas
Idioma:
En
Ano de publicação:
2012
Tipo de documento:
Article