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Clinical Variations in Assessment of Bull's-eye Maculopathy
Malaika M. Kurz-Levin, MD;
Anthony S. Halfyard, PhD;
Catey Bunce, MSc;
Alan C. Bird, MD;
Graham E. Holder, PhD
Arch Ophthalmol. 2002;120:567-575.
Objectives To evaluate the phenotypic variation in bull's-eye maculopathy and seek
possible correlations between functional loss and clinical appearance.
Methods From January 1, 1999, to September 30, 2000, we prospectively examined
patients with bull's-eye lesions. Age of onset, duration of symptoms, visual
acuity, clinical appearance, and autofluorescence images were recorded, the
area of atrophy measured, and electrophysiologic investigations performed.
Results Forty-seven patients, including 6 sibling pairs, met the study entry
criteria. On the basis of autofluorescence imaging, 3 distinct groups were
identified. Group 1 showed a distinct ring of increased autofluorescence surrounding
an area of decreased autofluorescence. In group 2, the ring of increased autofluorescence
was not present. Group 3 displayed a speckled appearance within the affected
area. All patients had evidence of central sparing in an area of centrally
increased autofluorescence. There was significant correlation with the age
of onset, visual acuity, and duration of disease. Electrophysiologic tests
revealed that 28 patients had macular dysfunction only, 14 had cone-rod dystrophy,
3 had rod-cone dystrophy, and only 2 (monozygotic twins) had cone dystrophy.
The correlation between electrophysiologic and autofluorescence data was poor.
The sibling pairs had concordant autofluorescence appearance, but electrophysiologic
grouping differed in 2 pairs.
Conclusions Bull's-eye maculopathy represents a heterogeneous group of disorders.
The clinical appearance was not helpful in assessing the degree of retinal
dysfunction. The difference in qualitative characteristics of functional loss
between siblings implies that these attributes do not necessarily reflect
the influence of the primary mutation.
From the Medical Retina Service (Drs Kurz-Levin and Bird) and Departments
of Epidemiology (Dr Bunce) and Electrophysiology (Dr Holder), Moorfields Eye
Hospital, and Department of Visual Science, Institute of Ophthalmology (Dr
Halfyard), London, England.
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