a.t . , a 43-year - old nigerian lady , presented to the eye clinic of the university college hospital , ibadan with gradual blurring of vision in both eyes for 4 years .
ocular examination showed unaided visual acuity of 6/12 ou corrected to 6/9 in both eyes .
dilated fundoscopy showed a well circumscribed cystic fovea lesion with a fluid level and an atrophic center in the right eye , while the left eye showed a scar in the macula ( fig . 1 and fig .
the patient was prescribed with spectacles , counseled and placed on close observation with amslers grid for the development of choroidal neovascular membrane that will require urgent treatment .
this is probably the first case of best macular dystrophy to be reported in ibadan , nigeria and possibly sub - saharan africa to the best of the author 's knowledge .
it has also been reported in caucasians and asians , the other cases seen in african americans were associated with sickle cell trait .
the pathophysiology of best 's disease is explained by abnormality in the retinal pigment epithelium ( rpe ) with resultant abnormal ionic transport leading to the accumulation of lipofuscin in the rpe cells and sub - rpe space in the macular area .
the vitelliruptive stage may herald visual deterioration which becomes worse in the atrophic stage due to the presence of choroidal neovascular membrane .
our patient presented with the early stages of the disease , hence the good visual acuity .
the patient will be under observation and follow - up so as to detect changes amenable to treatment .
choroidal neovascular membrane ( cnvm ) from best 's disease has been reported to respond well to intravitreal antivegf [ 7 , 8 ] .
fundus flourescein angiography is essential in confirming the presence of cnvm and should be done when patient presents with sudden deterioration in vision . if a cnvm develops , then a corresponding area of hyperfluorescence with leakage will be found .
since the disease is an autosomal dominant disorder , other family members will benefit from regular fundus examinations , and amslers grid is a valuable tool for monitoring central vision .
an electrophysiologic test such as electrooculogram ( eog ) is specific for confirming the presence of the disease in relatives even in the absence of clinical signs and symptoms .
a severe decrease occurs in light response , reflected by an arden ( light - peak / dark - trough ) ratio of 1.11.5 ( the normal arden ratio is 1.8 ) .
the full - field electroretinogram ( erg ) result is normal in best 's disease .
a focal erg or multifocal erg , concentrating on macular function , reveals abnormal function corresponding to the area of anatomical disruption .
the pathophysiology of best 's disease is explained by abnormality in the retinal pigment epithelium ( rpe ) with resultant abnormal ionic transport leading to the accumulation of lipofuscin in the rpe cells and sub - rpe space in the macular area .
the vitelliruptive stage may herald visual deterioration which becomes worse in the atrophic stage due to the presence of choroidal neovascular membrane .
our patient presented with the early stages of the disease , hence the good visual acuity .
the patient will be under observation and follow - up so as to detect changes amenable to treatment .
choroidal neovascular membrane ( cnvm ) from best 's disease has been reported to respond well to intravitreal antivegf [ 7 , 8 ] .
fundus flourescein angiography is essential in confirming the presence of cnvm and should be done when patient presents with sudden deterioration in vision .
if a cnvm develops , then a corresponding area of hyperfluorescence with leakage will be found .
since the disease is an autosomal dominant disorder , other family members will benefit from regular fundus examinations , and amslers grid is a valuable tool for monitoring central vision .
an electrophysiologic test such as electrooculogram ( eog ) is specific for confirming the presence of the disease in relatives even in the absence of clinical signs and symptoms .
a severe decrease occurs in light response , reflected by an arden ( light - peak / dark - trough ) ratio of 1.11.5 ( the normal arden ratio is 1.8 ) .
the full - field electroretinogram ( erg ) result is normal in best 's disease .
a focal erg or multifocal erg , concentrating on macular function , reveals abnormal function corresponding to the area of anatomical disruption .
this is a report of best macular dystrophy in ibadan , sub - saharan africa .
 best macular dystrophy is reported to be rare in africans . 
 it is a hereditary disease that starts in childhood and progresses through some stages before visual symptoms occur . 
 this case report presents a 43-year - old nigerian with the disease and stresses the importance of regular eye exams of patients and relatives to detect changes such as choroidal neovascular membrane amenable to treatment . 
Case Presentation
Discussion
Pathophysiology
Conclusion