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The British Journal of Ophthalmology logoLink to The British Journal of Ophthalmology
letter
. 1999 Feb;83(2):250–251. doi: 10.1136/bjo.83.2.250

A case of reversible blindness in maple syrup urine disease

O BACKHOUSE, R LEITCH, D THOMPSON, A KRISS, D CHARRIS, P CLAYTON, I RUSSELL-EGGITT
PMCID: PMC1722938  PMID: 10396207

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Figure 1  .

Figure 1  

Computed tomogram showing swelling of white matter.

Figure 2  .

Figure 2  

Computed tomogram showing changes in white matter appearances after acute presentation.    

Figure 3  .

Figure 3  

The right eye flash ERG and binocular flash VEP recorded at presentation. (Note, positivity is downwards). Averaged lower lid skin ERGs from the right and left eyes were of normal size and similar for each eye. No consistent flash VEP was discernible at the occipital midline.

Figure 4  .

Figure 4  

ERG and VEPs following treatment by dietary restriction. The top two traces show the flash ERG and flash VEP. A normal well defined retinal response was again recorded from both eyes (the right ERG is illustrated). On this occasion a broad positive flash VEP was consistently detected. Binocular pattern reversal stimulation with a range of check sizes also elicited consistent responses (P100 to 100 minute checks arrowed, note the higher display gain for the PVEPs compared with flash VEP and ERG). PVEPs were larger and better defined for the large and moderate check sizes, indicating moderate acuity levels.


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