Homonymous field defect as the first manifestation of Creutzfeldt-Jakob disease.
OBJECTIVE
Although in the early stage of Creutzfeldt-Jakob disease most patients have obvious dementia, we found that the disease can be diagnosed in patients solely on the basis of a visual system disorder.


METHODS
We examined three patients who initially complained of a nonspecific, insidious visual disturbance.


RESULTS
The three patients were found to have Creutzfeldt-Jakob disease, confirmed by histopathologic analysis. Each patient had a homonymous hemianopsia on the first neuro-ophthalmologic examination. The initial neurologic, neuroimaging, and electrophysiologic examinations were not conclusive. The subsequent rapid deterioration in the neurologic status, including dementia and typical electroencephalographic changes, was suggestive of Creutzfeldt-Jakob disease.


CONCLUSIONS
At onset of Creutzfeldt-Jakob disease, patients may have a homonymous hemianopsia despite normal results of magnetic resonance imaging of the brain and neurologic examination.
Vargas
M E
ME
Department of Ophthalmology, New York University Medical Center, New York, USA.
Kupersmith
M J
MJ
Savino
P J
PJ
Petito
F
F
Frohman
L P
LP
Warren
F A
FA
D000368:Aged; D001706:Biopsy; D001921:Brain / Q000473:pathology; D007562:Creutzfeldt-Jakob Syndrome / Q000150:complications / Q000175:diagnosis*; D004569:Electroencephalography; D006423:Hemianopsia / Q000175:diagnosis* / Q000209:etiology; D006801:Humans; D008297:Male; D008875:Middle Aged; D058609:Visual Field Tests; D014794:Visual Fields