SIMULTANAGNOSIA AS THE INITIAL SIGN OF DEGENERATIVE DEMENTIA

In a study of 10 patients with degenerative brain disease that manifes ted as simultanagnosia, our aims were (1) to elucidate their clinical, neuropsychologic, and radiologic findings to determine whether these patients might represent a group distinguishable from those with typic al Alzheimer's disease and (2) to help clinicians recognize this entit y. All patients were initially examined by ophthalmologists because of visual difficulties, and the simultanagnosia remained undiagnosed unt il nonophthalmologic complaints developed. Optic ataxia developed in s ix patients, and all patients had mildly impaired eye movements. All 1 0 patients could identify colors appropriately. Nine patients had lang uage deficits (anomia, decreased auditory comprehension, alexia, and a graphia) but were fluent and had relative preservation of sentence rep etition, and four performed in the normal range on a test of associati ve fluency. Two patients scored in the normal range on memory tests, a ll had preserved insight, and nine had no family history of degenerati ve dementia. The mean age at onset of the disorder was 60 years (range , 50 to 69). Neuroimaging disclosed prominent bilateral occipitopariet al atrophy in nine patients and generalized atrophy in one. With this unusual but consistent clinical, neuropsychologic, and anatomic profil e, these patients are clinically distinguishable from those with typic al Alzheimer's disease, but until a specific cause has been found, we cannot be certain that they constitute a specific biologic entity. Cli nicians should consider this diagnosis in relatively young patients wh o have slowly progressive nonocular visual complaints.