Simultanagnosia as the Initial Sign of Degenerative Dementia

NEILL R. GRAFF-RADFORD, JAMES P. BOLLING, FRANKLIN EARNEST, ELIZABETH A. SHUSTER, RICHARD J. CASELLI, PAUL W. BRAZIS

Research output: Contribution to journalArticle

51 Scopus citations

Abstract

In a study of 10 patients with degenerative brain disease that manifested 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 typical Alzheimer's disease and (2) to help clinicians recognize this entity. All patients were initially examined by ophthalmologists because of visual difficulties, and the simultanagnosia remained undiagnosed until nonophthalmologic complaints developed. Optic ataxia developed in six patients, and all patients had mildly impaired eye movements. All 10 patients could identify colors appropriately. Nine patients had language deficits (anomia, decreased auditory comprehension, alexia, and agraphia) but were fluent and had relative preservation of sentence repetition, and four performed in the normal range on a test of associative fluency. Two patients scored in the normal range on memory tests, all had preserved insight, and nine had no family history of degenerative dementia. The mean age at onset of the disorder was 60 years (range, 50 to 69). Neuroimaging disclosed prominent bilateral occipitoparietal atrophy in nine patients and generalized atrophy in one. With this unusual but consistent clinical, neuropsychologic, and anatomic profile, these patients are clinically distinguishable from those with typical Alzheimer's disease, but until a specific cause has been found, we cannot be certain that they constitute a specific biologic entity. Clinicians should consider this diagnosis in relatively young patients who have slowly progressive nonocular visual complaints.

Original languageEnglish (US)
Pages (from-to)955-964
Number of pages10
JournalMayo Clinic proceedings
Volume68
Issue number10
DOIs
StatePublished - Jan 1 1993

ASJC Scopus subject areas

  • Medicine(all)

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