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Progressive Focal Degenerative Disease of the Posterior Associative Cortex

Published online by Cambridge University Press:  18 September 2015

Ezzedine Attig ,
Jean Jacquy ,
Patrick Uytdenhoef  and
Hervé Roland
Ezzedine Attig*
Affiliation:
Department of Neurology (E.A., J.J., P.U., H.R.), Hopital Civil de Charleroi, Charleroi, Belgium, affiliated with the Free University of Brussels; and the Department of Neurology (E.A.), Hotel-Dieu Hospital, Montreal
Jean Jacquy
Affiliation:
Department of Neurology (E.A., J.J., P.U., H.R.), Hopital Civil de Charleroi, Charleroi, Belgium, affiliated with the Free University of Brussels; and the Department of Neurology (E.A.), Hotel-Dieu Hospital, Montreal
Patrick Uytdenhoef
Affiliation:
Department of Neurology (E.A., J.J., P.U., H.R.), Hopital Civil de Charleroi, Charleroi, Belgium, affiliated with the Free University of Brussels; and the Department of Neurology (E.A.), Hotel-Dieu Hospital, Montreal
Hervé Roland
Affiliation:
Department of Neurology (E.A., J.J., P.U., H.R.), Hopital Civil de Charleroi, Charleroi, Belgium, affiliated with the Free University of Brussels; and the Department of Neurology (E.A.), Hotel-Dieu Hospital, Montreal
*
Dept. of Neurology, Hotel-Dieu Hospital, 3840 Saint-Urbain, Montreal, PQ, Canada H2W 1T8
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Abstract:

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A 72-year-old man developed a very progressive neuropsychologic deficit 6 years ago, beginning with isolated visual topographic memory disturbances and visuo-constructive apraxia without additional manifestations of dementia. The syndrome worsened thereafter with the emergence of visual agnosia, simultagnosia, psychic paralysis of gaze, auditivo-verbal agnosia, and recently an amnestic syndrome with confabulation and confusion (at the end of 1989). CT scans, which remained unchanged over the years, showed mild, focal atrophic changes revealed by dilatation of the right occipital horn. His angiograms were normal. Two SPECT studies (with HMPAO measurements), performed 6 years from the onset, detected marked hypoperfusion in both parieto-occipital regions, mainly on the right side. Progressive focal degenerative disease without dementia is a relatively new syndrome, especially in cases with progressive aphasia.1 As noted in our patient, progressive disturbances initially localized in the right parieto-occipital region followed by posterior bilobar degeneration (pronounced on the right side) without dementia until late in the course may represent another exceptionally reported characteristic of this new syndrome. It is suggested that this variant of the Mesulam syndrome is more likely explained by progressive atrophy of the Alzheimer type.

Type
Research Article
Information
Canadian Journal of Neurological Sciences , Volume 20 , Issue 2 , May 1993 , pp. 154 - 157
Copyright
Copyright © Canadian Neurological Sciences Federation 1993

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