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Unilateral Creutzfeldt-Jakob Disease Presenting as Rapidly Progressive Aphasia

Published online by Cambridge University Press:  18 September 2015

Andrew Kirk  and
L.C. Ang
Andrew Kirk*
Affiliation:
Division of Neurology, Royal University Hospital, University of Saskatchewan, Saskatoon
L.C. Ang
Affiliation:
Department of Pathology (Neuropathology), Sunnybrook Health Science Centre, University of Toronto, Toronto
*
Division of Neurology, Royal University Hospital, 103 Hospital Drive. Saskatoon. Saskatchewan, Canada S7N 0W8
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Abstract:

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A 64-year-old man presented with a three day history of progressive Broca’s aphasia, followed within 3 weeks by exclusively right-sided myoclonus, rigidity, and dystonia. Within 4 weeks he was globally aphasie. He died within 7 weeks of onset. In the final week, rigidity and myoclonus became bilateral. CT and MRI were normal. SPECT showed diminished perfusion of the left hemisphere. EEG showed periodic discharges on the left. At autopsy, there were marked cortical spongiform change, neuronal loss, and gliosis throughout the left hemisphere and in the right occipital cortex. Elsewhere in the right hemisphere, spongiform change was non-existent to minimal. There was moderate spongiform change in the molecular layer of the cerebellar cortex, much more marked on the left. Clinical and pathological unilateral cerebral predominance extended to the ipsilateral cerebellum. Creutzfeldt-Jakob disease is an important consideration in patients with rapidly progressive unilateral cerebral signs associated with a movement disorder.

Résumé:

Résumé:

Aphasie progressant rapidement comme tableau initial chez un patient atteint de Creutzfeldt-Jakob unilatéral. Un homme âgé de 64 ans a consulté pour une aphasie de Broca ayant progressé sur une période de trois jours, suivie trois semaines plus tard par des myoclonies, de la rigidité et de la dystonie localisées exclusivement à l’hémicorps droit. Quatre semaines après le début de la maladie, il présentait une aphasie globale et il est décédé trois semaines plus tard, soit sept semaines après le debut de la maladie. Pendant la dernière semaine de sa maladie, la rigidité et les myoclonies sont devenues bilatérales. La tomodensitométrie et la résonance magnétique nucléaire étaient normales alors que le SPECT a montré une diminution de la perfusion à l’hémisphère gauche. L’EEG a montré des décharges périodiques à gauche. A l’autopsie, it existait des modifications spongifonnes corticales importantes, une perte neuronale et de la gliose dans tout l’hémisphère gauche et dans le cortex occipital droit. Ailleurs dans l’hémisphère droit, les modifications spongiformes étaient absentes ou minimes, alors qu’elles étaient modérées dans la couche moléculaire du cortex cérébelleux, surtout à gauche. La prédominance unilatérale clinique et anatomopathologique s’étendait au cervelet ipsilatéral. La maladie de Creutzfeldt-Jakob est un diagnostic qu’il est important de considérer chez les patients qui présentent des signes d’atteinte cérébrale unilatérale qui progressent rapidement et qui sont accompagnés d’un désordre du mouvement.

Type
Original Articles
Information
Canadian Journal of Neurological Sciences , Volume 21 , Issue 4 , November 1994 , pp. 350 - 352
Copyright
Copyright © Canadian Neurological Sciences Federation 1994

References

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