Creutzfeldt-Jakob Disease with Mixed Transcortical Aphasia: Insights into Echolalia

Aphasia is a common manifestation of Creutzfeldt-Jakob disease (CJD), and investigation of the linguistic disorders of CJD patients may provide insights into the neurobiological mechanisms of language and aphasia. We report an autopsy-confirmed case of CJD in which the presenting symptom was change...

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Bibliographic Details
Main Authors: S. E. McPherson, J. D. Kuratani, J. L. Cummings, J. Shih, P. S. Mischel, H. V. Vinters
Format: Article
Language:English
Published: Wiley 1994-01-01
Series:Behavioural Neurology
Online Access:http://dx.doi.org/10.3233/BEN-1994-73-415
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Summary:Aphasia is a common manifestation of Creutzfeldt-Jakob disease (CJD), and investigation of the linguistic disorders of CJD patients may provide insights into the neurobiological mechanisms of language and aphasia. We report an autopsy-confirmed case of CJD in which the presenting symptom was change in language abilities. The patient ultimately evidenced mixed transcortical aphasia (MTA) with echolalia. Disruption of frontal-subcortical circuits with environmental dependency accounts for the symptoms in MTA, including intact repetition and echolalia. Observation in this patient and a review of the literature suggest that frontal-subcortical circuit dysfunction may contribute to the syndrome of echolalia. This hypothesis offers an alternative explanation to “isolation” of the speech area as the cause of MTA.
ISSN:0953-4180
1875-8584