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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| Format: | Article |
| Language: | English |
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Wiley
1994-01-01
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| Series: | Behavioural Neurology |
| Online Access: | http://dx.doi.org/10.3233/BEN-1994-73-415 |
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| author | S. E. McPherson J. D. Kuratani J. L. Cummings J. Shih P. S. Mischel H. V. Vinters |
| author_facet | S. E. McPherson J. D. Kuratani J. L. Cummings J. Shih P. S. Mischel H. V. Vinters |
| author_sort | S. E. McPherson |
| collection | DOAJ |
| description | 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. |
| format | Article |
| id | doaj-art-10cfaf81c7914c91ad2c8ce504fe4fb8 |
| institution | Kabale University |
| issn | 0953-4180 1875-8584 |
| language | English |
| publishDate | 1994-01-01 |
| publisher | Wiley |
| record_format | Article |
| series | Behavioural Neurology |
| spelling | doaj-art-10cfaf81c7914c91ad2c8ce504fe4fb82025-02-03T01:00:30ZengWileyBehavioural Neurology0953-41801875-85841994-01-0173-419720310.3233/BEN-1994-73-415Creutzfeldt-Jakob Disease with Mixed Transcortical Aphasia: Insights into EcholaliaS. E. McPherson0J. D. Kuratani1J. L. Cummings2J. Shih3P. S. Mischel4H. V. Vinters5Department of Psychiatry and Biobehavioral Science, UCLA School of Medicine, Los Angeles, CA, USADepartment of Neurology, UCLA School of Medicine, Los Angeles, CA, USADepartment of Psychiatry and Biobehavioral Science, UCLA School of Medicine, Los Angeles, CA, USADepartment of Neurology, University of New Mexico, USADepartment of Pathology and Laboratory Medicine, UCLA School of Medicine, Los Angeles, CA, USADepartment of Pathology and Laboratory Medicine, UCLA School of Medicine, Los Angeles, CA, USAAphasia 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.http://dx.doi.org/10.3233/BEN-1994-73-415 |
| spellingShingle | S. E. McPherson J. D. Kuratani J. L. Cummings J. Shih P. S. Mischel H. V. Vinters Creutzfeldt-Jakob Disease with Mixed Transcortical Aphasia: Insights into Echolalia Behavioural Neurology |
| title | Creutzfeldt-Jakob Disease with Mixed Transcortical Aphasia: Insights into Echolalia |
| title_full | Creutzfeldt-Jakob Disease with Mixed Transcortical Aphasia: Insights into Echolalia |
| title_fullStr | Creutzfeldt-Jakob Disease with Mixed Transcortical Aphasia: Insights into Echolalia |
| title_full_unstemmed | Creutzfeldt-Jakob Disease with Mixed Transcortical Aphasia: Insights into Echolalia |
| title_short | Creutzfeldt-Jakob Disease with Mixed Transcortical Aphasia: Insights into Echolalia |
| title_sort | creutzfeldt jakob disease with mixed transcortical aphasia insights into echolalia |
| url | http://dx.doi.org/10.3233/BEN-1994-73-415 |
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