Affiliations: Department of Psychiatry and Biobehavioral Sciences, UCLA | Department of Neurology, UCLA | Behavioral Neuroscience Section, Psychiatry Service, West Los Angeles Veteran Affairs Medical Center | Department of Neurology, University of New Mexico | Department of Pathology and Laboratory Medicine, UCLA School of Medicine, Los Angeles, CA,USA
Note: [] Correspondence to: S.E. McPherson, UCLA NPI, 760 Westwood Plaza, Los Angeles, CA 90024-1759, USA
Abstract: 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.
