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 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.
Department of Psychiatry and Biobehavioral Sciences, UCLA.
S E
SE
McPherson
Department of Neurology, UCLA.
J D
JD
Kuratani
Department of Psychiatry and Biobehavioral Sciences, UCLA Department of Neurology, UCLA Behavioral Neuroscience Section, Psychiatry Service, West Los Angeles Veteran Affairs Medical Center.
J L
JL
Cummings
Department of Neurology, University of New Mexico.
J
J
Shih
Department of Pathology and Laboratory Medicine, UCLA School of Medicine, Los Angeles, CA,USA.
P S
PS
Mischel
Department of Pathology and Laboratory Medicine, UCLA School of Medicine, Los Angeles, CA,USA.
H V
HV
Vinters
Creutzfeldt-Jakob disease; Echolalia; Frontal-subcortical circuit; Mixed transcortical aphasia