Mixed Transcortical Aphasia in Association with Neurodegenerative Disease
Congratulations Drs. Doty and Heilman on the publication of “Mixed Transcortical Aphasia in Association with Neurodegenerative Disease,” which was published in the April 28th edition of Neurology.
Objective: To describe a novel case of mixed transcortical aphasia in association with neurodegenerative disease.
Background: Mixed transcortical aphasia is a rare speech disorder characterized by reduced spontaneous speech and decreased comprehension with intact repetition. This type of aphasia has been seen previously in patients with brain infarction, particularly with ischemia of watershed areas which isolate Broca’s area, Wernicke’s areas, and the arcuate fasciculus from rest of the brain.
Design/Methods: A case report.
Results: we report a 55 year old right-handed female who gradually developed language difficulties over two years. Symptoms began with difficulty finding words and word production. On neuropsychologic testing, she was found to have impaired spontaneous speech, impaired comprehension and naming, with intact repetition. Her MRI showed atrophy in the left frontal, parietal and anterior temporal lobe, without evidence of ischemia.
Conclusions: Primary progressive aphasia (PPA) is a neurodegenerative disease manifested by a progressive decline in language capabilities. To date, three types of PPA have been described: a semantic variant and two non-semantic variants called nonfluent/agrammatic and logopenic variants. Semantic PPA patients have difficulty comprehending word meaning and anomia but are fluent with grammatically correct speech. Nonfluent variants present with speech apraxia, grammatical errors with preserved word comprehension (Gorno Tempini 2011). To our knowledge this is the first case associated with neurodegenerative disease. Hence, this case represents a new variant of primary progressive aphasia.