Pervasive autobiographical memory impairments in Huntington's disease

Autobiographical memory dysfunction is a pervasive feature of neurodegenerative disorders, but less is known about the integrity of autobiographical memory in Huntington's disease (HD). Deficits in anterograde verbal episodic memory on traditional neuropsychological tests have been detected in HD, however, whether personally-relevant autobiographical retrieval is also affected is unknown. We examined autobiographical memory performance in 26 participants genetically confirmed to have HD who were in the peri-manifest stage of the disease (including 12 in the late premanifest stage and 14 who were early diagnosed), and 24 matched controls using the Autobiographical Interview (AI), a semi-structured interview assessing retrieval of autobiographical details from discrete epochs across the lifetime. Relative to controls, people with HD exhibited global episodic autobiographical memory impairments, regardless of recency or remoteness of the memory being retrieved. While specific cues bolstered the retrieval of episodic (internal) details in HD participants, their performance remained significantly below that of controls. Moreover, following probing, people with HD retrieved more extraneous (external) details not directly related to the autobiographical event they originally retrieved, including semantic details, repetitions, and metacognitive statements. Our results reveal marked autobiographical memory dysfunction in HD, not directly attributable to strategic retrieval deficits, and suggest that autobiographical memory impairment may represent an overlooked feature of the cognitive phenotype of HD.