Right ventral frontal hypometabolism and abnormal sense of self in a case of disproportionate retrograde amnesia

Résumé En

We report the case of a 42-year-old man (patient CL) who developed a particular profile of amnesia with two dates of onset. At the first onset, the patient suffered a mild/lmoderate injury that accounts for an initial anterograde and mild retrograde memory impairment. At the second onset, 8 months later, he suffered a sudden and persistent loss of personal identity and severe retrograde amnesia. We report an extensive neuropsychological investigation of his memory systems carried out 18 months after the second onset. Results indicated mild executive dysfunction (primary memory), intact procedural skills and perceptual representational system. In accordance with Kopelman's methodological recommendations, we have reliably compared post- and pre-onset semantic and episodic memory using strict matched procedures. We found that post-onset, though not pre-onset semantic (autobiographical and nonautobiographical) memory was entirely preserved. Post-onset episodic autobiographical memory was not intact, however, although it was clearly less affected compared with the total absence of the pre-onset memory. Moreover, a novel and high standard investigation of the subjective states of consciousness, which accompanied retrieval of autobiographical memories via the Remember/lKnow (R/lK) paradigm with a long time interval from the present, demonstrated a deterioration of R responses compared to matched controls. Interestingly, this result showed deficient autonoetic consciousness and suggested an underlying accelerated forgetting rate for post-onset autobiographical episodic memories. Last, a [18F] fluorodeoxyglucose resting PET study revealed a significant right-sided ventral frontal lobe hypometabolism in the absence of overt structural lesions. The involvement of this region is consistent with CL's autobiographical retrograde amnesia and his inability to re-experience information concerning the self across time. In our particular case, characterised by two dates of onset, the attribution of causality is thoroughly examined in terms of CL's organic and psychogenic aspects.