Apport du test de mémoire sémantique 42 items (TMS-42) en consultation mémoire

International audienc

Assessment of semantic memory with the SMT-42

International audienc

Démences : où sont les corps de Lewy ?

La démence à corps de Lewy (DCL) est la deuxième cause de démence dégénérative du sujet âgé, dans les grandes séries autopsiques. Dans la réalité quotidienne des centres mémoire pourtant, la DCL représente une faible proportion des diagnostics cliniques, avec une forte disparité entre les centres. Plusieurs raisons peuvent rendre compte de la faible sensibilité du diagnostic de DCL : l’imprécision et la subjectivité des critères diagnostiques existants ; la place insuffisante donnée à certains signes non-moteurs (troubles du comportement en sommeil paradoxal, dysautonomie) ; enfin et surtout l’association quasi constante de la pathologie de Lewy à une pathologie de type Alzheimer, qui domine rapidement le phénotype clinique. À l’heure de l’essor des thérapies ciblées contre les agrégats protéiques, de nouvelles échelles cliniques permettant d’appréhender la coexistence de la pathologie de Lewy dans la maladie d’Alzheimer sont plus que jamais nécessaires

A phenotype of atypical apraxia of speech in a family carrying SQSTM1 mutation.

SQSTM1 mutations, coding for the p62 protein, were identified as a monogenic cause of Paget disease of bone and of amyotrophic lateral sclerosis. More recently, SQSTM1 mutations were identified in few families with frontotemporal dementia. We report a new family carrying SQSTM1 mutation and presenting with a clinical phenotype of speech apraxia or atypical behavioral disorders, associated with early visuo-contructional deficits. This study further supports the implication of SQSTM1 in frontotemporal dementia, and enlarges the phenotypic spectrum associated with SQSTM1 mutations

Neurobiol Aging

GRN mutations are frequent causes of familial frontotemporal degeneration. Although there is no clear consensual threshold, plasma progranulin levels represent an efficient biomarker for predicting GRN mutations when decreased. We evaluated plasma levels to determine whether it could also predict age at onset, clinical phenotype, or disease progression in 160 GRN carriers. Importantly, progranulin levels were influenced by gender, with lower levels in male than in female patients in our study. Although we found no correlation with age at onset or with clinical phenotype, we confirmed that decreased level predicts GRN mutations, even in presymptomatic carriers more than four decades before disease onset. We also provided first evidence for the stability of levels throughout longitudinal trajectory in carriers, over a 4-year time span. Finally, we confirmed that progranulin levels constitute a reliable, cost-effective marker, suitable as a screening tool in patients with familial frontotemporal degeneration, and more broadly in patients without family history or with atypical presentations who are less likely to be referred for molecular diagnosis

Dementia Caregiver Burden: A Research Update and Critical Analysis

Purpose of Review: This article provides an updated review of the determinants of caregiver burden and depression, with a focus on care demands and especially the differential effects of various neuropsychiatric symptoms or symptom clusters. Moreover, studies on caregivers for frontotemporal and Lewy body dementias were referred to in order to identify differences and similarities with the mainstream literature based largely on Alzheimer caregivers. Recent Findings: As a group, neuropsychiatric symptoms are most predictive of caregiver burden and depression regardless of dementia diagnosis, but the effects appear to be driven primarily by disruptive behaviors (e.g., agitation, aggression, disinhibition), followed by delusions and mood disturbance. Disruptive behaviors are more disturbing partly because of the adverse impact on the emotional connection between the caregiver and the care-recipient and partly because they exacerbate difficulties in other domains (e.g., caring for activities of daily living). In behavioral variant frontotemporal dementia, not only are these disruptive behaviors more prominent but they are also more disturbing due to the care-recipient’s insensitivity to others’ feelings. In Lewy body dementia, visual hallucinations also appear to be distressing. Summary: The disturbing nature of disruptive behaviors cuts across dementia conditions, but the roles played by symptoms that are unique or particularly serious in a certain condition need to be explored further

Increased Pupil Size during Future Thinking in a Subject with Retrograde Amnesia

International audienceRecent research has assessed pupil size during past thinking in patients with retrograde amnesia. Building on this research, we assessed pupil size during future thinking in a retrograde amnesia patient. To this end, we measured pupil size during past and future thinking in L, a 19-year-old, right-handed man free of neurological/psychiatric disorders except for retrograde amnesia that occurred after an episode of fugue. During a past thinking condition, we invited L to retrieve retrograde events (i.e., events that occurred before amnesia) and anterograde events (i.e., events that occurred after amnesia). During a future thinking condition, we invited him to imagine events that might occur the following week, the following month, and in the new year. Past and future thinking occurred while L’s pupil size was monitored with eye-tracking glasses. L demonstrated higher specificity during future than during past thinking. Critically, the results demonstrated a larger pupil size during future than during past thinking. The larger pupil size during future thinking observed in L can be attributed to the high cognitive load involved in future thinking. Our study not only demonstrates preserved future thinking in a patient with dissociative retrograde amnesia, but also shows that pupillometry can be used for the physiological assessment of future thinking in retrograde amnesia patients

Eye movements of recent and remote autobiographical memories: fewer and longer lasting fixations during the retrieval of childhood memories

International audienc

Loneliness of residents in retirement homes during the COVID-19 crisis

International audienc