Marion Houot

Hôpital La Pitié Salpêtrière (Groupe Hospitalier "La Pitié Salpêtrière - Charles Foix") · Département des Maladies du Système Nerveux

Neurodegenerative dementias have a profound impact on higher-order cognitive and behavioural functions. Investigating macroscale functional networks through cortical gradients provides valuable insights into the neurodegenerative dementia process and overall brain function. This approach allows for the exploration of unimodal-multimodal differentia...

Background and Purpose Frontotemporal dementia (FTD) is a neurodegenerative disorder characterized by pervasive personality and behavioural disturbances with severe impact on patients and caregivers. In current clinical practice, treatment is based on nonpharmacological and pharmacological approaches. Unfortunately, trial‐based evidence supporting...

I-FP-CIT SPECT enables the detection of presynaptic dopaminergic denervation. It allows to differentiate degenerative parkinsonian syndromes from secondary parkinsonian syndromes or essential tremor, and patients with suspected dementia with Lewy bodies from those with other dementia subtypes. The aim of our study was to evaluate the appropriatenes...

Background Sleep‐disordered breathing (SDB; including stridor and sleep apnea syndromes) is frequent in multiple system atrophy (MSA), but the immediate effect of continuous positive airway pressure (CPAP) therapy is incompletely determined. Objective We sought to evaluate the acute effect and safety of CPAP therapy on SDB and sleep architecture,...

Background: Understanding the nature and extent of sensorimotor decline in aging individuals and those with neurocognitive disorders (NCD), such as Alzheimer's disease, is essential for designing effective music-based interventions. Our understanding of rhythmic functions remains incomplete, particularly in how aging and NCD affect sensorimotor sy...

Background In early‐stage Parkinson's disease (PD), rapid eye movement (REM) sleep behavior disorder (RBD) predicts poor cognitive and motor outcome. However, the baseline significance and disease evolution associated with isolated REM sleep without atonia (iRWA, ie, enhanced muscle tone during 8.7% of REM sleep, but no violent behavior) are not we...

Bi-allelic pathogenic variants in PRKN are the most common cause of autosomal recessive Parkinson’s disease (PD). 647 patients with PRKN -PD were included in this international study. The pathogenic variants present were characterised and investigated for their effect on phenotype. Clinical features and progression of PRKN -PD was also assessed. Am...

Background Respiratory disorders remain incompletely described in multiple sclerosis (MS), even though they are a frequent cause of death. Methods The objective was to describe respiratory disorders in MS patients with Expanded Disability Status Score (EDSS) ⩾ 6.5. Diaphragm dysfunction was defined by at least two of the seven criteria: clinical s...

In Parkinson’s disease (PD), it remains unclear whether sleep disorders including insomnia, REM sleep behavior disorder (RBD), excessive daytime sleepiness (EDS), restless legs syndrome (RLS) and sleep-disordered breathing (SDB), are isolated or combined, interact with each other and are associated with clinical factors. We sought to determine the...

Neurodegenerative dementias have a profound impact on higher-order cognitive and behavioural functions. Investigating macroscale functional networks through cortical gradients provides valuable insights into the neurodegenerative dementia process and overall brain function. This approach allows for the exploration of unimodal-multimodal differentia...

Background Understanding the nature and extent of sensorimotor decline in ageing individuals and those with neurocognitive disorders NCD, such as Alzheimer’s disease, is essential for designing effective music-based interventions. Objective Our understanding of rhythmic functions remains incomplete, particularly in how ageing and NCD affect sensor...

Background While not originally developed for this purpose, evidence suggests that the Healthy Aging Brain Care Monitor questionnaire (HABC‐M; Monahan et al, 2012, 2014) may be a valuable instrument for assessing unawareness of cognitive decline (i.e., anosognosia) from preclinical stages of Alzheimer’s disease (AD) (Cacciamani et al, 2017). This i...

Background Cerebral amyloid angiopathy (CAA) diagnostic is of special interest due to its association with cognitive impairment, Alzheimer disease (AD) and Amyloid‐Related Imaging Abnormalities (ARIA) in trials with anti‐amyloid immunotherapies. We aimed to assess the frequency of CAA diagnosis and associated lesions in subjects with cognitive comp...

Background: Though not originally developed for this purpose, the Healthy Aging Brain Care Monitor (HABC-M) seems a valuable instrument for assessing anosognosia in Alzheimer's disease (AD). Objectives: Our study aimed at 1) investigating the validity of the HABC-M (31 items), and its cognitive, psychological, and functional subscales, in discri...

Background and purpose: The specific effects of antiseizure medications (ASMs) on cognition are a rich field of study, with many ongoing questions. The aim of this study was to evaluate these effects in a homogeneous group of patients with epilepsy to guide clinicians to choose the most appropriate medications. Methods: We retrospectively identi...

Background: Seizures are a frequent complication of strokes. The initial severity of the stroke is a risk factor for both seizure occurrence and poor functional recovery. Aim: To determine whether epilepsy has a negative impact on functional recovery or is just a proxy for the initial severity of the stroke. Patients and methods: We conducted...

Unawareness of memory deficits is an early manifestation in patients with Alzheimer's disease (AD), which often delays diagnosis. This intriguing behavior constitutes a form of anosognosia, whose neural mechanisms remain largely unknown. We hypothesized that anosognosia may depend on a critical synaptic failure in the error-monitoring system, which...

Temporal lobe epilepsy (TLE) can induce various difficulties in recognizing emotional facial expressions (EFE), particularly for negative valence emotions. However, these difficulties have not been systematically examined according to the localization of the epileptic focus. For this purpose, we used a forced-choice recognition task in which faces...

Abstract Background There is a need for a reliable, easy-to-use, widely available, and validated tool for timely cognitive impairment identification. We created a computerized cognitive screening tool (Santé-Cerveau digital tool (SCD-T)) including validated questionnaires and the following neuropsychological tests: 5 Word Test (5-WT) for episodic m...

GRN mutations are among the main genetic causes of frontotemporal dementia (FTD). Considering the progranulin involvement in lysosomal homeostasis, we aimed to evaluate if plasma lysosphingolipids (lysoSPL) are increased in GRN mutation carriers, and whether they might represent relevant fluid-based biomarkers in GRN-related diseases.We analyzed fo...

Background Mesial temporal lobe epilepsy with hippocampal sclerosis (MTLE-HS) is usually associated with a poor response to antiseizure medications. We focused on MTLE-HS patients who were seizure free on medication to: (1) determine the clinical factors associated with seizure freedom and (2) develop a machine-learning classifier to better earlier...

Background Little is known about risk factors for mortality in older patients with COVID‐19 and neuropsychiatric conditions. Methods We conducted a multicentric retrospective observational study at Assistance Publique‐Hôpitaux de Paris. We selected inpatients aged 70 years or older, with COVID‐19 and preexisting neuropsychiatric comorbidities and/...

Background and objectives: GRN variants are a frequent cause of familial frontotemporal dementia (FTD). Monitoring disease progression in asymptomatic carriers of genetic variants is a major challenge in delivering preventative therapies before clinical onset. This study aimed to assess the usefulness of fluorodeoxyglucose (FDG)-PET in identifying...

We investigated the presence of misfolded alpha-Synuclein (α-Syn) in minor salivary gland biopsies in relation to substantia nigra pars compacta (SNc) damage measured using magnetic resonance imaging in patients with isolated rapid eye movement sleep behavior disorder (iRBD) and Parkinson’s disease (PD) as compared to healthy controls. Sixty-one pa...

Aim: To validate a French version of the Multiple Sclerosis Intimacy and Sexuality Questionnaire 15 which examines patients' perception of the effect of multiple sclerosis symptoms on their sexual activity. Methods: After completing a translation/re-translation process to ensure linguistic and content validity, the Multiple Sclerosis Intimacy an...

Botulinum neurotoxin (BoNT) is a useful therapeutic option to treat dystonic manifestations. Data on its efficiency on dystonia associated with Parkinson’s disease (PD) or atypical parkinsonism (AP) are scarce and no comparison of the efficiency of BoNT has been performed between these diseases and between the different localizations of dystonia in...

Background and purposeSeizures represent a core symptom of autoimmune encephalitides with specific therapeutic issues. To date, patients with new-onset seizures or established epilepsy are not systematically tested for autoimmune antibodies. We aimed to identify clinical and paraclinical criterion that could help to select patients requiring additi...

Unawareness of memory deficits is an early manifestation in patients with Alzheimer’s disease (AD). This intriguing behavior constitutes a form of anosognosia and has neural mechanisms that remain largely unknown. Here, we hypothesized that it may result from a failure in the error-monitoring system, which would prevent AD patients from being aware...

Introduction Dans l’épilepsie temporale mésiale, la présence d’une sclérose hippocampique est fortement associée avec une pharmacorésistance. Néanmoins, des travaux ont montré que 25 % des patients peuvent être libres de crises sous traitement antiépileptique. Objectifs L’objectif de notre étude est de définir les caractéristiques cliniques et par...

Introduction La dystonie est un symptôme fréquent dans les syndromes parkinsoniens dégénératifs, à l’origine d’un handicap notable. Peu de données sont disponibles sur l’efficacité de la toxine botulique dans cette indication. Objectifs Ce travail visait à modéliser l’efficacité de la toxine botulique sur les dystonies associées aux syndromes park...

Introduction Les expansions de C9orf72 sont la cause génétique principale de démence fronto-temporale et sclérose latérale amyotrophique. Les essais thérapeutiques à venir nécessitent une connaissance approfondie des trajectoires des biomarqueurs structuraux. Objectifs Nous avons cherché à définir le profil d’atrophie de la substance grise et sa p...

Background Temporary disruption of the blood-brain barrier (BBB) using pulsed ultrasound leads to the clearance of both amyloid and tau from the brain, increased neurogenesis, and mitigation of cognitive decline in pre-clinical models of Alzheimer’s disease (AD) while also increasing BBB penetration of therapeutic antibodies. The goal of this pilot...

Purpose Human neuronal activity, recorded in vivo from microelectrodes, may offer valuable insights into physiological mechanisms underlying human cognition and pathophysiological mechanisms of brain diseases, in particular epilepsy. Continuous and long-term recordings are necessary to monitor non predictable pathological and physiological activiti...

About two-thirds of patients with minor strokes are discharged home. However, these patients may have difficulties returning to their usual living activities. To investigate the factors associated with successful home discharge, our aim was to provide a decision tree (based on clinical data) that could identify if a patient discharged home could re...

Anosognosia, or the lack of awareness of one's own impairment, is frequent for memory deficits in patients with Alzheimer's disease (AD). Although often related to frontal dysfunctions, the neural mechanisms of anosognosia remain largely unknown. We hypothesized that anosognosia in AD may result from a failure in the error-monitoring system, thus p...

Background We examined the association between preclinical Alzheimer's disease (AD) and undergoing anesthesia and surgery (“surgery” henceforth) in a cohort of elderly individuals with a subjective cognitive decline (SCD). Methods Individuals with SCD (N = 268) were enrolled in a longitudinal follow-up study. Participants underwent comprehensive y...

Objective. To highlight specific characteristics of seizure semiology and EEG features associated with different subtypes of autoimmune encephalitis (AE). Methods. We systematically reviewed the seizure semiology and all the EEG recordings from patients with AE managed in a tertiary referral centre for epilepsy and a neuro‐intensive care unit. Each...

COVID-19, caused by the SARS-CoV-2 virus, has spread since December 2019 and was declared a pandemic by the World Health Organization. An initial Chinese cohort of 214 COVID-19 patients reported a high frequency of neurological symptoms (36%), including non-specific manifestations such as headache, confusion and myalgia, but also strokes and seizur...

Objective Lateral temporal lobe epilepsies (LTLE) are poorly characterized heterogeneous epilepsies. As the lateral temporal lobe supports distinct functions, we hypothesized that neuropsychological profiles could differ according to the localization of the seizure focus within the lateral temporal lobe. Methods We retrospectively examined the neu...

Background Alzheimer’s disease (AD) patients are often unaware of their memory deficits even at early stage of disease. Anosognosia has been related to frontal dysfunction although its underlying neural mechanism is still unknown. We hypothesized that it may result from an inability to monitor ongoing behavior due to a failure in the error‐monitori...

Patients with Alzheimer’s disease (AD) suffer from various types of memory distortions. We showed that confabulations are plausible memories, mainly reflecting the recall of repeated personal events mistakenly considered by confabulating patients as specific and unique events. The aim of this study is to see whether the notion that over-learned inf...

The G4C2-repeat expansion in C9orf72 is the most common cause of frontotemporal dementia and of amyotrophic lateral sclerosis. The variability of age at onset and phenotypic presentations is a hallmark of C9orf72 disease. In this study, we aimed to identify modifying factors of disease onset in C9orf72 carriers using a family-based approach, in pai...

Background: The TNI-93 is a quick memory test designed for all patients regardless of their education level. A significant proportion of patients with Alzheimer's disease (AD) are illiterate or poorly educated, and only a few memory tests are adapted for these patients. Objective: In this study we aimed at assessing the diagnostic value of the T...

Background: Temporary disruption of the blood-brain barrier (BBB) using pulsed ultrasound leads to the clearance of both amyloid and tau from the brain, increased neurogenesis, and mitigation of cognitive decline in pre-clinical models of Alzheimer’s disease (AD) while also increasing BBB penetration of therapeutic antibodies. The goal of this pilo...

C9orf72 repeat expansions are rarely associated with primary progressive aphasias (PPA). In-depth characterization of the linguistic deficits, and the underlying patterns of grey-matter atrophy in PPA associated with the C9orf72 expansions (PPA-C9orf72) are currently lacking. In this study, we comprehensively analyzed a unique series of 16 patients...

Introduction: We aim to understand how patients with Alzheimer's disease (AD) are treated by identifying in a longitudinal fashion the late-life changes in patients' medical history that precede and follow AD diagnosis. Methods: We use prescription history of 34,782 patients followed between 1996 and 2019 by French general practitioners. We comp...

Objective Neurofilament light chain (NfL) is a promising biomarker in genetic frontotemporal dementia (FTD) and amyotrophic lateral sclerosis (ALS). We evaluated plasma neurofilament light chain (pNfL) levels in controls, and their longitudinal trajectories in C9orf72 and GRN cohorts from presymptomatic to clinical stages. Methods We analysed pNfL...

Background: Identifying a poor degree of awareness of cognitive decline (ACD) could represent an early indicator of Alzheimer's disease (AD). Objectives: (1) to understand whether there is evidence of poor ACD in the pre-dementia stages of AD; (2) to summarize the main findings obtained investigating ACD in AD; (3) to propose a conceptual framework...

Background Therapeutic research into Alzheimer’s disease (AD) has been dominated by the amyloid cascade hypothesis (ACH) since the 1990s. However, targeting amyloid in AD patients has not yet resulted in highly significant disease-modifying effects. Furthermore, other promising theories of AD etiology exist. Objective We sought to directly investi...

A variety of neuropsychiatric complications has been described in association with Covid-19 infection. Large scale studies presenting a wider picture of these complications and their relative frequency are lacking. The objective of our study was to describe the spectrum of neurological and psychiatric complications in patients with Covid-19 seen in...

There is substantial experimental evidence for dysregulation of several microRNA (miRNA) expression levels in Alzheimer’s disease (AD). MiRNAs modulate critical brain intracellular signaling pathways and are associated with AD core pathophysiological mechanisms. First, we conducted a real-time quantitative PCR-based pilot study to identify a set of...

Objective To determine relative frequencies and linguistic profiles of primary progressive aphasia (PPA) variants associated with progranulin ( GRN ) mutations, and study their neuroanatomical correlates. Methods PPA patients carrying GRN mutations (PPA- GRN ) were selected amongst a national prospective research cohort of 1,696 frontotemporal dem...

Combining multimodal biomarkers could help in the early diagnosis of Alzheimer's disease (AD). We included 304 cognitively normal individuals from the INSIGHT-preAD cohort. Amyloid and neurodegeneration were assessed on ¹⁸F-florbetapir and ¹⁸F-fluorodeoxyglucose PET, respectively. We used a nested cross-validation approach with non-invasive feature...

Background Cognitive impairment is an important comorbidity of refractory temporal lobe epilepsy (TLE). We aimed to explore the impact of (i) specific lesions, such as dysembryoplastic neuroepithelial tumor (DNET), dysplasia, or hippocampal sclerosis, (ii) focus localization (medial versus lateral) and (iii) focus lateralization (right versus left)...

Introduction À l’approche de thérapies ciblées dans les formes génétiques de démence frontotemporale (DFT) et sclérose latérale amyotrophique (SLA), l’identification des biomarqueurs d’évolution présymptomatique et conversion clinique représente un enjeu majeur. Objectifs Nous souhaitions évaluer les niveaux plasmatiques de la chaîne légère des ne...

Introduction Les troubles respiratoires (TbR) sont peu décrits chez les patients atteints de SEP, bien qu’ils jouent un rôle primordial dans le fardeau de la maladie. Première cause de mortalité, ils peuvent subitement s’aggraver, en cas d’infection pulmonaire par exemple. Objectifs Objectif principal : décrire trois TbR dans la SEP : – dysfonctio...

Introduction Tous les sujets Alzheimer asymptomatiques ne vont pas déclarer de symptômes cliniques de la maladie. Cibler les personnes à haut risque de progression vers un stade prodromal semble pertinent. Objectifs Déterminer chez des sujets asymptomatiques les facteurs de risque de progression vers un stade prodromal de la maladie d’Alzheimer (M...

Background. Patients with isolated cervical carotid artery occlusion not eligible to recanalization therapies but with compromised intracranial hemodynamics may be at risk of further clinical events. Apart from lying flat until spontaneous recanalization or adjustment of the collateral circulation hopefully occurs, no specific treatment is currentl...

Introduction Growing evidence highlighting a social cognition (SC) deficit in Parkinson’s disease (PD) have recently emerged, while pointing to ambiguous findings. Our objective was to determine if SC abilities impairment is frequent at the early stages of PD. Methods 109 patients with idiopathic PD diagnosed within the past four years (ICEBERG co...

Introduction : Mechanically ventilated patients admitted to the intensive care unit (ICU) for generalized convulsive status epilepticus (GCSE) are a heterogeneous population. Our objective was to evaluate the number of patients who fulfilled the diagnostic criteria for refractory GCSE and describe their initial management and prognosis. Methods :...

Background: A significant proportion of Alzheimer disease (AD) patients are illiterate or poorly educated, and only a few memory tests are adapted for these patients. The TNI-93 is a quick memory test that was designed for all patients regardless of their education level. In the present study we aimed at assessing the diagnostic value of the TNI-93...

Background It has recently been proposed that the awareness of cognitive decline (ACD) may already be slightly reduced in the preclinical phase of AD, in the presence of subtle cognitive changes. This is a recent field of research, and the few studies available do not allow to appreciate the actual evolution of ACD in the pre‐dementia phases of AD....

Background Neuropathological bases of suspected non‐Alzheimer’s disease pathophysiology (SNAP) have recently been identified as frequent causes of progressive isolated amnestic non Alzheimer’s disease (AD) neurodegenerative diseases 1,2 . Nonetheless, their in‐vivo profiles have so far been only poorly characterized. The goal of this study is to sp...

Background While memory performance is well studied in preclinical Alzheimer's disease (AD), there are relatively few studies concerning memory errors. However, in the clinical and prodromal stages, intrusions are very frequent, and the specific error profile appears to be particularly diacritical compared to other diseases. In this study, we explo...

Background Early biomarkers are needed to identify individuals at high risk of preclinical Alzheimer’s disease (AD) (Jack et al. , 2018). Electroencephalography (EEG) is a non‐invasive and cheap technique that would be an interesting screening tool for the preclinical stage of AD. Method We included participants from the INSIGHT‐preAD cohort, whic...

Background As not all the asymptomatic Alzheimer Disease (AD) subjects will progress to symptomatic stages, targeting those individuals at high risk of progression to prodromal AD is relevant, especially for disease‐modifier therapeutic trials. The aim of this study is to determine markers of progression to prodromal AD in cognitively normal indivi...

Background Early biomarkers are needed to identify individuals at high risk of preclinical Alzheimer’s disease (AD) (Jack et al. , 2018). Electroencephalography (EEG) is a non‐invasive and cheap technique that would be an interesting screening tool for the preclinical stage of AD. Method We included participants from the INSIGHT‐preAD cohort, whic...

Neuroinflammation, a key early pathomechanistic alteration of Alzheimer's disease, may represent either a detrimental or a compensatory mechanism or both (according to the disease stage). YKL-40, a glycoprotein highly expressed in differentiated glial cells, is a candidate biomarker for in vivo tracking neuroinflammation in humans. We performed a l...

Objective To describe the spectrum of neurological and psychiatric complications in patients with Covid-19 seen in a multidisciplinary center over six months. Methods We conducted a retrospective, observational study on all patients showing neurological or psychiatric symptoms in the context of Covid-19 seen in the Department of Neurology and Psyc...

Background Lack of awareness of cognitive decline (ACD) is common in late-stage Alzheimer’s disease (AD). Recent studies showed that ACD can also be reduced in the early stages. Methods We described different trends of evolution of ACD over 3 years in a cohort of memory-complainers and their association to amyloid burden and brain metabolism. We s...