Gait analysis in demented subjects: Interests and perspectives

Gait disorders are more prevalent in dementia than in normal aging and are related to the severity of cognitive decline. Dementia-related gait changes (DRGC) mainly include decrease in walking speed provoked by a decrease in stride length and an increase in support phase. More recently, dual-task related changes in gait were found in Alzheimer’s disease (AD) and non-Alzheimer dementia, even at an early stage. An increase in stride-to-stride variability while usual walking and dual-tasking has been shown to be more specific and sensitive than any change in mean value in subjects with dementia. Those data show that DRGC are not only associated to motor disorders but also to problem with central processing of information and highlight that dysfunction of temporal and frontal lobe may in part explain gait impairment among demented subjects. Gait assessment, and more particularly dual-task analysis, is therefore crucial in early diagnosis of dementia and/or related syndromes in the elderly. Moreover, dual-task disturbances could be a specific marker of falling at a pre-dementia stage

Idiopathic Parkinson's disease phenotype related to C9ORF72 repeat expansions: contribution of the neuropsychological assessment.

International audienceBACKGROUND: Expanded GGGGCC hexanucleotide repeats in the non-coding region of the C9ORF72 gene was recently identified as being responsible for over 40% of the cases of amyotrophic lateral sclerosis associated with frontotemporal lobar degeneration, in various extrapyramidal syndromes including supranuclear gaze palsy and corticobasal degeneration, and in addition, has been found to be a rare genetic cause of isolated Parkinsonism. To our knowledge, there is no published data concerning the neuropsychological evaluation of patients diagnosed with idiopathic Parkinson's disease related with C9ORF72 repeat expansions. CASE PRESENTATION: We report the results of the comprehensive neuropsychological evaluation in a newly described case in the literature (the sixth) of a patient presenting isolated idiopathic Parkinson's disease associated with C9ORF72 repeat expansions.The decrease in the patient's prefrontal functions resulted in a slight decrease in global efficiency. These abnormalities did not appear to be different, with respect to the deficit observed and the intensity of the cognitive impairment, from those classically observed in cases of sporadic idiopathic Parkinson's disease. Our patient also exhibited a significant impairment in visual gnosis. CONCLUSIONS: If confirmed in other patients, visuoperceptive deficits in idiopathic Parkinson's disease could represent a red flag that should prompt the clinician to perform addition diagnostic procedures. A thorough neuropsychological assessment may prove to be useful for detecting idiopathic Parkinson's disease in patients who are suspected of having repeat abnormalities of C9ORF72 expansions

Molecular Imaging of Microglial Activation in Amyotrophic Lateral Sclerosis

There is growing evidence of activated microglia and inflammatory processes in the cerebral cortex in amyotrophic lateral sclerosis (ALS). Activated microglia is characterized by increased expression of the 18 kDa translocator protein (TSPO) in the brain and may be a useful biomarker of inflammation. In this study, we evaluated neuroinflammation in ALS patients using a radioligand of TSPO, 18F-DPA-714. Ten patients with probable or definite ALS (all right-handed, without dementia, and untreated by riluzole or other medication that might bias the binding on the TSPO), were enrolled prospectively and eight healthy controls matched for age underwent a PET study. Comparison of the distribution volume ratios between both groups were performed using a Mann-Whitney’s test. Significant increase of distribution of volume ratios values corresponding to microglial activation was found in the ALS sample in primary motor, supplementary motor and temporal cortex (p = 0.009, p = 0.001 and p = 0.004, respectively). These results suggested that the cortical uptake of 18F-DPA-714 was increased in ALS patients during the ‘‘time of diagnosis’’ phase of the disease. This finding might improve our understanding of the pathophysiology of ALS and might be a surrogate marker of efficacy of treatment on microglial activation

Association of angiitis of central nervous system, cerebral amyloid angiopathy, and Alzheimer’s disease: Report of an autopsy case

The association of angiitis of central nervous system (ACNS) with cerebral amyloid angiopathy (CAA) suggests a physiopathological relationship between these two affections. Few cases are reported in patients with Alzheimer’s disease (AD). We describe here a clinicopathological case associating ACNS, CAA, and AD. We discuss the aetiology of ACNS and its relationship with cerebral deposition of beta A4 amyloid protein (βA4)

Recognition-based memory through familiarity assessment in severe Alzheimer’s disease

peer reviewe

Encephalopathy associated with autoimmune thyroid disease in patients with Graves' disease: clinical manifestations, follow-up, and outcomes

<p>Abstract</p> <p>Background</p> <p>The encephalopathy associated with autoimmune thyroid disease (EAATD) is characterized by neurological/psychiatric symptoms, high levels of anti-thyroid antibodies, increased cerebrospinal fluid protein concentration, non-specific electroencephalogram abnormalities, and responsiveness to the corticosteroid treatment in patients with an autoimmune thyroid disease. Almost all EAATD patients are affected by Hashimoto's thyroiditis (HT), although fourteen EAATD patients with Graves' disease (GD) have been also reported.</p> <p>Methods</p> <p>We have recorded and analyzed the clinical, biological, radiological, and electrophysiological findings and the data on the therapeutic management of all GD patients with EAATD reported so far as well as the clinical outcomes in those followed-up in the long term.</p> <p>Results</p> <p>Twelve of the fourteen patients with EAATD and GD were women. The majority of GD patients with EAATD presented with mild hyperthyroidism at EAATD onset or shortly before it. Active anti-thyroid autoimmunity was detected in all cases. Most of the patients dramatically responded to corticosteroids. The long term clinical outcome was benign but EAATD can relapse, especially at the time of corticosteroid dose tapering or withdrawal. GD and HT patients with EAATD present with a similar clinical, biological, radiological, and electrophysiological picture and require an unaffected EAATD management.</p> <p>Conclusions</p> <p>GD and HT equally represent the possible background condition for the development of EAATD, which should be considered in the differential diagnosis of all patients with encephalopathy of unknown origin and an autoimmune thyroid disease, regardless of the nature of the underlying autoimmune thyroid disease.</p

Etude phénotypique des démences extrapyramidales (apport de la neuropsychologie dans le diagnostic différentiel.)

Les situations cliniques associant des troubles moteurs et une détérioration cognitive sont fréquentes et déroutantes pour le clinicien, qui est confronté à la difficile question du diagnostic différentiel entre plusieurs cadres nosographiques dont la maladie de Parkinson avec démence (MPD) et la démence à corps de Lewy Diffus (DCLD). Dans ce travail, nous avons étudié les caractéristiques neuropsychologiques pouvant différencier les deux affections. Nous avons, dans une première étude, montré que la mémoire de reconnaissance visuelle était altérée de façon différente. Dans un second travail, nous avons spécifié les caractéristiques de ces altérations en les rapprochant des classiques profils cortical et sous cortical de démence. Nous avons ainsi montré que la MPD présentait une altération de la mémoire de reconnaissance visuelle intermédiaire entre la Maladie d Alzheimer et la DCLD. Enfin, dans une dernière partie, nous proposons des perspectives de recherche dans la continuité de ces travaux.Clinical manifestations associating motor and cognitive impairment are frequently encountered and difficult for the clinician who is required to address the problem of making the correct differential diagnosis, particularly to differentiate Parkinson's disease with dementia (PDD) from Lewy bodies dementia (LBD). In this study, we examined the neuropsychological characteristics which allow us to differentiate the two disorders. In the first study, we demonstrated that visual recognition memory is disturbed differently in the two cases. In a second study, we specified the characterisstics of the modifications encountered by using the classic "cortical" and "subcortical" dementia profiles. We also showed that, in PDD, the alteration in visual recognition memory is intermediary between Alzheimer's disease and LBD. Finally, in the last part of our study, we suggest future avenues of research needed to complete our work.TOURS-Bibl.électronique (372610011) / SudocSudocFranceF

Association of angiitis of central nervous system, cerebral amyloid angiopathy, and Alzheimer&rsquo;s disease: Report of an autopsy case

C&eacute;dric Annweiler1, Marc Paccalin2, Gilles Berrut3, Caroline Hommet4, Christian Lavigne1, Jean-Paul Saint-Andr&eacute;5, Olivier Beauchet11Department of Geriatrics and Internal Medicine, Angers University Hospital, France; 2Department of Geriatrics, Poitiers University Hospital, France; 3Department of Geriatrics, Nantes University Hospital, France; 4Department of Geriatrics, Tours University Hospital, France; 5Department of Anatomopatholgy, Angers University Hospital, FranceAbstract: The association of angiitis of central nervous system (ACNS) with cerebral amyloid angiopathy (CAA) suggests a physiopathological relationship between these two affections. Few cases are reported in patients with Alzheimer&rsquo;s disease (AD). We describe here a clinicopathological case associating ACNS, CAA, and AD. We discuss the aetiology of ACNS and its relationship with cerebral deposition of beta A4 amyloid protein (&beta;A4).Keywords: cerebral angiopathy, Alzheimer&rsquo;s diseas

Olfaction: a potential cognitive marker of psychiatric disorders

International audienceCognitive deficits are well documented in psychiatric disorders, particularly in schizophrenia and depression. Cognitive activity roots in perceptions. However, research on sensorial alterations in psychiatric conditions has mainly focused on visual or auditory processes and less on olfaction. Here, we examine data on olfactory deficits in psychiatric patients using a systematic review of recent publications. Schizophrenic patients are mainly characterized by no reliable change in odour sensitivity and by a deficit in odour identification, recognition and discrimination. Depressed patients principally exhibit a deficit in the hedonic aspects of this perception, even if, in some case, alterations in sensitivity or identification are also found. Changes in odour perception are also found in dementia and in some neurodegenerative disease, but in this case alterations concern all aspects of the sensorial experience (detection threshold, identification and recognition). Taken together, these data indicate that olfactory abnormalities might be a marker of psychiatric conditions, with a specific pattern for each disease