Peripheral Neuropathy and VIth Nerve Palsy Related to Randall Disease Successfully Treated by High-Dose Melphalan, Autologous Blood Stem Cell Transplantation, and VIth Nerve Decompression Surgery

Randall disease is an unusual cause of extraocular motor nerve (VI) palsy. A 35-year-old woman was hospitalized for sicca syndrome. The physical examination showed general weakness, weight loss, diplopia related to a left VIth nerve palsy, hypertrophy of the submandibular salivary glands, and peripheral neuropathy. The biological screening revealed renal insufficiency, serum monoclonal kappa light chain immunoglobulin, urinary monoclonal kappa light chain immunoglobulin, albuminuria, and Bence-Jones proteinuria. Bone marrow biopsy revealed medullar plasma cell infiltration. Immunofixation associated with electron microscopy analysis of the salivary glands showed deposits of kappa light chains. Randall disease was diagnosed. The patient received high-dose melphalan followed by autostem cell transplantation which led to rapid remission. Indeed, at the 2-month followup assessment, the submandibular salivary gland hypertrophy and renal insufficiency had disappeared, and the peripheral neuropathy, proteinuria, and serum monoclonal light chain had decreased significantly. The persistent diplopia was treated with nerve decompression surgery of the left extraocular motor nerve. Cranial nerve complications of Randall disease deserve to be recognized

Voluntary imitation in alzheimer's disease patients

Although Alzheimer's disease (AD) primarily manifests as cognitive deficits, the implicit sensorimotor processes that underlie social interactions, such as automatic imitation, seem to be preserved in mild and moderate stages of the disease, as is the ability to communicate with other persons. Nevertheless, when AD patients face more challenging tasks, which do not rely on automatic processes but on explicit voluntary mechanisms and require the patient to pay attention to external events, the cognitive deficits resulting from the disease might negatively affect patients' behavior. The aim of the present study was to investigate whether voluntary motor imitation, i.e., a volitional mechanism that involves observing another person's action and translating this perception into one's own action, was affected in patients with AD. Further, we tested whether this ability was modulated by the nature of the observed stimulus by comparing the ability to reproduce the kinematic features of a human demonstrator with that of a computerized-stimulus. AD patients showed an intact ability to reproduce the velocity of the observed movements, particularly when the stimulus was a human agent. This result suggests that high-level cognitive processes involved in voluntary imitation might be preserved in mild and moderate stages of AD and that voluntary imitation abilities might benefit from the implicit interpersonal communication established between the patient and the human demonstrator

Increased physical fitness is associated with higher executive functioning in people with dementia

Physical fitness (PF) has been associated with improved cognition in older age, but less is known about its effects on different cognitive domains in individuals diagnosed with dementia. We explored the associations between PF and cognitive performance in 40 healthy elderly and 30 individuals with dementia. Participants completed a battery of standardized cognitive tests (Mini-Mental State Exam, Verbal Fluency, Prospective and Retrospective Memory Questionnaire, Clock Drawing, and California Verbal Learning Test) and were classified into high versus low levels of PF based on their score on the Physical Fitness Questionnaire. Analyses took into account age, gender, education, occupation, head injury, Internet use, brain training, and past levels of exercise and revealed overall benefits of PF, in particular for the people with dementia. Discriminant analysis showed high accuracy of reclassification, with most errors being due to the misclassification of dementia cases as healthy when they had high PF. The first discriminant function accounted for 83% of the variance. Using individual estimates of this function, which reflected global cognitive performance, confirmed the beneficial role of PF in dementia, even when taking into account age, past level of exercise, and the number of years since the dementia diagnosis. Finally, univariate analyses confirmed the differential sensitivity of the cognitive tests, with MMSE and clock drawing showing reliable interaction effects. This work shows that PF is associated with a reduced level of cognitive deterioration expected with dementia, especially in executive functioning and provides empirical support for the cognitive benefits of interventions promoting PF for individuals with dementia

Eye Movements Affect Postural Control in Young and Older Females

Visual information is used for postural stabilization in humans. However, little is known about how eye movements prevalent in everyday life interact with the postural control system in older individuals. Therefore, the present study assessed the effects of stationary gaze fixations, smooth pursuits, and saccadic eye movements, with combinations of absent, fixed and oscillating large-field visual backgrounds to generate different forms of retinal flow, on postural control in healthy young and older females. Participants were presented with computer generated visual stimuli, whilst postural sway and gaze fixations were simultaneously assessed with a force platform and eye tracking equipment, respectively. The results showed that fixed backgrounds and stationary gaze fixations attenuated postural sway. In contrast, oscillating backgrounds and smooth pursuits increased postural sway. There were no differences regarding saccades. There were also no differences in postural sway or gaze errors between age groups in any visual condition. The stabilizing effect of the fixed visual stimuli show how retinal flow and extraocular factors guide postural adjustments. The destabilizing effect of oscillating visual backgrounds and smooth pursuits may be related to more challenging conditions for determining body shifts from retinal flow, and more complex extraocular signals, respectively. Because the older participants matched the young group's performance in all conditions, decreases of posture and gaze control during stance may not be a direct consequence of healthy aging. Further research examining extraocular and retinal mechanisms of balance control and the effects of eye movements, during locomotion, is needed to better inform fall prevention interventions

Effectiveness of balance training exercise in people with mild to moderate severity Alzheimer's disease: protocol for a randomised trial

BACKGROUND: Balance dysfunction and falls are common problems in later stages of dementia. Exercise is a well-established intervention to reduce falls in cognitively intact older people, although there is limited randomised trial evidence of outcomes in people with dementia. The primary objective of this study is to evaluate whether a home-based balance exercise programme improves balance performance in people with mild to moderate severity Alzheimer's disease. METHODS/DESIGN: Two hundred and fourteen community dwelling participants with mild to moderate severity Alzheimer's disease will be recruited for the randomised controlled trial. A series of laboratory and clinical measures will be used to evaluate balance and mobility performance at baseline. Participants will then be randomized to receive either a balance training home exercise programme (intervention group) from a physiotherapist, or an education, information and support programme from an occupational therapist (control group). Both groups will have six home visits in the six months following baseline assessment, as well as phone support. All participants will be re-assessed at the completion of the programme (after six months), and again in a further six months to evaluate sustainability of outcomes. The primary outcome measures will be the Limits of Stability (a force platform measure of balance) and the Step Test (a clinical measure of balance). Secondary outcomes include other balance and mobility measures, number of falls and falls risk measures, cognitive and behavioural measures, and carer burden and quality of life measures. Assessors will be blind to group allocation. Longitudinal change in balance performance will be evaluated in a sub-study, in which the first 64 participants of the control group with mild to moderate severity Alzheimer's disease, and 64 age and gender matched healthy participants will be re-assessed on all measures at initial assessment, and then at 6, 12, 18 and 24 months. DISCUSSION: By introducing a balance programme at an early stage of the dementia pathway, when participants are more likely capable of safe and active participation in balance training, there is potential that balance performance will be improved as dementia progresses, which may reduce the high falls risk at this later stage. If successful, this approach has the potential for widespread application through community based services for people with mild to moderate severity Alzheimer's disease. TRIAL REGISTRATION: The protocol for this study is registered with the Australian New Zealand Clinical Trials Registry (ACTRN12608000040369)

Lewy body disease: clinical and pathological "overlap syndrome" between synucleinopathies (Parkinson disease) and tauopathies (Alzheimer disease)

This article is part of the Topical Collection on Movement Disorders.International audienceLewy body disease (LBD) is a neurodegenerative disease resulting in dementia. It shares clinical and pathological features with Parkinson disease (PD), the most frequent synucleinopathy, Parkinson disease dementia (PDD), and Alzheimer disease (AD), a tauopathy. Even though the diagnostic criteria for these neurodegenerative diseases are clearly established, and recently revised for LBD, their precise clinical diagnosis is often difficult because LBD, PD, PDD, and AD share epidemiological, clinical, and pathological characteristics. This manuscript discusses current understanding of overlapping symptoms and the particular features of LBD, PD, and AD. It also describes features that could facilitate the diagnosis of each of these diseases. We concluded that the concept of neurodegenerative "overlap" syndrome, which includes the accepted diagnosis of LBD, may be taken in account and should contribute to clarifying LBD and definitions of close differential diagnoses. This should allow clinicians to suspect LBD at an earlier stage and provide better patient care

[Psychomotor disadaptation syndrome].

International audiencePsychomotor disadaptation syndrome (PDS) was first described by the Geriatrics School of Dijon (France), three decades ago, under the name «psychomotor regression syndrome». Over time, the original clinical features remained unchanged. However, progress has been made in its pathophysiology understanding and care, hence the new name, PDS, appeared in the 1990s. The PDS is also called sub-cortico-frontal dysfunction syndrome since the 2000s. It corresponds to a decompensation of posture, gait and psychomotor automatisms, related to an alteration of the postural and motor programming, which is a consequence of sub-cortico-frontal lesions. The clinical features of PDS associate backward disequilibrium, nonspecific gait disorders and neurological signs (akinesia, reactional hypertonia, impaired reactive postural responses and protective reactions, etc.). Psychological disorders of PDS are a fear of standing and walking in its acute form (the post-fall syndrome), or a bradyphrenia and anhedonia in its chronic form. The PDS occurrence results from the combination of three factors implicated in the reduction in functional reserves related to the alteration of the sub-cortico-frontal structures: ageing, chronic afflictions and acute situations, which induce a decrease in cerebral blood flow. The PDS management must be multidisciplinary, including the physician, the physiotherapist, the psychologist, nurses and care assistants

Cognition Impairment and Gait Disorders in Older Adults

Thanks to the increase in life expectancy linked to scientific and medical progress and improvements in hygiene conditions, the population of people aged 75 years and over continues to grow worldwide, particularly in industrialized countries [...

Experience of medical students mentoring in an acute geriatric unit: Use of evidence-based medicine

International audiencen a retrospective study, we studied the learning experience of second and third year undergraduate medical students in the acute geriatric unit of a University Hospital.Students who did not receive Evidence based medicine (EBM) classes had lower grades than those who did in the final theoretical and practical evaluations (p < .01).Our experience suggested that the implementation of EBM in the curriculum through small, structured semiology courses tailored to the specificities of the elderly improved Evidence based practices for undergraduate medical students. EBM should improve instruction and mentoring during undergraduate students' clinical rotation