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

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)

[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

[Backward disequilibrium in the elderly: review of symptoms and proposition of a tool for quantitative assessment].

International audiencePURPOSE: Although there is currently no epidemiological data on backward disequilibrium, this disturbance of posture does not seem to be rare in frail elderly. ACTUALITIES AND STRONG POINTS: Backward disequilibrium is characterized by the following criteria: the location of buttocks on the anterior side of the seat while the trunk rested at the back of the armchair in the sitting position; an inadequate forward of the trunk and a backward projection of the trunk outside the base of support during sit-to-stand; and a posterior projection of the center of mass outside the base of support in the standing position. Several pathological situations either somatic (degenerative, ischemic and traumatic brain lesions), psycho-somatic (psychomotor disadaptation syndrome, extended bed confining, non-use) or psychological (depression) affections can entail backward disequilibrium. Falls, loss of autonomy and the risk of the vicious circle with its causes are the main consequences of backward disequilibrium. PROSPECTS AND PROJECTS: Although the geriatrician is familiarized with backward disequilibrium, there is no scale to quantify it. In this paper we review causes, consequences and management of backward disequilibrium, and in order to assess it, we propose a semi-quantitative scale, based on some activities of everyday living which are sitting position, sit-to-stand, back-to-sit and standing position. So, a backward disequilibrium score could be determined

Backward disequilibrium in elderly subjects

Patrick Manckoundia1,2, France Mourey1,2, Dominic Pérennou2,3, Pierre Pfitzenmeyer1,21Department of Internal Medicine and Geriatrics, University Hospital, Dijon, France; 2INSERM/ERIT-M 0207 Motricity-Plasticity University of Burgundy, Dijon, France; 3Department of Neurological Rehabilitation, University Hospital, Dijon, FranceAbstract: Backward disequilibrium is observed frequently in daily clinical practice. However, there are no epidemiological data concerning this postural disorder. Defined by a posterior position of the centre of mass with respect to the base of support, backward disequilibrium is abnormal postural behavior, usually characterized by a posterior trunk tilt in standing and sitting positions, which predisposes subjects to backward falls. Many afflictions whether they are somatic (degenerative, ischemic and traumatic brain lesions), psychosomatic (psychomotor disadaptation syndrome, confinement to bed, nonuse situations) or psychological (depression) can cause backward disequilibrium. A vicious circle of falls, and loss of autonomy can arise and this is the main consequence of backward disequilibrium. Thus, in this paper, we review backward disequilibrium in elderly subjects with regard to the causes, consequences, assessment, and management.Keywords: backward disequilibrium, balance, elderly subject, falls, postur

Decreased trunk angular displacement during sitting down: an early feature of aging

BACKGROUND AND PURPOSE: Trunk motion plays an important role in achieving both sit-to-stand and stand-to-sit transfers. However, these 2 body transfers depend on different postural and mechanical constraints. Although the effects of aging on sit-to-stand transfers have been widely studied, there is a lack of information concerning stand-to-sit transfers. The aim of this study was to determine how angular displacements of the trunk and shank are affected by aging during sit-to-stand and stand-to-sit transfers. SUBJECTS: Ten community-dwelling older adults (mean age=75.9 years, SD=3.2) and 9 young adults (mean age=26.8 years, SD=4.7) volunteered to participate. METHODS: Maximal angular displacements of the trunk and shank with respect to the vertical (ie, orientation angles) were measured, during standing up and sitting down, using an optoelectronic movement analyzer. RESULTS For standing up, there was no difference between the young and older adults with regard to both maximal orientation angles. During sitting down, the maximal shank orientation angle was not affected by age, whereas the older adults had a smaller trunk motion compared with the young adults (approximately 10 degrees less). DISCUSSION AND CONCLUSION: The results showed that older adults tended to minimize the forward body displacement during sitting down. This strategy could be seen as an adaptive mechanism to decrease the risk of anterior disequilibrium. The authors suggest that this feature could be used as an early marker of aging on postural control

''Pneumonia due to Yersinia enterocolitica''

Jarrot, P. -A. | Mahmoudi, R. | Novella, J. -L. | Manckoundia, P.International audienc

Successful Management of Heparin-Induced Thrombocytopenia Using Argatroban in a Very Old Woman: A Case Report

Thrombosis due to heparin-induced thrombocytopenia (HIT) is rare but has a severe prognosis. Its management is not always easy, particularly in old patients with renal insufficiency. A 95-year-old woman was hospitalized for dyspnea. Curative treatment with unfractionated heparin was started because pulmonary embolism was suspected. Disseminated intravascular coagulation was then suspected because of thrombocytopenia, hypoprothrombinemia, hypofibrinogenemia, and a positive ethanol gelation test. The first immunoassay for HIT was negative. On the 12th day of hospitalization, bilateral cyanosis of the toes occurred associated with recent deep bilateral venous and arterial thrombosis at duplex ultrasound. New biological tests confirmed HIT and led us to stop heparin and to start argatroban with a positive clinical and biological evolution. Venous and arterial thrombosis associated with thrombocytopenia during heparin treatment must be considered HIT whatever the biological test results are. Argatroban is a good alternative treatment in the elderly