Bone mineral density in adults with arthrogryposis multiplex congenita: a retrospective cohort analysis.

The primary objective of this study was to evaluate the prevalence of low femoral and lumbar spine bone mineral density (BMD) in adults with arthrogryposis multiplex congenita (AMC). We performed a retrospective cohort analysis of adults with AMC who were enrolled in the French Reference Center for AMC and in the Pediatric and Adult Registry for Arthrogryposis (PARART, NCT05673265). Patients who had undergone dual-energy X-ray absorptiometry (DXA) and/or vitamin D testing were included in the analysis. Fifty-one patients (mean age, 32.9 ± 12.6 years) were included; 46 had undergone DXA. Thirty-two (32/51, 62.7%) patients had Amyoplasia, and 19 (19/51, 37.3%) had other types of AMC (18 distal arthrogryposis, 1 Larsen). Six patients (6/42, 14.3%) had a lumbar BMD Z score less than - 2. The mean lumbar spine Z score (- 0.03 ± 1.6) was not significantly lower than the expected BMD Z score in the general population. Nine (9/40, 22.5%) and 10 (10/40, 25.0%) patients had femoral neck and total hip BMD Z scores less than - 2, respectively. The mean femoral neck (- 1.1 ± 1.1) and total hip (- 1.2 ± 1.2) BMD Z scores in patients with AMC were significantly lower than expected in the general population (p < 0.001). Femoral neck BMD correlated with height (rs = 0.39, p = 0.01), age (rs = - 0.315, p = 0.48); total hip BMD correlated with height (rs = 0.331, p = 0.04) and calcium levels (rs = 0.41, p = 0.04). Twenty-five patients (25/51, 49.0%) reported 39 fractures. Thirty-one (31/36, 86.1%) patients had 25-hydroxyvitamin D levels less than 75 nmol/l, and 6 (6/36, 16.7%) had 25-hydroxyvitamin D levels less than 75 nmol/l. Adults with AMC had lower hip BMD than expected for their age, and they more frequently showed vitamin D insufficiency. Screening for low BMD by DXA and adding vitamin D supplementation when vitamin D status is insufficient should be considered in adults with AMC, especially if there is a history of falls or fractures.

Clinimetric properties of relevant criteria for assessing writing and drawing orientation after right hemisphere stroke.

BACKGROUND: Writing and drawing orientation is rarely assessed in clinical routine, although it might have a potential value in detecting impaired verticality perception after right hemispheric stroke (RHS). Assessment tools and criteria must be conceived and validated. We therefore explored the clinimetric properties of a set of quantitative writing and drawing orientation criteria, their ranges of normality, and their tilt prevalence in RHS individuals. NEW METHODS: We asked 69 individuals with subacute RHS and 64 matched healthy controls to write three lines and to copy the Gainotti Figure (house and trees). We determined six criteria referring to the orientation of writing and drawing main axes: for writing, the line and margin orientations, and for drawing, the tree, groundline, wall, and roofline orientations. Orientations were measured by using an electronic protractor from specific landmarks positioned by independent evaluators. RESULTS: The set of criteria fulfilling all clinimetric properties (feasibility, measurability, reliability) comprised the line orientation of the writing and the wall and roofline orientations of the drawing. Writing and drawing tilts were frequent after RHS (about 30% by criterion). COMPARISON WITH EXISTING METHODS: So far, graphomotor orientation was mostly tested qualitatively and could not be objectively appreciated in absence of validated tools and criteria, and without ranges of normality. Writing and drawing tilts may now be assessed both in routine clinical practice and research. CONCLUSIONS: Our study paves the way for investigating the clinical determinants of graphomotor tilts, including impaired verticality perception, to better understand their underlying mechanisms.

Looking at hospitalized persons throughout the prism of the handicap.

OBJECTIVE: To describe the disability status of non-selected hospitalized persons. METHODS AND FINDINGS: We conducted a cross-sectional survey to assess activity limitations of every person older than 18 years hospitalized in a regional university hospital covering all medical fields. Evaluators rated, on a scale from 0 to 4, 22 selected items of the International Classification of Functioning (ICF), covering the 6 following domains: learning and applying knowledge, general tasks and demands, communication, mobility, self-care, and interpersonal interactions and relationships. Univariate and multivariate analyses were performed to analyze the prevalence, severity and profile of the handicap in terms of sociodemographic characteristics and care pathways. RESULTS: Among 1572 eligible persons, 1267 (81%) were surveyed (mean age 62.7±20.4years; 655 males [51.7%]). Overall, 82% showed at least one activity limitation. For 52%, disability was severe or total for at least one ICF item. Prevalence of disabilities was higher for mobility (75%) and self-care domains (63%). Disability was strongly related to age: age older than 80years versus 18 to 44years (OR=12.8 95% CI 6.4-27.9]; P<0.01). Disability was associated with hospitalization in rehabilitation units (96%; OR=4.3 [95% CI 2.2-5.3]; P<0.01). Severe disability was associated with hospitalization in critical care units (OR=6.7 [CI 3.2-15.1]; P<0.001) and psychiatry units (OR=5.3 [CI 2.7-11.4]; P<0.001). CONCLUSION: Handicap was common in hospitalized persons, involving all 6 tested ICF activity domains, particularly mobility and self-care. This study alerts care givers, hospital administrators, and in general, people influencing health policies about the need to plan actions to reduce activity limitations of hospitalized persons, whatever the cause of the hospitalization.

Visual verticality perception after stroke: A systematic review of methodological approaches and suggestions for standardization.

OBJECTIVE: Visual vertical (VV) measurements are being increasingly used for routine clinical assessment of spatial cognition, to investigate otolithic vestibular function and identify altered verticality perception as a possible cause of postural disorders after stroke. The objective of this paper was to synthesize knowledge of assessment methods for testing VV after stroke. METHODS: This systematic review, following the PRISMA statement, involved a search for articles in MEDLINE via PubMED published up to November 2015 by using the search terms "visual vertical," "verticality perception" and "stroke". We included only case or group studies on VV perception after hemispheric, brainstem or cerebellar strokes. Two authors independently assessed data on patients' and VV assessment characteristics, outcome measures, ranges of normality and psychometric properties. RESULTS: We assessed reports for 61 studies (1982 patients) of VV for hemispheric (n=43), brainstem (n=18) or cerebellar (n=8) stroke. VV assessment procedures varied widely in paradigm, type of stimulus, patient posture, number of trials and outcome measures. However, on the basis of recent studies it is recommended assessing VV in absolute darkness, with an even number of trials, from 6 to 10, with the body maintained upright. Under these conditions, normal VV orientation (mean of VV estimates) can be considered from -2.5° to 2.5° and is highly reliable for use in clinical practice and research. A difference ≥ 2° between repeated measures for a given patient can be interpreted as a real change in VV perception. Myriad of protocols have been proposed, for which psychometric properties must be better analyzed. CONCLUSIONS: This first review of VV assessment methods after stroke shows a great heterogeneity of procedures, settings and parameters, among which only some are eligible for standardization to limit measurement errors and better interpret the results.

The effects of mirror therapy on pain and motor control of phantom limb in amputees: A systematic review.

BACKGROUND AND OBJECTIVE: Phantom limb pain (PLP) is a major problem after limb amputation. Mirror therapy (MT) is a non-pharmacological treatment using representations of movement, the efficacy of which in reducing PLP remains to be clarified. Here, we present the first systematic review on MT efficacy in PLP and phantom limb movement (PLM) in amputees (lower or upper limb). METHODS: A search on Medline, Cochrane Database and Embase, crossing the keywords "Phantom Limb" and "Mirror Therapy" found studies which were read and analyzed according the PRISMA statement. RESULTS: Twenty studies were selected, 12 on the subject of MT and PLP, 3 on MT and PLM, 5 on MT and both (PLP and PLM). Among these 20 studies, 5 were randomized controlled trials (163 patients), 6 prospective studies (55 patients), 9 case studies (40 patients) and methodologies were heterogeneous. Seventeen of the 18 studies reported the efficacy of MT on PLP, but with low levels of evidence. One randomized controlled trial did not show any significant effect of MT. As to the effect of MT on PLM, the 8 studies concerned reported effectiveness of MT: 4 with a low level of evidence and 4 with a high level of evidence. An alternative to visual illusion seems to be tactile or auditory stimulation. CONCLUSION: We cannot recommend MT as a first intention treatment in PLP. The level of evidence is insufficient. Further research is needed to assess the effect of MT on pain, prosthesis use, and body representation, and to standardize protocols.

Assessing the visual vertical: how many trials are required?

BACKGROUND: The visual vertical (VV) consists of repeated adjustments of a luminous rod to the earth vertical. How many trials are required to reach consistency in this measure? This question has never been addressed despite the widespread clinical use of the measurement in stroke rehabilitation. METHODS: VV perception was assessed (10 trials) in 117 patients undergoing rehabilitation after a first hemisphere stroke. The intraclass correlation coefficient (ICC) and standard error of measurement (SEM) were calculated for each patient category: with contralesional VV bias (n = 48), ipsilesional VV bias (n = 17) and normal VV (n = 52). RESULTS: For patients with VV biases, 6 trials were required to reach high inter-trial reliability (contralesional: ICC = 0.9, SEM = 1.36°; ipsilesional: ICC = 0.896, SEM = 0.96°). For patients with normal VV, a minimum of 10 trials was required (ICC = .728, SEM = 1.13°). A set of 6 trials correctly classified 96 % of patients. CONCLUSIONS: In the literature, 10 is the most frequently used number of trials used to assess VV orientation. Our study shows that 10 trials are required to adequately measure VV orientation in non-selected subacute stroke patients. For complex protocols imposing a decrease in the number of trials in each condition, 6 trials are needed to identify VV biases in most patients.

The Cognitive Assessment scale for Stroke Patients (CASP) vs. MMSE and MoCA in non-aphasic hemispheric stroke patients.

INTRODUCTION: CASP specifically assesses post-stroke cognitive impairments. Its items are visual and as such can be administered to patients with severe expressive aphasia. We have previously shown that the CASP was more suitable than the Mini Mental State Examination (MMSE) and Montreal Cognitive Assessment (MoCA) in aphasic patients. Our objective was to compare the above scales in non-aphasic stroke patients, and assess to what extent the solely visual items of the CASP were problematic in cases of neurovisual impairments. METHODS: Fifty non-aphasic patients admitted to Physical Medicine and Rehabilitation (PM&R) units after a recent left- or right-hemisphere stroke were evaluated with the CASP, MMSE and MoCA. We compared these three scales in terms of feasibility, concordance, and influence of neurovisual impairments on the total score. RESULTS: Twenty-nine men and 21 women were included (mean age 63 ± 14). For three patients, the MoCa was impossible to administer. It took significantly less time to administer the CASP (10 ± 5 min) than the MoCA (11 ± 5 min, P=0.02), yet it still took more time than MMSE administration (7 ± 3 min, P<10(-6)). Neurovisual impairments affected equally the total scores of the three tests. Concordance between these scores was poor and only the CASP could specifically assess unilateral spatial neglect. CONCLUSION: The sole visual format of the CASP scale seems suitable for administration in post-stroke patients.

Feasibility of the cognitive assessment scale for stroke patients (CASP) vs. MMSE and MoCA in aphasic left hemispheric stroke patients.

INTRODUCTION: Post-stroke aphasia makes it difficult to assess cognitive deficiencies. We thus developed the CASP, which can be administered without using language. Our objective was to compare the feasibility of the CASP, the Mini Mental State Examination (MMSE) and the Montreal Cognitive Assessment (MoCA) in aphasic stroke patients. MATERIAL AND METHODS: All aphasic patients consecutively admitted to seven French rehabilitation units during a 4-month period after a recent first left hemispheric stroke were assessed with CASP, MMSE and MoCA. We determined the proportion of patients in whom it was impossible to administer at least one item from these 3 scales, and compared their administration times. RESULTS: Forty-four patients were included (age 64±15, 26 males). The CASP was impossible to administer in eight of them (18%), compared with 16 for the MMSE (36%, P=0.05) and 13 for the MoCA (30%, P=0.21, NS). It was possible to administer the CASP in all of the patients with expressive aphasia, whereas the MMSE and the MoCA could not be administered. Administration times were longer for the CASP (13±4min) than for the MMSE (8±3min, P<10(-6)) and the MoCA (11±5min, P=0.23, NS). CONCLUSION: The CASP is more feasible than the MMSE and the MoCA in aphasic stroke patients.

The assessment and treatment of postural disorders in cerebellar ataxia: a systematic review.

Gait and balance disorders are often major causes of handicap in patients with cerebellar ataxia. Although it was thought that postural and balance disorders in cerebellar ataxia were not treatable, recent studies have demonstrated the beneficial effects of rehabilitation programs. This article is the first systematic review on the treatment of postural disorders in cerebellar ataxia. Nineteen articles were selected, of which three were randomized, controlled trials. Various aetiologies of cerebellar ataxia were studied: five studies assessed patients with multiple sclerosis, four assessed patients with degenerative ataxia, two assessed stroke patients and eight assessed patients with various aetiologies. Accurate assessment of postural disorders in cerebellar ataxia is very important in both clinical trials and clinical practice. The Scale for the Assessment and Rating of Ataxia (SARA) is a simple, validated measurement tool, for which 18 of the 40 points are related to postural disorders. This scale is useful for monitoring ataxic patients with postural disorders. There is now moderate level evidence that rehabilitation is efficient to improve postural capacities of patients with cerebellar ataxia - particularly in patients with degenerative ataxia or multiple sclerosis. Intensive rehabilitation programs with balance and coordination exercises are necessary. Although techniques such as virtual reality, biofeedback, treadmill exercises with supported bodyweight and torso weighting appear to be of value, their specific efficacy has to be further investigated. Drugs have only been studied in degenerative ataxia, and the level of evidence is low. There is now a need for large, randomized, controlled trials testing rehabilitation programs suited to postural and gait disorders of patients with cerebellar ataxia.

Measuring verticality perception after stroke: why and how?

About 80 papers dealing with verticality after stroke have been published in the last 20years. Here we reviewed the reasons and findings that explain why measuring verticality perception after stroke is interesting. Research on verticality perception after stroke has contributed to improve the knowledge on brain mechanisms, which build up and update a sense of verticality. Preliminary research using modern techniques of brain imaging has shown that the posterior lateral thalamus and the parietal insular cortex are areas of interest for this internal model of verticality. How they interact and are critical remains to be investigated. From a clinical standpoint, it has now been clearly established that biases in verticality perception are frequent after a stroke, causing postural disorders. Measuring the postural vertical with the wheel paradigm has allowed elucidating the mechanisms of lateropulsion, leading or not to a pushing. Schematically, patients with a hemispheric stroke align their erect posture with an erroneous reference of verticality, tilted to the side opposite the lesion. In patients with a brainstem stroke lateropulsion is usually ipsilesional, and results rather from a pathological asymmetry of tone, through vestibulo-spinal mechanisms. These evolutions of concepts and measurement standards of verticality representation should guide the emergence of rehabilitation programs specifically dedicated to the sense of verticality after stroke. Indeed, several pilot studies using appropriate somatosensory stimulation suggest the possibility to recalibrate the internal model of verticality biased by the stroke, and to improve uprightness. Vestibular stimulations seem to be less relevant and efficient.

[Arterial bypass iterative thrombosis and cancer: three cases]. / Thromboses itératives de pontages artériels et cancer : à propos de trois cas.

Cancer associated with venous thromboembolic disease has been recognized since Trousseau, but a link between cancer and iterative arterial thrombosis is rarely described. We report three cases of patients with iterative bypass thrombosis in whom cancer was subsequently diagnosed: lung cancer in one patient and hepatocarcinoma and bladder cancer in the others. Smoking and hypertension were risk factors in both patients. The link between arterial thrombosis and cancer is probably multifactorial. In case of iterative arterial bypass thrombosis, the search for cancer is as useful as the control of cardiovascular risk factors and the search for antiphospholipid syndrome, since patient management can be affected.

Interest of workplace support for returning to work after a traumatic brain injury: a retrospective study.

OBJECTIVE: To analyse usefulness of the SPASE programme, a coordinated facility programme to assist traumatic brain injury (TBI) persons in returning to work and retaining their job in the ordinary work environment. DESIGN: A retrospective study including 100 subjects aged over 18 who had suffered traumatic brain injury (GOS 1 or 2). The criterion for return to work (RTW) success was the ability to return to the job he/she had before the accident or to a new professional activity. RESULTS: Factors associated with RTW success were at short-term (2-3 years): the presence of significant workplace support OR=15.1 [3.7-61.7], the presence of physical disabilities OR=0.32 [0.12-0.87] or serious traumatic brain injury OR=0.22 [0.07-0.66]. At medium-term (over 3 years) these factors were: significant workplace support OR=3.9 [1.3-11.3] and presence of mental illness OR=0.15 [0.03-0.7]. CONCLUSION: This study suggests that a case coordination vocational programme may facilitate the return and maintain to work of TBI persons. It reveals that the workplace support is a key factor for job retention in the medium-term.

Achilles tendon vibration shifts the center of pressure backward in standing and forward in sitting in young subjects.

OBJECTIVE: An Achilles tendon (AT) vibration is known to disrupt the postural control in standing ("vibration-induced falling", VIF) and to backward tilt the postural vertical in restrained sitting position, suggesting a link between AT vibration and internal representations involved in postural control. A recalibration of some troubles in body orientation by oriented sensory manipulations could be of great clinical interest. In order to use the VIF paradigm in a procedure suitable for a rehabilitation context, AT vibration deserves to be more investigated in sitting, for security reasons, and first in young participants. METHOD: In 12 healthy participants (6 men/6 women; 23.3±1.9 years), posturographic data to AT vibrations (85 Hz) were recorded over 30 s standing and 40 s sitting trials. RESULTS: Surprisingly, four types of differences were found between standing and sitting AT vibrations: presence/absence of VIF and kinesthetic illusion, opposite directions of the centre of foot pressure (CoP) displacements, different temporal profiles. In standing: VIF without kinesthetic illusion, rapid backward shift of CoP with a peak of -54.6±11.3 mm (with respect to baseline P<0.001) 2.8±0.2 s after vibration onset. In sitting: surprising systematic forward shift of the CoP throughout the duration of AT vibration with a maximum of 27.9±18.9 mm (P<0.05 with respect to the baseline) 19.6±0.3 s after vibration onset, associated with a kinesthetic illusion in most subjects. CONCLUSION: The present study invalidates our idea to extend in sitting the VIF paradigm but the unexpected results open a new window about the basic mechanisms underlying muscle vibration effects.