Sensor assisted self-management in Parkinson's disease: A feasibility study of ambulatory posture detection and feedback to treat stooped posture.

INTRODUCTION: A stooped posture is one of the characteristic motor symptoms of patients with Parkinson's disease, and has been linked to impairments in daily activities and quality of life. We aimed to test the efficacy, safety, practical utility and user-friendliness of a posture correction and vibrotactile trunk angle feedback device (the UpRight) in the home setting of patients with Parkinson's disease with a stooped posture. It was hypothesized that ambulatory use of the UpRight would be safe, feasible and result in a less stooped posture, i.e. a lower trunk angle during daily activities. METHODS: 15 patients wore the UpRight during a baseline period of 1 week (no feedback), followed by an intervention period of 1 week (feedback). RESULTS: We found a significant decrease (average -5,4°) in trunk angle from baseline period to intervention period without the occurrence of adverse events. In addition, patients found the device usable and beneficial to posture. CONCLUSION: Use of the feedback and correction device has a positive effect on ambulatory trunk angles. The device appears to be both safe and useful for self-management of stooped posture in patients with Parkinson's Disease.

Impact of physical therapy for Parkinson's disease: a critical review of the literature.

A systematic review of the literature found 23 randomized clinical trials reflecting specific core areas of physical therapy (PT), that is, transfer, posture, balance, reaching and grasping, gait, and physical condition. All studies were of moderate methodological quality. Important limitations of the studies were: (1) insufficient statistical power (type II error); (2) poor methodological quality due to inadequate randomization and blinding procedures; (3) insufficient contrast in dosage and treatment between experimental and control groups; and (4) lack of appropriate measurement instruments able to identify clinically meaningful changes according to the International Classification of Functioning (ICF). In the last 5 years, the methodological quality of RCTs has shown substantial improvement. Most high-quality studies investigated the effects of exercise therapy, including the use of external rhythms to improve gait and gait-related activities. The results of these trials suggest that the effects of PT are task- and context-specific. This indicates that the tasks that are trained tend not to generalize to related activities that are not directly trained in the rehabilitation programme itself, and suggests that future programmes should train meaningful tasks preferably in patients' home environment. In addition, the decline in treatment effects after an intervention has ended suggests the need for permanent treatment of patients with PD, i.e. chronic treatment for this chronic disease. Future studies should aim to develop responsive measurement instruments able to monitor meaningful changes in activities, as well as better understanding of insufficiently understood symptoms such as freezing, rigidity and bradykinesia and greater insight into neurophysiological mechanisms underlying training-induced changes in activities such as improved gait performance by rhythmic cueing.

The effect of rhythmic somatosensory cueing on gait in patients with Parkinson's disease.

BACKGROUND AND AIMS: Gait and gait related activities in patients with Parkinson's disease (PD) can be improved with rhythmic auditory cueing (e.g. a metronome). In the context of a large European study, a portable prototype cueing device was developed to provide an alternative for rhythmic auditory cueing: rhythmic somatosensory cueing (RSC, a miniature vibrating cylinder attached to the wrist). We investigated whether PD patients could adapt their walking pattern using RSC under conditions of changing walking speed and the presence of potentially distracting visual flow while walking on a treadmill. METHODS: A total of 17 patients with PD participated (mean age 63.4+/-10.3 years; Hoehn-Yahr score 2.5+/-0.9, mean Unified Parkinson's Disease Rating Scale score 49.8+/-13.7, mean disease duration 7.7+/-5.1 years). They performed systematic walking speed manipulations under 4 conditions in a random order: (1) no cue, no visual flow, (2) no cue, visual flow, (3) cue, no visual flow and (4) cue, visual flow. Visual flow in the form of a virtual corridor that moved at the current walking speed was projected on a 2 x 2 m rear-projection screen. The cueing rhythm was set at -10% of preferred stride frequency at each speed. Stride frequency was assessed using peaks in the trajectories of thigh sagittal plane segmental angles. RESULTS: Walking with RSC resulted in lower stride frequencies, and thus larger step lengths (p-values <0.05), regardless of walking speed. The presence of visual flow did not impair the use of RSC, as evidenced by the lack of differences between conditions 3 and 4 (p>0.05). CONCLUSION: Rhythmic somatosensory cueing may be a viable alternative for auditory cueing and is robust to changes in walking speed and visual distractors.

The effects of visual rhythms and optic flow on stride patterns of patients with Parkinson's disease.

This study was aimed at determining the effects of rhythmic visual cueing under changing visual conditions on stride frequency in patients with Parkinson's disease (PD; n = 21) and healthy age matched controls (n = 7) while walking at different speeds on a treadmill. Stride frequency and stride length in patients with PD as well as controls were not rigidly coupled to walking speed and could be manipulated with walking speed as well as by using spatial and temporal rhythmic visual cues.

Measuring gait and gait-related activities in Parkinson's patients own home environment: a reliability, responsiveness and feasibility study.

The aim of this study was to assess reliability, responsiveness and feasibility of gait and gait related tests in the home of patients with Parkinson's disease (PD). The Unified Parkinson's Disease Rating Scale, a timed walking test, the Timed Get Up and Go test the Berg Balance Scale and the Functional Reach test were applied by three independent observers on 26 PD patients. Moderate to high Intraclass Correlation Coefficients were found, ranging from 0.74 to 0.88 and 0.64 to 0.87 for the intra- and inter-observer reliability, respectively. All test showed Reliable Change Indexes under 11% and the whole test battery was applicable within 30 min.

Distinctive neurological phenotype associated with partial trisomy of chromosome 16.

We present a case of a live born female infant who presented in early life with a movement disorder, lack of developmental progress and neutropenia. Extensive neuro-metabolic investigation was non-diagnostic. Chromosome analysis of cultured lymphocyte cells showed an abnormal chromosome 16 with additional material noted in the proximal long arm. Additional fluorescence in situ hybridisation studies identified this additional material to represent a duplication of the long arm of chromosome 16 between 16q11.2 and 16q21. There was progressive decline and death by 10 months. Dystonia cortical blindness and neutropenia have not been a reported feature of trisomy 16 to date.

Acute disseminated encephalomyelitis after mastoid surgery in a child.

We present a case of acute disseminated encephalomyelitis after mastoid surgery in an 11-year-old child. The aim of this paper is to increase awareness about a previously unreported and an unusual neurological complication of cholesteatoma and mastoid surgery.

Effects of external rhythmical cueing on gait in patients with Parkinson's disease: a systematic review.

OBJECTIVE: To critically review studies evaluating the effects of external rhythmical cueing on gait in patients with Parkinson's disease. METHODS: Articles published from 1966 to January 2005 were searched by two physiotherapists in MEDLINE, PiCarta, PEDRo, Cochrane, DocOnline, CINAHL and SUMSEARCH. To be included, articles had to investigate the effects of external rhythmical cueing (i.e., auditory, visual or tactile cueing) on gait parameters in patients with idiopathic Parkinson's disease. Both controlled and noncontrolled studies were included. Based on the type of design and methodological quality a meta-analysis or best-evidence synthesis was applied. RESULTS: Twenty-four studies (total number of patients = 626) out of the 159 screened studies were evaluated in this systematic review. Two out of 24 were randomized controlled trails (RCT), both of high methodological quality. One RCT did not focus specifically on external rhythmical cueing of individual patients with Parkinson's disease, but on group exercises in general, including walking with cues. All other studies were pre-experimental studies. Best-evidence synthesis showed strong evidence for improving walking speed with the help of auditory cues. Insufficient evidence was found for the effectiveness of visual and somatosensory cueing. CONCLUSION: Only one high-quality study, specifically focused on the effects of auditory rhythmical cueing, suggesting that the walking speed of patients with Parkinson's disease can be positively influenced. However, it is unclear whether positive effects identified in the laboratory can be generalized to improved activities of daily living (ADLs) and reduced frequency of falls in the community. In addition, the sustainability of a cueing training programme remains uncertain.

The effects of physical therapy in Parkinson's disease: a research synthesis.

OBJECTIVE: To present a critical review and meta-analysis of studies evaluating the effects of physical therapy in patients suffering from Parkinson's disease (PD), in terms of neurologic signs, activities of daily living (ADLs), and walking ability. DATA SOURCES: Articles published from 1966 to May 1999 were compiled by means of MEDLINE, Cochrane register of controlled trials, and CINAHL using combinations of the key words Parkinson's disease, exercise, exercise therapy, physical therapy, and group training. References presented in relevant publications were also examined. Articles written in English, German, or Dutch were included. STUDY SELECTION: Studies had to meet the following selection criteria: (1) patients with PD were included in the intervention study, (2) the effects of physical therapy (PT) were evaluated, (3) the study could be classified as true or quasi-experiment, and (4) the study was published in a journal or book. DATA EXTRACTION: Two reviewers assessed independently the methodologic quality of the data of each included study. One reviewer extracted relevant meta-analysis data. DATA SYNTHESIS: For each outcome measure the estimated effect size and the summary effect size (SES) were calculated, using fixed (ie, Hedges's g) and random effects models. The meta-analysis resulted in a significant homogeneous SES with regard to ADLs (.40; confidence interval [CI] = .17-.64) and stride length (.46; CI = .12-.82). The SES with regard to walking speed showed a significant heterogeneous SES, which remained significant after applying a random effects model (.49; CI = .21-.77). The SES with regard to neurologic signs was not significant (.22; CI = -.08 to .52). The small number of studies included and the shortcomings of the methodologic quality of these studies, however, bias the results of the present study. CONCLUSIONS: The results of the present research synthesis support the hypothesis that Parkinson patients benefit from PT added to their standard medication.

Renal function after pediatric cardiac transplantation: the effect of early cyclosporin dosage.

BACKGROUND: There is little data on renal function in pediatric heart transplant recipients. Early rejection is a major concern and most units run high cyclosporin A (CyA) levels during the 2 to 3 months after transplantation. We sought to document long-term renal function after transplantation and to assess influence of early CyA levels. METHODS: We reviewed all of our pediatric transplants between June 1985 and August 1998 who survived longer than 6 months (n = 54). Glomerular filtration rate (GFR) was estimated at 1, 2, 4, and 8 years posttransplantation using the Schwartz formula: GFR (mL/min/1.73m(2)) = [Ht(cm)/creatinine(micromol/L)] x X We also analyzed whether change in renal function correlated with trough CyA levels. RESULTS: Median age at transplant was 4 years and median follow-up was 5 years. Survival rates were 87% at 1 year and 80% at 5 years. Mean GFR pretransplant was 79 +/- 19 mL/min/1.73 m(2), reflecting prerenal impairment. One year later, mean GFR was 72 mL/min/1.73 m(2); after 2 years it was 65 mL/min/1.73 m(2), after 4 years (n = 35) it was 60 mL/min/1.73 m(2), and after 8 years (n = 14) it was 57 mL/min/1.73 m(2). CyA levels during the first 2 months correlated with the change in GFR during the first year (r(2) = 0.21). CONCLUSIONS: This study demonstrates for the first time that decline in renal function after heart transplantation correlates with early CyA exposure; this dysfunction persists even when CyA doses are subsequently reduced.

Shoulder function and patient well-being after various types of neck dissections.

Preserving the accessory nerve results in a better outcome of the shoulder function after neck dissection. However, little is known about the impact of preserving a cervical contribution to the accessory nerve. This study describes the shoulder function after different types of neck dissections, with the emphasis on the significance of the cervical contribution to the accessory nerve. Fifty-nine patients who underwent neck dissections of various types were included. Thirty-eight patients underwent unilateral radical or modified radical neck dissections, and 21 patients underwent bilateral neck dissections. All the patients were assessed subjectively and objectively, using a questionnaire and an inclinometer. Radical neck dissections inflicted significantly more morbidity than modified radical neck dissections. Preserving a cervical contribution to the accessory nerve did not decrease pain complaints or functional impairment. However, there might be some improvement in range of motion, especially exorotation and anteflexion. Preserving the accessory nerve has a positive influence on shoulder function and complaints. Preserving a cervical contribution does not decrease morbidity significantly.

Pyruvate dehydrogenase deficiency presenting as dystonia in childhood.

Two individuals with pyruvate dehydrogenase (PDH) deficiency due to missense mutations in the gene for the E1alpha subunit (PDHA1) presented during childhood with dystonia. The first patient, a male, presented at age 4 years with dystonia affecting the lower limbs, which responded to treatment with combined carbidopa and levodopa. The second patient, a female, was first investigated at age 6 years because of a dystonic gait disorder. In both patients, the main clue to the biochemical diagnosis was a raised concentration of lactate in the cerebrospinal fluid. PDH activity was significantly reduced in cultured fibroblasts in both cases. Dystonia is a previously unrecognized major manifestation of PDH deficiency and is of particular interest as the mutations in the PDHA1 gene in these patients have both been identified previously in individuals with typical presentations of the condition.

Incidence of shoulder pain after neck dissection: a clinical explorative study for risk factors.

BACKGROUND: It is the purpose of this study to determine the incidence of shoulder pain and restricted range of motion of the shoulder after neck dissection, and to identify risk factors for the development of shoulder pain and restricted range of motion. METHODS: Clinical patients who underwent a neck dissection completed a questionnaire assessing shoulder pain. The intensity of pain was assessed using a visual analog scale (100 mm). Range of motion of the shoulder was measured. Information about reconstructive surgery and side and type of neck dissection was retrieved from the medical records. RESULTS: Of the patients (n = 177, mean age 60.3 years [SD, 11.9]) 70% experienced pain in the shoulder. Forward flexion and abduction of the operated side was severely reduced compared to the non-operated side, 21 degrees and 47 degrees, respectively. Non-selective neck dissection was a risk factor for the development of shoulder pain (9.6 mm) and a restricted shoulder abduction (55 degrees ). Reconstruction was risk factor for a restricted forward flexion of the shoulder (24.5 degrees ). CONCLUSIONS: Shoulder pain after neck dissection is clinically present in 70% of the patients. Non-selective neck dissection is a risk factor for shoulder pain and a restricted abduction. Reconstruction is a risk factor for a restricted forward flexion of the shoulder.