Neurological gait assessment.

Gait disorders are a common feature of neurological disease. The gait examination is an essential part of the neurological clinical assessment, providing valuable clues to a myriad of causes. Understanding how to examine gait is not only essential for neurological diagnosis but also for treatment and prognosis. Here, we review aspects of the clinical history and examination of neurological gait to help guide gait disorder assessment. We focus particularly on how to differentiate between common gait abnormalities and highlight the characteristic features of the more prevalent neurological gait patterns such as ataxia, waddling, steppage, spastic gait, Parkinson's disease and functional gait disorders. We also offer diagnostic clues for some unusual gait presentations, such as dystonic, stiff-person and choreiform gait, along with red flags that help differentiate atypical parkinsonism from Parkinson's disease.

Application of the Scale for the Assessment and Rating of Ataxia (SARA) in pediatric oncology patients: A multicenter study.

Posterior cranial fossa (PCF) tumors in childhood are often associated with ataxia as well as other motor, neurobehavioral and linguistic impairment. The use of a reliable outcome measure is mandatory to evaluate the severity of impairment and monitor rehabilitation effectiveness. The aim of this work is to explore the validity of the Scale for the Assessment and Rating of Ataxia (SARA) in pediatric subjects with ataxia secondary to PCF tumor resection and evaluate the influence of age and comorbidities. Seventy eight patients (3-18 years) were recruited in 5 centers from 2016 to 2018. The age effect on SARA was analyzed by correlating total SARA scores and item scores with age and gradually excluding youngest subjects. The comorbidity effect was evaluated by comparing the ataxia-only group vs a group of subjects with ataxia + dysfunction of cranial nerves or cerebellar mutism (CM) and a group of patients with ataxia + hemiparesis. Several negative correlations between SARA scores and age were found under age 9. Differences between ataxia-only group and the other two groups were closely associated with specific comorbidities (e.g. speech disturbance in cranial nerves or CM group (p value < 0.001) and gait, stance, sitting and finger chase in the hemiparetic group (mean p value 0.022)).

Home Biofeedback for the Treatment of Dyssynergic Defecation: Does It Improve Quality of Life and Is It Cost-Effective?

OBJECTIVES: Biofeedback therapy, whether administered at home or in office settings, is effective for dyssynergic defecation (DD). Whether home biofeedback improves quality of life (QOL) and is cost-effective when compared with office biofeedback is unknown. METHODS: QOL was assessed in 8 domains (SF-36) at baseline and after treatment (3 months), alongside economic evaluation during a randomized controlled trial (RCT) comparing home and office biofeedback in patients with DD (Rome III). Costs related to both biofeedback programs were estimated from the hospital financial records, study questionnaires, and electronic medical records. A conversion algorithm (Brazier) was used to calculate the patient's quality-adjusted life years (QALYs) from SF-36 responses. Cost-effectiveness was expressed as incremental costs per QALY between the treatment arms. RESULTS: One hundred patients (96 female patients, 50 in each treatment arm) with DD participated. Six of the 8 QOL domains improved (P < 0.05) in office biofeedback, whereas 4 of the 8 domains improved (P < 0.05) in home biofeedback; home biofeedback was noninferior to office biofeedback. The median cost per patient was significantly lower (P < 0.01) for home biofeedback ($1,112.39; interquartile range (IQR), $826-$1,430) than for office biofeedback ($1,943; IQR, $1,622-$2,369), resulting in a cost difference of $830.11 The median QALY gained during the trial was 0.03 for office biofeedback and 0.07 for home biofeedback (P = NS). The incremental cost-effectiveness ratio was $20,752.75 in favor of home biofeedback. DISCUSSION: Biofeedback therapy significantly improves QOL in patients with DD regardless of home or office setting. Home biofeedback is a cost-effective treatment option for DD compared with office biofeedback, and it offers the potential of treating many more patients in the community.

Evaluation of the use of the Scale for the Assessment and Rating of Ataxia (SARA) in healthy volunteers and patients with schizophrenia.

OBJECTIVE: The Scale for the Assessment and Rating of Ataxia (SARA) is a semi-quantitative assessment used to evaluate ataxia. The goal of these studies was to assess and evaluate the utility of this instrument in a Healthy Volunteer (HV) group and subjects with Schizophrenia (SCZ). METHODS: Two studies were completed to collect SARA data, in a HV group and in a stable SCZ group. 177 HVs (18-65 years) and 16 SCZs (18-58 years) provided written consent and were assessed using the SARA. Of 177 HV subjects, 88 had 2 SARA assessments (within 2 days of initial visit) while all 16 SCZ had 3 SARA assessments (within 14 days of initial visit). RESULTS: For the HV group, the mean score ±â€¯Std for the SARA on visit-1 was 0.39 ±â€¯0.72, and 0.34 ±â€¯0.64 for visit-2. The Pearson correlation coefficient between visit-1 and visit-2 was 0.7486 and an ICC of 0.743. For the SCZ group, the mean score for the SARA was 0.63 ±â€¯0.65 on visit-1, 0.84 ±â€¯1.19 on visit-2, and 0.84 ±â€¯0.94 on visit-3. The Pearson correlation coefficient between visit-1 and visit-2 was 0.6545, between visit-1 and visit-3 was 0.6635 and between visit-2 and visit-3 was 0.7613 and an ICC of 0.650. There was no significant difference in baseline SARA scores between the HV and SCZ group p = .063. A statistically significant positive association between age and total SARA scores was observed in HV (r = 0.345) and SCZ (r = 0.676). CONCLUSIONS: A strong association was observed in both the HV and SCZ groups in the reassessment of signs of ataxia using the SARA scale. Both groups demonstrated minimal signs of ataxia, with no statistically significant difference between the two groups in their visit-1 scores.

Clinical assessment of dysphagia in neurodegeneration (CADN): development, validity and reliability of a bedside tool for dysphagia assessment.

Screening assessments for dysphagia are essential in neurodegenerative disease. Yet there are no purpose-built tools to quantify swallowing deficits at bedside or in clinical trials. A quantifiable, brief, easy to administer assessment that measures the impact of dysphagia and predicts the presence or absence of aspiration is needed. The Clinical Assessment of Dysphagia in Neurodegeneration (CADN) was designed by a multidisciplinary team (neurology, neuropsychology, speech pathology) validated against strict methodological criteria in two neurodegenerative diseases, Parkinson's disease (PD) and degenerative ataxia (DA). CADN comprises two parts, an anamnesis (part one) and consumption (part two). Two-thirds of patients were assessed using reference tests, the SWAL-QOL symptoms subscale (part one) and videofluoroscopic assessment of swallowing (part two). CADN has 11 items and can be administered and scored in an average of 7 min. Test-retest reliability was established using correlation and Bland-Altman plots. 125 patients with a neurodegenerative disease were recruited; 60 PD and 65 DA. Validity was established using ROC graphs and correlations. CADN has sensitivity of 79 and 84% and specificity 71 and 69% for parts one and two, respectively. Significant correlations with disease severity were also observed (p < 0.001) for PD with small to large associations between disease severity and CADN scores for DA. Cutoff scores were identified that signal the presence of clinically meaningful dysphagia symptomatology and risk of aspiration. The CADN is a reliable, valid, brief, quantifiable, and easily deployed assessment of swallowing in neurodegenerative disease. It is thus ideally suited for both clinical bedside assessment and future multicentre clinical trials in neurodegenerative disease.

Assessment of speech in early-onset ataxia: a pilot study.

AIM: The aim of the study was to determine whether paediatric ataxia speech subscores are reliably applicable for international early-onset ataxia (EOA) databases. If so, we reasoned that ataxia speech subscores should be associated with ataxia scores and involve high interobserver agreement, including those for internationally applicable Scale for Assessment and Rating of Ataxia (SARA) syllable repetition tasks (SARASRT). METHOD: Three independent paediatric neurologists and a speech therapist scored speech in 52 healthy children (mean age 10y, range 4-16y) and 40 individuals with EOA (mean age 15y, range 5-34y). We compared ataxia speech subscores for the association with age and ataxia scores as well as interobserver reliability. RESULTS: In healthy children, ataxia speech subscores were moderately associated with age (International Cooperative Ataxia Rating Scale [ICARS]: r=-0.515; SARA: r=-0.321; p<0.05) and with ataxia scores (ICARS: r=0.552; SARA: r=0.336; p<0.05), and revealed slight to moderate interobserver agreement (ICARS-intraclass correlation coefficient [ICC]: 0.380; SARA-ICC: 0.185; SARASRT-ICC: 0.509). In EOA, speech subscores have a strong association with ataxia scores (ICARS: r=0.735; SARA: r=0.730; p<0.001) and revealed substantial to nearly perfect interobserver agreement (ICARS-ICC: 0.812; SARA-ICC: 0.854; SARASRT-ICC: 0.724). INTERPRETATION: Early-onset ataxia speech subscores are associated with ataxia and also reveal high interobserver agreement, including those internationally applicable to SARASRT. We conclude that SARASRT appears to be applicable for EOA databases. However, before syllable repetition tasks are included, we would advise to wait for the results published by the international Childhood Ataxia and Cerebellar Group.

Quantitative assessment of interutterance stability: application to dysarthria.

PURPOSE: Following recent attempts to quantify articulatory impairment in speech, the present study evaluates the usefulness of a novel measure of motor stability to characterize dysarthria. METHOD: The study included 8 speakers with ataxic dysarthria (AD), 16 speakers with hypokinetic dysarthria (HD) as a result of Parkinson's disease, and 24 unimpaired control participants. Each participant performed a series of sentence repetitions under habitual, fast, and slow speaking rate conditions. An algorithm to measure utterance-to-utterance spectro-temporal variation (UUV; Cummins, 2009) was used. Speech rate and intelligibility were also measured. RESULTS: UUV scores were significantly correlated with perceptually based intelligibility scores. There were significant differences in UUV between control speakers and the AD but not the HD groups, presumably because of differences in intelligibility in the samples used and not because of differences in pathology. Habitual speaking rate did not correlate with UUV scores. All speaker groups had greater UUV levels in the slow conditions compared with habitual and fast speaking rates. CONCLUSIONS: UUV results were consistent with those of other variability indices and thus appear to capture motor control issues in a similar way. The results suggest that the UUV could be developed into an easy-to-use clinical tool that could function as a valid and reliable assessment and outcome measure.

Early recognition of pelvic floor dyssynergia and colorectal assessment in Parkinson's disease associated with bowel dysfunction.

AIM: Slow colonic transit time (CTT) and pelvic floor dyssynergia (PFD) are major contributors to constipation in patients with Parkinson's disease (PD). However, no symptom survey yet exists that effectively differentiates the contributing aetiologies. The significance of individual pelvic floor musculature behaviours and their relationship with colorectal dysmotility in constipated patients with PD are still controversial and need further clarification. We aimed to investigate how differentiated constipation-related symptoms of PD patients with constipation may identify constipation groupings and to register the pathophysiological features of the pelvic musculature. METHOD: Our subjects undertook CTT, defaecography and the Knowles-Eccersley-Scott Symptom questionnaire. The pathological aetiologies were categorized as group 1 (slow CTT) and/or group 2 (puborectalis syndrome) and/or group 3 (pubococcygeus syndrome), in accordance with the CTT and defaecography results. RESULTS: Constipation-related symptoms such as incomplete evacuation and defaecation difficulty yielded high post-test probabilities (81% and 88%, respectively) in groups 3 and 2, but a low post-test probability in group 1 (58%). Changes in the anorectal angle and perineum descent during straining were significantly correlated with CTT (r = 0.57 and r = 0.61, respectively) and with each other (r = 0.82). CONCLUSION: Our findings that neural control of the puborectalis and pubococcygeus, along with colorectal peristalsis, were in a similar state of degeneration is key information that should assist physicians to instigate more effective management for colonic dysmotility or PFD.

Assessment of patient and caregiver needs in fragile X-associated tremor/ataxia syndrome by utilizing Q-sort methodology.

OBJECTIVES: Psychosocial stressors faced by patients with fragile X-associated tremor/ataxia syndrome (FXTAS) and their caregivers have not been systematically explored. FXTAS is a neurodegenerative disease occurring in approximately 45% of elderly male carriers and 8-16% of female carriers of the fragile X mental retardation one premutation. This study investigated the subjective needs of patients with FXTAS and their family caregivers, by utilizing Q-sort methodology. METHOD: Patients with FXTAS and their caregivers seen during January 2005 to June 2007 participated. The Q-sort was composed of 17 (eight formal and nine informal) items, designed to explore emotional, informational, and instrumental needs of patients with FXTAS and their caregivers. Item scores were generated from 1 = least important to 7 = most important. Analysis included descriptive statistics for all the demographic and outcome variables. Generalized estimating equations were used to identify which of the need domains were perceived as most important by the participants. RESULTS: A total of 24 patients (79% men, mean age 65.6 ± 6.4 years) with FXTAS and 18 caregivers (11% men, mean age 63.6 ± 6.2 years) completed the Q-sort. Both patients and caregivers rated informational needs as most important, followed by emotional and, finally, by instrumental needs. Participants lacked many important resources, in particular those addressing instrumental needs. CONCLUSION: Providers should be educated and able to provide timely information and referrals to formal services, as well as to informal resources, including the National Fragile X Foundation online support network (www.fragilex.org).

Advances in diagnostic assessment of fecal incontinence and dyssynergic defecation.

Disorders of the anorectum and pelvic floor affect approximately 25% of the population. Their evaluation and treatment have been hindered by a lack of understanding of underlying mechanism(s) and a working knowledge of the diagnostic advances in this field. A meticulous evaluation of anorectal structure and its function can provide invaluable insights to the practicing gastroenterologist regarding the pathogenic mechanism(s) of these disorders. Also, significant new knowledge has emerged over the past decade that includes the development of newer diagnostic tools such as high-resolution manometry and magnetic resonance defecography as well as a better delineation of the clinical and pathophysiologic subtypes of constipation and incontinence. This article provides an up-to-date review on the role of diagnostic tests in the evaluation of fecal incontinence and constipation with dyssynergic defecation.