We present a clinimetric assessment of the Movement Disorder Society (MDS)-sponsored revision of the Unified Parkinson's Disease Rating Scale (MDS-UPDRS). The MDS-UDPRS Task Force revised and expanded the UPDRS using recommendations from a published critique. The MDS-UPDRS has four parts, namely, I: Non-motor Experiences of Daily Living; II: Motor Experiences of Daily Living; III: Motor Examination; IV: Motor Complications. Twenty questions are completed by the patient/caregiver. Item-specific instructions and an appendix of complementary additional scales are provided. Movement disorder specialists and study coordinators administered the UPDRS (55 items) and MDS-UPDRS (65 items) to 877 English speaking (78% non-Latino Caucasian) patients with Parkinson's disease from 39 sites. We compared the two scales using correlative techniques and factor analysis. The MDS-UPDRS showed high internal consistency (Cronbach's alpha = 0.79-0.93 across parts) and correlated with the original UPDRS (rho = 0.96). MDS-UPDRS across-part correlations ranged from 0.22 to 0.66. Reliable factor structures for each part were obtained (comparative fit index > 0.90 for each part), which support the use of sum scores for each part in preference to a total score of all parts. The combined clinimetric results of this study support the validity of the MDS-UPDRS for rating PD.

/pdf/movement-disorder-society-sponsored-revision-of-the-unified-3shjr1g3wc.pdf

Movement Disorder Society-sponsored revision of the Unified Parkinson's Disease Rating Scale (MDS-UPDRS): scale presentation and clinimetric testing results.

Objective: Parkinson’s disease (PD) is a progressive neurological disorder characterised by a large number of motor and non-motor features that can impact on function to a variable degree. This review describes the clinical characteristics of PD with emphasis on those features that differentiate the disease from other parkinsonian disorders. Methods: A MedLine search was performed to identify studies that assess the clinical characteristics of PD. Search terms included “Parkinson’s disease”, “diagnosis” and “signs and symptoms”. Results: Because there is no definitive test for the diagnosis of PD, the disease must be diagnosed based on clinical criteria. Rest tremor, bradykinesia, rigidity and loss of postural reflexes are generally considered the cardinal signs of PD. The presence and specific presentation of these features are used to differentiate PD from related parkinsonian disorders. Other clinical features include secondary motor symptoms (eg, hypomimia, dysarthria, dysphagia, sialorrhoea, micrographia, shuffling gait, festination, freezing, dystonia, glabellar reflexes), non-motor symptoms (eg, autonomic dysfunction, cognitive/neurobehavioral abnormalities, sleep disorders and sensory abnormalities such as anosmia, paresthesias and pain). Absence of rest tremor, early occurrence of gait difficulty, postural instability, dementia, hallucinations, and the presence of dysautonomia, ophthalmoparesis, ataxia and other atypical features, coupled with poor or no response to levodopa, suggest diagnoses other than PD. Conclusions: A thorough understanding of the broad spectrum of clinical manifestations of PD is essential to the proper diagnosis of the disease. Genetic mutations or variants, neuroimaging abnormalities and other tests are potential biomarkers that may improve diagnosis and allow the identification of persons at risk.

https://jnnp.bmj.com/content/jnnp/79/4/368.full.pdf

Parkinson’s disease: clinical features and diagnosis

The clinical diagnosis of Parkinson's disease rests on the identification of the characteristics related to dopamine deficiency that are a consequence of degeneration of the substantia nigra pars compacta. However, non-dopaminergic and non-motor symptoms are sometimes present before diagnosis and almost inevitably emerge with disease progression. Indeed, non-motor symptoms dominate the clinical picture of advanced Parkinson's disease and contribute to severe disability, impaired quality of life, and shortened life expectancy. By contrast with the dopaminergic symptoms of the disease, for which treatment is available, non-motor symptoms are often poorly recognised and inadequately treated. However, attention is now being focused on the recognition and quantitation of non-motor symptoms, which will form the basis of improved treatments. Some non-motor symptoms, including depression, constipation, pain, genitourinary problems, and sleep disorders, can be improved with available treatments. Other non-motor symptoms can be more refractory and need the introduction of novel non-dopaminergic drugs. Inevitably, the development of treatments that can slow or prevent the progression of Parkinson's disease and its multicentric neurodegeneration provides the best hope of curing non-motor symptoms.

https://www.thelancet.com/pdfs/journals/laneur/PIIS1474-4422(06)70373-8.pdf

Non-motor symptoms of Parkinson's disease: diagnosis and management

The Movement Disorder Society Task Force for Rating Scales for Parkinson's disease (PD) prepared a critique of the Hoehn and Yahr scale (HY). Strengths of the HY scale include its wide utilization and acceptance. Progressively higher stages correlate with neuroimaging studies of dopaminergic loss, and high correlations exist between the HY scale and some standardized scales of motor impairment, disability, and quality of life. Weaknesses include the scale's mixing of impairment and disability and its non-linearity. Because the HY scale is weighted heavily toward postural instability as the primary index of disease severity, it does not capture completely impairments or disability from other motor features of PD and gives no information on nonmotor problems. Direct clinimetric testing of the HY scale has been very limited, but the scale fulfills at least some criteria for reliability and validity, especially for the midranges of the scale (Stages 2-4). Although a "modified HY scale" that includes 0.5 increments has been adopted widely, no clinimetric data are available on this adaptation. The Task Force recommends that: (1) the HY scale be used in its original form for demographic presentation of patient groups; (2) when the HY scale is used for group description, medians and ranges should be reported and analysis of changes should use nonparametric methods; (3) in research settings, the HY scale is useful primarily for defining inclusion/exclusion criteria; (4) to retain simplicity, clinicians should "rate what you see" and therefore incorporate comorbidities when assigning a HY stage; and (5) because of the wide usage of the modified HY scale with 0.5 increments, this adaptation warrants clinimetric testing. Without such testing, however, the original five-point scales should be maintained.

/pdf/movement-disorder-society-task-force-report-on-the-hoehn-and-58mf2wq2ef.pdf

Movement Disorder Society Task Force report on the Hoehn and Yahr staging scale: status and recommendations.

This article presents the revision process, major innovations, and clinimetric testing program for the Movement Disorder Society (MDS)-sponsored revision of the Unified Parkinson's Disease Rating Scale (UPDRS), known as the MDS-UPDRS. The UPDRS is the most widely used scale for the clinical study of Parkinson's disease (PD). The MDS previously organized a critique of the UPDRS, which cited many strengths, but recommended revision of the scale to accommodate new advances and to resolve problematic areas. An MDS-UPDRS committee prepared the revision using the recommendations of the published critique of the scale. Subcommittees developed new material that was reviewed by the entire committee. A 1-day face-to-face committee meeting was organized to resolve areas of debate and to arrive at a working draft ready for clinimetric testing. The MDS-UPDRS retains the UPDRS structure of four parts with a total summed score, but the parts have been modified to provide a section that integrates nonmotor elements of PD: I, Nonmotor Experiences of Daily Living; II, Motor Experiences of Daily Living; III, Motor Examination; and IV, Motor Complications. All items have five response options with uniform anchors of 0 = normal, 1 = slight, 2 = mild, 3 = moderate, and 4 = severe. Several questions in Part I and all of Part II are written as a patient/caregiver questionnaire, so that the total rater time should remain approximately 30 minutes. Detailed instructions for testing and data acquisition accompany the MDS-UPDRS in order to increase uniform usage. Multiple language editions are planned. A three-part clinimetric program will provide testing of reliability, validity, and responsiveness to interventions. Although the MDS-UPDRS will not be published until it has successfully passed clinimetric testing, explanation of the process, key changes, and clinimetric programs allow clinicians and researchers to understand and participate in the revision process.

/pdf/movement-disorder-society-sponsored-revision-of-the-unified-25kj25ssf9.pdf

Movement Disorder Society-sponsored revision of the Unified Parkinson's Disease Rating Scale (MDS-UPDRS): Process, format, and clinimetric testing plan.

We assessed the clinometric characteristics of rating scales used for the evaluation of motor impairment and disability of patients with Parkinson's disease (PD), conducting a systematic review of PD rating scales published from 1960 to the present. Thirty studies describing clinometrics of 11 rating scales used for PD were identified. Outcome measures included validity (including factor structure), reliability (internal consistency, inter-rater, and intrarater) and responsiveness. We traced three impairment scales (Webster, Columbia University Rating Scale [CURS] and Parkinson's Disease Impairment Scale), four disability scales (Schwab and England, Northwestern University Disability Scale [NUDS], Intermediate Scale for Assessment of PD, and Extensive Disability Scale), and four scales evaluating both impairment and disability (New York University, University of California Los Angeles, Unified Parkinson's Disease Rating Scale [UPDRS], and Short Parkinson Evaluation Scale). The scales showed large differences in the extent of representation of items related to signs considered responsive to dopaminergic treatment or to those signs that appear late in the disease course and lack responsiveness to treatment. Regardless of the scale, there was a conspicuous lack of consistency concerning inter-rater reliability of bradykinesia, tremor, and rigidity. Overall disability items displayed moderate to good inter-rater reliability. The available evidence shows that CURS, NUDS, and UPDRS have moderate to good reliability and validity. In contrast to their widespread clinical use for assessment of impairment and disability in PD, the majority of the rating scales have either not been subjected to an extensive clinometric evaluation or have demonstrated clinometric shortcomings. The CURS, NUDS, and UPDRS are the most evaluated, valid, and reliable scales currently available.

Systematic evaluation of rating scales for impairment and disability in Parkinson's disease.

An association between HLA-DR13 and patients with complex regional pain syndrome (CRPS) who progressed towards multifocal or generalized tonic dystonia was recently reported. We now report on a new locus, centromeric in HLA-class I, which was significantly associated with a spontaneous development of CRPS, suggesting an interaction between trauma severity and genetic factors conferring CRPS susceptibility. Additionally, an association with the D6S1014 locus was found, supporting the previous finding of an association with HLA-DR13.

Susceptibility loci for complex regional pain syndrome

To examine the quality and quantity of physiotherapy care in Parkinson's disease (PD) in the Netherlands, postal questionnaires were sent to 300 PD patients. Patients who had received treatment by a physiotherapist at some stage during the previous year were asked to give approval to contact their therapist. Subsequently, postal questionnaires were sent to 129 physiotherapists who had recently treated the patients that were identified in the first stage of the study. Completed questionnaires were returned by 235 patients and 99 therapists. Sixty percent of patients were currently treated by a physiotherapist, most of them for more than six months. Ninety-five patients had serious problems within at least one of the four core areas of physiotherapy practice in PD (posture, balance, gait, transfers) and were therefore regarded as requiring referral to physiotherapy. Yet, 41% of them received no treatment at the time of enquiry. On the other hand, 75 patients had no serious problems within these core areas, yet half of them currently received treatment. The main goals for treatment were improvement of gait, general physical condition, posture or balance. Treatment mainly comprised active exercises. 60% of patients were treated by therapists without specific interest or education in PD. Most patients were presently satisfied with the treatment, but 11% had previously changed to another therapist because of dissatisfaction. In the Netherlands, referral rates of PD patients to physiotherapy are high and treatment duration is lengthy. Referral seems, to some extent, arbitrary. Scientific evidence for the effectiveness of physiotherapy in PD remains limited.

Physiotherapy in Parkinson's disease: utilisation and patient satisfaction.

Dyskinesias are most prevalent in patients with Huntington's disease (HD), patients with Parkinson's disease (PD) who have received chronic levodopa therapy, and in patients who have been treated with neuroleptics (tardive dyskinesia ITD]). Recent therapeutic developments have fueled a growing interest in the clinimetrics of dyskinesias. For dyskinesias in HD, few rating scales are available, but data on validity, reliability, and responsiveness are scarce. Only the interrater reliability of facial dyskinesias has been evaluated and found to be low. Many subjective rating scales for dyskinesias in PD exist, but only the Dyskinesia Rating Scale has undergone sufficient clinimetric evaluation. For TD, numerous rating scales are available, many of them with ample data on reliability and validity. Objective assessment of dyskinesias has been attempted with a number of techniques. All these methods require a laboratory setting, rendering them susceptible to influence of stress. Moreover, they provide only a momentary assessment of dyskinesia severity and fail to take into account diurnal fluctuations. In view of the methodologic shortcomings in the assessment of dyskinesias, more effort needs to be put into strengthening currently available modes of assessment or designing new ones. In the future ambulatory accelerometry might prove to be of value in this field.

A review of the assessment of dyskinesias.

Objectives. To explore the feasibility of intravenous magnesium administration as a potential candidate intervention for a large size trial in Complex Regional Pain Syndrome Type 1 (CRPS 1).

Design. Randomized clinical trial.

Setting. Outpatient pain clinic.

Patients. Ten CRPS 1 patients.

Interventions. Eight patients received 70 mg/kg magnesium sulphate infusions in 4 hours for 5 days. For blinding purposes, 2 patients received equal amount NaCl 0.9% solutions (data not analyzed or presented). Interventions were accompanied by standardized physical therapy.

Outcome Measures. Pain was assessed using an 11-point Box scale (three times daily for a week) and the McGill Pain Questionnaire. Skin sensitivity was measured with the Semmes Weinstein Monofilaments, (other) impairments with the Impairment Level Sumscore. In addition, functional limitations (Radboud Skills Questionnaire, questionnaire rising and sitting down) and quality of life (Short Form-36 [SF-36], EuroQol) were evaluated. Assessments were performed at baseline, 1, 3, 6, and 12 weeks after intervention.

Results. Mild systemic side effects were experienced and the infusions were locally well tolerated. Pain was significantly reduced at all follow up compared with baseline (T1: P = 0.01, T3: P = 0.04, T6: P = 0.02, T12: P = 0.02). McGill sensory subscale improved significantly at T1 (number of words chosen: P = 0.03 and pain rating index: P = 0.03). Impairment level ( P = 0.03) and quality of life (EuroQol P = 0.04, SF-36 physical P = 0.01) were significantly improved at T12. No improvement was found for skin sensitivity and functional limitations.

Conclusion. Intravenous magnesium significantly improved pain, impairment and quality of life and was well tolerated. The results of this pilot study are encouraging and suggest that magnesium IV as a treatment in CRPS 1 should be further explored in a large size formal trial design.

http://www.rsdcanada.org/parc/english/resources/CollinsIVmagnesium2011.pdf

Bob J. van Hilten

Papers

Systematic evaluation of rating scales for impairment and disability in Parkinson's disease.

Susceptibility loci for complex regional pain syndrome

Physiotherapy in Parkinson's disease: utilisation and patient satisfaction.

A review of the assessment of dyskinesias.

Intravenous magnesium for complex regional pain syndrome type 1 (CRPS 1) patients: a pilot study.