Anette Schrag

Movement Disorder Society-Sponsored Revision of the Unified Parkinson's Disease Rating Scale (MDS-UPDRS): Scale Presentation and Clinimetric Testing Results

We present a clinimetric assessment of the Movement Disorder Society (MDS)‐sponsored revision of the Unified Parkinsons 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 Parkinsons disease from 39 sites. We compared the two scales using correlative techniques and factor analysis. The MDS‐UPDRS showed high internal consistency (Cronbachs alpha = 0.79–0.93 across parts) and correlated with the original UPDRS (ρ = 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.

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

This article presents the revision process, major innovations, and clinimetric testing program for the Movement Disorder Society (MDS)–sponsored revision of the Unified Parkinsons Disease Rating Scale (UPDRS), known as the MDS‐UPDRS. The UPDRS is the most widely used scale for the clinical study of Parkinsons 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.

Depression rating scales in Parkinson's disease: critique and recommendations.

Depression is a common comorbid condition in Parkinsons disease (PD) and a major contributor to poor quality of life and disability. However, depression can be difficult to assess in patients with PD due to overlapping symptoms and difficulties in the assessment of depression in cognitively impaired patients. As several rating scales have been used to assess depression in PD (dPD), the Movement Disorder Society commissioned a task force to assess their clinimetric properties and make clinical recommendations regarding their use. A systematic literature review was conducted to explore the use of depression scales in PD and determine which scales should be selected for this review. The scales reviewed were the Beck Depression Inventory (BDI), Hamilton Depression Scale (Ham‐D), Hospital Anxiety and Depression Scale (HADS), Zung Self‐Rating Depression Scale (SDS), Geriatric Depression Scale (GDS), Montgomery‐Asberg Depression Rating Scale (MADRS), Unified Parkinsons Disease Rating Scale (UPDRS) Part I, Cornell Scale for the Assessment of Depression in Dementia (CSDD), and the Center for Epidemiologic Studies Depression Scale (CES‐D). Seven clinical researchers with clinical and research experience in the assessment of dPD were assigned to review the scales using a structured format. The most appropriate scale is dependent on the clinical or research goal. However, observer‐rated scales are preferred if the study or clinical situation permits. For screening purposes, the HAM‐D, BDI, HADS, MADRS, and GDS are valid in dPD. The CES‐D and CSDD are alternative instruments that need validation in dPD. For measurement of severity of depressive symptoms, the Ham‐D, MADRS, BDI, and SDS scales are recommended. Further studies are needed to validate the CSDD, which could be particularly useful for the assessment of severity of dPD in patients with comorbid dementia. To account for overlapping motor and nonmotor symptoms of depression, adjusted instrument cutoff scores may be needed for dPD, and scales to assess severity of motor symptoms (e.g., UPDRS) should also be included to help adjust for confounding factors. The HADS and the GDS include limited motor symptom assessment and may, therefore, be most useful in rating depression severity across a range of PD severity; however, these scales appear insensitive in severe depression. The complex and time‐consuming task of developing a new scale to measure depression specifically for patients with PD is currently not warranted.

How does Parkinson's Disease affect quality of life? A comparison with quality of life in the general population

Adequate provision of appropriate healthcare resources for patients with chronic neurologic disorders such as Parkinsons disease (PD) requires knowledge of the impact of the illness on their life. Quality of life (QoL) instruments measure the impact of the disease on general well‐being that cannot be fully appreciated by clinical rating scales and allow comparisons with other patient groups and the general population.

Differentiation of atypical parkinsonian syndromes with routine MRI

Objective: To evaluate the use of routine MRI in differentiating between patients with progressive supranuclear palsy (PSP), multiple system atrophy (MSA), corticobasal degeneration (CBD) and control subjects. Methods: Two neuroradiologists rated blindly and independently axial T2-weighted and proton density MR images of 54 patients with MSA, 35 patients with PSP, 5 patients with CBD, and 44 control subjects. Results: More than 70% of patients with PSP and more than 80% of patients with cerebellar predominant MSA could be classified correctly with 0.5-T or 1.5-T scans, and no patient in these groups was misclassified. In the remaining patients an unequivocal differentiation could not be made. However, only approximately 50% of patients with parkinsonism-predominant MSA could be classified correctly, and 19% of them (all of whom had had 0.5-T scans) were misclassified. Conclusions: Characteristic findings on routine MRI, either 1.5 T or 0.5 T, can contribute to the identification of MSA and PSP. However, in a minority of patients no unequivocal diagnosis can be made using MRI findings alone.

What Are the Most Important Nonmotor Symptoms in Patients with Parkinson's Disease and Are We Missing Them?

Nonmotor symptoms (NMS) are increasingly recognized as important and neglected aspects of Parkinsons disease (PD). We evaluated their relative frequency and comparative impact on health‐related quality of life (Hr‐QoL) using validated questionnaires. In addition, we assessed the rate of reporting of NMS in neurology clinics compared with their subjective impact on patients. We used a range of validated clinimetric scales of motor and nonmotor symptoms and Hr‐QoL to assess consecutive patients with PD. Reporting of NMS was assessed by comparison with case note documentation. A mean of 11 of 30 NMS per patient were elicited on the NMS questionnaire of which on average 4.8 were reported in the clinical notes (44%). The most common NMS were autonomic (particularly urinary). The Hr‐QoL scores correlated most strongly with autonomic dysfunction (r = 0.84; particularly urinary and gastrointestinal symptoms), mood (r = 0.74), fatigue (r = 0.74), sleep problems (nocturnal r = 0.55; daytime somnolence r = 0.65), pain (r = 0.56), and psychosis (r = 0.55, all p < 0.0001) followed by UPDRS motor score (r = 0.48, p < 0.0001). Greater motor fluctuations (r = 0.57) and dyskinesia (r = 0.43, both p < 0.0001) were also associated with worse Hr‐QoL. In multivariate analysis, depression had the strongest association with Hr‐QoL (adjusted R2 = 0.53, p = 0.005) followed by fatigue, thermoregulatory, gastrointestinal, and cardiovascular autonomic function (especially orthostatic hypotension), daytime somnolence, and urinary problems. This study demonstrates that a autonomic dysfunction, psychiatric complications, pain, fatigue, and sleep problems are major correlates of poor Hr‐QoL. However, whilst psychiatric problems are increasingly documented, many symptoms (particularly those possibly perceived as embarrassing or unrelated) remain under‐reported.

Meta-analysis of early nonmotor features and risk factors for Parkinson disease.

To evaluate the association between diagnosis of Parkinson disease (PD) and risk factors or early symptoms amenable to population‐based screening.

Neuropsychiatric symptoms in Parkinson's disease†‡

Neuropsychiatric symptoms are common in Parkinsons disease, even at the earliest stages, and have important consequences for quality of life and daily functioning, are associated with increased carer burden and increased risk for nursing home admission. In addition to cognitive impairment, a wide range of neuropsychiatric symptoms have been reported. In this article, the epidemiology, clinical course, diagnosis, and management of some of the most common neuropsychiatric symptoms in PD are discussed: depression, anxiety, apathy, fatigue, and psychotic symptoms. Although much is known regarding the prevalence and course of these symptoms, the empirical evidence for how to manage these symptoms is limited at best. There is thus an urgent need for systematic studies for the pharmacological and non‐pharmacological management of these symptoms.

Quality of life and depression in Parkinson's disease

This paper reviews the literature on health-related quality of life (Hr-QoL) and depressive disorders, and the relationship between them, in patients with Parkinsons disease (PD). PD is associated with reduced Hr-QoL, including motor and non-motor physical consequences of the disease, emotional well-being and social functioning. While this effect is greater in advanced disease stages, there is no close relationship between disease duration and impact on quality of life, and the relationship between clinical rating scales and Hr-QoL scores is only moderate. On the other hand, presence and severity of depression in PD strongly correlates with Hr-QoL scores, and a number of studies have reported depression as the main determinant of poor HR-QoL scores. Despite being the main determinant of poor Hr-QoL and being recognized as an important problem by clinicians, until recently depression in PD has received relatively little attention in research studies. It is known that depression and anxiety occur more frequently in PD than in controls. Depression occurs in a bimodal pattern in PD, with increased rates at the onset and a later peak in advanced disease. Both anxiety and depression can also occur before the first motor symptoms of PD and predate the diagnosis of PD, indicating that these co-morbidities are manifestations of the underlying disease process of PD. Imaging studies have demonstrated abnormalities of dopaminergic, noradrenergic and serotonergic functioning with some correlation with severity of depression. The overall relationship between disease severity and rate of depression (except for off-period related depression) is poor, suggesting that nigrostriatal dysfunction alone is not sufficient to explain depressive symptoms in PD. Other factors are likely to contribute to occurrence and severity of depression in PD, either due to extrastriatal pathology or due to psychological and environmental factors leading to reactive depression. Thus, it is likely that depression in PD is multifactorial. The investigation of depression in PD is complicated by diagnostic difficulties in measuring and diagnosing depression in patients with PD due to the considerable overlap between symptoms of PD and depression. While a number of treatment approaches have been suggested, double-blind randomized controlled trials to demonstrate improvement of depression and overall Hr-QoL in PD are warranted.

Psychogenic movement disorders

Purpose of reviewThis review summarizes the progress made in the area of psychogenic movement disorders (PMDs) over the past 2 years, and a simplified classification of diagnostic certainty is proposed that incorporates electrophysiological assessment. Recent findingsFunctional magnetic resonance imaging studies have demonstrated altered blood flow in conversion disorders that may reflect changes in synaptic activity. Electrophysiological testing shows limitations in distinguishing between psychogenic and organic propriospinal myoclonus and dystonia. Recent evidence cautions against the uncritical acceptance of all cases of posttraumatic myoclonus and ‘jumpy stump’ as being organic in nature. ‘Essential palatal tremor’ is recognized as a rather heterogeneous group of tremors that includes psychogenic tremor. Two recent studies evaluating the long-term prognosis of psychogenic tremor differ in the degree of unfavorable outcome. Different groups of PMDs might have distinctive gait characteristics with prognostic, diagnostic, or therapeutic value. Two recent reviews provide comprehensive information on the understudied area of PMDs in children. SummaryThe diagnosis of PMDs should not be regarded as a diagnosis of exclusion. Careful clinical assessment is critical, and imaging or electrophysiological studies may provide important insights and confirmation of the diagnosis though some cases remain challenging and current assessments fail to provide needed clarification. Treatment is often delayed, contributing to a largely unfavorable long-term outcome. Well designed randomized control trials that validate and compare therapeutic options are urgently required.