Milan Grofik

Relationship between the non-motor items of the MDS-UPDRS and Quality of Life in patients with Parkinson's disease

The Movement Disorder Society-Unified Parkinsons Disease Rating Scale (MDS-UPDRS) is a newly developed comprehensive tool to assess Parkinsons disease (PD), which covers a wider range of non-motor PD manifestations than the original UPDRS scale. The aim of this study was to assess the relationship between the MDS-UPDRS and Quality of Life (QoL) and to analyze the relationship between individual MDS-UPDRS non-motor items and QoL. A total of 291 PD patients were examined in a multicenter Slovak study. Patients were assessed by the MDS-UPDRS, HY scale and PDQ39. Data were analyzed using the multiple regression analyses. The mean participant age was 68.0 ± 9.0 years, 53.5% were men, mean disease duration was 8.3 ± 5.3 years and mean HY was 2.7 ± 1.0. In a multiple regression analysis model the PDQ39 summary index was related to MDS-UPDRS parts II, I and IV respectively, but not to part III. Individual MDS-UPDRS non-motor items related to the PDQ39 summary index in the summary group and in the non-fluctuating patients subgroup were pain, fatigue and features of dopamine dysregulation syndrome (DDS). In the fluctuating PD patient subgroup, PDQ39 was related to pain and Depressed mood items. Other MDS-UPDRS non-motor items e.g. Anxious mood, Apathy, Cognitive impairment, Hallucinations and psychosis, Sleep problems, Daytime sleepiness and Urinary problems were related to some PDQ39 domains. The overall burden of NMS in PD is more important in terms of QoL than motor symptoms. Individual MDS-UPDRS non-motor items related to worse QoL are especially pain and other sensations, fatigue and features of DDS.

Unique case of eleven Bell's palsy episodes

Bells palsy (BP) is a peripheral facial nerve paralysis of unknown etiology. It is not a life-threatening condition; however, incomplete recovery may leave an individual stigmatized functionally, occupationally as well as socially. Recurrent paralyses are seldom, noted in 7–8% of all BP cases. More than two BP relapses are even less frequent. Adour et al. (1977) reported only two patients with four BP episodes from 1700 patients. Only one patient with more than four BP recurrences in the group containing 2414 BP cases was reported by Yanagihara et al. (1984). The highest reported number of BP recurrences in the accessible literature has been nine. We are presenting an unusual patient who suffered a total of eleven relapses of an idiopathic facial nerve palsy. Description of the case along with review of the relevant literature are discussed.

Differences in MDS‐UPDRS Scores Based on Hoehn and Yahr Stage and Disease Duration

The Movement Disorder Society Unified Parkinsons Disease Rating Scale (MDS‐UPDRS) is a newly developed tool to assess Parkinsons disease (PD). Changes in scores on the scale over the course of PD, including increasing disease duration and Hoehn and Yahr (HY) stages, have not been described. The objectives of this study were to analyze MDS‐UPDRS scores on Parts I through IV and their differences based on HY stage and disease duration in a large cohort of patients with PD.

Relationship between the MDS-UPDRS and Quality of Life: A large multicenter study of 3206 patients

BACKGROUND The relationship between Health-Related Quality of Life (HRQoL) and MDS-UPDRS has not been fully studied so far. The aim of this study was to evaluate the relationship between all MDS-UPDRS components and HRQoL in a representative international cohort of PD patients. METHODS We collected demographic and disease-related data as well as MDS-UPDRS and PDQ8 scales. Data were analyzed using correlations between PDQ8 and all MDS-UPDRS items, subsequently two hierarchical multiple regressions were performed, first between the scores of the MDS-UPDRS Parts and PDQ8 and second between individual items from those Parts demonstrating significant relationship to PDQ8 scores in the first regression. LASSO regression analyses were performed to evaluate the relationship between PDQ8 and all individual MDS-UPDRS items. RESULTS A total of 3206 PD patients were included in the study. In the first regression analysis, PDQ8 was significantly related to MDS-UPDRS parts I and II, but not to III and IV. In the second regression model, significant contributions to PDQ8 were found for Part I items Fatigue, Pain, Depressed mood, Apathy; and Part II items Dressing, Doing hobbies, Freezing, Speech and Tremor. In the LASSO analysis, six Part I, seven Part II, three Part III and one Part IV items contributed to PDQ8 scores. The five items most significantly related to the model were Depressed mood, Dressing, Apathy, Pain and Fatigue. CONCLUSIONS This is so far the largest study related to HRQoL issues in PD. Restrictions in activities of daily living and non-motor symptoms significantly contribute to HRQoL in PD.

Validation of the Official Slovak Version of the Unified Dyskinesia Rating Scale (UDysRS).

After successful clinimetric testing of the Unified Dyskinesia Rating Scale (UDysRS), a program for translation and validation of non-English versions of the UDysRS was initiated. The aim of this study was to validate and confirm the factor structure of the Slovak translation of the UDysRS. We examined 251 patients with Parkinsons disease and dyskinesia using the Slovak version of the UDysRS. The average age of our sample was 65.2 ± 9.2 years and average disease duration was 10.9 ± 5.0 years. Slovak data were compared using confirmatory factor analysis with the Spanish data. To be designated as the official Slovak UDysRS translation, the comparative fit index (CFI) had to be ≥0.90 relative to the Spanish language version. Exploratory factor analysis was performed to explore the underlying factor structure without the constraint of a prespecified factor structure. For all four parts of the Slovak UDysRS, the CFI, in comparison with the Spanish language factor structure, was ≥0.98. Isolated differences in the factor structure of the Slovak UDysRS were identified by exploratory factor analysis compared with the Spanish version. The Slovak version of the UDysRS was designated as an official non-English translation and can be downloaded from the website of the International Parkinson and Movement Disorder Society.

Effects of rasagiline on freezing of gait in Parkinson's disease - an open-label, multicenter study.

AIMS Freezing of gait is a disabling symptom in advanced Parkinsons disease. Positive effects have been suggested with MAO-B inhibitors. We report on an open label clinical study on the efficacy of rasagiline as add-on therapy on freezing of gait and quality of life in patients with Parkinsons disease. METHODS Forty two patients with freezing of gait were treated with 1 mg rasagiline daily as an add-on therapy. Patients were assessed at baseline and after 1, 2 and 3 months of treatment. Freezing of gait severity was assessed using the Freezing of Gait Questionnaire, motor impairment by the modified MDS UPDRS part III, and quality of life using the PDQ-39 questionnaire. RESULTS Patients treated with rasagiline had a statistically significant decrease in FoG-Q score and modified MDS UPDRS score after 1, 2 and 3 months of therapy. A moderately strong (r = 0.686, P = 0.002) correlation between the effects on mobility and freezing of gait was found. We also observed a statistically significant improvement in global QoL and in the subscales mobility, ADL, stigma and bodily discomfort in patients after 3 months of rasagiline therapy. A significant correlation (r = 0.570, P = 0.02) between baseline FoG-Q score and the baseline score for the PDQ Mobility subscale was found. CONCLUSION In our study rasagiline as add-on antiparkinsonian therapy significantly improved mobility, freezing of gait and quality of life. The positive effect on freezing of gait appears to be related to improvement of mobility.

14. Autoimmune lower motor neurone disorder – Case report

Motor neurone diseases (MND) traditionally include a broad spectrum of neuromuscular disorders. First, we have to mention sporadic forms of ALS (amyotrophic lateral sclerosis) with mixed clinical presentation (syndromes with upper and lower motor neurone signs), then the isolated central type (primary lateral sclerosis, PLS) and at last the isolated peripheral type (primary muscular atrophy, PMA). The existence of familiar hereditary and genetic MND forms is well known, e.g. superoxide dismutase (SOD-1) and dynactin gene mutations are known to cause PMA variants also called LMND (lower motor neurone disease). Selected LMND cases are associated to specific autoantibodies. For example an asymmetric lower motor neurone syndrome with predominant distal involvement is associated to IgM anti-GM1 or to IgM anti-GalNAc-GD1a. Another LMND variant is an asymmetric lower motor neurone syndrome with particular damage of proximal upper extremities muscles (also known as Vulpian–Bernhard syndrome, brachial amyotrophic diplegia or flail arm syndrome) can be associated in 10–20% cases to anti-asialo GM1 autoantibodies. We present a patient’s case report with suspected Vulpian–Bernhard syndrome with bilateral non-symmetric cervico-brachial amyotrophy without sensory impairment. The autoimmune aetiology was clearly proven by clinical responsiveness to immunosuppression/immunomodulation therapy mainly to repetitive intravenous human polyclonal immunoglobulin application (IVIG).