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Background: Parkinson's disease (PD) has a negative effect on oral health and orofacial function, but the subjective experience of orofacial symptoms and their impact on the quality of life is not fully investigated. In addition, knowledge of how to improve the subjective oral symptoms is lacking. Objectives: To assess the self‐reported orofacial function and oral health in patients with PD. Furthermore, to investigate the effect of interventions for improvement of oral hygiene and function on oral health–related quality of life (OHRQoL). Methods: A randomised controlled study with delayed intervention was conducted in 29 patients with moderate to advanced PD. Patients were instructed in a standardised exercise programme for the jaw and orofacial muscles and given an individualised oral hygiene programme. The effect on self‐reported orofacial function and OHRQoL was measured after 2 and 4 months using the Nordic Orofacial Test—Screening (NOT‐S), the oral health impact profile (OHIP‐14), self‐reported drooling score and subjective mastication ability. Results: Self‐reported oral health and function before the intervention was significantly correlated to the severity and duration of PD. The NOT‐S and drooling score were significantly improved by the interventions after 2 months and the OHIP‐14 after 4 months. Conclusion: The interventions improve the self‐reported orofacial function and OHRQoL. These simple interventions can be implemented in the allied multidisciplinary health care surrounding the PD patient. [ABSTRACT FROM AUTHOR]

Background: Parkinson's disease (PD) is a progressive neurodegenerative disorder. It is associated with reduced oral health and impaired oro‐facial function, but besides recommendations of dental visits and drooling treatment, there are little documented odontological treatment options. Objectives: To evaluate the effect of standardised home exercise jaw opening and chewing programmes, as well as home oral hygiene measures instructed and controlled by a trained dentist. Methods: Twenty‐nine patients (median 65 years) with moderate to advanced PD participated in the project after informed consent. They were followed at a Neurology Department, were able to cooperate, and had stable medical treatment and nine also deep brain stimulation. The interventions were individual dental hygiene instruction, training of jaw opening (JawTrainer), and lip and chewing exercises (Ulmer Oral Screen and Proxident Fluoride Gum). The study was performed as a randomised controlled study. The treatment effect was evaluated after 2 and 4 months. The primary outcome was maximum unassisted jaw opening capacity, chewing time of a standardised apple slice, and the Simplified Debris Index. Results were analysed with Wilcoxon matched pairs test and Mann‐Whitney U test (significance level P <.05). Results: Jaw opening, chewing time and hygiene were significantly improved 2 months from the start of the invention, respectively, 6%, 49%, and 25%, and the improvement was still significant after 4 months. No significant changes were found after the 2‐month control period without intervention. Conclusion: The simple measures had a substantial and significant clinical effect which is promising despite the progressive nature of the PD. [ABSTRACT FROM AUTHOR]

Objective. Drooling in neurodegenerative diseases is associated with social impediment. Previous treatments of drooling have little effect or are effective but with severe side effects. Therefore, there is a need to test new methods such as the use of botulinum toxin type A (BTX-A). Material and methods. This open, prospective study deals with treatment of drooling in 12 patients with amyotrophic lateral sclerosis and three with Parkinson's disease. Injections of BTX-A (Botox) were given into the parotid (25--40 units) and submandibular (15--30 units) glands with ultrasonographic guidance. After BTX-A treatment, the patients were followed for 2 months with evaluations every second week by means of self-assessed rating scales for drooling intensity, discomfort and treatment effect, and determination of unstimulated whole saliva (UWS) flow rate, and inorganic and organic UWS composition. The treatment was repeated up to four times, but seven patients dropped out shortly after the first treatment due to marked worsening of their disease-related condition. Results. Drooling and flow were reduced ( P < 0.05) 2 weeks after treatment, without side-effects. The maximal reductions during the observation period were 40% for drooling and 30% for flow. There was a systematic variation in flow during the observation period, with an initial decrease and then an increase followed by a second decrease. Amylase activity and total protein concentration generally increased with decreasing flow ( P ≤≤ 0.03). Conclusion. Inhibition of acetylcholine release from postganglionic parasympathetic nerve endings by injection of BTX-A into salivary glands seemed useful for secondary sialorrhoea, although cyclic variations in flow may occur, possibly due to transitory sprouting and regeneration. [ABSTRACT FROM AUTHOR]

Abstract: Background: Parkinson''s disease (PD) and multiple system atrophy (MSA) are neurodegenerative diseases that can be difficult to diagnose and distinguish from each other. Study aims and methods: Patients with PD and MSA and controls were studied with magnetic resonance imaging (MRI) using tissue segmentation and outlining of regions in order to identify regional volume changes that might be useful in the diagnosis of the two diseases. Results: Patients with PD had significantly larger intracranial volumes (ICVs) and significantly smaller putaminal and sustantia nigra volumes than controls. MSA patients had significantly smaller substantia nigra and caudate volumes than controls but normal intracranial volume. In both patient groups there was a further trend towards smaller amygdala volumes. Discussion: Increased ICV in PD patients is a new finding that may be explained by genetic factors or compensatory responses to early CNS damage. Atrophy of the amygdala in MSA patients has not been demonstrated with MR before. It might explain why these patients can have hyposmia. The putaminal atrophy found in the PD group may be a trait of the later stages of PD. Segmentation of the substantia nigra can be a useful aid in the diagnosis of PD and MSA. [Copyright &y& Elsevier]

BACKGROUND: MR diffusion tensor imaging (DTI) appears to be a powerful method to investigate the neuronal and axonal fibre distribution in the human brain. Changes in diffusion characteristics of water molecules in the white matter can be estimated as the apparent diffusion coefficient (ADC) and the fractional anisotropy index (FA). OBJECTIVES: To characterize DTI changes at three different levels in the corticospinal tract (CST) (corona radiata, internal capsule and pons) in order to elucidate if pathogenesis of ALS is due to an anterograde or retrograde axonal degeneration. METHODS: We studied eight ALS patients with clinical signs of upper motor neuron involvement. The patients were compared with 11 healthy age-matched controls. RESULTS: ADC was significantly increased in the CST in ALS patients at the level of the internal capsule and also increased in the pons, but without statistical significance. ADC was unchanged at the level of the corona radiata. FA was significantly reduced at the lowest level (pons), only tended to be reduced in the internal capsule, but was also unchanged in the corona radiata. CONCLUSIONS: Segmentation of the CST into three regions supports the hypothesis of a 'dying back' mechanism in ALS and suggests that ADC is a more sensitive measure than FA to detect pathological changes in ALS. [ABSTRACT FROM AUTHOR]

Objectives: Persons with Parkinson's disease (PD) have a higher prevalence of oral diseases and orofacial dysfunction, but knowledge about the use of dental care and whether their dental care needs are met is sparse. This study aimed to investigate the dental attendance and usage of dental care services of the total PD population in Denmark and compare it with a control group. Methods: National registers were used to identify the total PD population in Denmark (n = 6874) and to obtain data on their dental care from 2015 to 2019. These data were compared with a five‐fold age‐, gender‐ and geographically matched control group without PD (n = 34 285). Register data on age, gender, civil status, educational level, income, nursing homes status and mortality were also collected and adjusted for in the analyses. The dental attendance was analysed using χ2‐test with Bonferroni correction, and the type of dental care services was analysed using negative binomial regression analysis. Results: A significantly higher proportion of persons with PD were irregular attenders of the dental care system (21.0%), compared with the control group (16.9%). Persons with PD had a significantly higher overall usage of dental cares services. Most prominent was the high usage of treatment services, where persons with PD had a 1.50 times higher incidence rate of tooth extractions and a 1.71 times higher incidence rate of tooth fillings in the five years compared with the control group. Conclusion: Persons with PD are more often irregular users of dental care and receive more treatment services than the control group. This indicates a need for high‐quality prophylactic initiatives to prevent high filling and tooth extraction rates. Furthermore, this knowledge can be used by clinicians and decision makers to ensure optimal dental care for persons with PD. [ABSTRACT FROM AUTHOR]

Aim: This is a long-term open follow-up of a prospective double-blind crossover study, where electrodes were bilaterally implanted in both the Subthalamic nucleus (STN) and internal pallidum (GPi) in patients with isolated dystonia.Methods: Patients with isolated dystonia were included to undergo surgery with Deep Brain stimulation (DBS) and after randomization, in a double-blind cross-over study, receiving bilateral stimulation of either STN or GPi for 6 months in each target. Preoperative and postoperative assessments with the Burke-Fahn-Marsden Dystonia Rating Scale (BFMDRS) and the 36-item Short Form Health Survey (SF-36) were performed. In this long-term follow-up (LFU), these ratings were repeated, and patients were evaluated with cognitive tests.Results: 21 patients were included in the protocol, 9 patients with generalized dystonia, 12 with a diagnosis of cervical dystonia. The mean duration of disease was 19.3 years, age at time of surgery 50.1 years. Fourteen patients participated in the LFU. At a mean follow-up of 10.2 years (range 4.8-15.4), BFMDRS movement score was improved with a mean of 36% (p < 0.05) compared with baseline. At LFU both a statistically significant improvement of stimulation in STN on BFMDRS movement score (p = 0.029) and Gpi (p = 0.008) was demonstrated, no significant difference was found between the two targets (p = 0.076). SF-36 improved for both targets.Conclusion: In this study we performed a long-term follow-up in 14 patients with cervical or generalized dystonia, who received stimulation in GPi, STN or both. The mean follow-up time was more than 10 years. Our data support a long-term effect of both STN-DBS and GPi-DBS in dystonia with equal effect and safety for up to 15 years. STN has been proven a viable safe and effective target and may be used as an alternative to GPi in both adult-onset cervical dystonia and generalized dystonia. [ABSTRACT FROM AUTHOR]

Introduction: Amyotrophic lateral sclerosis (ALS) is a progressive motor neuron disease with great heterogeneity. Biological prognostic markers are needed for the patients to plan future supportive treatment, palliative treatment, and end-of-life decisions. In addition, prognostic markers are greatly needed for the randomization in clinical trials.Objective: This study aimed to test the ALS Functional Rating Scale-Revised (ALSFRS-R) progression rate (ΔFS) as a prognostic marker of survival in a Danish ALS cohort.Methods: The ALSFRS-R score at test date in association with duration of symptoms, from the onset of symptoms until test date, (defined as ΔFS') was calculated for 90 Danish patients diagnosed with either probable or definite sporadic ALS. Median survival time was then estimated from the onset of symptoms until primary endpoint (either death or tracheostomy). ΔFS' was subjected to survival analysis using Cox proportional hazards modelling, log-rank test, and Kaplan-Meier survival analysis.Results and Conclusions: Both ΔFS' and age was found to be strong predictors of survival of the Danish ALS cohort. Both variables are easily obtained at the time of diagnosis and could be used by clinicians and ALS patients to plan future supportive and palliative treatment. Furthermore, ΔFS', is a simple, prognostic marker that predicts survival in the early phase of disease as well as at later stages of the disease. [ABSTRACT FROM AUTHOR]

Background: Deep brain stimulation of the subthalamic nucleus (STN‐DBS) is well established and the most effective treatment for advanced Parkinson's disease (PD). However, little is known of the long‐term effects. Objectives: The aim of this study was to examine the long‐term effects of STN‐DBS in PD and evaluate the effect of reprogramming after more than 8 years of treatment. Methods: A total of 82 patients underwent surgery in Copenhagen between 2001 and 2008. Before surgery and at 8 to 15 years follow‐up, the patients were rated with the Unified Parkinson's Disease Rating Scale (UPDRS) with and without stimulation and medicine. Furthermore, at long‐term follow‐up, the patients were offered a systemic reprogramming of the stimulation settings. Data from patients' medical records were collected. The mean (range) age at surgery was 60 (42–78) years, and the duration of disease was 13 (5–25) years. A total of 30 patients completed the long‐term follow‐up. Results: The mean reduction of the motor UPDRS by medication before surgery was 52%. The improvement of motor UPDRS with stimulation alone compared with motor UPDRS with neither stimulation nor medication was 61% at 1 year and 39% at 8 to 15 years after surgery (before reprogramming). Compared with before surgery, medication was reduced by 55% after 1 year and 44% after 8 to 15 years. After reprogramming, most patients improved. Conclusions: STN‐DBS remains effective in the long run, with a sustained reduction of medication in the 30 of 82 patients available for long‐term follow‐up. Reprogramming is effective even in the late stages of PD and after many years of treatment. View Supplementary Video 1 [ABSTRACT FROM AUTHOR]

Parkinsonism and attention deficit hyperactivity disorder (ADHD) are widespread brain disorders that involve disturbances of dopaminergic signaling. The sodium-coupled dopamine transporter (DAT) controls dopamine homeostasis, but its contribution to disease remains poorly understood. Here, we analyzed a cohort of patients with atypical movement disorder and identified 2 DAT coding variants, DAT-Ile312Phe and a presumed de novo mutant DAT-Asp421Asn, in an adult male with early-onset parkinsonism and ADHD. According to DAT single-photon emission computed tomography (DAT-SPECT) scans and a fluoro-deoxy-glucose-PET/MRI (FDG-PET/MRI) scan, the patient suffered from progressive dopaminergic neurodegeneration. In heterologous cells, both DAT variants exhibited markedly reduced dopamine uptake capacity but preserved membrane targeting, consistent with impaired catalytic activity. Computational simulations and uptake experiments suggested that the disrupted function of the DAT-Asp421Asn mutant is the result of compromised sodium binding, in agreement with Asp421 coordinating sodium at the second sodium site. For DAT-Asp421Asn, substrate efflux experiments revealed a constitutive, anomalous efflux of dopamine, and electrophysiological analyses identified a large cation leak that might further perturb dopaminergic neurotransmission. Our results link specific DAT missense mutations to neurodegenerative early-onset parkinsonism. Moreover, the neuropsychiatric comorbidity provides additional support for the idea that DAT missense mutations are an ADHD risk factor and suggests that complex DAT genotype and phenotype correlations contribute to different dopaminergic pathologies. [ABSTRACT FROM AUTHOR]

Purpose: To examine the diagnostic sensitivity and specificity of dopamine transporter SPECT imaging with a highly dopamine transporter selective radioligand. The study included consecutively enrolled, drug-naive patients with an average short history of parkinsonian motor symptoms, referred for diagnostic scanning. Methods: The study group comprised 288 patients naive to antiparkinson treatment who were enrolled as they were admitted for a diagnostic SPECT scan with the radioligand [I]- N-(3-iodoprop-2E-enyl)-2-β-carbomethoxy-3β-(4-methylphenyl)nortropane (I-PE2I). After the diagnostic scanning, patients were followed clinically with an average follow-up of 19.7 ± 12.5 months. Results: A diagnosis could be clinically settled in 189 patients and among these patients, a dopamine transporter scan had a sensitivity of 88% and a specificity of 91% for discrimination between patients with and without striatal neurodegeneration. In cognitively impaired patients (Mini Mental State Examination <27) the specificity was 75% and the sensitivity 95%. A striatal anterior-posterior ratio (APR) of >2 differentiated between idiopathic Parkinson's disease and atypical parkinsonian syndromes with a specificity of 84% and a sensitivity of 63%. Conclusion: In drug-naive patients with subtle clinical parkinsonian motor symptoms, dopamine transporter scan using I-PE21 has a high sensitivity and specificity in distinguishing between patients with and without striatal neurodegeneration. The specificity is lower in patients who are also cognitively impaired. Calculation of the striatal APR can assist in differentiating between idiopathic Parkinson's disease and atypical parkinsonian syndromes. [ABSTRACT FROM AUTHOR]

Bakke M, Larsen SL, Lautrup C, Karlsborg M. Orofacial function and oral health in patients with Parkinson's disease. Eur J Oral Sci 2011; 119: 27-32. © 2011 Eur J Oral Sci No comprehensive study has previously been published on orofacial function in patients with well-defined Parkinson's disease (PD). Therefore, the aim of this study was to perform an overall assessment of orofacial function and oral health in patients, and to compare the findings with matched control subjects. Fifteen outpatients (nine women and six men, 61-82 yr of age; Hoehn & Yahr Stages 2-4; and with motor impairment ranging from 17 to 61 according to the Unified Parkinson's Disease Rating Scale, Objective Motor Part III) were examined in their 'on' state together with 15 age- and gender-matched controls. Orofacial function and oral health were assessed using the Nordic Orofacial Test, masticatory ability, performance and efficiency, oral stereognosis, jaw opening, jaw muscle tenderness, the Oral Health Impact Profile-49, number of natural teeth, and oral hygiene. Orofacial dysfunction was more prevalent, mastication and jaw opening poorer, and impact of oral health on daily life more negative, in patients with PD than in controls. The results indicate that mastication and orofacial function are impaired in moderate to advanced PD, and with progression of the disease both orofacial and dental problems become more marked. It is suggested that greater awareness of the special needs in PD patients and frequent dental visits are desirable to prevent dental diseases and decay and to support masticatory function. [ABSTRACT FROM AUTHOR]

Abstract: Background: Restless legs syndrome (RLS) is a neurological movement disorder, which often causes sleep problems. However, the comorbidity of this disorder is not well known. This study aimed to document the prevalence of RLS in the general population of Sweden and to identify factors associated with this condition. Methods: A cross-sectional study was performed in Sweden. One thousand subjects aged 18–90 years old underwent telephone interviews. The questionnaire assessed such factors as sleep variables, depressive mood, treatment of diabetes mellitus, and treatment with drugs for depression during the previous four-week period. RLS was diagnosed based on the minimal criteria provided by the International RLS Study Group. Results: The prevalence of RLS was 5% (5.7% in women, 3.5% in men). Severe or very severe RLS symptoms during the previous week were noted by 64% of the RLS subjects. Factors associated with RLS were insomnia, excessive daytime sleepiness, periodic limb movements in sleep, and depressed mood. Those affected by RLS were not more often consumers of drugs for depression than non-RLS subjects. Conclusions: RLS is prevalent in the general population in Sweden. RLS negatively influenced sleep and was associated with depressed mood. Antidepressive drug treatment was not associated with RLS. [Copyright &y& Elsevier]

Abstract: Background and Purpose: To estimate prevalence and severity (using the International Restless Legs Syndrome Study Group Rating Scale (IRLS)) and to identify risk factors of restless legs syndrome (RLS). Patients and Methods: Population-based cross-sectional study; 2005 randomly selected adults 18 years and above participated in a telephone interview in Norway and Denmark, employing the next-birthday technique. Results: Of the cross-section, 11.5% fulfilled the diagnostic criteria for RLS. Half of these reported the symptoms as moderate to very severe. Mean duration of the complaint was 10 years. Prevalence was higher in females than in males (13.4 vs 9.4%) and lowest in the youngest age group (18–29 years, 6.3%). From 30 years and above, no clear age-related difference was seen. Main predictors of RLS were insomnia (odds ratios: 1.71–3.16) and symptoms of periodic limb movements in sleep (3.20–7.85). The response rate was 47%, making the results less reliable. Conclusions: This study indicates that there is a high occurrence of RLS among adults. Main predictors are insomnia and periodic limb movements in sleep. [Copyright &y& Elsevier]