Subthalamic nucleus physiology is correlated with deep brain stimulation motor and non-motor outcomes.

Subthalamic nucleus deep brain stimulation is commonly indicated for symptomatic relief of idiopathic Parkinson's disease. Despite the known improvement in motor scores, affective, cognitive, voice and speech functions might deteriorate following this procedure. Recent studies have correlated motor outcomes with intraoperative microelectrode recordings. However, there are no microelectrode recording-based tools with predictive values relating to long-term outcomes of integrative motor and non-motor symptoms. We conducted a retrospective analysis of the outcomes of patients with idiopathic Parkinson's disease who had subthalamic nucleus deep brain stimulation at Tel Aviv Sourasky Medical Centre (Tel Aviv, Israel) during 2015-2016. Forty-eight patients (19 women, 29 men; mean age, 58 ± 8 years) who were implanted with a subthalamic nucleus deep brain stimulation device underwent pre- and postsurgical assessments of motor, neuropsychological, voice and speech symptoms. Significant improvements in all motor symptoms (except axial signs) and levodopa equivalent daily dose were noted in all patients. Mild improvements were observed in more posterior-related neuropsychological functions (verbal memory, visual memory and organization) while mild deterioration was observed in frontal functions (personality changes, executive functioning and verbal fluency). The concomitant decline in speech intelligibility was mild and only partial, probably in accordance with the neuropsychological verbal fluency results. Acoustic characteristics were the least affected and remained within normal values. Dimensionality reduction of motor, neuropsychological and voice scores rendered six principal components that reflect the main clinical aspects: the tremor-dominant versus the rigidity-bradykinesia-dominant motor symptoms, frontal versus posterior neuropsychological deficits and acoustic characteristics versus speech intelligibility abnormalities. Microelectrode recordings of subthalamic nucleus spiking activity were analysed off-line and correlated with the original scores and with the principal component results. Based on 198 microelectrode recording trajectories, we suggest an intraoperative subthalamic nucleus deep brain stimulation score, which is a simple sum of three microelectrode recording properties: normalized neuronal activity, the subthalamic nucleus width and the relative proportion of the subthalamic nucleus dorsolateral oscillatory region. A threshold subthalamic nucleus deep brain stimulation score >2.5 (preferentially composed of normalized root mean square >1.5, subthalamic nucleus width >3 mm and a dorsolateral oscillatory region/subthalamic nucleus width ratio >1/3) predicts better motor and non-motor long-term outcomes. The algorithm presented here optimizes intraoperative decision-making of deep brain stimulation contact localization based on microelectrode recording with the aim of improving long-term (>1 year) motor, neuropsychological and voice symptoms.

Multidisciplinary intensive outpatient rehabilitation program for patients with moderate-to-advanced Parkinson's disease.

BACKGROUND: Intensive, multi-disciplinary, rehabilitation programs for patients with Parkinson's disease (PWPs) have shown to be effective. However, most programs are based on in-patient service, which is expensive. OBJECTIVE: To demonstrate the feasibility of a multidisciplinary, intensive, outpatient rehabilitation program (MIOR) for moderate to advanced Parkinson's Disease (H&Y≥2). METHOD: The MIOR program takes place at a community rehabilitation center ('Ezra Le'Marpe'), 3 times a week, 5 hours, 8 weeks, and includes 20 PWPs in each cycle. The multi-disciplinary team includes physical, occupational, speech and hydro therapists. Additional activities include, social work groups, boxing, dancing and bridge. RESULTS: Data was collected retroactively for the first two years. Data analysis includes 158 patient files who completed the program (mean disease duration 10.1±6 and mean H&Y stage 2.8±0.67). Assessments were performed at the beginning and end of the intervention. Positive results were collected: improvement in number of falls (p < 0.0001), Functional Independence Measure (p < 0.0001), quality of life (p < 0.01), balance (p < 0.0001), upper limb function (p < 0.0001) and paragraph reading vocal intensity (p < 0.01). CONCLUSIONS: MIOR is a feasible program, showing positive results in moderate to advanced PWP's, improving quality of life, daily function, and motor performance. The current outcomes demonstrate feasibility of MIOR in addition to medical treatment.

Dysphagia characteristics in Huntington's disease patients: insights from the Fiberoptic Endoscopic Evaluation of Swallowing and the Swallowing Disturbances Questionnaire.

BACKGROUND: Huntington's disease (HD) is a neurodegenerative disease characterized by increasing dysphagia as the disease progresses. Specific characteristics of the HD dysphagia are not well defined. OBJECTIVE: To characterize the swallowing disturbances of HD patients, to evaluate the feasibility of Fiberoptic Endoscopic Evaluation of Swallowing (FEES) in assessing dysphagia in HD patients, and to discern the relation between FEES findings and patients' self-report on dysphagia symptoms and swallowing related quality of life (SWAL-QOL). METHOD: A retrospective case series in a tertiary referral center. All recruited HD patients underwent Bed Side Swallowing Evaluation (BSE), FEES, the Unified Huntington's Disease Rating Scale (UHDRS), and the Montreal Cognitive Assessment (MoCA). All completed the Swallowing Disturbances Questionnaire (SDQ) and the SWAL-QOL questionnaire. RESULTS: Fourteen HD patients were recruited. All were able to complete the FEES study. The FEES demonstrated delayed swallowing reflex, solid food residues, and pre/post swallowing spillage in most patients (50%, 53.5%, 83.3%, and 87.5%, respectively). The mean SDQ score was 13.2. Significant correlations were found between the SWAL-QOL fear of eating score; the SDQ oral, pharyngeal, and total scores; and the FEES parameters of pureed and solid food bolus flow time. Significant correlations were also found between the total UHDRS score, the volitional cough score, and the SWAL-QOL disease burden score. CONCLUSION: HD patients exhibit prominent unique oropharyngeal dysphagia features that may serve as a marker of disease progression. The FEES and the SDQ are valuable tools for detecting these features in HD patients with swallowing disturbance.

Two Ethnic Clusters with Huntington Disease in Israel: The Case of Mountain Jews and Karaites.

BACKGROUND: Worldwide prevalence estimates of Huntington disease (HD) vary widely, with no reliable information regarding the Jewish population in Israel. METHODS: This specialized tertiary single-center cross-sectional study assessed clinical, cognitive, and demographic characteristics of 84 HD patients who were treated at the Movement Disorder Unit of the Tel Aviv Medical Center, Israel. RESULTS: Our cohort was composed of one-third Ashkenazi Jews, 27% Mountain Jews (Caucasus Jews), 18% Sephardi Jews, and 21% Karaites, with both Mountain Jews and Karaites over-represented compared to their relevant proportion in the population of the state of Israel, which is less than 1%. No between-group differences were detected regarding the number of CAG (cytosine-adenine-guanine) repeats, age at onset, disease duration, years from symptom onset to diagnosis, gender, years of education, Unified Huntington Disease Rating Scale scores, or the Montreal Cognitive Assessment scores. CONCLUSION: We detected clustering of HD among the population treated at our Medical Center, which has the only specialized HD clinic in the country, with a high percentage of HD among 2 relatively small subpopulations of Jews: Mountain Jews and Karaites.

Tradução e adaptação cultural do swallowing disturbance questionnaire para o português-brasileiro / Translation and cultural adaptation of swallowing disturbance questionnaire for brazilian portuguese

RESUMO Objetivo: traduzir e adaptar culturalmente para o português brasileiro o Swallowing disturbance questionnaire (SDQ) para detecção de risco de disfagia em indivíduos com Doença de Parkinson (DP). Métodos: realizou-se tradução para o português, retradução para o inglês, análise de confiabilidade do instrumento, sendo a versão final aplicada aos participantes e realizado o reteste após duas semanas. Selecionou-se os participantes em um ambulatório de distúrbios do movimento de um hospital de referência no Rio Grande do Sul. Incluiu-se indivíduos com diagnóstico de DP. Excluiu-se indivíduos com alteração de linguagem ou audição que impossibilitasse a compreensão do questionário e com diagnóstico de outras doenças neurológicas. Resultados: a amostra foi composta por 23 indivíduos. Os participantes responderam a todas as questões. Não houve eliminação de nenhuma questão. Na análise da confiabilidade teste-reteste o coeficiente de correlação intraclasse do escore final nos dois momentos foi de 0,912 com p<0,001 (95%IC=0,792-0,963), demonstrando que os dados são altamente homogêneos. Na análise por questão, não houve diferença significante entre os dois momentos de aplicação. O valor de α de Cronbach do instrumento foi de 0,63. Conclusão: houve equivalência cultural do SDQ para o português brasileiro, com boa confiabilidade interna do instrumento.

ABSTRACT Purpose: to translate and culturally adapt to Brazilian Portuguese Swallowing the disturbance questionnaire (SDQ) for dysphagia detection risk in individuals with Parkinson's disease (PD). Methods: this was translated into Portuguese, back-translation into English, instrument reliability analysis, the final version applied to participants and conducted retesting after two weeks. It was selected participants at a clinic for movement disorders of a reference hospital in Rio Grande do Sul. It was included individuals diagnosed with PD. It was excluded individuals with impaired speech or hearing that prevented the understanding of the questionnaire and diagnosis of other neurological diseases. Results: the sample consisted of 23 individuals. The participants answered all questions. There was no question of disposal. In the analysis of test-retest reliability intraclass correlation coefficient of the final score in the two periods was 0.912 with p <0.001 (95% CI = 0.792 to 0.963), demonstrating that the data are highly homogeneous. In the analysis by question, there was no significant difference between the two application times. The value of α Cronbach instrument was 0.63. Conclusion: there was cultural equivalence of the SDQ for Brazilian Portuguese, with good internal reliability of the instrument.

Interdisciplinary teamwork for the treatment of people with Parkinson's disease and their families.

Parkinson's disease (PD) is a chronic progressive neurodegenerative and multidimensional disease that involves a range of disabling motor and nonmotor symptoms. These symptoms can have a major impact on the quality of life of PD patients. The focus of this article is to stress the importance of the interdisciplinary team intervention approach in the treatment of patients with PD. The team approach uses experts in PD from different health care professions, including a neurologist, a nurse, a speech and language therapist, a physiotherapist, a social worker, a psychiatrist, an occupational therapist, a sexologist, and a dietician. The major aim of the team and of teamwork is to provide professional care in all motor and nonmotor aspects of PD throughout the course of the disease. There are different models of multidisciplinary teams: inpatient facility, community rehabilitation facility, and synchronized multiprofessional treatment in the community. The Tel Aviv Sourasky Medical Center model of interdisciplinary care was designed to create a coordinated multidisciplinary team in the Movement Disorders Unit. The role of each team member and their professional objective are described. Their collaboration is by design a promotion of a team goal for maintaining and enhancing the quality of life of PD patients and their families.

Video-assisted swallowing therapy for patients with Parkinson's disease.

BACKGROUND: Conventional swallowing therapy for patients with Parkinson's disease (PD) and swallowing difficulties has poor carryover to everyday life. Herein, we test the effectiveness of visual information while treating swallowing disturbances in patients with PD. METHODS: Forty two non-demented PD patients with swallowing disturbances were randomly divided into two groups. An experimental group received video-assisted swallowing therapy (VAST) and a control group (n = 21) was given conventional therapy. Both groups were given 6 interventional sessions by the same speech and swallowing therapist. Patients in the VAST group were exposed to video of the swallowing process in general as well as of their own, as part of all therapy sessions. Swallowing function was assessed before and post-intervention by fiberoptic endoscopic evaluation of swallowing (FEES). Quality of life, quality of care and the degree of pleasure from eating were also assessed by questioners pre and post-intervention. RESULTS: There was a significant improvement in swallowing functions following both interventions. The FEESs demonstrated a significantly greater reduction in food residues in the pharynx in the VAST group compared to the conventional treatment group. There were significant group improvement in some parameters of the quality of life, quality of care and pleasure of eating scales. CONCLUSION: In cognitively intact patients with PD with swallowing disturbances VAST was associated with improved swallowing related QOL and less food residues in the pharynx.

Appreciation of humor is decreased among patients with Parkinson's disease.

OBJECTIVE: To test whether appreciation of humor might be a non-motor function affected by Parkinson's disease (PD). METHODS: Thirty-nine PD patients and 38 healthy controls participated in this study. Appreciation of humor and effect of the presentation method utilized were assessed. Sense of humor was evaluated by the sense of humor questionnaire (SHQ-6). Humor appreciation was tested using three methods of presentation: videos, audio sketches and pictorial cartoons, each portraying both obvious and non-obvious humor content. Depression, anxiety, cognition, disease severity and quality of life were measured by standardized questionnaires and correlated with humor outcomes. RESULTS: Patients with PD rated humor content lower than controls on every method of presentation as well as on the SHQ-6 (p = 0.004). The greatest between-group difference was noted when the material was presented visually via pictorial cartoons (p < 0.0001). In addition, obvious humor content was rated higher than non-obvious content by the PD group in all three presentation methods (p < 0.05). The degree of depression and anxiety did not influence these results. CONCLUSIONS: Patients with PD have a decreased sense of humor compared to healthy controls. Utilizing audio methods of presentation and humor in an obvious mode appears to be the preferred approach for eliciting responses to humor in a PD population.

Swallowing disturbance questionnaire for detecting dysphagia.

OBJECTIVES: To assess the accuracy of the swallowing disturbance questionnaire (SDQ) that had originally been designed and validated for detecting swallowing problems among patients with Parkinson's disease and was now applied for identifying patients with dysphagia associated with various other etiologies. STUDY DESIGN: Prospective. METHODS: One-hundred patients with the complaint of swallowing disturbances who underwent a full swallowing survey at the Tel-Aviv Voice and Swallowing Disorder Clinic participated. They all filled in the SDQ. The collected data included patient characteristics, medical history, and the results of an oromotor examination and a fiberoptic endoscopic evaluation of swallowing (FEES). The SDQ results were compared to the FEES and oromotor examination findings. RESULTS: The responses to the questions in the SDQ were highly correlated with the findings of the oral part of the oromotor examination (85.71% sensitivity, 87.6% specificity). Items on the laryngopharyngeal phase reliably assessed dysphagia symptoms in correlation to the FEES examination (67.3% sensitivity, 76.7% specificity). The total SDQ score correlated with the total oromotor and the FEES scores (79.7% sensitivity, 73% specificity). CONCLUSIONS: The SDQ is a sensitive and accurate tool for identifying patients with true swallowing disturbances arising from different etiologies and for indicating the need for more in-depth instrumental swallowing evaluations.

Anxiety, depression and swallowing disorders in patients with Parkinson's disease.

Swallowing disturbances (SDs), anxiety and depression are commonly present in Parkinson's disease (PD) patients. We hypothesized that there is an association between the presence of SDs and the PD affective state. Sixty-nine PD patients were assessed for the presence of SDs by undergoing cognitive screening with the Mini Mental State Examination (MMSE), completing three inventories: a swallowing disturbance questionnaire (SDQ), the Spielberger manual for the trait anxiety and Beck depression inventories. All patients underwent clinical swallowing evaluations by a speech and language pathologist (SLP). Patients diagnosed with SDs were also assessed by fiberoptic endoscopic evaluation of swallowing (FEES) performed by an ENT and SLP. Thirty-eight patients experienced SDs, the other 31 did not. The clinical characteristics of the two groups were matched. Patients with SDs experienced increased anxiety and depression compared to patients without SDs. Comparisons between patients who scored in the two opposite ends of the anxiety and depression ranges demonstrated that the most anxious and depressed patients reported more swallowing difficulties (SDQ scores) compared with the least anxious and depressed ones. In addition, the most anxious patients had significantly increased disease severity and decreased MMSE scores compared with the least anxious patients. Disease severity was also increased in the most depressed patients compared with the least depressed ones. Advanced disease emerged as being associated with high anxiety levels and greater numbers of SDs. The contribution of anxiety or depression to the development or worsening of SDs and their role in treatment strategy warrant further investigation.

Validation of a swallowing disturbance questionnaire for detecting dysphagia in patients with Parkinson's disease.

Underreporting of swallowing disturbances by Parkinson's disease (PD) patients may lead to delay in diagnosis and treatment, alerting the physician to an existing dysphagia only after the first episode of aspiration pneumonia. We developed and validated a swallowing disturbance questionnaire (SDQ) for PD patients and compared its findings to an objective assessment. Fifty-seven PD patients (mean age 69 +/- 10 years) participated in this study. Each patient was queried about experiencing swallowing disturbances and asked to complete a self-reported 15-item "yes/no" questionnaire on swallowing disturbances (24 replied "no"). All study patients underwent a physical/clinical swallowing evaluation by a speech pathologist and an otolaryngologist. The 33 patients who complained of swallowing disturbances also underwent fiberoptic endoscopyic evaluation of swallowing (FEES). According to the ROC test, the "optimal" score (where the sensitivity and specificity curves cross) is 11 (sensitivity 80.5%, specificity 81.3%). Using the SDQ questionnaire substantially reduced Type I errors (specifically, an existing swallowing problem missed by the selected cutoff point). On the basis of the SDQ assessment alone, 12 of the 24 (50%) noncomplaining patients would have been referred to further evaluation that they otherwise would not have undergone. The SDQ emerged as a validated tool to detect early dysphagia in PD patients.