Patient preference of device-based treatment of Parkinson's disease.

INTRODUCTION: Subthalamic nucleus deep brain stimulation (STN-DBS), continuous subcutaneous apomorphine infusion (APO), and levodopa-carbidopa intestinal gel infusion (LCIG) are treatments used to treat severe motor fluctuations and dyskinesia in patients with advanced levodopa responsive Parkinson's disease (PD), who can no longer be managed with available combinations of oral medications. This study aims to evaluate patient choice of one of three device-based treatment methods. METHODS: A total of 58 patients clinically diagnosed with PD were included in the study. Eligibility for device-based treatment of PD patients with motor symptoms despite optimal medical treatment was assessed based on Hoehn & Yahr Stages, and Unified Parkinson's Disease Rating Scale-Part III. All three device-based treatment methods were thoroughly explained with on-hand demonstrations. Preferences and reasons for choice were recorded. RESULTS: Nineteen patients were ineligible for STN-DBS due to neurological causes. A total of 23 patients preferred STN-DBS, 23 preferred APO, and only one patient preferred LCIG. Thirteen patients preferred to continue oral medical treatment, while two patients positively approached both STN-DBS and APO. CONCLUSION: The most common reason patients declined STN-DBS and LCIG was concerned about the surgical operation, while the most common reason APO was declined was its frequent administration of the injection. While STN-DBS was preferred by younger, less severe patients, APO was preferred by older patients who had a longer duration of disease.

No Effect of Different Stimulation Conditions on Verbal Fluency and Visuospatial Orientation in Patients with Subthalamic Nucleus Deep Brain Stimulation.

BACKGROUND: Subthalamic nucleus deep brain stimulation is an effective treatment for the symptomatic treatment of Parkinson's disease. Apart from the obvious motor benefits, some cognitive side effects have been reported, particularly in verbal fluency. OBJECTIVES: Our aim was to evaluate the effects of the stimulation on verbal fluency and visuospatial orientation with changing stimulation conditions in 35 patients with Parkinson's disease. METHODS: Patients were randomized for their stimulation conditions as 'both on', 'both off', 'right on', and 'left on' and underwent verbal fluency and visuospatial orientation tasks during their drug-on periods. Letter and categorical fluency tasks and Benton's Judgment of Line Orientation Test were used for assessment. RESULTS: Overall, 6 patients were excluded due to dementia or depression. For verbal fluency, the number of words they produced in 1 min was similar in four stimulation conditions (p > 0.05). No significant difference was found between stimulation conditions in the spatial orientation task. CONCLUSIONS: We were unable to find any significant changes in verbal fluency and visuospatial orientation task scores with different stimulation conditions. This result suggests that either stimulation has no effect on given domains or the effect is so small that more detailed batteries are required to detect the difference.

Risk disclosure in prodromal Parkinson's disease - A survey of neurologists.

INTRODUCTION: In the absence of a disease-modifying treatment and prognostic uncertainty, ethics of risk disclosure in prodromal Parkinson's disease (PD) is challenging. Previous studies highlighted several facets of these challenges from the perspective of involved parties. However, to date, the view of neurologists who may encounter individuals with prodromal PD remained unrepresented. Moreover, cross-cultural differences intrinsic to the ethics of risk disclosure are yet to be elucidated. Therefore, we investigated the attitude of neurologists toward risk disclosure in prodromal PD. METHODS: In this observational study, Turkish neurologists were invited to fill out a questionnaire evaluating their stance on risk disclosure regarding an individual with polysomnography-confirmed REM sleep behavior disorder, which is the strongest risk factor for PD. RESULTS: More than 90% of the participating 222 neurologists were familiar with prodromal PD. While 15.3% stated that the risk should be disclosed in any case, 6.8% chose no disclosure. The remaining 77.9% favored disclosure only under certain circumstances, the plurality of which was the individual's consent to know about the risk. After reminding the potential neuroprotective effects of exercise and diet, neurologists who chose the option of "no disclosure" decreased to 3.2% (McNemar's test p = 0.008). No significant differences among the neurologists were found regarding sex, academic title, or field of interest. CONCLUSION: The majority of the neurologists found it appropriate to disclose the risk of future PD only if the individual expresses a desire to know. Also, recognition of the impact of lifestyle factors on PD is important in prognostic counseling.

Caregivers' Impression About the Effect of Subthalamic Nucleus Deep Brain Stimulation in the Treatment of Parkinson's Disease.

AIM: To determine caregivers' impressions of the impact of STN-DBS on motor and non-motor symptoms of Parkinson's disease (PD) in patients who underwent subthalamic nucleus deep brain stimulation (STN-DBS), evaluate the relationship of these changes with disease characteristics, and examine their contribution to the daily lives of patients. MATERIAL AND METHODS: The caregivers of patients who underwent STN-DBS were interviewed over the telephone. All telephone interviews were recorded, and changes in the motor and non-motor symptoms of the patients after STN-DBS were evaluated with a standardized questionnaire. RESULTS: Of the 173 patients with PD who underwent STN-DBS between 2005 and 2015, 62 who could be contacted by telephone were enrolled in the study. The mean age of the patients was 59.71 ± 9.78 years (range: 33-77 years). The mean disease duration was 15.62 ± 8.66 years (Range: 4-50 years). STN-DBS was performed on average 3.88 ± 2.6 years earlier (range: 1-11 years). According to the caregivers of the patients, there was a reduction in "off" periods in 79% of the patients, tremor in 58.1%, dyskinesia in 59.6%, depression in 46.8 %, pain symptoms in 41.9%, and improvement in sleep problems in 43.6% after STN-DBS. Moreover, 80.6% of the patients reported an improvement in their daily life activities after STN-DBS. CONCLUSION: From the caregivers' perspective, there was an improvement in the non-motor symptoms as well as the motor symptoms of patients with PD after STN-DBS, and this had a positive effect on the activities of daily lives in the majority of patients. Telephone interviews can be considered as an alternative method in the follow-up of patients with PD, especially when they cannot be assessed face-to-face.

Cross-Cultural Differences in Stigma Associated with Parkinson's Disease: A Systematic Review.

BACKGROUND: Stigma is an important social attitude affecting the quality of life (QoL) of people with Parkinson's disease (PwP, PD) as individuals within society. OBJECTIVE: This systematic review aimed to 1) identify the factors associated with stigma in PD and 2) demonstrate culture-based diversity in the stigmatization of PwP. We also reported data from the Turkish PwP, which is an underrepresented population. METHODS: Following the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) statement, a literature search of the PubMed/Medline electronic database was performed covering the last 26 years. Articles on self-perceived stigma in PD with a sample size >  20 and quantitative results were included. Data were extracted by independent reviewers. RESULTS: After screening 163 articles, 57 were eligible for review, most of which were from Europe or Asia. Only two studies have been conducted in South America. No study from Africa was found. Among the 61 factors associated with stigma, disease duration, sex, and age were most frequently studied. A comparison of the investigated factors across the world showed that, while the effect of motor impairment or treatment on stigma seems to be culture-free, the impact of sex, education, marriage, employment, cognitive impairment, and anxiety on stigma may depend on culture. CONCLUSION: The majority of the world's PD population is underrepresented or unrepresented, and culture may influence the perception of stigma in PwP. More diverse data are urgently needed to understand and relieve the challenges of PwP within their society.

Combined Unilateral Radiofrequency Lesioning of the Motor Thalamus, Field of Forel, and Zona Incerta: A Series of Cases With Dystonia.

BACKGROUND: Dystonia is a group of disorders characterized by involuntary slow repetitive twisting movements and/or abnormal posture. Surgical options such as neuromodulation through deep brain stimulation and neuroablative procedures are available for patients who do not respond to conservative treatment. OBJECTIVE: To present our series of patients with dystonia who were treated with stereotactic combined unilateral radiofrequency lesioning of the motor thalamus, field of Forel, and zona incerta. METHODS: Medical records of 50 patients with dystonia who were treated with unilateral combined lesions were reviewed. Outcomes of the surgical procedure were evaluated using the Burke-Fahn-Marsden Dystonia Rating Scale (with movement and disability subscales) and Unified Parkinson's Disease Rating Scale-tremor items. RESULTS: Based on the symptoms, patients were categorized as having generalized dystonia (34%), hemidystonia (30%), and dystonic tremor (DT) (36%). Primary/idiopathic dystonia, primary genetic/hereditary dystonia, and secondary dystonia accounted for 16%, 4%, and 80% of patients, respectively. The mean follow-up duration was 156.2 ± 88.9 mo. The overall improvement in the Burke-Fahn-Marsden Dystonia Rating Scale scores (movement and disability, respectively) was 57.8% and 36.4% in generalized dystonia, 60.0% and 45.8% in hemidystonia, and 65.6% and 56.8% in DT. Patients with DT showed an 83.3% improvement in mean Unified Parkinson's Disease Rating Scale tremor score. Patients with cerebral palsy showed mean improvements of 66.7% in movement scores and 50.8% in disability scores. No mortality or major morbidity was observed postoperatively. CONCLUSION: Stereotactic radiofrequency unilateral combined thalamotomy, campotomy, and zona incerta lesions may be an effective surgical alternative for patients with dystonia, especially those with secondary dystonia resistant to deep brain stimulation.

Secondary social anxiety in hyperkinesias.

This is a comparison study that is aimed to investigate and compare the frequency and severity of secondary social anxiety disorder (SAD) in patients with hyperkinesias, which is associated with a significant sense of disfigurement and compromised social interaction. Patients with hemifacial spasm (n = 20), cervical dystonia (n = 20), and essential tremor (n = 20) were evaluated by SCID-I, Liebowitz Social Anxiety Scale, Hamilton Anxiety and Depression Rating Scales, and Sheehan Disability Scale. The DSM-IV H criterion excluding social anxiety related to a medical condition was disregarded for the diagnosis of secondary SAD. The control group (n = 60) consisted of matched healthy subjects. The frequency of the diagnosis and severity of symptoms were compared and associations with sociodemographic and clinical factors were explored. There was no difference between three patient groups in terms of the frequency or the severity of secondary SAD. Younger age and depressive symptoms were associated with the severity of secondary SAD, while severity or duration of the movement disorder or social disability was not. This study revealed a high frequency of secondary SAD in hyperkinesias, emphasizing the need for psychiatric assessment, especially for younger and depressed patients, who seem to be at greater risk.

Re-emergent tremor in Parkinson's disease: Clinical and accelerometric properties.

Re-emergent tremor (RET) and the classical parkinsonian rest tremor were considered as two different phenomena of the same central tremor circuit. However, clinical and accelerometric characteristics of these tremors were not previously compared in a single study. We evaluated disease characteristics and accelerometric measurements of two tremor types in 42 patients with Parkinson's disease. Disease specific features and accelerometric measurements of peak frequency, amplitude at peak frequency and the root mean square (RMS) amplitude of two tremor types were compared. Eighteen patients had RET and the mean latency of the RET was 9.48 (±9.2)s. Groups of only rest tremor and RET did not differ significantly in age of disease onset, disease duration and severity and mean levodopa equivalent dose. Comparison of peak frequency and amplitude at peak frequency were not different between the groups, but RMS amplitude was significantly higher in the RET group (p=0.03). RMS amplitude of RET was also correlated with disease severity (r=.48, p=0.04). These results support the previous notion that rest tremor and RET are analogue, both are triggered by the same central ossilator with RET being only the suppression of the rest tremor due to arm repositioning.

Minimal change nephrotic syndrome with stiff-person syndrome: is there a link?

Stiff-person syndrome is a rare, likely immune-mediated neurological disorder characterized by painful spasms and progressive symmetric rigidity of the axial and proximal limb muscles. Rigidity of truncal muscles and continuous contraction of the agonist and antagonist muscles caused by involuntary motor-unit firing at rest are the hallmarks of stiff-person syndrome. Immunosuppressive therapy has induced remission in patients with stiff-person syndrome. We report a patient with stiff-person syndrome with minimal change nephrotic syndrome (MCNS). The pathophysiologic states of stiff-person syndrome and MCNS are unclear. T-Cell-dependent mechanisms are highly suspected for the pathogenesis of both. The diagnosis of stiff-person syndrome was made on the basis of clinical and laboratory findings, and both MCNS and stiff-person syndrome resolved completely with immunosuppressive therapy. To our knowledge, this is the first case of stiff-person syndrome in association with MCNS in the literature.

Sudden onset of oromandibular dystonia after cerebellar stroke.

BACKGROUND: We present the case of a 65-year-old female with sudden-onset involuntary mouth opening, deviation of the jaw, facial grimacing, and tongue movements that started 6 months prior to her admission. CASE REPORT: She was diagnosed with oromandibular dystonia. Differential diagnosis of oromandibular dystonia and various etiologies were investigated. Neuroimaging studies revealed a left cerebellar infarction. DISCUSSION: To our knowledge, this case is the first oromandibular dystonia presenting with cerebellar ischemic stroke. Possible roles of the cerebellum for the pathophysiology of oromandibular dystonia are discussed.

Management of a hemidystonic patient with thalamotomy, campotomy and cervical dorsal root entry zone operation.

Several medical and surgical procedures have been presented for treatment of dystonia. Thalamotomy, pallidotomy, and campotomy are some of the surgical choices. This study presents a patient with dystonia who underwent a cervical dorsal root entry zone (DREZ) operation after thalamotomy and campotomy. A 23-year-old man who was resistant to medical treatment presented with left hemidystonia. Thalamotomy and campotomy were performed. The patient remarkably benefited from the procedure but dystonic complaints in his left arm continued. A cervical DREZ operation was performed 5 years after the first operation and the dystonic complaints decreased after the surgery. This article presents a new aspect for the treatment of dystonia. Based on the outcomes of the treatment, DREZ operation may be suggested as an alternative surgical treatment for patients with segmental dystonia located in the extremities.

Idiopathic localized crossed (left side of the upper part of the body, right side of the lower part of the body) hyperhidrosis: successful treatment of facial area with botulinum a toxin injection.

BACKGROUND: Localized hyperhidrosis either may be due to an organic cause or may be idiopathic. Most cases of localized hyperhidrosis are reported to be idiopathic and unilateral. OBJECTIVE AND RESULT: We report a case of idiopathic localized hyperhidrosis with extensive involvement on crossed sides of the body. The hyperhidrotic area on the forehead was treated with a single session of botulinum A toxin injection with a quite successful result, and the area remained free of symptoms during a follow-up period of 5 months. CONCLUSION: All documented cases of idiopathic localized hyperhidrosis are located on one side of the body. To the best of our knowledge, idiopathic localized crossed hyperhidrosis has not previously been reported. The botulinum A toxin injection produced a rapid and successful result on the facial area.