Survey of the Knowledge, Attitudes, and Practices of Neurologists Regarding Exercise in Parkinson’s Disease

Background@#and Purpose Exercise and physiotherapy can exert potentially beneficial effects on the motor and nonmotor features of Parkinson’s disease (PD). We conducted an email survey to assess the knowledge, attitudes, and practices of neurologists regarding exercise among patients with PD. @*Methods@#A total of 222 neurologists from the Korean Movement Disorder Society and the Korean Society of Neurologists completed the survey and were classified into 4 clusters using the k-means clustering algorithm based on their institute types, the proportions of PD patients in their clinics, and the number of years working as neurologists. @*Results@#Specialists working at referral hospitals (Clusters 1 and 2) were more confident than general neurologists (Clusters 3 and 4) about exercise improving the general motor features of PD. Specialists recommended more-frequent intense exercise compared with physicians not working at referral hospitals. The specialists in Cluster 1, representing >50% of PD patients in the clinics at referral hospitals, recommended exercise regardless of the disease stage, whereas the general neurologists in Clusters 3 and 4 recommended low-intensity exercise at an early stage of disease. Although most of the respondents agreed with the need for PD patients to exercise, less than half had prescribed rehabilitation or physiotherapy. More than 90% of the respondents answered that developing an exercise/physiotherapy protocol for PD would be helpful. @*Conclusions@#Specialists were more confident than general neurologists about the effect of exercise and recommended more-intense activities regardless of the disease stage. These results highlight the need to develop clinical practice guidelines and PD-specialized exercise protocols to provide optimal care for PD patients.

Trends in Physiotherapy Interventions and Medical Costs for Parkinson’s Disease in South Korea, 2011–2020

Objective@#Physiotherapy (PT), which is an effective strategy for managing Parkinson’s disease (PD), can influence health care utilization. We analyzed trends in health care utilization, PT interventions, and medical costs among patients with PD. @*Methods@#Using data from the Korean National Health Insurance Service from 2011 to 2020, we analyzed the number of patients with PD and their health care utilization and assessed the odds ratio (OR) for receiving regular PTs. @*Results@#Over 10 years, 169,613 patients with PD were included in the analysis. The number of patients with PD increased annually from 49,417 in 2011 to 91,841 in 2020. The number of patients with PD receiving PT increased from 4,847 (9.81%) in 2011 to 13,163 (14.33%) in 2020, and the number of PT prescriptions increased from 81,220 in 2011 to 377,651 in 2019. Medical costs per patient with PD increased from 1,686 United States dollars (USD) in 2011 to 3,202 USD in 2020. The medical expenses for each patient with PD receiving PT increased from 6,582 USD in 2011 to 13,475 USD in 2020. Moreover, regular PTs were administered to 31,782 patients (18.74%) and were administered only through hospitalization. Those patients in their 50s with disabilities demonstrated a high OR for regular PTs, whereas those aged 80 years or older and residing outside of Seoul had a low OR. @*Conclusion@#The PD burden increased in South Korea between 2011 and 2020, as did health care utilization and medical costs. A significant increase in medical expenses can be associated with increased PD incidence and PT interventions. Regular PT applications remain restricted and have barriers to access.

Parainfectious Anti-Glial Fibrillary Acidic Protein-Associated Meningoencephalitis

Movement disorders associated with glial fibrillary acidic protein (GFAP) autoantibodies have rarely been reported as ataxia or tremors. A 32-year-old man with headache and fever, initially diagnosed with viral meningoencephalitis, showed gradual improvement with empirical treatment. Two weeks after the illness, he suddenly developed orofacial, tongue, and neck dyskinesia accompanied by oculomotor abnormalities, which developed into severe generalized choreoballism. Brain magnetic resonance imaging (fluid-attenuated inversion recovery) showed signal hyperintensities in the bilateral globus pallidus interna. The clinical picture suggested an acute inflammatory trigger of secondary autoimmune encephalitis. The autoimmune antibody test was positive for GFAP, with the strongest reactivity in the cerebrospinal fluid (CSF) before treatment and decreased reactivity in serial CSF examinations during immunotherapy. Dyskinesia gradually improved to the extent that it could be controlled with only oral medications. This patient presented with parainfectious GFAP meningoencephalitis with distinctive clinical features and imaging findings.

Investigation of Nocturnal Hypokinesia and Health-Related Quality of Life in Parkinsonian Patients with the Korean Version of the Nocturnal Hypokinesia Questionnaire

Objective@#To assess nocturnal hypokinesia using the Korean version of the Nocturnal Hypokinesia Questionnaire (NHQ-K) in Parkinson’s disease (PD) patients across disease stages. @*Methods@#We developed the NHQ-K and performed questionnaire-based interviews with 108 PD patients from three referral hospitals. Clinical associations of nocturnal hypokinesia and its impact on health-related quality of life (HRQoL) were also analyzed. @*Results@#The NHQ-K showed acceptable internal consistency (0.83) and interrater reliability (0.95). Nocturnal hypokinesia significantly affected HRQoL in PD patients at both the early and advanced stages (adjusted p < 0.001). Increased severity of nocturnal hypokinesia was associated with dyskinesias, off-period disability, apathy, and anxious mood in PD patients (adjusted p < 0.01) after controlling for disease severity and medication dose. @*Conclusion@#The NHQ-K is useful for screening nocturnal hypokinesia in PD patients. Given the high impact of nocturnal hypokinesia on HRQoL, comprehensive management of nocturnal disability is needed for PD patients.

Investigation of Nocturnal Hypokinesia and Health-Related Quality of Life in Parkinsonian Patients with the Korean Version of the Nocturnal Hypokinesia Questionnaire

Objective@#To assess nocturnal hypokinesia using the Korean version of the Nocturnal Hypokinesia Questionnaire (NHQ-K) in Parkinson’s disease (PD) patients across disease stages. @*Methods@#We developed the NHQ-K and performed questionnaire-based interviews with 108 PD patients from three referral hospitals. Clinical associations of nocturnal hypokinesia and its impact on health-related quality of life (HRQoL) were also analyzed. @*Results@#The NHQ-K showed acceptable internal consistency (0.83) and interrater reliability (0.95). Nocturnal hypokinesia significantly affected HRQoL in PD patients at both the early and advanced stages (adjusted p < 0.001). Increased severity of nocturnal hypokinesia was associated with dyskinesias, off-period disability, apathy, and anxious mood in PD patients (adjusted p < 0.01) after controlling for disease severity and medication dose. @*Conclusion@#The NHQ-K is useful for screening nocturnal hypokinesia in PD patients. Given the high impact of nocturnal hypokinesia on HRQoL, comprehensive management of nocturnal disability is needed for PD patients.

Alternating Hemiplegia of Childhood in Korea: a Case Report

Alternating hemiplegia of childhood (AHC) is a rare neurodevelopmental disorder characterized by recurrent paroxysmal hemiplegic attacks that affect one or the other side of the body. Up to 74% of patients with AHC have a pathologic variant in the ATP1A3 gene. After the introduction of next-generation sequencing, intermediate cases and atypical cases have expanded the clinical spectrum of ATP1A3-related disorders. Herein, we report the first case of AHC in Korea. A 33-year-old man visited our hospital with recurrent hemiplegic and dystonic episode after his first birthday. He was completely normal between episodes and did not have any ataxia, but brain magnetic resonance imaging showed cerebellar atrophy. He also had pes planovalgus deformity. Whole exome sequencing revealed a heterozygous G947R variant in the ATP1A3 gene (c.2839G > C, rs398122887), which is a known pathologic variant. This atypical case of AHC demonstrates the importance of the clinical approach in diagnosing ATP1A3-related disorders.

Smoking Cessation, Weight Change, and Risk of Parkinson’s Disease: Analysis of National Cohort Data

Background@#and Purpose: To determine whether the postcessation weight gain modifies the protective effect of smoking on the development of Parkinson’s disease (PD). @*Methods@#This nationwide cohort study included 3,908,687 Korean males aged ≥40 years who underwent at least 2 health checkups biennially between 2009 and 2015. They were grouped into current smokers; quitters with body mass index (BMI) increase, maintenance, and decrease; and never smokers. The occurrence of incident PD was tracked, and Cox proportional-hazard models were used to adjust for potential confounding factors. We also analyzed the impact of weight change regardless of smoking status in the study population. @*Results@#There were 6,871 incident PD cases observed during the 13,059,208 person-years of follow-up. The overall risk of PD was significantly lower in quitters than in never smokers [hazard ratio (HR)=0.78, 95% confidence interval (CI)=0.70–0.86]. The risk of PD was still lower in quitters with BMI increase (HR=0.80, 95% CI=0.65–0.98) and in those with BMI maintenance (HR=0.77, 95% CI=0.68–0.87). This tendency was also observed in quitters with BMI decrease (HR=0.76, 95% CI=0.55–1.06), although it was not as robust as in the other two groups.With respect to weight change alone, BMI increase (HR=1.10, 95% CI=1.02–1.18) but not BMI decrease (HR=1.06, 95% CI=0.98–1.14) significantly increased the PD risk compared to BMI maintenance. @*Conclusions@#Postcessation weight gain in males did not offset the protective impact of smoking on PD development, although overall weight gain predicted an increased risk of PD.

Young-Onset Parkinson’s Disease with Impulse Control Disorder Due to Novel Variants of F-Box Only Protein 7

F-box only protein 7 (FBXO7) is a rare monogenic cause of hereditary Parkinson’s disease (PD) with an autosomal recessive mode of inheritance and a broad spectrum of clinical manifestations. Here, we report a de novo PD patient with onset at the age of 28 with novel compound heterozygous variants in the FBXO7 gene (c.1162C>T, p.Gln388X; c.80G>A, p.Arg27His). The clinical features of the patient were problematic impulse control disorder behaviors and pyromania, and pyramidal signs were negative. We describe the novel pathogenic variants of the FBXO7 gene with detailed clinical pictures to report the expanding genotypes and phenotypes of FBXO7-associated parkinsonism.

Successful Pallidal Deep Brain Stimulation in a Patient with Childhood-Onset Generalized Dystonia with ANO3 Mutation

No abstract available.

Intravascular lymphoma as a Potential Cause of Recurrent Embolic Stroke of Undetermined Source

No abstract available.

Spinocerebellar ataxia type 2 presenting with chorea: Korean cases

@#Spinocerebellar ataxia type 2 (SCA2) is an expanded CAG repeat disorder in ATXN2 gene with a wide range of clinical phenotypes. Chorea has been reported as one of extrapyramidal symptoms of SCA2 patients, but has not been reported in Korea. Here, we report two Korean cases of SCA2 presenting with chorea: one showed generalized chorea in young onset SCA2 with 57 CAG repeats, and the other showed mild chorea in the hands in adult onset SCA2 with 40 CAG repeats. This report documents the phenotype of chorea in Korean patients with SCA2

Embolic Infarction with Hypercoagulability Caused by Subclavian Pseudoaneurysm

Traumatic subclavian artery injury is a rare complication of clavicular fracture. An arterial injury can present as a pseudoaneurysm, which can cause a hypercoagulable state, thrombus formation, deep vein thrombosis, and distal embolism. We report a case of subclavian artery pseudoaneurysm that presented as embolic stroke in a 81-year-old man at 2015 1 month after clavicular fracture.

Reversible Cerebral Vasoconstriction Syndrome Presenting with Bilateral Occipital Hemorrhage after the Subcutaneous Injection of Synthetic Gonadotropin Releasing Hormone

Reversible cerebral vasoconstriction syndrome (RCVS) is a heterogeneous group of cerebrovascular disease. The pathophysiology of RCVS is unknown, but a disturbance in cerebral vascular tone is one of hypothesis. Long-term use of Gonadotropin-releasing hormone (GnRH) agonists can induce a pseudomenopausal state in which estrogen production are suppressed. It might lead to reduced arterial relaxation by estrogen withdrawal. We report a case of RCVS after the injection of synthetic analogue of GnRH.

Spinal Myoclonus Responding to Continuous Intrathecal Morphine Pump

Spinal myoclonus is a sudden, brief, and involuntary movement of segmental or propriospinal muscle groups. Spinal myoclonus has occasionally been reported in patients undergoing opioid therapy, but the pathophysiology of opioid-induced myoclonus has not been elucidated yet. Here, we present two patients with spinal segmental myoclonus secondary to ischemic and radiation myelopathy. Conventional medications did not help treat persistent myoclonus in both legs. Continuous intrathecal morphine infusion was implanted for pain control in one patient, which relieved spinal myoclonus entirely. This experience led to the application of this method with a second patient, leading to the same gratifying result. Spinal myoclonus reemerged as soon as the morphine pumps were off, which confirmed the therapeutic role of opioids. In contrast to the opioid-induced myoclonus, these cases show a benefit of opioids on spinal myoclonus, which could be explained by synaptic reorganization after pathologic insults in the spinal cord.