In the early stages or atypical manifestation of parkinsonism, dopamine transporter imaging can assist the early diagnosis. We describe a 19 year-old man presenting with progressive gait disturbance, cervical dystonia and head tremor. 18F-FP-CIT PET (FluoroPropyl-Carbomethoxylodopropyl-nor-BTropane positron emission tomography) was done and interpreted as normal at other hospital, and his diagnosis remained baffling. He visited our hospital several months later, and the FP-CIT PET image was reviewed by the nuclear medicine physician in our hospital, who also interpreted it as normal. However, we reviewed his FP CIT-PET image because his clinical picture was strongly suggestive of juvenile parkinsonism. After adjusting the window setting of the PET image, we could appreciate the decreased uptake in the bilateral basal ganglia. Thus he was finally diagnosed as juvenile parkinsonism and gene test confirmed Park2 gene mutation. In conclusion, proper window setting is important during visual assessment of dopamine transporter imaging.
Parkinson Disease

Traditionally, schizophrenia is considered to be a result of dopaminergic hyperactivity while dopaminergic deficiency underlies Parkinson’s disease (PD). This opposing pathophysiology makes comorbid schizophrenia and PD seemingly impossible; however, they do coexist rarely in clinical practice. We present four patients with paranoid schizophrenia diagnosed in their youth who developed parkinsonian symptoms on a stable regimen of quetiapine or clozapine after several years. The diagnosis of comorbid schizophrenia and PD was made mainly according to clinical observation. In addition, dopamine transporter (DAT) imaging with 18F-FP-CIT PET was done in two patients, which showed normal DAT density. It is believed that dopaminergic dysfunction in distinct dopaminergic pathways may explain the coexistence of these two disorders

OBJECTIVE: Many people dealing with Parkinson’s disease (PD) turn to complementary and alternative medicine when searching for a cure or relief from symptoms. Acupuncture is widely used in the Korean PD population to alleviate symptoms and in hopes of curing the illness. However, acupuncture use for PD patients has only recently begun to be studied scientifically and is still considered an unproven treatment for PD. Therefore, there is an urgent need for acupuncture to be studied, validated and used for PD. Thus, our study’s aim is to examine how many acupuncture studies in PD are registered and reported in Korea. METHODS: The registries Clinicaltrials.gov and the Clinical Research Information Service (CRIS) and the search engine PubMed were searched to find relevant human clinical studies involving acupuncture therapy in PD patients. We examined the registration of trials, the posting and publication of results, and whether published articles were registered. RESULTS: In Clinicaltrials.gov, one completed trial was found with published results. In CRIS, one completed trial was found with published results. A total of 6 publications were found in our study: 2 articles were registered, but only 1 had the registered trial number listed in the article. CONCLUSION: Acupuncture is popular among the PD population in Korea regardless of its unproven safety and efficacy. Despite the pressing need for clinical trials, the number of studies listed in the registries was small, and only a few publications were registered. More effort and rigor are needed to validate the efficacy and safety of acupuncture for PD.
Acupuncture Therapy ; Acupuncture* ; Complementary Therapies ; Hope ; Humans ; Information Services ; Korea* ; Parkinson Disease* ; Publications ; Registries ; Search Engine

Previously, we defined DRD as a syndrome of selective nigrostriatal dopamine deficiency caused by genetic defects in the dopamine synthetic pathway without nigral cell loss. DRD-plus also has the same etiologic background with DRD, but DRD-plus patients have more severe features that are not seen in DRD because of the severity of the genetic defect. However, there have been many reports of dystonia responsive to dopaminergic drugs that do not fit into DRD or DRD-plus (genetic defects in the dopamine synthetic pathway without nigral cell loss). We reframed the concept of DRD/DRD-plus and proposed the concept of DRD look-alike to include the additional cases described above. Examples of dystonia that is responsive to dopaminergic drugs include the following: transportopathies (dopamine transporter deficiency; vesicular monoamine transporter 2 deficiency); SOX6 mutation resulting in a developmentally decreased number of nigral cells; degenerative disorders with progressive loss of nigral cells (juvenile Parkinson's disease; pallidopyramidal syndrome; spinocerebellar ataxia type 3), and disorders that are not known to affect the nigrostriatal dopaminergic system (DYT1; GLUT1 deficiency; myoclonus-dystonia; ataxia telangiectasia). This classification will help with an etiologic diagnosis as well as planning the work up and guiding the therapy.
Ataxia ; Classification ; Diagnosis ; Dopamine ; Dopamine Agents ; Dystonia* ; Humans ; Parkinson Disease ; Spinocerebellar Ataxias ; Vesicular Monoamine Transport Proteins

BACKGROUND AND PURPOSE: Research on stem cells (SC) is growing rapidly in neurology, but clinical applications of SC for neurological disorders remain to be proven effective and safe. Human clinical trials need to be registered in registries in order to reduce publication bias and selective reporting. METHODS: We searched three databases—clinicaltrials.gov, the Clinical Research Information System (CRIS), and PubMed—for neurologically relevant SC-based human trials and articles in Korea. The registration of trials, posting and publication of results, and registration of published SC articles were examined. RESULTS: There were 17 completed trials registered at clinicaltrials.gov and the CRIS website, with results articles having been published for 5 of them. Our study found 16 publications, of which 1 was a review article, 1 was a protocol article, and 8 contained registered trial information. CONCLUSIONS: Many registered SC trials related to neurological disorders are not reported, while many SC-related publications are not registered in a public registry. These results support the presence of biased reporting and publication bias in SC trials related to neurological disorders in Korea.
Bias (Epidemiology) ; Humans ; Information Systems ; Korea ; Nervous System Diseases* ; Neurology ; Publication Bias ; Publications ; Registries ; Stem Cells*

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.

BACKGROUND: Pregnancy considerably alters cardiovascular dynamics, and thereby affects the transition of blood pressure after delivery in women. We aimed to analyze the association between parity and blood pressure in Korean adult women. METHODS: We included 8,890 women who participated in Korean National Health and Nutrition Examination Survey between 2010 and 2012. We divided the population according to the menopause status and analyzed the association between parity and blood pressure by using multiple regression analysis, and on hypertension, by using logistic regression analysis. RESULTS: Systolic and diastolic blood pressures were significantly associated with parity in premenopausal women (beta=-0.091 [P<0.001] and beta=-0.069 [P<0.001], respectively). In the analysis that excluded women receiving antihypertensive medication, the systolic and diastolic blood pressure of postmenopausal women were significantly associated with parity (beta=-0.059 [P=0.022] and beta=-0.054 [P=0.044], respectively). Parity was found to prevent hypertension after adjustment for confounders in postmenopausal women (odds ratio, 0.55; 95% confidence interval, 0.310-0.985). CONCLUSION: We found that parity prevented hypertension in Korean women.
Adult ; Blood Pressure* ; Female ; Humans ; Hypertension ; Logistic Models ; Menopause ; Nutrition Surveys* ; Parity* ; Pregnancy

Some studies have demonstrated that chemotherapy drugs enhance sensitivity to anesthetics owing to its systemic toxicity, while others have demonstrated that chemotherapy drugs have no effect. This study aimed to determine whether neoadjuvant chemotherapy influences the effect-site concentration (Ce) of propofol for sedation in patients withbreast cancer.Methods: This study included patients aged 19–75 years who were scheduled to undergobreast cancer surgery under general anesthesia. Patients who received neoadjuvant chemotherapy were assigned to group C, whereas those who never received chemotherapy wereassigned to group N. Propofol was administered through an effect-site target-controlled infusion, and the Modified Observer’s Assessment of Alertness/Sedation scale (MOAA/S) scoreand Bispectral Index (BIS) were recorded. When the plasma concentration and Ce wereequal to the target Ce, and if the MOAA/S score did not change, the target Ce was increasedby 0.2 μg/ml; otherwise, the Ce was maintained for 2 min and then increased. This processwas repeated until the MOAA/S score became 0.Results: No significant differences were observed in Ce values at each sedation level between both groups. Ce values for loss of consciousness (LOC) of groups C and N were 2.76± 0.29 and 2.67 ± 0.27 μg/ml (P = 0.285), respectively. However, the BIS value at LOC ofgroup C (63.87 ± 7.04) was lower than that (68.44 ± 6.01) of group N (P = 0.018).Conclusions: Neoadjuvant chemotherapy for breast cancer has no effect on the Ce ofpropofol for sedation.

Background: This study aimed to investigate 1) long-term outcomes of deep brain stimulation (DBS), such as mortality after DBS as well as the causes of death, 2) demographic and socioeconomic factors influencing mortality, and 3) comorbidities affecting mortality after DBS in patients with Parkinson’s disease (PD). Methods: This study analyzed the National Health Insurance Service-National Health Information Database. Data on patients with PD diagnosis codes from 2002 to 2019 were extracted and analyzed. Data on the causes of death were obtained by linking the causes of death to data from Statistics Korea. The Kaplan-Meier method with the log-rank test was used for survival analysis. Multivariate Cox regression analyses were used to estimate hazard ratios (HRs) and their 95% confidence intervals. Regarding comorbidities such as PD dementia and fracture, which did not satisfy the assumption for the proportional HR, timedependent Cox analysis with the Mantel-Byar method was used. Results: From 2005 to 2017, among 156,875 patients diagnosed with PD in Korea, 1,079 patients underwent DBS surgery, and 251 (23.3%) had died by 2019. The most common cause of death (47.1%) was PD. In the multivariate Cox regression analysis, the higher the age at diagnosis and surgery, the higher the mortality rate. The men and medical aid groups had significantly higher mortality rates. PD dementia and fracture were identified as risk factors for mortality. Conclusion Older age at diagnosis and surgery, being male, the use of medical aid, and the comorbidity of dementia and fractures were associated with a higher risk of mortality after DBS in patients with PD. Neurologists should consider these risk factors in assessing the prognosis of PD patients undergoing DBS.