Journal of Clinical Neurology最新文献

Amyloid positron-emission tomography (PET) is the optimal method for detecting amyloid plaque deposition in patients experiencing cognitive decline, which is essential for diagnosing Alzheimer's disease. However, its clinical application globally has been restricted by the high cost, short radiotracer half-life, and significant accessibility challenges. In particular, the lack of treatment options following diagnosis has been considered the largest obstacle to using amyloid PET as a diagnostic tool. Consequently, the current appropriate-use recommendations for amyloid PET tend to support restricting its use. However, the relatively low cost and superior accessibility of amyloid PET in South Korea have resulted in it being used much more frequently in clinical settings than in other countries. The recent introduction of disease-modifying drugs has increased the importance and frequency of amyloid PET usage. Considering these circumstances, this article presents expert opinions on the appropriate use of amyloid PET in South Korea based on existing recommendations and survey results from dementia experts in South Korea.

Background and purpose: To evaluate the clinical efficacy and safety of pregabalin in Korean adults with restless legs syndrome (RLS).

Methods: In this randomized, multicenter, double-blind, placebo-controlled trial, 78 patients with RLS with an International Restless Legs Scale (IRLS) score ≥15 were randomized 1:1 to receive either pregabalin (n=39) or placebo (n=39) for 12 weeks. The primary efficacy outcome was the change in the IRLS score, and the secondary outcomes included the Clinical Global Impression-Improvement scale and changes in scores on other RLS symptom questionnaires. Safety was assessed by monitoring treatment-emergent adverse events (TEAE). This study was registered at ClinicalTrials.gov (NCT04161027).

Results: At baseline, the mean IRLS scores were 23.2±5.8 in the pregabalin and 24.5±5.2 in the placebo group (p=0.297). After 12 weeks, the baseline-adjusted change in the IRLS score was -6.8 (95% confidence interval [CI] -9.3 to -4.3) in the pregabalin group and -5.4 (95% CI -7.9 to -2.8) in the placebo group, with no significant difference between the groups (p=0.420). The secondary efficacy outcomes did not differ between the two groups. The incidence of TEAE was similar between the two groups (48.7% vs. 51.3%, p=0.821), with dizziness being the most common TEAE.

Conclusions: This study failed to demonstrate the therapeutic effect of pregabalin compared with placebo for RLS in Korean adults. Possible reasons for the negative results include low dose, insufficient sample size, and substantial placebo response. Further investigations are warranted to optimize pregabalin therapy in Korean adults with RLS.

Structural magnetic resonance imaging (sMRI) plays a pivotal role in the evaluation of neurological disorders by providing high-resolution anatomical information. Recent advances in quantitative postprocessing techniques have expanded the utility of sMRI beyond visual assessments by enabling the detection of subtle morphological changes associated with various neurological and psychiatric conditions. This review summarizes current clinical applications of sMRI-based analysis, including brain volumetry, shape analysis, voxel-based morphometry (VBM), surface-based morphometry, source-based morphometry, and voxel-based lesion-symptom mapping (VLSM). Volumetric and shape-based analyses allow for assessments of region-specific atrophy and subregional morphological alterations, while VBM and surface-based morphometry provide complementary insights into tissue volumes and the architecture of the cortical surface. Source-based morphometry reveals network-level patterns of structural covariance, and VLSM directly correlates lesion locations with functional outcomes, particularly in stroke. It has been demonstrated that these methodologies are clinically relevant in conditions such as Alzheimer's disease, Parkinson's disease, multiple sclerosis, epilepsy, and major depressive disorder. By quantifying structural brain alterations that are not readily detectable using conventional imaging methods, these tools improve diagnostic accuracy, support prognostication, and facilitate monitoring of treatment effects. This review highlights the growing integration of sMRI postprocessing techniques into clinical neurology.

Background and purpose: We aimed to determine efficacy, safety, and predictors of response and side effects of lasmiditan in Korean patients with migraine.

Methods: We prospectively recruited patients who took lasmiditan at the Seoul National University Hospital between April 2023 and March 2024. We collected data on treatment, adverse events, and dosage adjustment profiles. Tolerability was defined as dosage maintenance or dosage increase. Logistic regression analyses were performed to identify predictors of a response to lasmiditan and adverse events.

Results: This study included 154 patients. At visit 1, 110 (71.4%) and 44 (28.6%) patients were prescribed 50 and 100 mg of lasmiditan daily, respectively, with a treatment response observed at visit 2 in 49 (44.5%) and 20 (45.5%) patients, respectively. Adverse events were reported in 75 (48.7%) patients. Multivariate logistic analyses showed that female sex (odds ratio [OR]= 4.51, 95% confidence interval [CI]=1.43-14.19, p=0.01) and higher daily dose (100 mg vs. 50 mg: OR=2.35, 95% CI=1.14-4.83, p=0.02) were independently associated with adverse events. Dosage increase, dosage reduction, and treatment discontinuation occurred in 56 (36.4%), 17 (11.0%), and 40 (26.0%) patients, respectively. Lasmiditan was well tolerated by 97 (63.0%) patients.

Conclusions: This was the first real-world study of lasmiditan in Korean patients with migraine. Although administered at a low dosage, lasmiditan was effective in approximately half of the patients. Dizziness was the most common adverse event, and it occurred at a higher rate than in clinical trials.

Traumatic brain injury (TBI) is one of the main mechanisms underlying health issues associated with functional and structural brain changes. While direct effects such as cognitive and physical impairments are well documented, recent research has linked TBI to neurodegenerative changes similar to dementia. TBI-related neurodegeneration includes progressive brain-tissue degeneration that leads to behavioral changes, cognitive decline, and dementia-like symptoms. The exact underlying mechanisms are complex, and include neuroinflammation, oxidative stress, excitotoxicity, and disruption to protein homeostasis. Neuroinflammation is controlled by the activation of astrocytes and microglia and causes neuronal damage and the prolonged release of proinflammatory cytokines. Oxidative stress damages cell and impairs mitochondrial function, while the accumulation of misfolded proteins such as tau and β-amyloid mimics the pathology of Alzheimer's disease. Excitotoxicity involves excessive neurotransmitter release that may lead to further injuries. Epidemiological studies show that the risk of dementia is increased after moderate-to-severe TBI and influenced by age and genetic factors. Current management strategies focus on symptom relief, and there is ongoing research aimed at improving the understanding of the underlying mechanisms and the development of effective treatments.

Background and purpose: Neuronal intranuclear inclusion disease (NIID) is a progressive neurodegenerative disease characterized by a wide range of clinical manifestations. GGC-repeat expansion in NOTCH2NLC was recently identified as the genetic cause of NIID. Here we report clinical, radiological, pathological, and genetic findings in NIID patients.

Methods: Twenty-five NIID patients from 22 unrelated families of Korean ancestry were reviewed from 9 referral centers in South Korea. We compared clinical features between sporadic and familial NIID patients. We classified NIID patients according to their prominent symptoms. The presence of GGC repeat expansion was analyzed in 19 patients.

Results: The 25 reviewed NIID patients comprised 12 (48.0%) sporadic and 13 (52.0%) familial cases, with the latter showing a significantly higher proportion of males (p=0.027). The patients were classified into three subtypes based on the prominent symptoms: NIID-Episodic (44.0%), NIID-EPS (extrapyramidal symptoms) (36.0%), and NIID-Dementia (20.0%). Most patients (92.0%) also exhibited other symptoms, including peripheral neuropathy (60.0%), bladder dysfunction (48.0%), or ophthalmic problems (56.0%). Hyperintensities along the corticomedullary junctions in diffusion-weighted imaging and extensive white-matter hyperintensities in fluid-attenuated inversion-recovery imaging were observed in 96.0% and 100% of the patients, respectively. GGC repeat expansion in NOTCH2NLC was identified in 6 sporadic and 10 familial cases. The number of GGC repeats was not correlated with the onset age or clinical symptoms.

Conclusions: This study has highlighted the diverse phenotypes and genetic profiles of Korean NIID patients, and provided valuable insights into this rare disorder.