Journal of Clinical Neurology最新文献

Vertigo and dizziness are amongst the most frequent presenting symptoms in the emergency room, accounting for up to 4% of all emergency consultations. The broadness of their differential diagnosis and the often transient nature of these symptoms pose a significant challenge to the treating physician. Combining various subtle oculomotor signs at the bedside has been very successful in distinguishing peripheral from central causes in acutely dizzy patients meeting diagnostic criteria for the acute vestibular syndrome (i.e., acute and prolonged vertigo or dizziness accompanied by nausea or vomiting, gait imbalance, motion intolerance, and [not mandatory] nystagmus). While the diagnostic accuracy of the HINTS (Head-Impulse-Nystagmus-Test-of-Skew) algorithm has been studied extensively, less is known about the value of various nystagmus patterns seen at the bedside in patients with an acute vestibular syndrome. Here we review both spontaneous and triggered presenting nystagmus patterns and discuss their impacts and limitations, including primary-gaze horizontal, vertical, and torsional nystagmus, nystagmus during eccentric gaze, and nystagmus triggered by stimuli such as head-shaking, hyperventilation, positional testing, vibration, and the Valsalva maneuver. We conclude that the usefulness of nystagmus patterns in discriminating peripheral and central causes strongly depends on the pattern seen and the type of testing performed, being highly predictive of a central cause for torsional and vertical spontaneous nystagmus, downbeat, or apogeotropic horizontal and treatment-refractory positional nystagmus. The predictive value for central causes was moderate only for vertical nystagmus after horizontal head-shaking ("perverted" head-shaking nystagmus) since it can also occur in peripheral cases, while the predictive value was low for vibration-induced nystagmus and Valsalva-induced nystagmus.

Background and purpose: Guillain-Barré syndrome (GBS) is an autoimmune-mediated disorder characterized by demyelinating or axonal injury of the peripheral nerve. Our aim is to determine whether serum amyloid A (SAA) is a biomarker of demyelinating injury and disease severity in patients with GBS.

Methods: This study retrospectively enrolled 40 patients with either the demyelinating or axonal GBS and sex- and age-matched controls with other neurological diseases as well as healthy subjects. The demographic and clinical features at entry were collected. The serum levels of the SAA isoforms SAA1, SAA2, and SAA4 were determined in the patients with GBS and the controls using the enzyme-linked immunosorbent assay and analyzed for the associations between levels of different SAA isoforms and the clinical features of the patients.

Results: The levels of SAA2 and SAA4 were significantly higher in patients with GBS than in both the other neurological disease controls and the healthy subjects (p<0.05 for all). The level of SAA1 did not differ between patients with GBS and the controls. The level of SAA2 was considerably higher in GBS patients with antecedent infection than in those without infection (p=0.020). The levels of different SAA isoforms were not associated with the disease severity or other clinical features of patients with GBS (p>0.05 for all).

Conclusions: Increased levels of SAA2 and SAA4 may only represent the acute inflammatory status and so cannot be utilized as biomarkers of the disease severity or demyelinating injury in patients with GBS.

Background and purpose: The National Brain Biobank of Korea (NBBK) is a brain bank consortium supported by the Korea Disease Control and Prevention Agency and the Korea National Institute of Health, and was launched in 2015 to support research into neurodegenerative disease dementia (NDD). This study aimed to introduce the NBBK and describes clinicopathological correlations based on analyses of data collected from the NBBK.

Methods: Four hospital-based brain banks have been established in South Korea: Samsung Medical Center Brain Bank (SMCBB), Seoul National University Hospital Brain Bank (SNUHBB), Pusan National University Hospital Brain Bank (PNUHBB), and Myongji Hospital Brain Bank (MJHBB). Clinical and pathological data were collected from these brain banks using standardized protocols. The prevalence rates of clinical and pathological diagnoses were analyzed in order to characterize the clinicopathological correlations.

Results: Between August 2016 and December 2023, 185 brain specimens were collected and pathologically evaluated (SNUHBB: 117; PNUHBB: 27; SMCBB: 34; MJHBB: 7). The age at consent was 70.8±12.6 years, and the age at autopsy was 71.7±12.4 years. The four-most-common clinical diagnoses were Alzheimer's disease (AD) dementia (20.0%), idiopathic Parkinson's disease (15.1%), unspecified dementia (11.9%), and cognitively unimpaired (CU) (11.4%). Most cases of unspecified dementia had a pathological diagnosis of central nervous system (CNS) vasculopathy (31.8%) or AD (31.8%). Remarkably, only 14.2% of CU cases had normal pathological findings. The three-most-common pathological diagnoses were AD (26.5%), CNS vasculopathy (14.1%), and Lewy body disease (13.5%).

Conclusions: These clinical and neuropathological findings provide a deeper understanding of the mechanisms underlying NDD in South Korea.

Background and purpose: The incidence of multiple sclerosis (MS) among older patients is increasing. Some of these patients develop the disease after the age of 50 years, a condition known as late-onset MS (LOMS). This study aimed to characterize MS in older patients (50-75 years-old) by comparing LOMS with adult-onset MS (AOMS).

Methods: We retrospectively analyzed data from 230 patients aged 50-75 years who attended a Portuguese tertiary referral center.

Results: This study included 189 AOMS patients aged 58 [54-63] years (median [interquartile range]) and 41 LOMS patients aged 67 [61-70] years. Females predominated in both the LOMS (70.7%) and AOMS (75.1%) groups. Primary progressive MS was more common in LOMS than AOMS patients (19.5% vs. 8.0%, p=0.03) and these two groups had equivalent proportions of relapsing-remitting MS (53.7% vs. 59.0%, p=0.55). The Expanded Disability Status Scale (EDSS) score at the diagnosis was higher in the LOMS patients (2 [1-4], p=0.03), but the current EDSS score did not differ significantly between the LOMS and AOMS patients (3.5 [1.75-6] vs. 3 [1.5-6], p=0.86). After adjusting or matching for age and disease duration, the current EDSS scores were not significantly different in the two groups. The proportion of patients currently receiving disease-modifying therapies was higher in LOMS patients (97.6%, p=0.02). A higher proportion of patients with a later onset had infratentorial involvement at a 5-year follow-up (86.7%, p=0.01). The time to an EDSS score of 6.0 was shorter for LOMS patients.

Conclusions: The LOMS patients presented with higher EDSS scores at the diagnosis, reaching a level of disability not significantly different from AOMS patients of the same age group despite a shorter disease course.

Background and purpose: The pain lateralization in cluster headache (CH) may be related to the asymmetry in the functions of the brain hemispheres. The right-sided dominance of pain in CH has been found inconsistently across studies, and so we aimed to characterize this and identify the factors influencing pain lateralization during current and previous bouts.

Methods: This study enrolled 227 patients from the Korean Cluster Headache Registry between October 2018 and December 2020. We evaluated the side of pain during current and previous bouts, demographic features, and clinical characteristics, including handedness. Multivariable logistic regression analyses were performed to identify factors associated with the side of pain.

Results: The 227 patients with CH included 131 (57.7%) with right-sided pain and 86 (37.9%) with left-sided pain during the current bout (p<0.001). The 189 patients with previous bouts of CH included 86.8% who consistently reported the same side of pain throughout multiple bouts (side-locked pain), with a higher prevalence of pain on the right than the left side (55.0% vs. 31.7%, p<0.001). Multivariable analyses revealed that higher age at diagnosis (odds ratio [OR]=1.045, p=0.031) and shorter CH attacks (OR=0.992, p=0.017) were associated with left-side-locked pain. However, handedness was not associated with the lateralization of left-side-locked pain.

Conclusions: This study has confirmed the predominance of right-sided pain throughout multiple CH bouts. We found that higher age at diagnosis and shorter CH attacks were associated with left-side-locked pain, suggesting that certain clinical factors are associated with the pain laterality. However, the underlying mechanisms linking these factors to lateralized pain remain unclear and therefore require further investigation.