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 leftside-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.

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 To determine whether the RNF213 p.R4810K mutation modifies the number of moyamoya disease manifestations and recurrent strokes in isolated intracranial arterial steno-occlusive disease (ICAD). Methods: This retrospective case–control study analyzed patients who visited the Asan Medical Center with steno-occlusive lesions in the M1 segment of the middle cerebral artery and terminal internal carotid artery, and underwent RNF213 genetic testing for screening moyamoya disease between January 2010 and November 2022. Patients with supportive findings of moyamoya disease or moderate-to-severe stenosis in the extracranial arteries were excluded.After matching antiplatelet drugs, the presentation of moyamoya disease and stroke recurrence were analyzed using chi-squared analysis and Kaplan–Meier survival curve analysis. Results: The 1,567 patients who underwent evaluations of RNF213 polymorphisms included 753 with ICAD, among whom females predominated (n=452, 60.0%) and 289 (38.4%) had an RNF213 mutation. The follow-up period was 2.47±3.51 years (mean±standard deviation; median=1.00 year, interquartile range=0–4 years). The risk of progression to moyamoya disease was higher in the RNF213-related-vasculopathy group than the RNF213-negative stenosis group (n=27 [9.3%] versus n=6 [1.3%], p<0.01), as were the risks of ischemic stroke (n=13 [4.5%] versus n=7 [1.5%], p=0.01) and hemorrhagic stroke (n=5 [1.7%] versus n=1 [0.2%], p=0.02, respectively). Furthermore, the presence of an RNF213 mutation was significantly associated with the risk of stroke recurrence (odds ratio=2.34, 95% confidence interval=1.44–3.80, p< 0.01). Conclusions Evaluations of RNF213 polymorphisms may help to identify patients with isolated ICAD at a high risk of progression to moyamoya disease and stroke.

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 headshaking, 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.