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Background and purpose: Primary lateral sclerosis (PLS) is a neurodegenerative disorder that primarily affects the central motor system. In rare cases, clinical features of PLS may overlap with those of progressive supranuclear palsy (PSP). We investigate neuroimaging features that can help distinguish PLS with overlapping features of PSP (PLS‐PSP) from PSP. Methods: Six patients with PLS‐PSP were enrolled between 2019 and 2023. We compared their clinical and neuroimaging characteristics with 18 PSP–Richardson syndrome (PSP‐RS) patients and 20 healthy controls. Magnetic resonance imaging, 18F‐flortaucipir positron emission tomography (PET), quantitative susceptibility mapping, and diffusion tensor imaging tractography (DTI) were performed to evaluate eight brain regions of interest. Area under the receiver operating characteristic curve (AUROC) was calculated. Results: Five of the six PLS‐PSP patients (83.3%) were male. Median age at symptom onset was 61.5 (52.5–63) years, and all had mixed features of PLS and PSP. Volumes of the pallidum, caudate, midbrain, and cerebellar dentate were smaller in PSP‐RS than PLS‐PSP, providing good discrimination (AUROC = 0.75 for all). The susceptibilities in pallidum, midbrain, and cerebellar dentate were greater in PSP‐RS compared to PLS‐PSP, providing excellent discrimination (AUROC ≥ 0.90 for all). On DTI, fractional anisotropy (FA) in the posterior limb of the internal capsule from the corticospinal tract was lower in PLS‐PSP compared to PSP‐RS (AUROC = 0.86), but FA in the superior cerebellar peduncle was lower in PSP‐RS (AUROC = 0.95). Pallidum flortaucipir PET uptake was greater in PSP‐RS compared to PLS‐PSP (AUROC = 0.74). Conclusions: Regional brain volume, tractography, and magnetic susceptibility, but not tau‐PET, are useful in distinguishing PLS‐PSP from PSP. [ABSTRACT FROM AUTHOR]

Background: Young‐onset multiple system atrophy (YOMSA) is defined as the onset of multiple system atrophy (MSA) before the age of 40 years old. YOMSA is rare and there is much uncertainty of the phenotype and natural history in patients with YOMSA. Objective: The objective is to evaluate the characteristics and disease course of patients with YOMSA. Methods: We retrospectively reviewed medical records of patients with MSA who were evaluated at all Mayo Clinic sites from 1998 to 2021. We identified patients with YOMSA and evaluated clinical characteristics, autonomic function testing results, and disease course. Results: Of 1496 patients with a diagnosis of clinically probable or clinically established MSA, 20 patients had YOMSA. The median age of onset was 39.1 (interquartile range [IQR] = 37.1, 40.1) years; 13 patients (65%) were male. MSA‐parkinsonism was the most common subtype (65%). The median duration of symptom onset to YOMSA diagnosis was 4.9 (IQR = 3.7, 9) years. At the time of medical record review, 17 patients were deceased with a median survival of 8.3 (IQR = 7, 10.9) years. Univariate analysis showed that initial onset of autonomic failure predicted unfavorable survival (hazard ratio = 2.89, P = 0.04) compared to those who presented with motor impairment only at onset. At the time of YOMSA diagnosis, composite autonomic severity score was available in 19 patients with a median of 5 (IQR = 4, 6.5). Conclusions: YOMSA resembles MSA in most aspects including phenotype and prognosis, although the diagnosis is usually delayed. The presence of autonomic failure at symptom onset may be a poor predictor for survival. [ABSTRACT FROM AUTHOR]

Background: Survival and prognostic factors following stroke occurrence differ between world regions. Studies investigating stroke features in the Middle-east region are scarce. We aimed to investigate 1-year survival and related prognostic factors of stroke patients in Central Iran. Materials and Methods: It is an observational analytical study conducted on patients registered in the Persian Registry of Cardiovascular Disease-Stroke (PROVE-Stroke) database. Records of 1703 patients admitted during 2015--2016 with the primary diagnosis of stroke in all hospitals of Isfahan, Iran were reviewed. Information regarding sociodemographic characteristics, clinical presentations, medications, and comorbidities were recorded. The living status of patients after 1 year from stroke was considered as 1-year survival. Results: Among 1345 patients with the final diagnosis of stroke, 970 (72.1%) were alive at the 1 year follow-up and the mean survival time based on Kaplan--Meier procedure was estimated 277.33 days. The hemorrhagic and ischemic types of stroke were reported in 201 (15.0%) and 1141 (84.8%) patients, respectively. Age (hazard ratio [HR] = 1.07, 95% confidence interval [CI] = 1.05--1.09), diabetes (HR = 1.49, 95% CI = 1.07--2.06), history of stroke or transient ischemic attack (HR = 1.81, 95% CI = 1.30--2.52), history of warfarin usage (HR = 1.73, 95% CI = 1.11--2.71), hospital complications of hemorrhage (HR = 3.89, 95% CI = 2.07--7.31), sepsis (HR = 1.78, 95% CI = 1.18--2.68), and hydrocephalus (HR = 3.43, 95% CI = 1.34--8.79), and modified Rankin Scale (mRS) ≥3 at the time of hospital dicharge (HR = 1.98, 95% CI = 1.27--3.07), were predictors of 1-year survival. Conclusion: Predictors of 1-year survival can be categorized into unchangeable ones, such as age, diabetes, previous stroke, and mRS. The changeable factors, such as hospital complications of infection and hemorrhage, guide physicians to pay greater attention to reduce the risk of mortality following stroke. [ABSTRACT FROM AUTHOR]

Background: Treatment options for secondary progressive multiple sclerosis (SPMS) are limitedly investigated. We aimed to compare the efficacy of rituximab (RTX) and glatiramer acetate (GA) in SPMS patients. Method: This open, randomized clinical trial was conducted on 84 SPMS patients, assigned to receive RTX or GA for 12 months. In RTX group, patients received 1 g intravenous RTX primarily and then every 6‐months. In GA group, patients received 40 mg of GA 3‐times/week subcutaneously. We measured EDSS as the primary outcome and neuroimaging findings, relapse rate (RR), and side effects as the secondary outcomes. Results: Seventy‐three patients completed the study (37 and 36 in RTX and GA groups, respectively). The mean EDSS increased from 3.05 ± 1.01 to 4.14 ± 0.91 in RTX group (p < 0.001) and from 3.22 ± 1.20 to 4.60 ± 0.67 in GA group (p < 0.001). No statistically significant difference was observed in EDSS between two groups (F(1, 67) = 3.377; p = 0.071). The number of active lesions in brain and cervical spine decreased with no difference between groups (p > 0.05). Also, RR decreased in both groups without significant difference between them (F(1, 67) = 0.390; p = 0.534). Non‐serious complications were observed in both groups. Conclusion: Neither RTX nor GA affects EDSS in SPMS patients. They are equally effective in the relapse control of these patients. [ABSTRACT FROM AUTHOR]