Subacute Progressive Severe Ataxic Sensory Neuropathy with Sjögren's Syndrome.

We herein report a 79-year-old woman with subacute progressive ataxic sensory neuropathy. The patient's symptoms began with numbness in the lower extremities, which rapidly deteriorated, resulting in gait disturbance and abnormal sensations in the extremities, reaching a peak over a period of approximately two months. Nerve conduction studies revealed pure axonal-type sensory polyneuropathy. The expeditious progression of the disease initially prompted suspicion of Guillain-Barré syndrome or paraneoplastic syndrome. Nevertheless, after comprehensive evaluations, the conclusive diagnosis was confirmed as ataxic sensory neuropathy with Sjögren's syndrome. Intensive immunotherapy was administered; however, it was ineffective in halting disease progression. Consequently, this case underscores the significance of an early comprehensive diagnosis and prompt immunotherapy for ataxic sensory neuropathy associated with Sjögren's.

The prevalence and incidence of multiple sclerosis over the past 20 years in northern Japan.

OBJECTIVES: The prevalence of multiple sclerosis (MS) in East Asia is thought to be lower than in Western countries. Globally, there is a trend of increasing MS prevalence. We investigated the changes in the prevalence and clinical phenotype of MS in the Tokachi province of Hokkaido in northern Japan, from 2001 to 2021. METHODS: Data processing sheets were sent to all related institutions inside and outside the Tokachi area of Hokkaido island in Japan and were collected from April to May 2021. The prevalence according to the Poser's diagnostic criteria for MS was determined on March 31, 2021. RESULTS: In 2021, the crude MS prevalence in northern Japan was 22.4/100,000 (95% confidence interval, 17.6-28.0). The prevalences of MS standardized by the Japanese national population in 2001, 2006, 2011, 2016, and 2021 were 6.9, 11.5, 15.3, 18.5, and 23.3, respectively. The female/male ratio was 4.0 in 2021, increased from 2.6 in 2001. We checked the prevalence using the 2017 revised McDonald criteria, and found only additional male patient who had not fulfilled Poser's criteria. The age- and sex-adjusted incidence of MS per 100,000 individuals increased from 0.09 in 1980-1984 to 0.99 in 2005-2009; since then, it has remained stable. The proportions of primary-progressive, relapsing-remitting, and secondary-progressive MS types in 2021 were 3%, 82%, and 15%, respectively. CONCLUSION: Our results demonstrated a consistent increase in the prevalence of MS among the northern Japanese over 20 years, particularly in females, and consistently lower rates of progressive MS in northern Japan than elsewhere in the world.

An epidemiological evaluation of COVID-19 in La paz, Bolivia.

INTRODUCTION: The Plurinational State of Bolivia (Bolivia) has experienced four major waves of coronavirus disease 2019 (COVID-19) so far. Although the ministry of health has been tracking morbidity and mortality through each wave, epidemiology of COVID-19 in Bolivia is not well defined, despite a need for more accurate measurement of the number of cases and deaths to allow for forecasting of the pandemic. This study examined prevalence of COVID-19 at community level, determinants of its occurrence and vaccine effectiveness. METHODS: We conducted a cross-sectional study in La Paz city on 2,775 individuals between March 2020 and February 2022. A structured questionnaire was used to collect data on COVID-19 morbidity, mortality and vaccination status. RESULTS: Of the 2,775 participants, 1,586 (57.1%) were infected with COVID-19, and 187 (6.7%) were suspected cases. The mortality rate was 2.9%. Sinopharm, Johnson & Johnson, Gamaleya, Pfizer-BioNtech, Moderna and AstraZeneka vaccines are in use, and all vaccines have demonstrated effectiveness in reducing the risk of onset. Risk for mortality was significantly lower in the vaccinated group with an odds ratio of 0.037 (95％ confidential interval: 0.01-0.10, p-value: <0.001). CONCLUSIONS: Actual prevalence of COVID-19 in La Paz (the prevalence rate: 63.8%, including suspected case) was higher than that reported by the Ministry of Health and Sports in Bolivia (7.5%). In addition, vaccination has contributed significantly to the control of the COVID-19 epidemic in Bolivia. We believe that our report will be useful for COVID-19 prevention strategies in Bolivia for the future.

Prior direct oral anticoagulant dosage and outcomes in patients with acute ischemic stroke and non-valvular atrial fibrillation: A sub-analysis of PASTA registry study.

BACKGROUND AND PURPOSE: Prescribing under-dose direct oral anticoagulants (DOACs) for non-valvular atrial fibrillation (NVAF) is alerted to increase cardiovascular events or death. However, the association between dose selection of DOACs and the clinical course remains unclear. This study aimed to propose a novel criterion for selecting the DOAC dose and investigate clinical characteristics of ischemic stroke (IS) under this criterion. METHODS: We assessed the pooled prospective multicenter registry data of stroke patients taking anticoagulant agents, including IS patients with NVAF and prior DOAC usage. The recommended dose according to the reduction criteria of each DOAC and the selected dose were identified for each patient, and patients were categorized into four groups: no alternative low-dose, selecting low-dose appropriately with all DOACs applicable for reduction criteria; selected low-dose, selecting low-dose appropriately or inappropriately despite at least one DOAC inapplicable for reduction criteria; selected standard-dose, appropriate standard-dose use; and absolute over-dose, inappropriate standard-dose regardless of criteria. We investigated the effects of dose selection of DOACs on short-term poor functional outcomes. RESULTS: 322 patients were included in the analysis. The prevalence of no alternative low-dose, selected low-dose, selected standard-dose, and absolute over-dose was 74 (23%), 144 (45%), 89 (27%), and 15 (5%), respectively. Multivariable analysis found that the selected low-dose group showed significantly poorer functional outcomes than the selected standard-dose group only in patients without renal dysfunction (OR, 2.60; 95% CI, 1.17-6.00; P = 0.0186). CONCLUSIONS: Selecting a low dose DOAC might be associated with poor functional outcomes in patients without renal dysfunction.

Characteristics of Ischemic Versus Hemorrhagic Stroke in Patients Receiving Oral Anticoagulants: Results of the PASTA Study.

Objective Limited data exist regarding the comparative detailed clinical characteristics of patients with ischemic stroke (IS)/transient ischemic attack (TIA) and intracerebral hemorrhage (ICH) receiving oral anticoagulants (OACs). Methods The prospective analysis of stroke patients taking oral anticoagulants (PASTA) registry, a multicenter registry of 1,043 stroke patients receiving OACs [vitamin K antagonists (VKAs) or non-vitamin K antagonist oral anticoagulant (NOACs)] across 25 medical institutions throughout Japan, was used. Univariate and multivariable analyses were used to analyze differences in clinical characteristics between IS/TIA and ICH patients with atrial fibrillation (AF) who were registered in the PASTA registry. Results There was no significant differences in cardiovascular risk factors, such as hypertension, diabetes mellitus, dyslipidemia, smoking, or alcohol consumption (all p>0.05), between IS/TIA and ICH among both NOAC and VKA users. Cerebral microbleeds (CMBs) [odds ratio (OR), 4.77; p<0.0001] were independently associated with ICH, and high brain natriuretic peptide/N-terminal pro B-type natriuretic peptide levels (OR, 1.89; p=0.0390) were independently associated with IS/TIA among NOAC users. A history of ICH (OR, 13.59; p=0.0279) and the high prothrombin time-international normalized ratio (PT-INR) (OR, 1.17; p<0.0001) were independently associated with ICH, and a history of IS/TIA (OR, 3.37; 95% CI, 1.34-8.49; p=0.0101) and high D-dimer levels (OR, 2.47; 95% CI, 1.05-5.82; p=0.0377) were independently associated with IS/TIA among VKA users. Conclusion The presence of CMBs, a history of stroke, natriuretic peptide and D-dimer levels, and PT-INR may be useful for risk stratification of either IS/TIA or ICH development in patients with AF receiving OACs.

Focused Ultrasound Thalamotomy for Refractory Essential Tremor: A Japanese Multicenter Single-Arm Study.

BACKGROUND: Several feasibility studies and a randomized, controlled, multicenter trial have demonstrated the safety and efficacy of unilateral transcranial magnetic resonance-guided focused ultrasound (FUS) lesioning of the ventral intermediate thalamic nucleus in treating essential tremor. OBJECTIVE: To evaluate the safety and efficacy of FUS thalamotomy in a Japanese patient cohort through a prospective, multicenter, single-arm confirmatory trial. METHODS: A total of 35 patients with disabling refractory essential tremor underwent unilateral FUS thalamotomy and were followed up for 12 post-treatment months. Safety was measured as the incidence and severity of treatment-related adverse events. Efficacy was measured as the tremor severity and quality of life improvements using the Clinical Rating Scale for Tremor and Questionnaire for Essential Tremor. RESULTS: The mean skull density ratio (SDR) was 0.47. There was a significant decrease in the mean postural tremor score of the treated hand from baseline to 12 mo by 56.4% (95% CI: 46.7%-66.1%; P < .001), which was maintained at last follow-up. Quality of life improved by 46.3% (mean overall Questionnaire for Essential Tremor score of 17.4 [95% CI: 12.1-22.7]) and there were no severe adverse events. The most frequent adverse event was gait disturbance and all events resolved. CONCLUSION: Unilateral FUS thalamotomy allowed significant and sustained tremor relief and improved the quality of life with an outstanding safety profile. The observed safety and efficacy of FUS thalamotomy were comparable to those reported in a previous multicenter study with a low SDR, and inclusion of the low SDR group did not affect effectiveness.

Multicenter Prospective Analysis of Stroke Patients Taking Oral Anticoagulants: The PASTA Registry - Study Design and Characteristics.

OBJECTIVES: The management of atrial fibrillation and deep venous thrombosis has evolved with the development of direct oral anticoagulants (DOAC), and oral anticoagulant (OAC) might influence the development or clinical course in both ischemic and hemorrhagic stroke. However, detailed data on the differences between the effects of the prior prescription of warfarin and DOAC on the clinical characteristics, neuroradiologic findings, and outcome of stroke are limited. DESIGN: The prospective analysis of stroke patients taking anticoagulants (PASTA) registry study is an observational, multicenter, prospective registry of stroke (ischemic stroke, transient ischemic attack, and intracerebral hemorrhage) patients receiving OAC in Japan. This study is designed to collect data on clinical background characteristics, drug adherence, drug dosage, neurological severity at admission and discharge, infarct or hematoma size, acute therapy including recanalization therapy or reverse drug therapy, and timing of OAC re-initiation. Patient enrollment started in April 2016 and the target patient number is 1000 patients. CONCLUSIONS: The PASTA prospective registry should identify the status of stroke patients taking OAC in the current clinical practice in Japan.

Vasospasm as a major complication after acute mechanical thrombectomy with stent retrievers.

OBJECTIVE: Mechanical thrombectomy using a stent retriever for acute large vessel occlusion is indispensable in stroke treatment, however, vasospasm may occur. The objective of this retrospective study was to investigate which cases are more likely to experience vasospasm after thrombectomy with stent retrievers. METHODS: We included 29 patients diagnosed with acute cardiogenic cerebral embolism who were treated with stent retrievers at our facility from December 2014 to December 2017. Atherothrombotic brain infarction cases were excluded because it was difficult to evaluate for vasospasms. Vasospasm was defined as reversible arterial narrowing of <80% of the normal vessel diameter after usage of the stent retriever. The age, sex, type of stent retriever, occlusion site, number of procedures, thrombolysis in cerebral infarction (TICI) grade, degree of vasospasm, intracranial hemorrhage by the procedure, and neurological outcomes were analyzed. RESULTS: Among the 29 cases, 12 (41.4%) resulted in vasospasm; nine cases were mild (20-50% stenosis) and 3 cases were severe (≥50% stenosis). Vasospasm frequently occurred in the distal part of the anterior circulation when compared to the proximal part. In addition, the frequency of vasospasm increased as the number of procedures increased. Pooled analysis showed significant difference in the intravenous tissue-type plasminogen activator group (P = 0.029). There was no significant difference in the other groups. CONCLUSION: Stent retrievers appear to cause vasospasm more than expected when including mild cases. Vasospasm tends to occur especially in cases with IV-tPA; prognosis is generally good, and it rarely requires any treatment.

Prevalence and clinical features of neuromyelitis optica spectrum disorders in northern Japan.

OBJECTIVE: To clarify the prevalence and clinical characteristics of neuromyelitis optica spectrum disorders (NMOSD) in Japan and compare them with those in other ethnic populations. METHODS: Data processing sheets were sent to all related institutions in northern Japan and were collected from April to May 2016. Prevalence was determined on March 31, 2016, using the 2015 International Panel for NMO Diagnosis criteria. RESULTS: The crude prevalence was 4.1/100,000 (95% confidence interval 2.2-6.9) for NMOSD in northern Japan, with a significantly higher number of female than male patients (female: male 12:2). The positivity for anti-aquaporin-4 antibody was 78.6%, and the mean age at onset was 45.2 years. All patients were subjected to preventive therapy in the form of treatment with steroids or immunosuppressive agents. CONCLUSIONS: Our results showed that the prevalence of NMOSD in the Japanese population is similar to that in Caucasians.

Usefulness of interventional embolization of the middle meningeal artery for recurrent chronic subdural hematoma: Five cases and a review of the literature.

PURPOSE: Although several strategies against recurrent chronic subdural hematoma (CSDH) have been proposed, no consensus has been established. Recently, middle meningeal artery (MMA) embolization has been proposed as radical treatment for recurrent CSDH. We wanted to estimate the usefulness of MMA embolization for recurrent CSDH. METHODS: From February 2012 to June 2013, 110 patients with CSDH underwent single burr-hole surgery with irrigation and drainage. Among these patients, 13 showed recurrent hematoma formation and were retreated surgically. Furthermore, repeated recurrence of CSDH was observed in six patients. Five of these six patients underwent middle meningeal artery (MMA) embolization with polyvinyl alcohol particles. All five patients with interventional treatment were observed for four to 60 weeks. RESULTS: No more recurrence of CSDH was observed in any of the patients. During the follow-up period, no patients suffered from any side effects or complications from the interventional treatment. CONCLUSION: MMA embolization with careful attention paid to the procedure might be a treatment of choice for recurrent CSDH.

An innovative technique for detecting the caudal end of occluded inferior petrosal sinus in cavernous arteriovenous fistula using intravascular ultrasonography--technical note.

INTRODUCTION: Although cavernous sinus (CS) dural arteriovenous fistulas (d-AVFs) are usually treated with transvenous embolization (TVE) via the inferior petrosal sinus (IPS), IPSs are sometimes thrombosed and angiographically invisible. In such cases, the first obstacle to TVE is detecting the entry to the IPS. We report a new technique for TVE via IPS using intravascular ultrasonography (IVUS). METHODS: Three consecutive cases of CS d-AVF with ipsilateral or bilateral IPS occlusion were involved in this study. On TVE, the orifice of the IPS was investigated with IVUS placed in the jugular vein or jugular bulb. RESULTS: This technique has been successfully adapted in all three cases. In two of these cases, IPS was well visualized with the help of IVUS, and TVE was successfully performed. CONCLUSION: To our knowledge, this is the first report to mention the usefulness of IVUS for detecting angiographically occult IPS.

Reversible splenial lesion in postpartum cerebral angiopathy: a case report.

Reversible lesions on magnetic resonance imaging that transiently restrict diffusion in the splenium of the corpus callosum (SCC) without any other accompanying lesions have been reported in various clinical conditions. We offer the first report of postpartum cerebral angiopathy with reversible SCC lesions.

Disturbed resting functional inter-hemispherical connectivity of the ventral attentional network in alpha band is associated with unilateral spatial neglect.

Unilateral spatial neglect (USN) is one of the most common symptoms of right hemisphere damage; its classical symptom is that patients fail to respond to information on their left side. It has been postulated that disturbance of 2 separate attentional networks relates to the occurrence of USN. However, little is known about the underlying mechanism and neuronal substrates. In this study, we measured spontaneous neural activity by means of magnetoencephalography in 13 patients with brain damage and 5 control subjects. To study the relationship between functional connectivity at rest and severity of USN symptoms, we determined the imaginary coherence values relating to the inter-hemispherical ventral and dorsal attentional networks, as well as the clinical severity of USN using neuropsychological tests and behavioral rating scales. The present results showed that inter-hemispherical connectivity in the ventral attentional network, especially between the left and right angular gyri, detected in the alpha band is associated with the severity of USN symptoms. This may suggest that connectivity of inter-hemispherical homologous regions of the ventral attentional network in the alpha band could be one of the biomarkers of attentional network imbalance occurring in patients with USN.

Carotid intima-media thickness and risk of cardiovascular events in high-risk patients. Results of the Osaka Follow-Up Study for Carotid Atherosclerosis 2 (OSACA2 Study).

BACKGROUND AND PURPOSE: There is epidemiological evidence that increased carotid intima-media thickness (IMT) is a predictor of cardiovascular disease (CVD) events. However, the significance of carotid IMT in high-risk patients in whom risk factors are managed clinically has not been adequately investigated. The purpose of this study was to determine the usefulness of carotid IMT measurement in such patients. METHODS: The study comprised 900 outpatients with cardiovascular risk factors or established atherosclerosis. Carotid IMT was calculated as the mean bilateral IMT of the common carotid artery, bifurcation, and internal carotid artery. Baseline vascular risk factors, medications, and history of CVD were recorded at the time of enrollment. The incidence of CVD events was determined prospectively. RESULTS: During a mean follow-up period of 2.6 years, there were 64 CVD events. The relative risk (RR) of a CVD event increased with increased IMT. Association between CVD events and carotid IMT was significant after adjustment for risk factors and history of CVD, showing an increased risk per IMT tertile from the middle tertile (RR, 2.5; 95% confidence interval [CI]: 1.0-6.3) to the highest (RR, 3.6; 95% CI: 1.4-9.0). When patients with a history of CVD were excluded (n = 574), the predictive value of IMT was significant even after adjustment for risk factors (hazard ratio per 1 SD IMT increase was 1.57 [95% CI: 1.11-2.20]). CONCLUSIONS: Carotid IMT is an independent predictor of vascular events in high-risk patients in whom risk factors are managed clinically.

Associations of soluble intercellular adhesion molecule-1 with carotid atherosclerosis progression.

Our previous study demonstrated that plasma concentration of high-sensitivity C-reactive protein (hs-CRP) is a marker of carotid atherosclerosis activity. In this study, we investigated whether plasma levels of soluble cell adhesion molecules have potential value to predict atherosclerosis progression. The study included 192 outpatients 40-82 years of age who were treated for traditional risk factor for cardiovascular disease. Patients underwent repeated ultrasonographic evaluation for 53+/-11 months. Severity of atherosclerosis was evaluated by the maximal intimal-medial thickness (max-IMT), plaque number (PN) and plaque score (PS, the sum of all plaque thicknesses). Blood samples were collected for measurement of hs-CRP, soluble intercellular adhesion molecule (sICAM-1) and sP-selectin at the time of baseline examination. The development of atherosclerosis was estimated by the formula: Deltavalue/year=(last value-baseline value)/number of follow-up years. Multivariate linear regression analysis revealed that sICAM-1 was associated with DeltaIMT/year and DeltaPS/year, which was not the case for sP-selectin. sICAM-1 was closely associated with DeltaIMT/year especially in patients with apparent atheromatous plaque. Our results suggested that levels of sICAM-1 might have predictive value of progression of carotid atherosclerosis independently of traditional risk factors and hs-CRP.

Higher levels of interleukin-6 are associated with lower echogenicity of carotid artery plaques.

BACKGROUND AND PURPOSE: Echo-lucent carotid plaques can be fragile and vulnerable to rupture, representing a risk factor for ischemic stroke. Given the studies showing that elevated levels of circulating inflammatory markers are predictive of cardiovascular events, we sought to determine whether higher levels of serum interleukin-6 (IL-6) and high-sensitivity C-reactive protein (hsCRP) are associated with lower echogenicity of carotid plaques. METHODS: The study comprised 246 patients who had carotid atherosclerotic plaques as evidenced by ultrasound. Using acoustic densitometry, we quantified the echogenicity of the largest plaque in each patient by integrated backscatter analysis. Serum IL-6 and hsCRP levels were determined in all patients. RESULTS: Both log-transformed IL-6 and hsCRP concentrations were negatively correlated with carotid plaque echogenicity (r=-0.28, P<0.001, and r=-0.14, P<0.05, respectively). When traditional atherosclerotic risk factors, plaque thickness, and medication use were controlled for, IL-6 levels were inversely associated with plaque echogenicity (beta=-0.21, P<0.01), whereas such an association was of borderline significance for hsCRP (beta=-0.12, P=0.06). CONCLUSIONS: Higher IL-6 levels, in addition to hsCRP levels, appear to be associated with lower echogenicity of carotid plaques, suggesting a link between inflammation and potential risk of plaques.

Equivalence of plaque score and intima-media thickness of carotid ultrasonography for predicting severe coronary artery lesion.

Carotid atherosclerosis appears to be predictive of myocardial infarction. Because several sonographical indices are available for carotid ultrasound (US), we compared "blindly" the potential utilities of those indices for predicting coronary lesions in 270 patients. Carotid atherosclerosis was evaluated by the following four indices: plaque score (PlaS), intima-media thickness (IMT) of common carotid artery (CCA-IMT), IMT of bulb to internal carotid artery (Bulb-ICA-IMT), and combined IMT measurement from all segments. The existence of coronary lesions was diagnosed by > 50% stenosis in diameter in coronary arteries. All indices were associated with coronary lesions independent of risk factors. By receiver-operating characteristic (ROC) curve analyses, ROC areas defined by Bulb-ICA-IMT (0.76 to 0.86), combined IMT (0.76 to 0.86) and PS (0.76 to 0.87) were greater than that defined by CCA-IMT (0.64 to 0.76). In conclusion, PlaS, Bulb-ICA-IMT and combined IMT are equally effective and could be better than CCA-IMT for predicting coronary lesions in a population with cardiovascular risk.

Carotid artery intima-media thickness and plaque score for the risk assessment of stroke subtypes.

As measures for the severity of carotid atherosclerosis, we compared common carotid artery (CCA) intima-media thickness (IMT) and plaque score (PS) for risk assessment of respective stroke subtypes. The subjects comprised 792 nonstroke and 311 stroke patients, including 72 with atherothrombotic infarction (AI), 113 with lacunar infarction (LI), 54 with cardioembolic infarction and 29 with cerebral hemorrhage. IMT was bilaterally measured on CCA far walls, and averaged. PS was obtained by summing up the maximum thickness of all plaques in bilateral carotid arteries. Both IMT and PS were greater in AI and LI patients than in nonstroke patients (all p < 0.05), but similar between other subtype and nonstroke patients. By receiver operating characteristic (ROC) curve analyses, both measures discriminated the likelihood for AI and LI (all p < 0.05), but not for other subtypes. When discriminating AI, ROC area defined by PS (0.80) was greater than that defined by IMT (0.68) (p < 0.05). Thus, although both CCA IMT and PS appear to help for risk assessment of AI and LI, risk of AI may be more effectively assessed by PS.