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Background@#and Purpose The onset of Huntington’s disease (HD) usually occurs before the age of 50 years, and the median survival time from onset is 15 years. We investigated survival in patients with late-onset HD (LoHD) (age at onset ≥60 years) and the associations of the number of mutant CAG repeats and age at onset (AAO) with survival in patients with HD. @*Methods@#Patients with genetically confirmed HD at six referral centers in South Korea between 2000 and 2020 were analyzed retrospectively. Baseline demographic, clinical, and genetic characteristics and the survival status as at December 2020 were collected. @*Results@#Eighty-seven patients were included, comprising 26 with LoHD (AAO=68.77±5.91 years, mean±standard deviation; 40.54±1.53 mutant CAG repeats) and 61 with common-onset HD (CoHD) (AAO=44.12±8.61 years, 44.72±4.27 mutant CAG repeats). The ages at death were 77.78±7.46 and 53.72±10.86 years in patients with LoHD and CoHD, respectively (p< 0.001). The estimated survival time was 15.21±2.49 years for all HD patients, and 10.74±1.95 and 16.15±2.82 years in patients with LoHD and CoHD, respectively. More mutant CAG repeats and higher AAO were associated with shorter survival (hazard ratio [HR]=1.05, 95% confidence interval [CI]=1.01–1.09, p=0.019; and HR=1.17, 95% CI=1.03–1.31, p=0.013; respectively) for all HD patients. The LoHD group showed no significant factors associated with survival after disease onset, whereas the number of mutant CAG repeats had a significant effect (HR=1.12, 95% CI=1.01–1.23, pp=0.034) in the CoHD group. @*Conclusions@#Survival after disease onset was shorter in patients with LoHD than in those with CoHD. More mutant CAG repeats and higher AAO were associated with shorter survival in patients with HD.
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Sensory Guillain-Barré syndrome (GBS) is a rare heterogeneous subgroup of GBS characterized by the primary involvement of sensory neurons resulting in a distinctive clinical presentation. Sensory GBS usually occurs with acute and monophasic sensory symptoms, and no or minimal muscle weakness. Sensory GBS patient show hypo- or areflexia, distal paresthesia, and normal cerebrospinal fluid finding or albumino-cytologic dissociation which are suggesting that these conditions are a GBS variant. Autonomic dysfunctions have rarely been reported in sensory GBS patient presenting with postural hypotension, abnormal heart rate response to respiration. In this case, we demonstrate a patient with autonomic symptoms dominant sensory GBS in systemic lupus erythematosus.
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@#Objective: Cerebral white matter changes (WMCs) and silent brain infarcts (SBIs) are common radiological findings in neurologically asymptomatic elderly people. Their presence is associated with an increased risk of stroke or dementia. Homocysteine (Hcy) can cause atherosclerosis and may thus act as a useful marker of stroke prior to symptom onset. This study aimed to evaluate the utility of Hcy as a surrogate marker for early intervention for stroke in community-dwelling healthy elderly persons with cerebrovascular lesions such as WMCs and SBIs. Methods: We assessed the relationship between the Hcy level and presence of WMCs/SBIs on brain computed tomography (CT) in 554 elderly individuals aged ≥65 years with no history of stroke or dementia. Results: The mean age of the participants was 74.43±7.32 years. Of the 554 patients, 106 (19.1%) had WMCs and/or SBI findings on brain CT. The mean Hcy level was higher in participants with WMCs/SBIs (P=0.001). The WMCs/SBIs group had a significantly greater number of participants with high Hcy levels than the non-WMCs/SBIs group (P<0.001). High Hcy levels (≥12.3 μmol/L) was significantly associated with WMCs and SBIs even after adjusting for age, Korean Mini Mental Status Examination score, education level, and vascular risk factors such as hypertension, diabetes, hyperlipidemia and smoking status. Conclusion: The result of our study suggests that regular monitoring of homocysteine level as a modifiable risk factor may be helpful for predicting the association of cerebrovascular lesions such as WMCs and SBIs for the prevention of future ischemic stroke and cognitive impairment.
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Background@#and Purpose: The incidence of ischemic stroke (IS) in young adults is increasing, and the associated large socioeconomic impact makes understanding IS in young adults important. We investigated the causes of and risk factors for IS in young adults, and their impact on outcomes. @*Methods@#The Stroke in Korean Young Adults (SKY) study is a standardized multicenter prospective study involving eight medical centers of the Republic of Korea. First-ever IS patients aged 18 years to 44 years were prospectively included in this study within 7 days of stroke onset.Their outcomes at 3 months were analyzed. @*Results@#This study enrolled 270 patients from April 2014 to December 2018, most (67.8%) of whom were male. About 41.5% of the patients had one or more vascular risk factors from among hypertension, diabetes mellitus, and dyslipidemia. However, only half of them had received regular treatment. Arterial dissection was more common in males, and systemic lupus erythematosus (SLE) and Moyamoya disease were more common in females. The outcome was favorable (modified Rankin Scale score of 0 or 1) in 81.9% of the patients at 3 months after stroke onset. More severe initial symptoms, higher initial glucose level, and SLE as a comorbidity were associated with unfavorable outcomes. @*Conclusions@#Young adult IS patients in Korea exhibit low awareness and poor management of their risk factors. Although the short-term outcome was relatively favorable in those patients, having SLE was associated with unfavorable outcomes. More attention needs to be paid for improving awareness and controlling risk factors in this population.
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Vertigo, dizziness, and disequilibrium are common symptoms following concussion or traumatic brain injury. Dizziness and vertigo may be the result of trauma to the peripheral vestibular system or the central nervous system, or, in some cases, may be due to anxiety, depression, or posttraumatic stress disorder; these mechanisms are not mutually exclusive. While most peripheral vestibular disorders can be identified by testing and examination, those without inner ear causes that have persisting complaints of dizziness and motion sickness are more difficult to understand and to manage. Some of these patients exhibit features compatible with vestibular migraine and may be treated successfully with migraine preventative medications. This paper reviews the neurological causes of persisting dizziness, the possible mechanisms, and the pathophysiology, as a framework for patient management and for future research.
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Humains , Anxiété , Lésions encéphaliques , Système nerveux central , Dépression , Lésion axonale diffuse , Sensation vertigineuse , Oreille interne , Migraines , Mal des transports , Troubles de stress post-traumatique , VertigeRésumé
Vasospasm after subarachnoid hemorrhage may alter intracranial hemodynamics. We report a case which showed significant size reduction of an unruptured intracranial aneurysm after resolution of vasospasm. This case may suggest that aneurysm size can be affected by vasospasm, which is presumably related to changes in hemodynamics or spontaneous thrombosis.
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BACKGROUND AND PURPOSE: Subclinical atherosclerotic plaques are common in patients with pontine infarctions (PIs) but without basilar artery (BA) stenosis. We hypothesized that BA plaque locations may differ by PI type and vertical location as well as vertebrobasilar artery geometry. METHODS: Ninety-six patients with PI but without BA stenosis on magnetic resonance imaging (MRI) and magnetic resonance angiography were enrolled. PIs were classified by type (paramedian, deep, or lateral) and vertical location (rostral, middle, or caudal). Patients underwent high-resolution MRI to evaluate BA plaque location (anterior, posterior, or lateral). The mid-BA angle on anteroposterior view and angle between the BA and dominant vertebral artery (BA-VA angle) on lateral view were measured. RESULTS: The PIs were paramedian (72.9%), deep (17.7%), and lateral (9.4%) type with a rostral (32.3%), middle (42.7%), and caudal (25.0%) vertical location. The BA plaque locations differed by PI type (P=0.03) and vertical location (P < 0.001); BA plaques were most frequent at the posterior wall in paramedian (37.1%) and caudal (58.3%) PIs and at the lateral wall in lateral (55.5%) and middle (34.1%) PIs. The BA-VA and mid-BA angles differed by BA plaque and PI vertical location; the greatest BA-VA angle was observed in patients with posterior plaques (P < 0.001) and caudal PIs (P<0.001). Greatest mid-BA angles were observed with lateral BA plaques (P=0.03) and middlelocated PIs (P=0.03). CONCLUSIONS: Greater mid-BA angulation may enhance lateral plaque formation, causing lateral and middle PIs, whereas greater BA-VA angulation may enhance posterior plaque formation, causing paramedian or caudal PIs.
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Humains , Artères , Artère basilaire , Infarctus du tronc cérébral , Sténose pathologique , Hémodynamique , Infarctus , Angiographie par résonance magnétique , Imagerie par résonance magnétique , Plaque d'athérosclérose , Artère vertébraleRésumé
BACKGROUND AND PURPOSE: Carotid plaques are a strong risk factor for ischemic stroke, and plaque rupture poses an even higher risk. Although many studies have investigated the pathogenic mechanisms of carotid plaque formation, few have studied the differences in molecular mechanisms underlying the rupture and non-rupture of carotid plaques. In addition, since early diagnosis and treatment of carotid plaque rupture are critical for the prevention of ischemic stroke, many studies have sought to identify the important target molecules involved in the rupture. However, a target molecule critical in symptomatic ruptured plaques is yet to be identified. METHODS: A total of 79 carotid plaques were consecutively collected, and microscopically divided into ruptured and non-ruptured groups. Quantitative polymerase chain reaction array, proteomics, and immunohistochemistry were performed to compare the differences in molecular mechanisms between ruptured and non-ruptured plaques. Enzyme-linked immunosorbent assay was used to measure the differences in ATP-binding cassette subfamily A member 1 (ABCA1) levels in the serum. RESULTS: The expression of several mRNAs and proteins, including ABCA1, was higher in ruptured plaques than non-ruptured plaques. In contrast, the expression of other proteins, including β-actin, was lower in ruptured plaques than non-ruptured plaques. The increased expression of ABCA1 was consistent across several experiments, ABCA1 was positive only in the serum of patients with symptomatic ruptured plaques. CONCLUSIONS: This study introduces a plausible molecular mechanism underlying carotid plaque rupture, suggesting that ABCA1 plays a role in symptomatic rupture. Further study of ABCA1 is needed to confirm this hypothesis.
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Humains , Marqueurs biologiques , Artères carotides , Diagnostic précoce , Test ELISA , Immunohistochimie , Réaction de polymérisation en chaîne , Protéomique , Facteurs de risque , ARN messager , Rupture , Accident vasculaire cérébralRésumé
BACKGROUND AND PURPOSE: Alzheimer's disease (AD) is the most common form of dementia which typically manifests as loss of memory and cognitive functions. Currently, available treatments for AD provide only symptomatic improvement and the benefit is minimal. Stem cell therapy (SCT) has been considered a promising treatment option for AD. We investigated the caregiver's perception about implementation of SCT in their AD patients, and determined the factors related to SCT. METHODS: A total of 100 caregivers, who cared for their AD patients, were interviewed at two hospitals. Structured open and closed questions about SCT for AD were asked by trained interviewers using the conventional in-person method. In addition, 60 dementia-related physicians were randomly interviewed via an e-mail questionnaire. RESULTS: Of the 100 subjects, 61 caregivers replied that they wanted their AD patients to receive SCT. Approximately 50% of the caregivers expected high improvement in cognitive function, behavioral and psychological symptoms, and activities of daily living, and physical improvements among their AD patients. However, physicians had much lower expectations of improvements in the above parameters. Multi-variate analysis revealed that female gender [odds ratio (OR): 3.747, 95% confidence interval (CI): 1.425–9.851] and familiarity with stem cells (OR: 3.873, 95% CI: 1.290–11.622) were independently associated with caregivers' desire that their AD patients should undergo SCT. The major source of information on SCT was television (76.7%), and the most reliable source of information on SCT was physicians (83.6%). CONCLUSIONS: In this study, many caregivers of AD patients fantasized and overestimated the need for SCT in comparison with physicians' expectation. Therefore, it is necessary for physicians to develop strategies for educating caregivers about the appropriate risks and benefits of SCT.
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Femelle , Humains , Activités de la vie quotidienne , Maladie d'Alzheimer , Aidants , Cognition , Démence , Courrier électronique , Mémoire , Méthodes , , Appréciation des risques , Cellules souches , TélévisionRésumé
Wernicke's encephalopathy is an acute neurological deterioration due to a reversible brain lesion caused by thiamine deficiency. Most of the affected patients are thiamine-depleted alcoholics, and the condition usually involves the medial thalami, mammillary bodies, and periaqueductal area. However, there are rare reports of lesions in the cerebellum and cerebral cortex, especially in patients with nonalcoholic Wernicke's encephalopathy. We report a case of nonalcoholic Wernicke's encephalopathy involving atypical diffuse cortical regions, and review previously reported cases.
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Humains , Alcooliques , Encéphale , Cervelet , Cortex cérébral , Corps mamillaires , Carence en thiamine , Encéphalopathie de Gayet-WernickeRésumé
BACKGROUND AND PURPOSE: The benefit of carotid endarterectomy (CEA) is directly influenced by the risk of perioperative adverse outcomes. However, patient-level risks and predictors including coronary stenosis are rarely evaluated, especially in Asian patients. The aim of this study was to determine the relationship between the vascular risk factors underlying CEA, including coronary stenosis, and postoperative outcome. METHODS: One hundred and fifty-three consecutive CEAs from our hospital records were included in this analysis. All patients underwent coronary computed tomography angiography before CEA. Data were analyzed to determine the vascular outcomes in patients with mild-to-moderate vs. severe coronary stenosis and high vs. standard operative risk, based on the criteria for high operative risk defined in the Stenting and Angioplasty with Protection in Patients at High Risk for Endarterectomy (SAPPHIRE) trial. The vascular outcome was defined as the occurrence of postoperative (< or =30 days) stroke, myocardial infarction (MI), or death. RESULTS: An adverse vascular outcome occurred in 8 of the 153 CEAs, with 6 strokes, 2 MIs, and 3 deaths. The vascular outcome differed significantly between the groups with mild-to-moderate and severe coronary stenosis (p=0.024), but not between the high- and standard-operative-risk groups (stratified according to operative risk as defined in the SAPPHIRE trial). Multivariable analysis adjusting for potent predictors revealed that severe coronary stenosis (odds ratio, 6.87; 95% confidence interval, 1.20-39.22) was a significant predictor of the early vascular outcome. CONCLUSIONS: Severe coronary stenosis was identified herein as an independent predictor of an adverse early vascular outcome.
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Humains , Oxyde d'aluminium , Angiographie , Angioplastie , Asiatiques , Coronarographie , Maladie des artères coronaires , Sténose coronarienne , Endartériectomie , Endartériectomie carotidienne , Archives administratives hospitalières , Infarctus du myocarde , Appréciation des risques , Facteurs de risque , Endoprothèses , Accident vasculaire cérébralRésumé
No abstract available.
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Infarctus cérébral , Endocardite , Syndrome hyperéosinophilique , InfarctusRésumé
No abstract available.
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Humains , Hématome épidural rachidien , Polyglobulie primitive essentielle , Ponction lombaire , Hémorragie meningéeRésumé
No abstract available.
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Humains , Embolie graisseuse , Spectroscopie par résonance magnétiqueRésumé
BACKGROUND: Seizures occur in 2-20% of stroke patients. Recent studies have reported that post-stroke seizures are associated with poorer functional outcomesand higher mortality. However there are no official guidelines on how to use antiepileptic drugs (AEDs) in stroke-related seizures. In this study we surveyed neurologists and neurosurgeons and compared the responses of subgroups categorized by department, specialty and workplace discrimination using a questionnaire containing questions concerning the present tendency to use AEDs in stroke patients. METHODS: 256 neurologists and neurosurgeons participated in the survey. The research instrument was a questionnaire comprising 9 parts and 30 questions. The questions concerned stroke mechanism, the prophylactic use of AEDs, and the choice of AED in early and late onset post-stroke seizures. RESULTS: Tendencies to use prophylactic AEDs in stroke differed depending on specialty and workplace(neurologist vs. neurosurgeon; 17.8% vs. 83.1%, p<0.001, hospital vs. university staff; 46.2% vs. 28.4%, p=0.05). The most commonly used prophylactic AEDs were valproic acid (75%) and levetiracetam (60%). Carbamazepine was the most commonly used AED and phenytoin and phenobarbital were still used in all subgroups to treat post-stroke seizures. CONCLUSIONS: There are significant differences between neurologists (17.8%) and neurosurgeons (83.1%) in the use of prophylactic AEDs after stroke. Valproic acid and levetiracetam are considered first-line prophylactic AEDs by neurosurgeon. Phenytoin and phenobarbital are still used in post-stroke seizure although they have been reported to have an adverse influence on motor recovery. We suggest that proper guidelines should be established for the use of AEDs in stroke-related seizures.
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Humains , Anticonvulsivants , Carbamazépine , , Phénobarbital , Phénytoïne , Piracétam , Enquêtes et questionnaires , Crises épileptiques , Accident vasculaire cérébral , Acide valproïqueRésumé
BACKGROUND AND PURPOSE: Aortic knob calcification (AC) is associated with increased risks of cardiovascular and cerebrovascular events. We evaluated the clinical importance of AC in ischemic stroke patients with intracranial (IC) stenosis using simple, non-invasive and routine chest radiography. METHODS: The presence of AC was assessed in a chest posteroanterior view in 307 acute ischemic stroke patients admitted from May 2009 to April 2010, and who underwent magnetic resonance angiography or distal subtraction angiography. The association of AC with IC stenosis was analyzed. RESULTS: Patient age (68.3+/-8.7 vs. 65.9+/-8.27 years, P=0.04), and the prevalence of IC stenosis (70.7 vs. 41.3%, P<0.01) were higher in patients with AC than in patients without calcification. After adjusting for age, gender and vascular risk factors, logistic regression analysis showed that AC (Odds ratio, 3.54; 95% confidence interval, 1.90 to 6.61, P<0.01) and age (Odds ratio, 1.79; 95% confidence interval, 1.01 to 3.19; P=0.04) were independent factors affecting IC stenosis. CONCLUSIONS: AC appears to be a reliable predictor for IC stenosis, an important mechanism of ischemic stroke.
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Humains , Angiographie , Athérosclérose , Sténose pathologique , Modèles logistiques , Angiographie par résonance magnétique , Prévalence , Facteurs de risque , Accident vasculaire cérébral , ThoraxRésumé
Simultaneous subarachnoid hemorrhage and infarction is a quite rare presentation in a patient with a spontaneous dissecting aneurysm of the anterior cerebral artery. Identifying relevant radiographic features and serial angiographic surveillance as well as mode of clinical manifestation, either hemorrhage or infarction, could sufficiently determine appropriate treatment. Enlargement of ruptured aneurysm and progressing arterial stenosis around the aneurysm indicates impending risk of subsequent stroke. In this setting, prompt treatment with stent-assisted endovascular embolization can be a reliable alternative to direct surgery. When multiple arterial dissections are coexistent, management strategy often became complicated. However, satisfactory clinical results can be obtained by acknowledging responsible arterial site with careful radiographic inspection and antiplatelet medication.