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Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS) is a brainstem predominant lymphocytic inflammatory disease, which often relapses without oral immunosuppressants. This report describes a 37-year-old male case of CLIPPERS without relapse for 1 year after early steroid treatment. He was admitted to our hospital because of sensory disturbance in the left side of his body and ataxic gait. Gadolinium-enhanced T

Background and Purpose: The difference among anterior and posterior circulation stroke regarding vascular risk factors is commonly known. Our aim is to verify the difference of clinical backgrounds between anterior and posterior circulation in hyperacute stroke with large vessel occlusion (LVO) caused by atherosclerotic occlusion (AO). Methods: This multicenter, retrospective study included LVO patients who were performed revascularization therapy (thrombectomy, intravenous thrombolysis, or both) between October 2017 and May 2021. The definition of AO was the fixed residual stenosis/occlusion at the initially occluded lesion confirmed in the second angiographic imaging performed 7 days later from symptom onset. The patients were dichotomized by LVO location, anterior or posterior. Traditional risk factors and laboratory data were compared. Results: Among the 1770 consecutive stroke patients admitted during the study period, 162 patients (104 men, median age 76 years old) were enrolled in our study. AO was diagnosed in 41 patients (25%; 27% of anterior circulation vs. 17% of posterior, P=0.291). In total cohort, patients with anterior circulation showed higher frequency of atrial fibrillation, lower glycosylated hemoglobin level, higher D dimer level, and higher brain natriuretic peptide level (Figure 1 black bar). The cohort of AO patients had a similar tendency (Figure 1 blue bar). Conclusion: One fourth of patients with LVO are AO. In anterior circulation stroke with AO, attention should be paid to stroke recurrence due to not only atherosclerosis but embolism.

To diagnose atherosclerotic occlusion mechanism in acute ischemic stroke patients with large vessel occlusion prior to revascularization therapy is challenging. The aim was to verify the relationship between atherosclerotic occlusion and non-culprit stenosis detected in urgent neuroimaging prior to interventional procedure.This study collected hyperacute stroke with large vessel occlusion, who underwent revascularization therapy (intravenous thrombolysis, thrombectomy, or both). An atherosclerotic occlusion was defined as an occlusion that did not recanalize or had residual stenosis at the initially occluded lesion, ensured in the second angiographic imaging performed after 1 week. The remaining patients who did not fulfill the definition of atherosclerotic occlusion was classified as embolic occlusion. A non-culprit stenosis was defined as a ≥ 50%-99% stenosis located other than the culprit occluded artery. Logistic regression analyses were performed to determine the factors independently associated with atherosclerotic occlusion.A total of 162 patients (104 men, median age 76 years old) were enrolled in our study. Forty one patients (25%) was atherosclerotic occlusion. Non-culprit stenosis was frequently observed in the atherosclerotic occlusion group than the embolic occlusion group (68% vs. 26%, P 0.001). The presence of non-culprit stenosis was independently associated with atherosclerotic occlusion (OR, 11.00; 95% CI, 3.96-30.52; P 0.001).In hyperacute stroke receiving endovascular therapy, non-culprit stenosis identification may be needed in order to perform an adequate revascularization, especially for atherosclerotic occlusion.

Bulbar symptoms in amyotrophic lateral sclerosis (ALS) are variable, reflecting bulbar and pseudobulbar palsy. The current study sought to characterize the pharyngeal findings in ALS using a fiberoptic laryngoscope and compare them with the findings of general neurological examination.We enrolled ALS patients with bulbar symptoms who were admitted between 2014 and 2020. All participants were evaluated on salivary status, velopharyngeal movement during speech and swallowing, pharyngeal constriction, and vocal cord movement using fiberoptic laryngoscopy. The laryngoscopic findings were compared with general neurological examination results.A total of 50 patients (31 men; median age: 69 years) were enrolled. Salivary residue in the hypopharynx was the most common abnormal finding on laryngoscopy (40 patients; 80%). Twenty-three patients (46%) exhibited velopharyngeal insufficiency, 18 of which exhibited good velopharyngeal closure in swallowing and poor velopharyngeal closure in speech. Thus, these patients presented speech-swallow dissociation (SSD) in velopharyngeal closure. Five patients (10%) exhibited unilateral weakness of the pharyngeal constrictor muscles in phonation. Compared with general neurological examinations, emotional incontinence was more frequent in patients who presented with SSD in velopharyngeal movement, compared with those who did not. None of the five patients with unilateral weakness of pharyngeal constriction showed curtain movement on examination via the mouth.The specific findings of laryngoscopy in ALS patients, such as SSD in velopharyngeal closure and laterality in pharyngeal constriction, could not be evaluated by general neurological examination via the mouth.

Although animal studies and studies on Parkinson’s disease (PD) suggest that dopamine deficiency slows the pace of the internal clock, which is corrected by dopaminergic medication, timing deficits in parkinsonism remain to be characterized with diverse findings. Here we studied patients with PD and progressive supranuclear palsy (PSP), 3–4 h after drug intake, and normal age-matched subjects. We contrasted perceptual (temporal bisection, duration comparison) and motor timing tasks (time production/reproduction) in supra- and sub-second time domains, and automatic versus cognitive/short-term memory–related tasks. Subjects were allowed to count during supra-second production and reproduction tasks. In the time production task, linearly correlating the produced time with the instructed time showed that the “subjective sense” of 1 s is slightly longer in PD and shorter in PSP than in normals. This was superposed on a prominent trend of underestimation of longer (supra-second) durations, common to all groups, suggesting that the pace of the internal clock changed from fast to slow as time went by. In the time reproduction task, PD and, more prominently, PSP patients over-reproduced shorter durations and under-reproduced longer durations at extremes of the time range studied, with intermediate durations reproduced veridically, with a shallower slope of linear correlation between the presented and produced time. In the duration comparison task, PD patients overestimated the second presented duration relative to the first with shorter but not longer standard durations. In the bisection task, PD and PSP patients estimated the bisection point (BP50) between the two supra-second but not sub-second standards to be longer than normal subjects. Thus, perceptual timing tasks showed changes in opposite directions to motor timing tasks: underestimating shorter durations and overestimating longer durations. In PD, correlation of the mini-mental state examination score with supra-second BP50 and the slope of linear correlation in the reproduction task suggested involvement of short-term memory in these tasks. Dopamine deficiency didn’t correlate significantly with timing performances, suggesting that the slowed clock hypothesis cannot explain the entire results. Timing performance in PD may be determined by complex interactions among time scales on the motor and sensory sides, and by their distortion in memory.

Background and Purpose: The association between the cerebral microbleed (CMB) count and outcomes in ischemic stroke has not been fully clarified. The aim of this study was to investigate the relationship between the CMB count and functional outcomes in patients with a minor ischemic stroke treated with antiplatelet therapy. Methods: Non-cardiogenic minor ischemic stroke (NIHSS score 4), and their clinical outcomes were compared. Deep white matter hyperintensities (DWMHs) were assessed using the Fazekas scale, and the relationship between DWMH burden and the CMB count was evaluated. A poor outcome was defined as a modified Rankin scale (mRS) score of 3-6 90 days after symptom onset. Logistic regression analysis was performed to evaluate whether the CMB count contributes to poor outcomes. Results: A total of 240 patients were enrolled, and their pre mRS scores were matched based on CMB presence. The median CMB count increased linearly with the Fazekas scale grade (P4 CMB group, P=0.002). Multivariate logistic regression analysis performed with well-known risk factors including DWMH showed that CMB burden (subgroups) was the independent factor associated with poor outcomes (odds ratio 1.75, 95% confidence interval 1.13-2.72, P=0.012), whereas the DWMH burden was not. Conclusion: The CMB count contributes independently to poor outcomes in minor ischemic stroke patients treated with antiplatelet therapy.

We report the case of a 43-year-old female patient who presented with symptoms of abnormal behavior, hearing loss, ataxic gait, central hyperventilation which had appeared over the course of one month. Brain MRI showed no abnormal findings in DWI and EEG did not indicate periodic synchronous discharge (PSD). Over the course of the same month, she also presented with central apnea that intermittently showed spontaneous improvement and reappearance. Cerebrospinal fluid 14-3-3 protein tested negative and there was no family history, but an abnormal prion protein was detected in the cerebrospinal fluid by the RT-QUIC assay. We diagnosed her with familial Creutzfeldt-Jakob disease (CJD) with an E200K mutation after genetic examination. Both high cortical signals on MRI and PSD on EEG were not recognized even in the advanced stage. Central apnea was presumed to be caused by disorders of the respiratory center of the brainstem. Hearing loss was also considered to be an obstacle at the brainstem level from the latency delay after the III wave in auditory brainstem response (ABR). The possibility of brain stem symptoms occurring in the early stages of CJD should be considered.

A 41-year-old man left for Mexico in May 2015. Right pulmonary nodule was detected at a health examination in May 2016, and he subsequently showed headache and slight fever. Contrast-enhanced magnetic resonance imaging of the brain revealed basilar meningitis, so he was admitted to our hospital. We considered imported mycosis due to his travel history to Mexico. We diagnosed histoplasmosis based on the presence of antibodies against Histoplasma in both serum and cerebrospinal fluid. Symptoms almost completely recovered with a liposomal formulation of amphotericin B. Central nervous system histoplasmosis is very rare in Japan. Immunocompetent hosts can develop histoplasmosis, and this pathology is important to consider in patients presenting with basilar meningitis and a positive travel history.

The association between the cerebral microbleed (CMB) count and outcomes in ischemic stroke has not been fully clarified. The aim of this study was to investigate the relationship between the CMBs count and functional outcomes in patients with a minor ischemic stroke treated with antiplatelet therapy METHODS: Non-cardiogenic minor ischemic stroke (NIHSS score 4 on admission) patients who were treated with antiplatelet therapy were enrolled. The patients were divided into four groups based on the number of CMBs (absent, 1, 2-4, and 4), and their clinical outcomes were compared. A poor outcome was defined as a modified Rankin scale (mRS) score of 3-6 90 days after symptom onset. Logistic regression analysis was performed to evaluate whether the CMBs count contributes to poor outcomes with well-known risk factors such as age, NIHSS score on admission, ischemic stroke recurrence, large artery atherosclerosis stroke subtype, and DWMHs.A total of 240 patients were enrolled, and their pre mRS scores were matched based on CMB presence. A higher burden of CMBs was linearly correlated with the incidence of poor outcomes (4% in the absent group, 8% in the 1 CMB group, 13% in the 2-4 CMB group, and 20% in the 4 CMB group, P = 0.002). Multivariate logistic regression analysis showed that CMBs count was one of the independent factor associated with poor outcomes (odds ratio 1.07, 95% confidence interval 1.02-1.12, P = 0.003).The CMBs count contributes independently to poor outcomes in minor ischemic stroke patients treated with antiplatelet therapy.

A 73-year-old man developed diplopia after the administration of pembrolizumab for lung adenocarcinoma. He had ptosis and external ophthalmoplegia without general muscle weakness. Serum CK levels were elevated. Although autoantibodies to acetylcholine receptor and muscle-specific kinase, the edrophonium test, and the repetitive nerve stimulation test were all negative, anti-titin autoantibody was positive, leading to the diagnosis of myasthenia gravis (MG). Muscle pathology showed necrotizing myopathy with tubular aggregates. Unlike previously reported cases of pembrolizumab-associated MG, the present case showed ocular MG. This is the first case of pembrolizumab-associated MG with anti-titin antibody, as well as the first case with tubular aggregates.

Purpose Vertebral artery hypoplasia (VAH) is a common congenital anatomical variation. In previous reports, it was unclear whether VAH was an independent risk factor for posterior circulation ischemia. The purpose of this study was to evaluate the impact of VAH on posterior circulation ischemia. Methods Subjects were patients with acute ischemic stroke who underwent brain magnetic resonance imaging (MRI) and carotid ultrasonography. Diagnostic criteria for VAH were as follows: (1) Vertebral artery (VA) diameter less than 2.5 mm; (2) VA diameter less than 3.0 mm and a difference in length equal to or greater than 1:1.7; (3) VA diameter less than 3.0 mm, peak systolic velocity less than 40 cm/second, and resistance index value greater than .75. The patients were categorized by the location of the ischemic stroke on MRI as follows: lesion in posterior circulation (P group), lesion in anterior circulation (A group), and multiple lesions in both the anterior and posterior circulations (AP group). Results We evaluated 129 consecutive patients. VAH was seen in 39, and VA occlusion was found in 15. The prevalence of VAH in the P group (44.4%) was significantly higher than that in the A + AP group (24.7%, P = .034). Multivariate regression analysis showed that large-artery atherosclerosis (odds ratio, 6.3; 95% confidence interval [CI], 1.3-30.1), posterior circulation ischemia (odds ratio, 12.0; 95% CI, 2.8-51.2), and VAH (odds ratio, 4.2; 95% CI, 1.2-15.0) were independent factors related to VA occlusion. Conclusion VAH was an independent factor related to VA occlusion. Therefore, VAH likely plays a role in posterior circulation ischemia.

Thrombolytic agents are infusion formulations, and some patients cannot be cannulated by a peripheral venous route. This report describes a patient with acute ischemic stroke who was administered alteplase following central venous catheter placement. An 82-year-old man with paroxysmal atrial fibrillation presented with left unilateral spatial neglect and left hemiparesis. Magnetic resonance imaging showed acute cerebral infarction located in the right cerebrum without occlusion of the main artery. The infarction was considered appropriately indicated for thrombolysis. However, no peripheral venous access could be secured, even by trained emergency room physicians. A central venous catheter was therefore placed in the right jugular vein and thrombolysis was performed. After treatment, neurological deficits completely resolved without any clinically serious bleeding. Venous catheter placement may be a safe alternative to peripheral vein access in such a circumstance.

Endomyocarditis in Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare complication, commonly involving an apical mass compatible with a thrombus. However, no previous report has discussed mobile structures detected by echocardiography in a patient with EGPA. A 53-year-old man with asthma presented with low-grade fever, consciousness disturbance, and vomiting. Magnetic resonance imaging showed multiple acute infarctions in the bilateral cerebrum and cerebellum. Remarkable eosinophilia was observed, and transthoracic echocardiography showed multiple mobile structures originating from the left ventricular septum. The day after admission, he developed left partial hemianopia and intracranial hemorrhage was identified in his right occipital lobe. Skin biopsy showed infiltration of eosinophils in the arterial wall, and we diagnosed EGPA. Myocardial biopsy was performed from the right ventricular wall, and eosinophilic infiltration into the endocardium and myocardium was observed. Endomyocarditis secondary to EGPA was confirmed, and steroid therapy was immediately initiated. Ten days after steroid therapy, the mobile structures in the left ventricle disappeared completely. He suffered no recurrence of stroke with continued steroid therapy. Mobile structures in the left ventricle in patients with active EGPA could be treated conservatively with steroid therapy.

A patient started anticoagulation for secondary prevention of stroke, achieving a prothrombin time-international normalized ratio within the optimal therapeutic range. The patient subsequently complained of pain in the left thigh on hospital day 25, and ultrasonography showed a large intramuscular hematoma in the left hamstring. The intramuscular hematoma gradually reduced without surgical intervention. Ultrasound examination plays an important role in precisely diagnosing intramuscular hematoma and monitoring changes in hematoma size.

Objective This study aimed to examine whether the volume of the olfactory bulbs and tracts (OB & T) on magnetic resonance imaging (MRI) is useful for differentiating Parkinson's disease (PD) from PD-related disorders. Methods The study group comprised 13 patients with PD, 11 with multiple system atrophy (MSA), five with progressive supranuclear palsy, and five with corticobasal degeneration (PSP/CBD). All patients were evaluated using the odor stick identification test for Japanese (OSIT-J), 123I-meta-iodobenzylguanidine (MIBG) scintigraphy, and brain MRI. OB & T areas on 1-mm-thick coronal images were measured and summed for volumes. We examined relationships between olfactory function and volume, and cardiovascular dysautonomia. We defined the cut-off values for OSIT-J score or MIBG uptake and OB & T volume to discriminate PD from PD-related disorders and calculated the proportional rate of PD in four categorized groups. Results OB & T volume was smaller in PD than in MSA or PSP/CBD (p

Background Carotid duplex ultrasonography (CUS) has been used to identify reversed vertebral artery flow (RVAF) at the extracranial cervical artery in some patients with subclavian steal syndrome. However, the characteristics of intracranial RVAF as evaluated by transcranial color flow imaging (TC-CFI), which can examine intracranial hemodynamics in a real-time and noninvasive fashion, remain unclear. The goal of this study was to analyze the prevalence of intracranial RVAF and its associated clinical characteristics. Methods Subjects were consecutive patients who underwent TC-CFI and CUS. We evaluated blood flow in both intracranial vertebral arteries (VAs) from the suboccipital echo window using TC-CFI. RVAF was defined as a flow signal directed toward the probe. We calculated the prevalence of intracranial RVAF in our subjects. Then, we investigated vascular condition (ie, site of lesion, stenosis, occlusion, and dissection) using magnetic resonance angiography, computed tomography angiography , and CUS in patients with intracranial RVAF. Results Seven hundred twenty patients (508 men; median age, 73 years) were included in this study from September 2007 to March 2013. Intracranial RVAF was seen in 12 patients (1.7%; 11 men; median age, 61 years). Among the 12 patients with intracranial RVAF, 8 patients (67%) had ischemia of the vertebrobasilar territory with distal VA occlusion, according to CUS. Of those patients, 6 (75%) had dissection of the VA. Conclusions TC-CFI detected intracranial RVAF in 1.7% of consecutive examinations in our facility. In vertebrobasilar territory stroke patients with intracranial RVAF, VA dissection may contribute to the development of stroke.

We report a case of complete Heerfordt syndrome accompanied by the involvement of small fiber neuropathy (SFN) manifesting as refracory facial pain. A 30-year-old man presented with pyrexia, a 2-week history of facial burning pain, and difficulty of mastication. After admission to our hospital, neurological examinations showed bilateral facial pain, trigeminal motor palsy, left facial nerve palsy, bilateral sensory neural deafness, uveitis and swelling of the parotid gland. Other examinations revealed bilateral hilar lymphadenopathy, high serum titer of angiotensin coenzyme, and no response in a tuberculin-tested, non-caseating epithelioid granuloma from lip biopsy, leading to the diagnosis of complete Heerfordt syndrome. Mandibular skin biopsy with immunostaining for PGP 9.5 showed SFN. High-dose corticosteroids proved somewhat effective against SFN as facial pain, but reducing the corticosteroid dose proved difficult, as symptoms were refractory to other immunosuppressants and pain-control drugs such as anti-epileptics and anti-depressants. The patient died of acute pancreatitis 3 years after disease onset. Autopsy showed no granuloma in hilar lymph node, trigeminal nerve, cranial base, nerve root, and muscle. SFN in this case probably represent a cause of refractory facial pain.

Background The goal of this study was to investigate the relationship between white matter lesions on magnetic resonance imaging and flow parameters in the middle cerebral artery (MCA) measured by transcranial color flow imaging. Methods Patients with acute ischemic stroke or transient ischemic attack were included. The relationship between severities of periventricular hyperintensity (PVH) and ultrasonographic parameters in the MCA was investigated. The frequency of PVH was calculated for different categories according to the presence or absence of 2 considerable parameters according to the value of area under the receiver operating characteristic curve. Results MCA flow was successfully measured in 203 temporal windows among 124 patients. After determining the cutoff value of end-diastolic velocity (EDV) and pulsatility index (PI) for the presence of PVH, 4 different categories were established: Category A, EDV more than 40 cm/second and PI less than .7; Category B, EDV more than 40 cm/second and PI more than .7; Category C, EDV less than 40 cm/second and PI less than .7; and Category D, EDV less than 40 cm/second and PI more than .7. The prevalence of PVH gradually increased along with category ( P Conclusions The evaluation of MCA parameters using the combination of PI and EDV may be useful for the prediction of PVH.

Purpose: Vertebral artery hypoplasia (VAH) is congenital anatomical variation, which is frequently observed in clinical situation. In previous reports, it was not unclear whether VAH was the independent risk factor for posterior circulation ischemia. The purpose of this study is to evaluate an impact of VAH on posterior circulation ischemia. Methods: Subjects were patients with acute ischemic stroke who underwent brain MRI and carotid ultrasonography. The diameter of vessel and flow velocities of extracranial vertebral artery (VA) was measured by carotid ultrasonography. Diagnostic criteria of VAH was as follows: 1) diameter of VA 0.75. We divided all patients into three groups by the location of the acute ischemic stroke evaluated by MRI: ischemic lesion on posterior circulation (P group), on anterior circulation (A group), and multiple lesions on both anterior and posterior circulation (AP group). Then, the prevalence rate of VAH was compared between P group and A+AP group. In order to evaluate independent factors of VA occlusion, we conducted multivariate regression analyses. Results: We evaluated a total of 129 consecutive patients (87 male, median age; 71 years). P group was 36 patients, and A+AP group was 93 patients. VAH was seen in 39 patients (31.5%), and VA occlusion was found in 15 patients. The prevalence rate of VAH in P group (44.4%) was significantly higher than in A+AP group (24.7%, p=0.034). In univariate analysis, the patients with VA occlusion were higher rates of hypertension (p=0.066), large artery atherosclerosis (p=0.095), posterior circulation ischemia (p=0.001), and the presence of VAH (p=0.038). Multivariate regression analysis demonstrated that large artery atherosclerosis (odds ratio, 6.3; 95% confidence interval, 1.3-30.1), posterior circulation ischemia (odds ratio, 12.0; 95% confidence interval, 2.8-51.2) and VAH (odds ratio, 4.2; 95% confidence interval, 1.2-15.0) were independently associated with the presence of VA occlusion. Conclusion: VAH was independent factor of VA occlusion, and should be associated with posterior circulation ischemia.