小出 玲爾

Primary central nervous system lymphoma (PCNSL) is a rare form of non-Hodgkin lymphoma, but its diagnosis is challenging in some cases. A brain biopsy is the gold standard for diagnosing PCNSL, but its invasiveness can be problematic. Thus, noninvasive imaging examinations have been developed for the pre-surgical diagnosis of PCNSL, including gadolinium-enhanced magnetic resonance imaging (MRI), 123I-N-isopropyl-p-iodoamphetamine single-photon emission computed tomography (123I-IMP SPECT), and positron emission tomography with 18F-fluorodeoxyglucose (18F-FDG PET). Here, we report the case of a 71-year-old woman with negative imaging findings for PCNSL, but who was diagnosed with PCNSL by a brain biopsy and histological analysis. Her imaging results were negative for gadolinium-enhanced cranial MRI, with low uptake in 123I-IMP SPECT and hypometabolism in 18F-FDG PET. However, a stereotactic brain biopsy from an abnormal lesion revealed that many round cells had infiltrated into the brain. Moreover, many infiltrating cells were positive for cluster of differentiation (CD)20 and CD79a, and proliferation marker protein Ki-67-positive cells accounted for nearly 80% of all cells. Based on these results, our final pathological diagnosis was PCNSL. The present case highlights the possibility of a PCNSL diagnosis even when all imaging-related examinations display negative results.

BACKGROUND: Although the relationship between amyotrophic lateral sclerosis (ALS) and cervical spondylotic myelopathy (CSM) is important, data relating to CSM complications in ALS remain lacking. PURPOSE: We aimed to investigate and validate the spinal cord conditions of ALS patients. MATERIALS AND METHODS: We recruited all patients diagnosed with ALS, Parkinson's disease (PD), or chronic inflammatory demyelinating polyneuropathy (CIDP) who were admitted to our department from April 1, 2017, to March 31, 2020. We analyzed the cervical or thoracolumbar magnetic resonance imaging (MRI) scans of these 128 patients. Data relating to spondylosis, cord compression, spinal canal diameter, spinal cord diameter, and the closest distance between the cervical spinal canal and cord were validated using MRI. RESULTS: Of the 128 patients, 52 had ALS, 48 had PD, and 28 had CIDP. The proportions of both cervical spondylosis and cervical cord compression were highest in the ALS group compared with the other patient groups (p < 0.05). The proportion of cervical spondylosis in ALS patients reached 38.3%, and that of cervical cord compression reached 53.2%. The closest distance between the cervical spinal canal and cord was also significantly smaller in ALS patients compared with CIDP patients (p < 0.05). In contrast to the cervical cord findings, there were no significant differences in the thoracolumbar cord between ALS patients and the other patient groups. CONCLUSIONS: Of the three disease groups, the proportion of CSM was highest in ALS patients. Furthermore, cervical cord conditions were significantly more crowded in the ALS patients than in the other patient groups.

Cardiac involvement has recently been the focus of sporadic late-onset nemaline myopathy (SLONM). However, right ventricular failure and pulmonary hypertension, in addition to repetitive cardiac arrest, are noteworthy characteristics of SLONM. We herein report a 66-year-old woman with SLONM whose main symptoms were cardiac arrest, right ventricular failure, and pulmonary hypertension. Despite permanent pacemaker replacement, cardiac arrest occurred repetitively, and even with continuous positive airway pressure, right ventricular failure and pulmonary hypertension persisted. The patient was finally diagnosed with SLONM by a muscle biopsy. Our case suggests the possibility of cardiovascular involvement in SLONM, especially right ventricular failure and pulmonary hypertension.

BACKGROUND: Many issues persist in the today's Alzheimer's disease (AD) screening and the breakthrough method is desired. OBJECTIVE: We aim to validate the association between venous reflux and AD, and to develop a new method for AD screening. METHODS: We examined spontaneous echo contrast, area, diameter, retrograde velocity, and anterograde velocity of the bilateral cervical internal jugular vein (IJV) using carotid ultrasonography. RESULTS: A total of 112 patients participated in this study, with 26 diagnosed as AD. The proportion of both or either IJV spontaneous echo contrast (+) occupied 25 of total 26 AD patients, which showed 96.2%of sensitivity and 98.5%negative predictive value. The IJV velocities also showed significant correlation with AD diagnosis, although the IJV area or diameter did not. CONCLUSION: Our results indicate that the validation of the spontaneous echo contrast or velocities of the IJV are convenient AD diagnosis screening methods and that the venous reflux disturbance correlates with AD development.

AIM: Aortic arch atherosclerosis, particularly complex aortic arch plaques (CAPs), is an important source of cerebral emboli. CAPs and atrial fibrillation (AF) often co-exist; however, the prevalence and risk of CAPs in acute ischemic stroke patients with AF is unclear. METHODS: In patients with acute ischemic stroke with non-valvular AF admitted to Jichi Medical University Hospital during April 2016 to September 2019, we retrospectively evaluated the presence of CAPs on transesophageal echocardiography (TEE). RESULTS: CAPs were observed in 41 (38.7 %) of 106 patients with non-valvular AF. Older age, diabetes mellitus, chronic kidney disease, low high-density lipoprotein cholesterol (HDL-C) levels, higher levels of glycohemoglobin A1c (HbA1c), higher CHADS2 and CHA2DS2-VASc scores, and intracranial or carotid artery stenosis were more frequently observed in CAPs-positive than in CAPs-negative patients. In multivariable analyses, older age (odds ratio [OR]: 1.2 per year increase; 95% confidence interval [CI]: 1.07-1.24; P＜0.0001), diabetes mellitus (OR: 4.7; 95%CI: 1.27-17.35; P＜0.05), and low HDL-C (OR: 0.95 per 1 mg/dl increase; 95%CI: 0.92-0.99; P ＜0.01) were independent risk factors for CAPs. The prevalence of CAPs was age-dependent, and there was a significantly higher risk in patients aged either 75-84 years or ＞84 years than in those aged ＜65 (OR: 7.6; 95%CI: 1.50-38.62, and OR: 32.1; 95%CI: 5.14-200.11, respectively). CONCLUSIONS: Even in patients with ischemic stroke with non-valvular AF, concomitant CAPs should be considered in older individuals and those who have diabetes or low HDL-C.

OBJECTIVES: Magnetic resonance angiography (MRA), three-dimensional computed tomography angiography, and cerebral angiography may be used to assess intracranial vertebrobasilar stenosis. However, these examinations cannot be performed at patients' bedsides. Our purpose was to develop a new bedside method to assess intracranial vertebrobasilar arterial stenosis. METHODS: We developed the new method using carotid duplex ultrasonography combined with the head-up test. A total of 141 subjects admitted between June 1, 2017 and March 31, 2019 were enrolled in this study. We calculated vertebral arterial peak systolic velocities (PSVs), end-diastolic velocities (EDVs), and mean velocities (MVs) at 0°, 16°, and 30° head-up angles. Vertebrobasilar arterial stenosis was confirmed using MRA. RESULTS: We excluded 28 subjects and included data for 113 subjects and 226 vessels in the final analysis. Cervical vertebral arterial PSV, EDV, and MV gradually decreased from 0° to 30° only in stenotic intracranial vertebral arteries. Sensitivity (probability of detection) was 75.5% and specificity (true negative rate) was 79.7% when EDV at the 30° head-up angle decreased ≥19.5% from the initial 0° head-up angle. Specificity was better (86.4%; sensitivity: 69.4%) when EDV was <9.1 cm/s at the 30° head-up angle. CONCLUSION: This new method easily detects intracranial vertebrobasilar arterial stenosis.

The treatment of ischemic stroke has recently witnessed dramatic developments. However, there are limited data on ischemic stroke characteristics in aged patients. As part of the South Tochigi Acute Ischemic Stroke Registry, we prospectively enrolled 636 consecutive acute ischemic stroke patients (within 7 days after the onset) who were ≥ 60 years of age and who were admitted to two independent institutes from April 1, 2016 to February 28, 2019. We analyzed three groups divided by age: early-aged (60-69 years), middle-aged (70-79 years), and oldest-aged (≥ 80 years). From the 636 subjects, 194 were early-aged, 215 were middle-aged, and 227 were oldest-aged. There were significant differences in the ischemic stroke subtypes in each aging group (p < 0.01). The proportion of cardioembolism was 22.2% in early-aged, 27.4% in middle-aged, and 41.4% in the oldest-aged patients. The proportion of patients with a modified Rankin Scale of 0-2 at 1 year after onset decreased to 42.2% in middle-aged and 17.8% in oldest-aged with cardioembolic ischemic stroke. The proportion of patients receiving anticoagulation therapy before admission was 25.6% (36.7% of atrial fibrillation [AF]) in early-aged, 39.0% (52.3% of AF) in middle-aged, and 18.1% (21.0% of AF) in oldest-aged patients (p < 0.001). Our study reports characteristics of clinical ischemic stroke in an aging population. The assessment of cardiogenic embolism is important for an aging population.

Isolated vertigo is an important symptom of posterior circulation stroke. It has been reported that 11.3% of patients with isolated vertigo have a stroke and that most lesions are located in the cerebellum, particularly in the posterior inferior cerebellar artery. We report the case of a 63-year-old man with multiple atherosclerotic risk factors and atrial fibrillation who showed repeated episodes of isolated vertigo. His repeated vertigo was short-lasting and was often triggered by body position, mimicking benign paroxysmal positional vertigo. Cranial computed tomography on the third hospital day showed left cerebellar infarction within the territory of the posterior inferior cerebellar artery. The vertigo was ameliorated on the fifth hospital day and warfarin was prescribed for secondary prevention. Clinicians should pay special attention to cases in which a patient presents isolated vertigo, even if it shows transient recurrence or is triggered by a positional change, especially in patients with multiple cerebrovascular risk factors.

Fatalities following intravenous recombinant tissue-type plasminogen activator therapy have been reported. Major fatal complications following intravenous recombinant tissue-type plasminogen activator therapy include intracranial hemorrhage, aortic dissection, and extracranial bleeding. However, the possibility that intravenous recombinant tissue-type plasminogen activator therapy itself paradoxically induces synchronized multiple cerebral novel infarctions has never been considered. We herein report the first case of bilateral internal carotid artery infarction with onset seizure following intravenous recombinant tissue-type plasminogen activator therapy for a vertebral-basilar artery infarction. A 75-year-old man was transferred to our hospital and diagnosed with acute ischemic stroke in the basilar artery. His National Institute of Health Stroke Scale score was 4. The intravenous recombinant tissue-type plasminogen activator therapy was initiated 234 minutes after stroke onset because no contraindications were present. Almost 2 hours after the intravenous recombinant tissue-type plasminogen activator therapy, the patient suddenly fell into a deep coma with generalized convulsions. A huge secondary infarction was found in the bilateral anterior circulation territories, and he died 7 days after stroke onset. This case alerts clinicians to the possibility of synchronized multiple cerebral infarctions following intravenous recombinant tissue-type plasminogen activator therapy as a dangerous complication in patients with multiple severe stenoses in the cerebral arteries.

Vertical gaze palsy is rarely a neurological symptom, although it has been observed in some cases. Here, we report the case of a patient presenting with complete upward and downward gaze palsy. In this case, a small lesion in the left rostral midbrain was observed on diffusion-weighted magnetic resonance (MR) images, and the lesion was considered to cause the ocular symptom. We consider that vertical gaze palsy is an important clue to an accurate topical diagnosis of a brain lesion.

Although foam sclerotherapy to varicose veins is now a popular treatment because of its high efficacy and safety, some neurologic complications have recently been reported. Presently, the effectiveness and safety of intravenous recombinant tissue-type plasminogen activator therapy to stroke following foam sclerotherapy remain unclear. Here, we report the case of a 68-year-old woman whose ischemic symptoms following foam sclerotherapy were treated by intravenous recombinant tissue-type plasminogen activator. After she was admitted, the venous thrombosis in her right soleus vein and a patent foramen ovale causing the right-to-left shunt were revealed. Thus, we diagnosed the ischemic symptoms were due to paradoxical embolism following foam sclerotherapy. After intravenous recombinant tissue-type plasminogen activator therapy, there was no complication and the outcome was good. Our case suggests the effectiveness and the safety of intravenous recombinant tissue-type plasminogen activator therapy to paradoxical embolism following foam sclerotherapy.