暂无摘要。

Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS) is a rare chronic central-nervous-system inflammatory disorder that became known only recently, and the pathogenesis of CLIPPERS remains poorly understood. This report presents clinical and radiological features of a rare case: a young female patient who rapidly died of suspected CLIPPERS. Helpful multiparametric MRI diagnostic criteria are proposed that can help discriminate CLIPPERS from non-CLIPPERS pathologies. We reviewed clinical history, symptoms, quantitative data from brain multiparametric MRI before and after treatment, and histopathological data. Perfusion-weighted imaging revealed a decrease in regional cerebral blood flow by 31% and in cerebral blood volume by 64%, with a moderate increase in transit time and in time to peak by up to 23% in affected pontine and cerebral white matter. As estimated by diffusion tensor imaging, there was elevated density of tracts (n/mm2) and a decrease of fraction anisotropy (×10-3 mm/s2) in the patient\'s pons as compared to a healthy control: density of tracts = 13.5 vs 12.4 and fraction anisotropy = 0.32 vs 0.45, respectively. Macromolecular proton fraction values proved to be reduced (15.8% and 14.5% in the control, respectively) in the patient\'s cerebral peduncles by 3% and in the pons by 4.1% and in a periventricular white matter lesion by 6.4% (11.3% in the normal-looking contralateral hemisphere). Based on our findings, we argue that quantitative MRI techniques may be a valuable source of biomarkers and reliable diagnostic criteria and can shed light on the pathogenesis and exact nosological position of this disorder.

UNASSIGNED: Cerebral small vessel diseases (SVDs) are a major cause of stroke and dementia. Yet, specific treatment strategies are lacking in part because of a limited understanding of the underlying disease processes. There is therefore an urgent need to study SVDs at their core, the small vessels themselves.
UNASSIGNED: This paper presents the rationale and design of the ZOOM@SVDs study, which aims to establish measures of cerebral small vessel dysfunction on 7T MRI as novel disease markers of SVDs.
UNASSIGNED: ZOOM@SVDs is a prospective observational cohort study with two years follow-up. ZOOM@SVDs recruits participants with Cerebral Autosomal Dominant Arteriopathy with Subcortical Infarcts and Leukoencephalopathy (CADASIL, N = 20), sporadic SVDs (N = 60), and healthy controls (N = 40). Participants undergo 7T brain MRI to assess different aspects of small vessel function including small vessel reactivity, cerebral perforating artery flow, and pulsatility. Extensive work-up at baseline and follow-up further includes clinical and neuropsychological assessment as well as 3T brain MRI to assess conventional SVD imaging markers. Measures of small vessel dysfunction are compared between patients and controls, and related to the severity of clinical and conventional MRI manifestations of SVDs.
UNASSIGNED: ZOOM@SVDs will deliver novel markers of cerebral small vessel function in patients with monogenic and sporadic forms of SVDs, and establish their relation with disease burden and progression. These small vessel markers can support etiological studies in SVDs and may serve as surrogate outcome measures in future clinical trials to show target engagement of drugs directed at the small vessels.

Staphylococcus aureus infectious endocarditis has a high mortality, major causes of death being cardiac failure, systemic embolism, and sepsis. Pseudoaneurysms, a rare complication of this infection, are not invariably fatal with appropriate treatment. A previously healthy 32-year-old man was found to have multiple cerebral infarctions, and infectious endocarditis with mitral valve vegetation was diagnosed by echocardiography. Because methicillin-resistant Staphylococcus aureus (MRSA) was identified from blood cultures, vancomycin was administered. Massive intracerebral hemorrhage in the left temporo-occipital lobe occurred in the patient on the 3rd day after admission, and the hematoma was completely removed surgically. Another hemorrhage was identified in the right occipital region on the 7th hospital day, which led the patient deep coma. Blood cultures on the 10th day were negative for MRSA; however, imaging studies revealed pseudoaneurysms in the superior mesenteric, hepatic, and left popliteal arteries 3 weeks after admission. No surgical indication was applied to these pseudoaneurysms because the patient remained comatose. On the 78th day after admission, the patient\'s blood pressure suddenly dropped and he died. Autopsy demonstrated massive bleeding in the abdominal cavity caused by rupture of the superior mesenteric artery pseudoaneurysm. Our patient\'s clinical course was fulminant, his endocarditis being complicated by cerebral infarctions, intracranial hemorrhages, and multiple pseudoaneurysms within 3 weeks of admission. In retrospect, he may have survived if emergency resection of the mitral valve vegetation had been performed on the first or second day of admission; however, the in-hospital mortality rate after such surgery is high.

Mitochondrial myopathy, encephalopathy, lactic acidosis, and stroke-like episodes (MELAS) syndrome presents with the features of herpes simplex encephalitis (HSE), which is rare and has been described in only a few case reports. Our case describes a 17-year-old female with no significant previous medical history presenting with an acute onset of fever, headache, and epilepsy, similar to HSE. Computed tomography of the brain showed bilateral basal ganglia calcification. Magnetic resonance imaging demonstrated gyriform restricted diffusion with T2-weighted images prolongation. Further investigation showed elevated blood lactate concentration at rest. Hence, MELAS was suspected and the diagnosis was confirmed by the presence of a nucleotide 3243 A→G mutation in the mitochondrial DNA. The clinical presentation and imaging studies of MELAS are variable and may mimic those of HSE. Infection may have also precipitated MELAS manifestation in this patient. Laboratory features, such as elevated lactate, basal ganglia calcification, and gyriform restricted diffusion may be helpful in identifying patients with MELAS.

暂无摘要。

•A patient exhibited IgG4-related hypothalamo-hypophysitis.•Prominent high-signal areas of swelling were observed in the hypothalamus, tuber cinereum, infundibulum, and bilateral optic nerve systems.•MRI T1WI with contrast media demonstrated enhanced neurohypophysis and cystic swelling, and compressed anterior pituitary.•MRI findings improved rapidly after 4 days of steroid therapy.

UNASSIGNED: Tacrolimus is a commonly used immunosuppressant medication after lung transplantation. In rare cases, tacrolimus causes a medication-induced optic neuropathy (TON) that can lead to significant vision loss.
UNASSIGNED: In this series, we describe three cases of TON, 1-10 years after medication use. Two patients were young (22yr and 33yr) females with cystic fibrosis. The last case was a 65yr male with idiopathic pulmonary fibrosis. In 2/3 cases tacrolimus serum levels were normal. Visual acuity ranged from 20/20 to 20/300, and vision loss occurred acutely to sub-acutely, over a span of 2-3 months.
UNASSIGNED: As presented here, TON can be highly variable. MRI findings are often non-specific, from normal brain findings to extensive white matter changes. There remains an unclear association with graft-versus-host disease and reduced kidney function. Visual findings are often subtle, including color vision aberration and peripheral visual field deficits, both of which usually require an ophthalmologic evaluation. When diagnosed in a timely fashion, TON is at least partially reversible in up to half of all cases. While rare, the cases described here support post-lung transplant ophthalmologic evaluation in those taking high-risk medications.

UNASSIGNED: Although silent brain infarction is an independent risk factor for subsequent symptomatic stroke and dementia in patients with nonvalvular atrial fibrillation, little is known regarding differences in risk factors for silent brain infarction between patients with paroxysmal and persistent nonvalvular atrial fibrillation.
UNASSIGNED: This study population consisted of 190 neurologically asymptomatic patients (mean age, 64 ± 11 years) with nonvalvular atrial fibrillation (119 paroxysmal, 71 persistent) who were scheduled for catheter ablation. All patients underwent brain magnetic resonance imaging to screen for silent brain infarction prior to ablation. Transthoracic and transesophageal echocardiography was performed to screen for left atrial abnormalities (left atrial enlargement, spontaneous echo contrast, or left atrial appendage emptying velocity) and complex plaques in the aortic arch.
UNASSIGNED: Silent brain infarction was detected in 50 patients (26%) [26 patients (22%) in paroxysmal vs. 24 patients (34%) in persistent, p = 0.09]. Multiple logistic regression analysis indicated that age and diabetes mellitus or chronic kidney disease (estimated glomerular filtration rate < 60 mL/min/1.73 m2) were associated with silent brain infarction in patients with paroxysmal nonvalvular atrial fibrillation (p < 0.05), whereas no modifiable risk factors of silent brain infarction were observed in patients with persistent nonvalvular atrial fibrillation.
UNASSIGNED: These findings suggest that intensive intervention for diabetes mellitus and renal impairment from the paroxysmal stage or ablation therapy at the time of paroxysmal stage to prevent progression to persistent nonvalvular atrial fibrillation may prevent silent brain infarction and consequently reduce the risk of future symptomatic stroke.

Hemolytic uremic syndrome is a frequent complication of shiga toxin producing Escherichia coli in pediatric population. It rarely affects adults with extremely rare neurological manifestation. We present a case of hemolytic uremic syndrome in a 64-year-old male who presented with a bloody diarrhea 30 minutes after eating an expired meat sandwich. Shiga-toxin producing Escherichia coli O157:H7 was confirmed as the causative agent. The patient developed neurological manifestations with persistent encephalopathy that ultimately leads to his death after 22 days of hospitalization. Magnetic resonance imaging findings was significant for signal changes in the thalami, tectum, insulae, and central pons, impressive of hemolytic uremic syndrome.