Charles Bonnet syndrome as first manifestation of occipital infarction

Purpose To describe a case of Charles Bonnet syndrome as the first manifestation of occipital infarction in a patient with preserved visual acuity. Observations We report a 78-year-old man followed in our department with a two-month-long history of visual hallucinations based on the vision of flowers and fruits intermittently, being perceived as unreal images. Best-corrected visual acuity was stable in the follow-up time being 20/20 in the right eye and 20/25 in the left eye. Extraocular muscle function testing, pupillary reflexes, biomicroscopy, fundus and optical coherence tomography examinations did not reveal any interesting findings. In order to rule out occipital pathology, orbital-cerebral magnetic resonance imaging was performed, showing an image compatible with the chronic ischemic right occipital lesion. The patient was diagnosed with Charles Bonnet syndrome secondary to occipital infarction and neurology decided that no treatment was required. 24-2 and 10-2 visual field tests showed no remarkable alterations and Full-field 120 point screening test showed nonspecific peripheral defects. Hallucinations improved over the months, being described as not annoying and increasingly infrequent. Conclusions and Importance Charles Bonnet syndrome is a condition characterized by the presence of recurrent and complex visual hallucinations in patients with visual pathway pathologic defects. Visual acuity or visual field loss is not a requirement for diagnosis. Charles Bonnet syndrome should be suspected in all patients with non-disturbing visual hallucinations, even though they present good visual acuteness. It will be essential to perform complementary explorations to identify the underlying pathology that allows the starting of a correct treatment option.

Left Atrial Remodeling and Cerebrovascular Disease Assessed by Magnetic Resonance Imaging in Continuously Monitored Patients

<b><i>Background:</i></b> Atrial remodeling is associated with future atrial fibrillation (AF) and stroke. AF has been associated with cognitive impairment and cerebral white matter lesions. We wished to investigate the possible direct association between atrial remodeling and cerebrovascular disease in patients with and without AF documented by implantable loop recorder (ILR). <b><i>Methods:</i></b> Cardiac and cerebral magnetic resonance imaging were acquired in a cross-sectional study, including participants ≥70 years of age with stroke risk factors without known AF. Cerebrovascular disease was visually rated using the Fazekas scale and number of lacunar strokes. Left atrial (LA) and ventricular volumes and function were analyzed. Associations between atrial remodeling and cerebrovascular disease were assessed with logistic regression models. The analyses were stratified according to sinus rhythm or any AF during 3 months of continuous ILR monitoring to account for subclinical AF. <b><i>Results:</i></b> Of 200 participants investigated, 87% had a Fazekas score ≥1 and 45% had ≥1 lacunar infarct. Within 3 months of ILR monitoring, AF was detected in 28 (14%) participants. For participants with sinus rhythm (<i>n</i> = 172), lower LA passive emptying fraction was associated with Fazekas score after multivariable adjustment (OR [95% CI]: 0.51 [0.27; 0.86] <i>p</i> = 0.02), and increased LA maximum (OR [95% CI]: 1.38 [1.07; 1.82] <i>p</i> = 0.01) and minimum volumes (OR [95% CI]: 1.48 [1.03; 2.17] <i>p</i> = 0.04) were associated with lacunar infarcts. There were no significant associations in patients with AF. <b><i>Conclusion:</i></b> In AF-free patients, as documented by ILR monitoring, we found an independent association between LA passive emptying fraction and Fazekas score and between atrial volumes and lacunar infarcts.

Microvascular proliferation in the clots: The key finding of acute subdural hematoma transforming into chronic subdural hematoma?

Background: Despite extensive investigations, the exact etiology of chronic subdural hematoma (CSDH) remains elusive. Organized CSDHs are a distinct but less-understood type of CSDH. Case Description: A 50-year-old hypertensive woman experienced headache without any previous head injury. At presentation, the patient showed no focal neurological deficits. Cranial computed tomography (CT) revealed a slightly compressive subdural hematoma that spontaneously regressed and no intracranial vascular lesions. Cerebral magnetic resonance imaging identified a non-enhancing nodular lesion in the subdural hematoma. After the patient presented disorientation and aphasia on post hospitalization day 14, CT showed a considerable enlargement of the subdural hematoma. Partial removal of the bi-layered hematoma was performed through a parietal craniotomy. Histological examination revealed microvascular proliferation in both the outer membrane and the nodular lesion. On postoperative day 35, CT demonstrated a remarkable resolution of the residual hematoma. Conclusion: Development of microvascular proliferation in the clots of an acute subdural hematoma may lead to its rapid enlargement as an organized CSDH. Organized CSDH can be managed by partial removal of the outer membrane and hematoma through a craniotomy.

A Combined Clinical and Serum Biomarker-Based Approach May Allow Early Differentiation Between Patients With Minor Stroke and Transient Ischemic Attack as Well as Mid-term Prognostication

Background: Early differentiation between transient ischemic attack (TIA) and minor ischemic stroke (MIS) impacts on the patient's individual diagnostic work-up and treatment. Furthermore, estimations regarding persisting impairments after MIS are essential to guide rehabilitation programs. This study evaluated a combined clinical- and serum biomarker-based approach for the differentiation between TIA and MIS as well as the mid-term prognostication of the functional outcome, which is applicable within the first 24 h after symptom onset.Methods: Prospectively collected data were used for a retrospective analysis including the neurological deficit at admission (National Institutes of Health Stroke Scale, NIHSS) and the following serum biomarkers covering different pathophysiological aspects of stroke: Coagulation (fibrinogen, antithrombin), inflammation (C reactive protein), neuronal damage in the cellular [neuron specific enolase], and the extracellular compartment [matrix metalloproteinase-9, hyaluronic acid]. Further, cerebral magnetic resonance imaging was performed at baseline and day 7, while functional outcome was evaluated with the modified Rankin Scale (mRS) after 3, 6, and 12 months.Results: Based on data from 96 patients (age 64 ± 14 years), 23 TIA patients (NIHSS 0.6 ± 1.1) were compared with 73 MIS patients (NIHSS 2.4 ± 2.0). In a binary logistic regression analysis, the combination of NIHSS and serum biomarkers differentiated MIS from TIA with a sensitivity of 91.8% and a specificity of 60.9% [area under the curve (AUC) 0.84]. In patients with NIHSS 0 at admission, this panel resulted in a still acceptable sensitivity of 81.3% (specificity 71.4%, AUC 0.69) for the differentiation between MIS (n = 16) and TIA (n = 14). By adding age, remarkable sensitivities of 98.4, 100, and 98.2% for the prediction of an excellent outcome (mRS 0 or 1) were achieved with respect to time points investigated within the 1-year follow-up. However, the specificity was moderate and decreased over time (83.3, 70, 58.3%; AUC 0.96, 0.92, 0.91).Conclusion: This pilot study provides evidence that the NIHSS combined with selected serum biomarkers covering pathophysiological aspects of stroke may represent a useful tool to differentiate between MIS and TIA within 24 h after symptom onset. Further, this approach may accurately predict the mid-term outcome in minor stroke patients, which might help to allocate rehabilitative resources.

Association of Blood Pressure, Its Treatment, and Treatment Efficacy With Volume of White Matter Hyperintensities in the Population-Based 1000BRAINS Study

White matter hyperintensities (WMHs) of presumed vascular origin are a frequent finding in cerebral magnetic resonance imaging of older people. They are attributed to small vessel disease and involved in the pathogenesis of cognitive decline. Since vascular risk factors, especially arterial hypertension, predispose to small vessel disease, we analyzed the association of systolic blood pressure (SBP), diastolic blood pressure (DBP), and antihypertensive medications with WMH volume in 560 participants of the 1000BRAINS study, drawn from the population-based Heinz Nixdorf Recall study (65.2±7.5 years; 51.4% men). Further, we analyzed treatment efficacy using a classification of 6 BP treatment groups defined by antihypertensive medication and level of BP: (1) untreated BP <120/<80 mm Hg, (2) untreated SBP 120 to 139 or DBP 80 to 89 mm Hg, (3) untreated BP ≥140 or ≥90 mm Hg, (4) treated BP <120/<80 mm Hg, (5) treated SBP 120 to 139 or DBP 80 to 89 mm Hg, and (6) treated BP ≥140 or ≥90 mm Hg. Median WMH volume (Q1–Q3) was 4.6 (3.0–7.8) cm 3 ; mean±SD of SBP and DBP was 128.6±17.4 and 76.1±9.8 mm Hg. In multivariable linear regression models, continuous SBP (β=0.63 cm 3 per 10 mm Hg [95% CI, 0.32–0.94]), DBP (0.64 cm 3 per 5 mmHg [95% CI, 0.37–0.91]), and antihypertensive treatment (1.23 cm 3 [95% CI, 0.14–2.23]) were significantly associated with WMH volume. Regarding treatment efficacy, only participants with hypertension despite treatment (treated BP ≥140 or ≥90 mm Hg) had significantly increased WMH volume (4.24 cm 3 [2.36–6.13]) compared with normotension without treatment (untreated BP <120/<80 mm Hg). Our results suggest that WMHs represent a marker of advanced hypertension pathology. Hence, early treatment should prevent WMHs.

Machine Learning-Based Analysis of Magnetic Resonance Radiomics for the Classification of Gliosarcoma and Glioblastoma

ObjectiveTo identify optimal machine-learning methods for the radiomics-based differentiation of gliosarcoma (GSM) from glioblastoma (GBM).Materials and MethodsThis retrospective study analyzed cerebral magnetic resonance imaging (MRI) data of 83 patients with pathologically diagnosed GSM (58 men, 25 women; mean age, 50.5 ± 12.9 years; range, 16-77 years) and 100 patients with GBM (58 men, 42 women; mean age, 53.4 ± 14.1 years; range, 12-77 years) and divided them into a training and validation set randomly. Radiomics features were extracted from the tumor mass and peritumoral edema. Three feature selection and classification methods were evaluated in terms of their performance in distinguishing GSM and GBM: the least absolute shrinkage and selection operator (LASSO), Relief, and Random Forest (RF); and adaboost classifier (Ada), support vector machine (SVM), and RF; respectively. The area under the receiver operating characteristic curve (AUC) and accuracy (ACC) of each method were analyzed.ResultsBased on tumor mass features, the selection method LASSO + classifier SVM was found to feature the highest AUC (0.85) and ACC (0.77) in the validation set, followed by Relief + RF (AUC = 0.84, ACC = 0.72) and LASSO + RF (AUC = 0.82, ACC = 0.75). Based on peritumoral edema features, Relief + SVM was found to have the highest AUC (0.78) and ACC (0.73) in the validation set. Regardless of the method, tumor mass features significantly outperformed peritumoral edema features in the differentiation of GSM from GBM (P &lt; 0.05). Furthermore, the sensitivity, specificity, and accuracy of the best radiomics model were superior to those obtained by the neuroradiologists.ConclusionOur radiomics study identified the selection method LASSO combined with the classifier SVM as the optimal method for differentiating GSM from GBM based on tumor mass features.

Postnatal Diagnostic Workup in Children With Arthrogryposis: A Series of 82 Patients

Objective: To describe a postnatal series of patients with arthrogryposis multiplex congenita by the causal mechanisms involved. Methods: In this single-center study, the local data warehouse was used to identify patients with arthrogryposis multiplex congenita. Patients were classified into different etiologic groups. Results: Of 82 patients included, the most frequent cause of arthrogryposis multiplex congenita was a neuromuscular disorder (39%), including skeletal muscle (n = 19), neuromuscular junction (n = 3), and peripheral nerve (n = 11) involvement. In other subgroups, 19 patients (23%) were classified by disorders in the central nervous system, 5 (6%) in connective tissue, 7 (8.5%) had mixed mechanisms, and 18 (22%) could not be classified. Contractures topography was not associated with a causal mechanism. Cerebral magnetic resonance imaging (MRI), electroneuromyography, and muscle biopsy were the most conclusive investigations. Metabolic investigations were normal in all the patients tested. Targeted or whole exome sequencing diagnostic rates were 51% and 71%, respectively. Thirty-three percent of patients died (early death occurred in patients with polyhydramnios, prematurity, and ventilatory dependency). Discussion: The benefits of a precise diagnosis in the neonatal period include more tailored management of arthrogryposis multiplex congenita and better genetic information.

Assessment of neurological function using the National Institute of Health Stroke Scale in patients with gliomas

Background The evaluation of treatment response in patients with gliomas is performed using the Response Assessment in Neuro-Oncology (RANO) criteria. These criteria are based on cerebral magnetic resonance imaging (MRI), steroid use and neurological function. However, a standardized tool for evaluating neurological function was lacking. We compared changes in the National Institute of Health Stroke Scale (NIHSS) to changes in the RANO categories to determine the relationship between clinical and neuroradiological findings. Methods We reviewed data on all adult patients with supratentorial gliomas WHO grade II-IV who were treated at the Cantonal hospital St. Gallen from 2008 – 2015. The NIHSS was performed prospectively at baseline and at 3-months intervals simultaneously to MRI. Associations between changes in the NIHSS and RANO categories were assessed using the Stuart-Maxwell test. Results Our cohort consisted of 61 patients from which 471 observations were analyzed. The most common histological diagnosis was glioblastoma (49.2%). In total, 74% of RANO categories and 81% of the NIHSS scores remained stable on follow-up. Statistically, contemporaneous changes in the RANO category did not correlate with changes in the NIHSS (p &lt; 0.0001). Conclusion The application of the NIHSS is easy and feasible in the heterogeneous population of glioma patients. In our cohort, the RANO categories did not reflect contemporaneous changes in the NIHSS. A validated clinical outcome measure with a well-defined minimal clinically important difference is warranted in neuro-oncological research and clinical practice.