Cerebral vasculitis as a clinical manifestation of neurosarcoidosis: A scoping review.

The occurrence of cerebral vasculitis in individuals with neurosarcoidosis (NS) is considered to be rare. Although the number of relevant publications has increased in recent years, evidence is mostly limited to case reports. To obtain a better understanding of this rare and severe manifestation of disease, we carried out a scoping review on cerebral vasculitis in patients diagnosed with NS. The results of the review indicate that the diagnosis of cerebral vasculitis in patients with NS is made especially in patients with systemic sarcoidosis. However, recurrent strokes in patients with NS remains the main indicator of cerebral vasculitis. A tissue biopsy is considered the gold standard to confirm the diagnosis despite occasional false-negative results. Glucocorticoids and steroid-sparing agents are the most successful current treatments. Favorable outcomes were observed with strategies targeting TNFα and B cells. The goal of this review is to summarize the current literature and treatment options for cerebral vasculitis in patients with NS.

First epileptic seizure and quality of life - A prospective study.

OBJECTIVE: Impaired QoL and depression are common in patients with chronic epilepsies; however, data on the impact of a first seizure on QoL are sparse. According to the current ILAE-definition of epilepsy, patients may be diagnosed with epilepsy immediately after the first seizure, if EEG and/or imaging findings are abnormal. Patients with normal findings in imaging and EEG are not diagnosed as having epilepsy. We investigated QoL in patients after a first seizure with and without a consecutive diagnosis of epilepsy to detect differences between groups within the first year after seizure. METHODS: We examined patients (n = 152) after a first epileptic seizure and six and 12 months thereafter using demographic, clinical and QoL-related questionnaire data (Short Form-36 Health Survey (SF-36), Quality of Life in Epilepsy Inventory-31 (QOLIE-31), Beck's depression inventory II (BDI-II)). RESULTS: Patients diagnosed with epilepsy after the first seizure showed a tendency of reduced mental health-related QoL six (p =.098) and 12 months (p =.092) after the first seizure compared to patients who were not diagnosed with epilepsy, but were diagnosed as having had a single first seizure. There were no significant differences between the two groups in physical health-related QoL. Multiple regression analyses showed that especially depressive symptoms explained 22.0 - 48.7 % of the variance in mental health-related QoL six (p <.001) and 12 months (p <.001) after the first seizure. Physical health-related QoL was especially predicted by age (p <.001), group (p =.002) and recurrent seizures (p = < 0.001). In PWE, there was a statistical trend with improving QOLIE-31 overall scores from six to 12 months (p =.086). CONCLUSION: Our results suggest that QoL may be impaired in patients diagnosed with epilepsy early, immediately after the onset of disease. Early follow-up monitoring from the beginning of patient career is important for possible interventions and to improve patients' daily life in the long term.

Morphometric correlates in patients with functional seizures with and without comorbid epilepsy.

OBJECTIVE: Functional seizures (FS) or psychogenic, non-epileptic seizures (PNES) are episodic alterations of behaviour with similar semiology to epileptic seizures but which are not caused by epileptic brain activity. Epilepsy patients show a high risk in developing FS; therefore, the purpose of this study is to examine morphometric correlates in patients with FS as well as in epilepsy patients with FS by comparing them separately to healthy controls (HC). METHODS: Twenty-one clinical three-dimensional (3D) T1-magnetic resonance imaging (MRI) scans of patients with FS (FS group) and 15 patients with FS and epilepsy (EFS group) were retrospectively compared with one control group of 21 age- and gender-matched HC. Two separate general linear model analyses were conducted via FreeSurfer version 6.0. RESULTS: The study population consisted of 21 FS patients (66.7% females, n = 14) with a median age at the time of the scan of 24 years (range 17-44 years); 15 EFS patients (80% females, n = 12) with a median age at the time of the scan of 27 years (range 16-43 years); and 21 healthy subjects (66.7% females, n = 14) with a median age at the time of the scan of 24 years (range 19-38 years). Both patient groups showed an increased Cth in the right prefrontal lobe: in the FS group in the right superior frontal, rostral middle frontal gyri and the right orbitofrontal cortex and, in the EFS group, in the right superior frontal gyrus and the right orbitofrontal cortex. Decreases in Cth were present in the right lateral occipital lobe in the FS group, while also in both hemispheres in the EFS group, namely the left paracentral, superior frontal, caudal middle frontal, lateral occipital and right superior frontal gyri. Neither group showed changes in curvature. CONCLUSION: These results suggest alterations in regions of emotional processing and executive control in patients with FS regardless of the presence of epilepsy.

Temporal encephaloceles and coexisting epileptogenic lesions.

OBJECTIVE: This study was performed to identify coexisting structural lesions in patients with epilepsy and known temporal encephaloceles (TEs). METHODS: Forty-seven structural magnetic resonance imaging (MRI) scans of patients with epilepsy and radiologically diagnosed TEs were retrospectively reviewed visually and using an automated postprocessing software, the Morphometric Analysis Program v2018 (MAP18), to depict additional subtle, potentially epileptogenic lesions in the 3D T1-weighted MRI data. All imaging findings were evaluated in the context of clinical and electroencephalographical findings. RESULTS: The study population consisted of 47 epilepsy patients (38.3% female, n = 18). The median age at the time of the scan was 40 years (range 12-81 years). Twenty-one out of 47 MRI scans (44.7%) showed coexisting lesions in the initial MRI evaluation; in 38.3% (n = 18) of patients, those lesions were considered probably epileptogenic. After postprocessing, probable epileptogenic lesions were identified in 53.2% (n = 25) of patients. Malformations of cortical development had initially been reported in 17.0% (n = 8) of patients with TEs, which increased to 38.3% (n = 18) after postprocessing. TEs and other epileptogenic lesions were considered equally epileptogenic in 21.3% (n = 10) of the cases in the initial MR reports and 25.5% (n = 12) of the cases after postprocessing. SIGNIFICANCE: Temporal encephaloceles are a potential cause of MRI-negative temporal lobe epilepsy. According to our data, TEs can occur with other lesions, suggesting that increased awareness is also required in patients with lesional epilepsy. TEs may not always be epileptogenic; hence, their occurrence with other structural pathologies may influence the presurgical evaluation and surgical approach. Finally, TEs can be associated with malformations of cortical development, which may indicate a common developmental etiology of those lesions.

Perception of memory performance after first seizure in patients with and without an epilepsy diagnosis.

OBJECTIVES: Memory complaints in patients with epilepsy have been well-studied. Although memory complaints are commonly reported by patients with chronic epilepsy, to date, few studies exist on memory complaints at the onset of epilepsy. The present study investigated the presence of memory complaints and their relation to mood and memory performance in patients after their first seizure. Thereby, we examined differences between individuals who received a diagnosis of epilepsy immediately with the occurrence of their first seizure and those who were diagnosed as having the first epileptic seizure, without fulfilling the ILAE criteria for the diagnosis of epilepsy. METHODS: Sixty-one patients participated in the study and completed, among others, a memory task and questionnaires on memory complaints and depression after their first epileptic seizure. We investigated the level of memory complaints and their correlation and accuracy in classification with a memory measure. We compared patients who received an epilepsy diagnosis after the first seizure with those who did not. RESULTS: Memory complaints did not correlate with objective memory performance. Classification into impaired/unimpaired showed low concordance between memory complaints and neuropsychological memory measures. After their first epileptic seizure, patients reported few memory complaints overall (10%), and there were no differences in memory complaints between patients with and without an epilepsy diagnosis. CONCLUSION: At epilepsy onset, in contrast to established epilepsies, memory complaints are rare. Although influences of anticonvulsant drugs and seizures are not present at the beginning of epilepsy, this substantial absence of memory complaints at epilepsy onset emphasizes the need for comprehensive neurological and psychological treatment early with the given diagnosis. Treatment should focus on anticonvulsant drug regimens, patients' concerns and convey realistic expectations.

Transcutaneous auricular vagus nerve stimulation influences gastric motility: A randomized, double-blind trial in healthy individuals.

BACKGROUND: Transcutaneous auricular vagus nerve stimulation (taVNS) has been investigated regarding its therapeutic properties in several several conditions such as epilepsy, migraine and major depressive disorder and was shown to access similar neural pathways as invasive vagus nerve stimulation. While the vagus nerve's role in gut motility is physiologically established, the effect of taVNS has scarcely been investigated in humans and yielded conflicting results. Real-time gastric magnetic resonance imaging (rtMRI) is an established reproducible method to investigate gastric motility non-invasively. OBJECTIVE: To investigate the influence of taVNS on gastric motility of healthy participants using rtMRI. METHODS: We conducted a randomized, double-blind study using high-frequency (HF) stimulation at 25Hz or low-frequency (LF) taVNS at 1Hz after ingestions of a standardized meal in 57 healthy participants. The gastric motility index (GMI) was determined by measuring the amplitude and velocity of the peristaltic waves using rtMRI. RESULTS: After HF taVNS, GMI was significantly higher than after LF stimulation (p = 0.005), which was mainly attributable to a higher amplitude of the peristaltic waves (p = 0.003). CONCLUSION: We provide evidence that 4-h of taVNS influences gastric motility in healthy human participants for the first time using rtMRI. HF stimulation is associated with higher amplitudes of peristaltic waves in the gastric antrum compared to LF stimulation. Further studies are needed to investigate the effect of different frequencies of taVNS and its therapeutic properties in conditions with impaired gastric motility.

Evaluation of a new wireless technique for continuous electroencephalography monitoring in neurological intensive care patients.

A novel wireless eight-channel electroencephalography (EEG) headset specially developed for ICUs was tested in regard of comparability with standard 10/20 EEG systems. The continuous EEG (cEEG) derivations via CerebAir EEG headset (Nihon Kohden Europe, Rosbach, Germany) and internationally standardized 10/20 reference EEGs as the diagnostic standard were performed in a mixed collective on a neurointensive care unit (neuro-ICU). The derivations were verified for comparability in detection of EEG background activity, epileptiform discharges, and seizure patterns. Fifty-two patients with vigilance reduction following serious neurological or metabolic diseases were included, and both methods were applied and further analyzed in 47. EEG background activity matched in 24 of 45 patients (53%; p = 0.126), epileptiform discharges matched in 32 (68%) patients (p = 0.162), and seizure activity matched in 98%. Overall, in 89% of the patients, cEEG detected the same or additional ICU-relevant EEG patterns. The tested wireless cEEG headset is a useful monitoring tool in patients with consciousness disorders. The present study indicates that long-term measurements with the wireless eight-channel cEEG lead to a higher seizure and epileptiform discharge detection compared to intermittent 10/20 EEG derivations in the ICU setting.

Efficacy, Tolerability, and Safety of Concentrated Intranasal Midazolam Spray as Emergency Medication in Epilepsy Patients During Video-EEG Monitoring.

BACKGROUND: An efficient, well tolerated, and safe emergency treatment with a rapid onset of action is needed to prevent seizure clusters and to terminate prolonged seizures and status epilepticus. OBJECTIVES: This study aimed to examine the efficacy, tolerability, and safety of intranasal midazolam (in-MDZ) spray in clinical practice. METHODS: In this retrospective, multicenter observational study, we evaluated all patients with peri-ictal application of in-MDZ during video-EEG monitoring at the epilepsy centers in Frankfurt and Marburg between 2 014 and 2017. For every patient, we analyzed the recurrence of any seizure or generalized tonic-clonic seizures after index seizures with and without in-MDZ administration. Treatment-emergent adverse events (TEAEs) were also evaluated. RESULTS: In-MDZ was used in 243 patients with epilepsy (mean age 35.5 years; range 5-76 years; 46.5% female) for treatment of 459 seizures. A median dose of in-MDZ 5 mg (i.e., two puffs; range 2.5-15 mg) was administered within a median time from EEG seizure onset until in-MDZ application of 1.18 min [interquartile range (IQR) 1.27], while median time from clinical seizure onset until in-MDZ administration was 1.08 min (IQR 1.19). In-MDZ was given within 1 min after EEG seizure onset in 171 seizures. An intraindividual comparison of seizures with and without application of in-MDZ was feasible in 171 patients, demonstrating that in-MDZ reduced the occurrence of any (Cox proportional-hazard model p < 0.001) and generalized tonic-clonic seizure (Cox proportional-hazard model p = 0.0167) over a period of 24 h. The seizure-free timespan was doubled from a median of 5.0 h in controls to a median of 10.67 h after in-MDZ administration. We additionally clustered in-MDZ administrations for the 119 patients who received in-MDZ more than once, comparing them with the index cases without in-MDZ. Even when considering subsequent seizures with in-MDZ administration, a patient receiving in-MDZ is still half as likely to incur another seizure in the upcoming 24 h as compared with when the same patient does not receive in-MDZ (hazard ratio 0.50; 95% CI 0.42-0.60; p < 0.01). In-MDZ was well tolerated without major adverse events. The most common side effects were irritation of the nasal mucosa [37 cases (8.1%)], prolonged sedation [26 cases (5.7%)], and nausea and vomiting [12 cases (2.6%)]. A decline in oxygen saturation was measured after 78 seizures (17%). CONCLUSION: We conclude that in-MDZ is a safe and efficient treatment option to prevent short-term recurrence of seizures. In-MDZ can be administered very quickly by trained staff within 1-2 min after seizure onset. No major cardiocirculatory or respiratory adverse events were observed.

First clinical postmarketing experiences in the treatment of epilepsies with brivaracetam: a retrospective observational multicentre study.

OBJECTIVES: Brivaracetam (BRV) is the latest approved antiepileptic drug and acts as a synaptic vesicle protein 2A ligand. The aim of the present study was to evaluate the efficacy and tolerability of BRV in the clinical setting. DESIGN: Retrospective, observational multicentre study. SETTING: We retrospectively collected clinical data of patients who received BRV in 10 epilepsy centres using a questionnaire that was answered by the reporting neurologist. PARTICIPANTS: Data of 615 epilepsy patients treated with BRV were included in the study. PRIMARY AND SECONDARY OUTCOME MEASURES: Efficacy regarding seizure frequency and tolerability of BRV were evaluated. Descriptive statistics complemented by X2 contingency tests and effect sizes were performed. RESULTS: Overall, 44% of the patients had a decreased, 38% a stable and 18% an increased seizure frequency. 17% of patients achieved seizure freedom after initiation of BRV. The seizure frequency decreased in 63% of 19 patients with BRV monotherapy. 27% reported adverse effects, but only 10% of patients with monotherapy. Brivaracetam was significantly more often associated with decreased seizure frequency in levetiracetam (LEV) naïve patients (p=0.012), but BRV also led to a decreased seizure frequency in 42% of patients who had been treated with LEV before, including 17% of patients who were completely seizure free. Adverse effects under LEV improved in 62% and deteriorated in 2% of patients after the switch to BRV. At latest follow-up (mean±SD = 26.3±6.5 months), 68% were still on BRV. CONCLUSIONS: The present study shows that results of the phase III studies on BRV match data from real life clinical settings. Brivaracetam seems to be a useful alternative in patients who have suffered adverse effects while taking LEV.

Assessment of Cardiac Involvement in Fabry Disease (FD) with Native T1 Mapping.

PURPOSE: Fabry disease (FD) is an X-linked multi-organ disorder of lysosomal metabolism with cardiac disease being the leading cause of death. Identifying early FD-specific pathologies is important in the context of maximum therapeutic benefit in these stages. Therefore, the aim of this study was to investigate the value of quantitative cardiac T1 mapping as a potential disease-specific surrogate. METHODS: 16 consecutive FD patients (9 female, 7 male; median age: 54 years, IQR 17) and 16 control patients (9 female, 7 male; median age: 52 years, IQR 20) were investigated at 1.5 Tesla. Native T1 mapping was performed using a modified look locker inversion recovery sequence (MOLLI) and native T1 times were measured within the septal myocardium at the midventricular short-axis section. Also functional parameters, left ventricular morphology, presence of late-gadolinium enhancement, cTnI- and Lyso-Gb3-Levels were evaluated. RESULTS: The median native septal T1 time for FD was 889.0 ms and 950.6 ms for controls (p < 0.003). LGE and positive cTnI values (0.26 ±â€Š0.21) were present in 5 FD patients (31.25 %), and left ventricular hypertrophy (LVH) was present in 4 FD patients (25.00 %). The 4 cTnI and 8 Lyso-Gb3 positive FD patients had significantly lower native T1 values (p < 0.05, respectively p < 0.01). Assuming a T1 cut-off value of 900 ms for the identification of increased cardiac lipid deposit, 9 patients with FD (56.25 %) had pathologic values (4 patients cTnI and 8 patients Lyso-Gb3 positive). Moreover, native septal T1 showed a good negative correlation to Lyso-Gb3 (r = - 0.582; p = 0.018). CONCLUSION: A pathologic cardiac native T1 time obviously reflects cardiac involvement in the scope of FD at tissue level. In the future native T1 mapping as an imaging biomarker might allow identification of early stages of cardiac involvement in FD before morphological changes are obvious. KEY POINTS: · Native T1 values are significantly decreased in Fabry disease.. · Native T1 shows promising correlation to cardiac and Fabry-specific biomarkers.. · Native T1 mapping might have great potential for early disease detection and therapy monitoring.. CITATION FORMAT: · Roller FC, Fuest S, Meyer M et al. Assessment of Cardiac Involvement in Fabry Disease (FD) with Native T1 Mapping. Fortschr Röntgenstr 2019; 191: 932 - 939.

What is the value of fibre-endoscopic evaluation of swallowing (FEES) in neurological patients? A cross-sectional hospital-based registry study.

OBJECTIVES: Fibre-endoscopic evaluation of swallowing (FEES) to detect dysphagia is gaining more and more importance as a diagnostic tool. Therefore, we have investigated the impact of FEES in neurological patients in a clinical setting. DESIGN: Cross-sectional hospital-based registry. SETTING: Primary acute care in a neurological department of a German university hospital. PARTICIPANTS: 241patients with various neurological diseases who underwent FEES procedure. PRIMARY AND SECONDARY OUTCOME MEASURES: Dysphagia and related comorbidities. RESULTS: 267 FEES were performed in 241 patients with various neurological diagnoses. Dysphagia was diagnosed in 68.9% of the patients. In only 33.1% of the patients, appropriate oral diet was chosen prior to FEES. A relevant dysphagia occurred more often in patients with structural brain lesions (83.1% vs 65.3%, P=0.001), patients with dysphagia had a longer hospitalisation (median 18 (IQR 12-30) vs 15 days (IQR 9.75-22.75), P=0.005) and had a higher mortality (8.4% vs 1.3%, P=0.041). When the oral diet was changed, we observed a lower pneumonia rate (36% vs 50%, P=0.051) and a lower mortality (3.7% vs 11.3%, P=0.043) in comparison to no change of oral diet. A restriction of oral diet was identified more often in older patients (median 75 years (IQR 66.3-82 years) vs median 72 years (IQR 60-79 years), P=0.01) and in patients with structural brain lesions (86.8% vs 73.1%, P=0.05). CONCLUSION: On clinical investigation, dysphagia was misjudged for the majority of the patients. FEES might help to compensate this drawback, revising the diet regime in nearly 70% of the patients.

One decade of oral anticoagulation in stroke patients: Results from a large country-wide hospital-based registry.

Background New evidence regarding stroke prevention in atrial fibrillation has been published. Implementing knowledge into clinical practice remains challenging. Aims To investigate oral anticoagulants in stroke patients documented in a nationwide registry. Methods The database is an obligatory federal-state-wide hospital-based registry that covers more than 95% of all ischemic strokes, transient ischemic attacks and intracerebral hemorrhages in a community of more than six million inhabitants (Hesse/Germany). We analyzed oral anticoagulants prescribed on discharge in patients with stroke or transient ischemic attack during 2006-2015. Results From 2006 to 2015, we annually selected median 20,895 patients. From 2006 to 2015, the proportion of patients treated with oral anticoagulants increased (9.8% to 24%). The annual proportion of patients with atrial fibrillation remained constant (median: 23%). In atrial fibrillation patients treated with oral anticoagulants, the age increased (median 2008/2009: 76.9 years vs. 2014/2015 79.4 years). The percentage of treated individuals in atrial fibrillation increased dramatically (2006: 30.1% to 2015: 74.5%); in 2015, 30.8% of these patients received vitamin K antagonists and 70.2% new oral anticoagulants. Independent factors associated with new oral anticoagulants therapy were a previous medication with new oral anticoagulants and a treatment on stroke unit. Between new oral anticoagulants- and vitamin K antagonists-treated patients (2015), no differences in age were noted (both mean: 79.4 years). Conclusions The new oral anticoagulants availability enhanced a general trend treating more target patients with oral anticoagulants.