Search

Background/Objectives: Cognitive impairment represents a core and prodromal clinical feature of cerebral amyloid angiopathy (CAA). We sought to assess specific cognitive domains which are mainly affected among patients with CAA and to investigate probable associations with neuroimaging markers and Cerebrospinal Fluid (CSF) biomarkers. Methods: Thirty-five patients fulfilling the Boston Criteria v1.5 or v2.0 for the diagnosis of probable/possible CAA were enrolled in this prospective cohort study. Brain Magnetic Resonance Imaging and CSF biomarker data were collected. Every eligible participant underwent a comprehensive neurocognitive assessment. Spearman's rank correlation tests were used to identify possible relationships between the Addenbrooke's Cognitive Examination—Revised (ACE-R) sub-scores and other neurocognitive test scores and the CSF biomarker and neuroimaging parameters among CAA patients. Moreover, linear regression analyses were used to investigate the effects of CSF biomarkers on the ACE-R total score and Mini-Mental State Examination (MMSE) score, based on the outcomes of univariate analyses. Results: Cognitive impairment was detected in 80% of patients, and 60% had a coexistent Alzheimer's disease (AD) pathology based on CSF biomarker profiles. Notable correlations were identified between increased levels of total tau (t-tau) and phosphorylated tau (p-tau) and diminished performance in terms of overall cognitive function, especially memory. In contrast, neuroimaging indicators, including lobar cerebral microbleeds and superficial siderosis, had no significant associations with cognitive scores. Among the CAA patients, those without AD had superior neurocognitive test performance, with significant differences observed in their ACE-R total scores and memory sub-scores. Conclusions: The significance of tauopathy in cognitive impairment associated with CAA may be greater than previously imagined, underscoring the necessity for additional exploration of the non-hemorrhagic facets of the disease and new neuroimaging markers. [ABSTRACT FROM AUTHOR]

Background: Intracranial atherosclerotic disease (ICAD) represents a leading cause of ischemic stroke worldwide, conferring increased risk of recurrent stroke and poor clinical outcomes among stroke survivors. Emerging evidence indicates a paradigm shift, pointing towards increasing detection rates of ICAD among White populations and an evolving epidemiological profile across racial and ethnic groups. The present review aims to provide a comprehensive overview of ICAD, focusing on its pathophysiology, diagnostic approach, and evolving epidemiological trends, including underlying mechanisms, advanced neuroimaging techniques for diagnostic evaluation, racial disparities in prevalence, and current and emerging management strategies. Main body: Atherosclerotic plaque accumulation and progressive arterial stenosis of major intracranial arteries comprise the pathophysiological hallmark of ICAD. In clinical practice, the diagnosis of intracranial artery stenosis (ICAS) or high-grade ICAS is reached when luminal narrowing exceeds 50% and 70%, respectively. Advanced neuroimaging, including high-resolution vessel wall MRI (HRVW-MRI), has recently enabled ICAD detection before luminal stenosis occurs. While earlier studies disclosed significant racial disparities in ICAS prevalence, with higher rates among Asians, Hispanics, and Blacks, recent evidence reveals rising detection rates of ICAD among White populations. Genetic, environmental and epigenetic factors have been suggested to confer an increased susceptibility of certain ethnicities and races to ICAD. Nevertheless, with improved accessibility to advanced neuroimaging, ICAD is increasingly recognized as an underlying stroke etiology among White patients presenting with acute ischemic stroke and stroke of undetermined etiology. While conventional management of ICAS entails risk factor modification, pharmacotherapy, and endovascular treatment in selected high-risk patients, substantial progress remains to be made in the management of ICAD at its early, pre-stenotic stages. Conclusion: ICAD remains a critical yet underappreciated risk factor for ischemic stroke across all populations, highlighting the need for increased awareness and improved diagnostic strategies. The emerging epidemiological profile of ICAD across racial groups necessitates a reassessment of risk factors, screening protocols and preventive strategies. Future research should focus on refining the diagnostic criteria and expanding the therapeutic options to cover the full spectrum of ICAD, with the aim of improving patient outcomes and reducing the global burden of intracranial atherosclerosis and stroke. [ABSTRACT FROM AUTHOR]

Background and purpose: Cervical artery dissection (CAD) represents a leading cause of unilateral lower cranial nerve IX–XII palsy, known as Collet–Sicard syndrome (CSS). High‐resolution magnetic resonance imaging (HR‐MRI) is widely used in the evaluation of patients with CAD, providing information regarding vessel wall abnormalities and intraluminal thrombus. Methods: We present a patient with palsy of multiple lower cranial nerves in the context of CSS, attributed to unilateral spontaneous internal carotid artery dissection. Results: We describe a 68‐year‐old man with unremarkable previous history, who presented with subacute, gradually worsening dysphagia and hoarse voice. Clinical examination revealed right‐sided palsy of cranial nerves IX–XII. Three‐dimensional fat‐saturated black‐blood T1‐weighted high‐resolution vessel wall imaging disclosed spontaneous dissection with intramural hematoma along the distal right internal carotid artery. Neck MRI showed inward displacement of right aryepiglottic fold, right pyriform sinus dilatation, and right true vocal cord in middle position, indicative of right vagus nerve palsy, atrophy of right trapezius and sternocleidomastoid muscles, due to right spinal accessory nerve palsy, and unilateral tongue atrophy with fatty infiltration, characteristic for right hypoglossal nerve palsy. Conclusions: This case highlights the utility of high‐resolution vessel wall imaging and especially fat‐saturated T1‐weighted black‐blood SPACE (sampling perfection with application‐optimized contrast using different flip‐angle evolutions) sequences in the accurate diagnosis of CAD, revealing the characteristic mural hematoma and intimal flap. HR‐MRI is also valuable in the recognition of indirect signs of lower cranial nerve compression. [ABSTRACT FROM AUTHOR]

Background and purpose: Orolingual angioedema (OA) represents a rare but life‐threatening complication among patients with acute ischemic stroke treated with intravenous thrombolysis with alteplase. Novel agents, including icatibant, are recommended in resistant patients with alteplase‐induced OA who have failed to respond to first‐line therapies including corticosteroids, antihistamines, and/or adrenaline. Methods: We present a patient with alteplase‐induced OA who showed substantial clinical improvement following the administration of icatibant. Results: We describe a 71‐year‐old woman with known arterial hypertension under treatment with angiotensin‐converting enzyme inhibitor, who presented with acute ischemic stroke in the territory of the right middle cerebral artery and received intravenous alteplase. During intravenous thrombolysis, the case was complicated with OA without any response to standard anaphylactic treatment including corticosteroids, dimetindene, and adrenaline. Thirty minutes after symptom onset, icatibant, a synthetic selective bradykinin B2‐receptor antagonist, was administered subcutaneously. Substantial symptomatic resolution was observed only following the icatibant administration. Conclusions: This case highlights the effectiveness of icatibant in the acute management of alteplase‐induced OA. In particular, icatibant administration, following first‐line therapies including corticosteroids, antihistamines, and/or adrenaline, may avert tracheostomy and intubation in resistant and refractory cases with OA following intravenous thrombolysis for acute ischemic stroke. [ABSTRACT FROM AUTHOR]

Background: Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disorder affecting upper and lower motor neurons. Some ALS patients exhibit concomitant nonmotor signs; thus, ALS is considered a multisystemic disorder. Pain is an important nonmotor symptom. Observational and case–control studies report high frequency of pain in ALS patients and it has been correlated with depression and quality of life. There are no specific scales for the assessment of pain and no randomized controlled trials (RCTs) regarding the drug management of pain in ALS. Aim: To systematically review the evidence for the nonpharmacological interventions (NPIs) in relieving pain in ALS, on March 2024, we searched the following databases: Pubmed, Scopus, Web of Science, and Cochrane. We also checked the bibliographies of trials identified to include further published or unpublished trials. Main results: A total of 1003 records were identified. Finally, five RCTs including 131 patients (64 in the intervention group and 67 in the control group) were included for meta-analysis. The interventions of the included RCTs consisted of muscle exercise, combined aerobics–strength intervention, and osteopathic manual treatment. The meta-analysis did not find a statistically significant difference in favor of NPIs for alleviating pain in ALS patients. Conclusions: ALS has a fulminant course and irreversibly leads to death. Pain in ALS patients, although a common nonmotor symptom, is often unrecognized and undertreated, and this is underlined by the lack of any RCTs on drug therapy for pain. Albeit NPIs are considered safe, as adverse effects are rarely reported, this systematic review did not provide sufficient evidence for a beneficial effect on pain. The scarceness of relevant literature highlights the need for future studies, with larger samples, more homogeneous in terms of interventions and population characteristics (stage of disease), and better choice of measurement scales to further investigate the efficacy, if any, of various pain interventions in ALS patients. [ABSTRACT FROM AUTHOR]

Background and Purpose: Transorbital sonography (TOS) provides a noninvasive tool to detect intracranial pressure by assessing optic nerve sheath diameter (ONSD) and optic disc elevation (ODE). The utility of TOS in the diagnosis of idiopathic intracranial hypertension (IIH) has been increasingly recognized. Methods: A single‐center case‐control study sought to compare TOS‐acquired ONSD and ODE among IIH‐cases versus patients with other neurological diseases (controls). Furthermore, a systematic review and meta‐analysis was conducted to present pooled mean differences and diagnostic measures of ONSD and ODE between IIH‐cases and controls. Results: In the single‐center study, consisting of 31 IIH‐cases and 34 sex‐ and age‐matched controls, ONSD values were higher among IIH‐cases than controls (p<.001), while ODE was more prevalent in cases (65% vs. 15%; p<.001). The receiver‐operating characteristic (ROC)‐curve analysis revealed that the optimal cutoff value of ONSD for predicting IIH was 5.15 mm, with an area under the curve (AUC) of 0.914 (95% confidence interval [CI]: 0.861‐0.967) and sensitivity and specificity values of 85% and 90%, respectively. In a meta‐analysis of 14 included studies with 415 IIH‐cases, ONSD and ODE values were higher in IIH‐cases than controls (mean difference in ONSD 1.20 mm; 95% CI: 0.96‐1.44 mm and in ODE 0.3 mm; 95% CI: 0.33‐0.67 mm). With regard to ONSD, pooled sensitivity, specificity, and diagnostic odds ratio were calculated at 85.5% (95% CI: 77.9‐90.8%), 90.7% (95% CI: 84.6‐94.5%), and 57.394 (95% CI: 24.597‐133.924), respectively. The AUC in summary ROC‐curve analysis was 0.878 (95% CI: 0.858‐0.899) with an optimal cutoff point of 5.0 mm. Conclusions: TOS has a high diagnostic utility for the noninvasive diagnosis of IIH and may deserve wider implementation in everyday clinical practice. [ABSTRACT FROM AUTHOR]

Background and purpose: Sporadic cerebral amyloid angiopathy (CAA) is a small vessel disease, resulting from progressive amyloid-β deposition in the media/adventitia of cortical and leptomeningeal arterioles. We sought to assess the prevalence of baseline characteristics, clinical and radiological findings, as well as outcomes among patients with CAA, in the largest study to date conducted in Greece. Methods: Sixty-eight patients fulfilling the Boston Criteria v1.5 for probable/possible CAA were enrolled and followed for at least twelve months. Magnetic Resonance Imaging was used to assess specific neuroimaging markers. Data regarding cerebrospinal fluid biomarker profile and Apolipoprotein-E genotype were collected. Multiple logistic regression analyses were performed to identify predictors of clinical phenotypes. Cox-proportional hazard regression models were used to calculate associations with the risk of recurrent intracerebral hemorrhage (ICH). Results: Focal neurological deficits (75%), cognitive decline (57%), and transient focal neurological episodes (TFNEs; 21%) were the most common clinical manifestations. Hemorrhagic lesions, including lobar cerebral microbleeds (CMBs; 93%), cortical superficial siderosis (cSS; 48%), and lobar ICH (43%) were the most prevalent neuroimaging findings. cSS was independently associated with the likelihood of TFNEs at presentation (OR: 4.504, 95%CI:1.258–19.088), while multiple (>10) lobar CMBs were independently associated with cognitive decline at presentation (OR:5.418, 95%CI:1.316–28.497). cSS emerged as the only risk factor of recurrent ICH (HR:4.238, 95%CI:1.509–11.900) during a median follow-up of 20 months. Conclusions: cSS was independently associated with TFNEs at presentation and ICH recurrence at follow-up, while a higher burden of lobar CMBs with cognitive decline at baseline. These findings highlight the prognostic value of neuroimaging markers, which may influence clinical decision-making. [ABSTRACT FROM AUTHOR]

Background and Purpose: Cerebral small vessel disease is a common manifestation among patients with Fabry disease (FD). As a biomarker of cerebral small vessel disease, the prevalence of impaired cerebral autoregulation as assessed by transcranial Doppler (TCD) ultrasonography was evaluated in FD patients and healthy controls. Methods: TCD was performed to assess pulsatility index (PI) and vasomotor reactivity expressed by breath‐holding index (BHI) for the middle cerebral arteries of included FD patients and healthy controls. Prevalence of increased PI (>1.2) and decreased BHI (<0.69) and ultrasound indices of cerebral autoregulation were compared in FD patients and controls. The potential association of ultrasound indices of impaired cerebral autoregulation with white matter lesions and leukoencephalopathy on brain MRI in FD patients was also evaluated. Results: Demographics and vascular risk factors were similar in 23 FD patients (43% women, mean age: 51 ± 13 years) and 46 healthy controls (43% women, mean age: 51 ± 13 years). The prevalence of increased PI (39%; 95% confidence interval [CI]: 20%‐61%), decreased BHI (39%; 95% CI: 20%‐61%), and the combination of increased PI and/or decreased BHI (61%; 95% CI: 39%‐80%) was significantly (p <.001) higher in FD patients compared to healthy controls (2% [95% CI: 0.1%‐12%], 2% [95% CI: 0.1%‐12%], and 4% [95% CI: 0.1%‐15%], respectively). However, indices of abnormal cerebral autoregulation were not associated independently with white matter hyperintensities and presented a low‐to‐moderate predictive ability for the discrimination of FD patients with and without white matter hyperintensities. Conclusions: Impaired cerebral autoregulation as assessed by TCD appears to be highly more prevalent among FD patients compared to healthy controls. [ABSTRACT FROM AUTHOR]

Background: Cerebral Amyloid Angiopathy-related inflammation (CAA-ri) is a distinct but rare subset of CAA. The greater availability of high resolution Magnetic Resonance Imaging (MRI) has currently allowed the increasing recognition and diagnosis of this entity, without the risk of a brain biopsy. However, in rare cases with typical clinical characteristics but uncommon neuroimaging findings at presentation, the brain-biopsy is required for an early and reliable diagnosis. Case description: A 71-year-old man with arterial hypertension presented due to 1-week history of headache, vomiting, disorientation and impaired consciousness. Brain MRI revealed multiple acute cortical/subcortical microinfarcts, scarce microbleeds, extensive right parietooccipital and left frontotemporal leptomeningeal enhancement. After an extensive diagnostic work-up, excluding infectious, neoplastic and autoimmune etiologies, the patient underwent brain-biopsy. Histology disclosed amyloid deposition in an arteriolar wall and the patient fulfilled diagnostic criteria for probable CAA-ri with supporting pathology. He received intravenous methylprednisolone, followed by oral tapering with steroids showing clinical and radiological improvement with complete resolution of gadolinium enhancement. Follow-up MRI revealed an increase of cerebral microbleeds and the patient fulfilled CAA-ri neuroimaging criteria. Conclusions: This case highlights the importance of continuous vigilance from clinical neurologists to detect CAA-ri diagnosis and the diagnostic value of brain-biopsy in CAA-ri patients with atypical neuroimaging presentation, such as acute microinfarcts. The early diagnosis and the prompt treatment initiation can improve the prognosis and the evolution of this rare disorder. [ABSTRACT FROM AUTHOR]

We present a case of a 47‐year‐old woman with a history of asthma and mononeuritis who presented with shortness of breath and fatigue. Heart failure was diagnosed and echocardiography revealed large floating thrombi attached to the left ventricular walls. Cardiac magnetic resonance imaging showed evidence of myocarditis and angiitis. Blood count revealed eosinophilia. She was diagnosed with eosinophilic granulomatosis with polyangiitis or Churg–Strauss syndrome (CSS) according to recently updated criteria. Medical management with specific aetiology (anticoagulation or immunosuppression) and heart failure treatment resulted in clinical improvement. We further discuss the diagnostic approach of CSS with cardiovascular complications and therapeutic management. [ABSTRACT FROM AUTHOR]

We describe the clinical presentation, radiological findings, treatment and outcomes of three patients with delayed leukoencephalopathy occurring after endovascular treatment (EVT) for cerebral aneurysms—a rare, albeit recurring, complication. The symptoms occurred 6 to 12 months following the EVT of the cerebral aneurysm. Characteristic imaging findings included high-signal changes on T2 images in the white matter without diffusion restriction predominantly at the distribution of the vascular territory of the catheterized arteries, coupled with patchy gadolinium enhancement or low susceptibility weighted imaging (SWI) signals within the white-matter lesions. Steroid pulse therapy is the treatment of choice and promptly improves clinical and imaging findings. Tapering or cessation of steroids may result in clinical and imaging relapses; close- and long-term follow-up for patients presenting this complication is warranted. [ABSTRACT FROM AUTHOR]

The clinical manifestations of proximal (extracranial) internal carotid artery occlusions (pICAOs) may range from asymptomatic to acute, large, and devastating ischemic strokes. The etiology and pathophysiology of the occlusion, intracranial collateral status and patient's premorbid status are among the factors determining the clinical presentation and outcome of pICAOs. Rapid and accurate diagnosis is crucial and may be assisted by the combination of carotid and transcranial duplex sonography, or a computed tomography/magnetic resonance angiography (CTA/MRA). It should be noted that with either imaging modalities, the discrimination of a pseudo-occlusion of the extracranial internal carotid artery (ICA) from a true pICAO may not be straightforward. In the absence of randomized data, the management of acute, symptomatic pICAOs remains individualized and relies largely on expert opinion. Administration of intravenous thrombolysis is reasonable and probably beneficial in the settings of acute ischemic stroke with early presentation. Unfortunately, rates of recanalization are rather low and acute interventional reperfusion therapies emerge as a potentially powerful therapeutic option for patients with persistent and severe symptoms. However, none of the pivotal clinical trials on mechanical thrombectomy for acute ischemic stroke randomized patients with isolated extracranial large vessel occlusions. On the contrary, several lines of evidence from non-randomized studies have shown that acute carotid endarterectomy, or endovascular thrombectomy/stenting of the ICA are feasible and safe, and pοtentially beneficial. The heterogeneity in the pathophysiology and clinical presentation of acute pICAOs renders patient selection for an acute interventional treatment a complicated decision-making process. The present narrative review will outline the pathophysiology, clinical presentation, diagnostic challenges, and possible treatment options for pICAOs. [ABSTRACT FROM AUTHOR]

Background and Purpose: Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disorder affecting upper and lower motor neurons. Some ALS patients exhibit concomitant nonmotor signs, and thus ALS is considered a multisystem disorder. The aim of this study is to investigate autonomous nervous system involvement in ALS. Methods: We investigated 21 ALS patients and 28 age‐matched controls. ALS patients were assessed for disease severity with the Revised‐ALS Functional Rating Scale (ALSFSR) and for the presence of autonomic symptoms with the Composite Autonomic Symptom Score scale. Sympathetic nervous system was evaluated by sympathetic skin response (SSR) and parasympathetic nervous system by ultrasonography of vagus nerve (VN) at the level of the thyroid gland. Results: SSR latencies were shorter and SSR amplitudes were higher in controls compared to ALS patients. The cross‐sectional area (CSA) of the VN was significantly smaller in ALS patients (mean CSA right/left: 1.73±0.62 mm2/1.47±0.53 mm2) compared to controls (mean CSA right/left: 2.91±0.79 mm2/2.30±0.80 mm2), right: p <. 001, left: p <. 001. There was a significant negative correlation between disease duration and CSA of left‐VN (r = –0.493, p =.023). This correlation was attenuated between disease duration and CSA of right‐VN (r = –0.419, p =.059). ALSFSR‐R was positively correlated to CSA of right‐VN (p =.006, r = 0.590). CSA of VN did not correlate with bulbar involvement. Conclusions: This study confirms the presence of autonomic dysfunction in ALS patients and provides evidence of VN atrophy that correlates with disease severity and duration and is independent of bulbar involvement. Degeneration of dorsal nucleus neurons of the VN is hypothesized. [ABSTRACT FROM AUTHOR]

Acute cerebral ischemia with underlying myelodysplastic syndrome mimicking vaccine-induced immune thrombotic thrombocytopenia Keywords: ischemic stroke; multilineage dysplasia; myelodysplastic syndrome; thrombosis with thrombocytopenia syndrome; vaccine-induced immune thrombotic thrombocytopenia (VITT) EN ischemic stroke multilineage dysplasia myelodysplastic syndrome thrombosis with thrombocytopenia syndrome vaccine-induced immune thrombotic thrombocytopenia (VITT) e9 e12 4 04/13/22 20220501 NES 220501 We have read with great interest the article by Krzywicka et al. describing the clinical characteristics of cerebral venous sinus thrombosis (CVST) cases following SARS-CoV-2 vaccination of which the European Medicines Agency was notified [1]. Ischemic stroke, multilineage dysplasia, myelodysplastic syndrome, vaccine-induced immune thrombotic thrombocytopenia (VITT), thrombosis with thrombocytopenia syndrome. [Extracted from the article]

The use and interpretation of diagnostic cerebrospinal fluid (CSF) biomarkers for neurodegenerative disorders, such as Dementia with Lewy bodies (DLB), represent a clinical challenge. According to the literature, the composition of CSF in DLB patients varies. Some patients present with reduced levels of amyloid, others with full Alzheimer Disease CSF profile (both reduced amyloid and increased phospho-tau) and some with a normal profile. Some patients may present with abnormal levels of a-synuclein. Continuous efforts will be required to establish useful CSF biomarkers for the early diagnosis of DLB. Given the heterogeneity of methods and results between studies, further validation is fundamental before conclusions can be drawn. [ABSTRACT FROM AUTHOR]

Background: Intracranial dural arteriovenous fistulas (dAVFs) are pathological anastomoses between meningeal arteries and dural venous sinuses or cortical veins. Diagnosis of dAVFs can be challenging due to their broad range of clinical manifestations and the lack of specificity in their symptoms. We present a rare case of a patient with bilateral dAVFs, who was referred to our department for carotid doppler ultrasonography/transcranial color Doppler. Case Report: A 67‐year‐old man was referred by his treating physician for neurovascular ultrasonography due to vision impairment associated with papilledema of both eyes. On ultrasound examination both external carotid arteries showed a "pseudo‐internalization" pattern with high end‐diastolic velocities and reduced resistance indices. Subsequent magnetic resonance and digital subtraction angiography revealed dAVF of both transverse sinuses, with occipital and medial meningeal arteries as feeders. Conclusions: Neurovascular sonography may serve as a screening and follow‐up tool when a DAVF in the transverse sinus/sigmoid sinus is suspected. [ABSTRACT FROM AUTHOR]

Background: Differences have been noted in the clinical presentation and mutational spectrum of CADASIL among various geographical areas. The aim of the present study was to investigate the mode of clinical presentation and genetic mutations reported in Greece. Methods: After a systematic literature search, we performed a pooled analysis of all published CADASIL cases from Greece. Results: We identified 14 studies that reported data from 14 families comprising 54 patients. Migraine with aura was reported in 39%, ischemic cerebrovascular diseases in 68%, behavioral‐psychiatric symptoms in 47% and cognitive decline in 60% of the patients. The mean (±SD) age of onset for migraine with aura, ischemic cerebrovascular diseases, behavioral‐psychiatric symptoms and cognitive decline was 26.2 ± 8.7, 49.3 ± 14.6, 47.9 ± 9.4 and 42.9 ± 10.3, respectively; the mean age at disease onset and death was 34.6 ± 12.1 and 60.2 ± 11.2 years. With respect to reported mutations, mutations in exon 4 were the most frequently reported (61.5% of all families), with the R169C mutation being the most common (30.8% of all families and 50% of exon 4 mutations), followed by R182C mutation (15.4% of all families and 25% of exon 4 mutations). Conclusions: The clinical presentation of CADASIL in Greece is in accordance with the phenotype encountered in Caucasian populations, but differs from the Asian phenotype, which is characterized by a lower prevalence of migraine and psychiatric symptoms. The genotype of Greek CADASIL pedigrees is similar to that of British pedigrees, exhibiting a high prevalence of exon 4 mutations, but differs from Italian and Asian populations, where mutations in exon 11 are frequently encountered. [ABSTRACT FROM AUTHOR]

This correspondence comments on a published article presenting a case of rhombencephalitis following SARS-CoV-2-vaccination with the mRNA vaccine BNT162b2 (Pfizer/BioNTech). We also present the case of a 47-year-old man who developed Guillain-Barré-syndrome and a fulminant encephalomyelitis 28 days after immunization with Ad26. COV2.S (Janssen/Johnson & Johnson). Based on the presented cases, we underscore the importance of clinical awareness for early recognition of overlapping neuroimmunological syndromes following vaccination against SARS-CoV-2. Additionally, we propose that that role of autoantibodies against angiotensin-converting enzyme 2 (ACE2) and the cell-surface receptor neuropilin-1, which mediate neurological manifestations of SARS-CoV-2, merit further investigation in patients presenting with neurological disorders following vaccination against SARS-CoV-2. [ABSTRACT FROM AUTHOR]

Introduction: Among congenital anomalies of the carotid artery circulation, the presence of a non-bifurcating carotid artery is extremely rare. Relevant cases with unilateral non-bifurcating carotid artery have scarcely been described in the literature. After extensive literature review, only one case with asymptomatic bilateral non-bifurcating carotid arteries associated with persistent proatlantal artery was identifed. Methods: We present the case of a 40-year-old man with recurrent cerebrovascular events presenting non-bifurcating carotid arteries bilaterally. Results: A 40-year-old man presented in the emergency department with a transient ischemic attack. Past medical history included prior ischemic stroke of unknown etiology in the distribution of the left middle cerebral artery, untreated hyperlipidemia and tobacco use. Complete work-up in order to identify the underlying mechanism of the patient’s recurrent cerebrovascular events was negative, except for the fnding of non-bifurcating carotid arteries bilaterally, associated with an extensive intracranial anastomosing arterial network. Long-term antiplatelet therapy and statins were administered as secondary stroke prevention therapy. Discussion: Previous reports suggest that non-bifurcating carotid arteries may be associated with atherosclerotic plaque formation in symptomatic cases due to shear stress, tortuosity or other local factors. However, in the absence of atherosclerosis, the pathogenic association of bilateral non-bifurcating carotid arteries with cerebrovascular events remains questionable, but may be considered when other stroke etiologies are excluded. [ABSTRACT FROM AUTHOR]

Cerebral venous thrombosis is an uncommon, yet life-threatening condition, affecting mainly young and middle-aged individuals. Moreover, it represents an underrecognised etiology of lobar intracerebral hemorrhage (ICH). The clinical course of CVT is variable in the first days after diagnosis and medical complications including pulmonary embolism (PE) may result in early neurological deterioration and death if left untreated. Case report. We describe a 46-year-old man with acute left hemiparesis and dysarthria in the context of lobar ICH due to underlying CVT of Trolard vein. Diagnosis was delayed because of misinterpretation of the initial neuroimaging study. Subsequently, the patient rapidly deteriorated and developed submassive PE and left iliofemoral venous thrombosis in the setting of previously undiagnosed hereditary thrombophilia (heterozygous prothrombin gene mutation G2021A). Emergent aspiration thrombectomy was performed resulting in the successful management of PE. A follow-up MRI study confirmed the thrombosed Trolard vein, thus establishing the CVT diagnosis. Anticoagulation treatment was immediately escalated to enoxaparine therapeutic dose resulting in clinical improvement of neurological deficits. Delayed diagnosis of cerebral venous thrombosis with underlying causes of lobar ICH may result in dire complications. Swift initiation of anticoagulants is paramount even in patients with lobar intracerebral hemorrhage as the initial manifestation of cerebral venous thrombosis. [ABSTRACT FROM AUTHOR]

Abstract Background Selective serotonin reuptake inhibitors (SSRIs) are the most commonly prescribed antidepressants for the treatment of depression in patients with Parkinson's Disease (PD) but data on their efficacy are controversial. Methods We conducted a systematic review and meta-analysis of randomized controlled trials to investigate the efficacy and acceptability of SSRIs in the treatment of depression in PD. Results Ten studies were included. In the comparison between SSRIs and Placebo (n = 6 studies), the combined risk ratio (random effects) was 1.08 (95% confidence interval: 0.77 - 1.55, p = 0.67). In the comparison between SSRIs and Tricyclic Antidepressants (TCAs) (n = 3 studies) the combined risk ratio was 0.75 (0.39 - 1.42, p = 0.37). An acceptability analysis showed that SSRIs were generally well tolerated. Conclusions These results suggest that there is insufficient evidence to reject the null hypothesis of no differences in efficacy between SSRIs and placebo in the treatment of depression in PD. Due to the limited number of studies and the small sample sizes a type II error (false negative) cannot be excluded. The comparison between SSRIs and TCAs is based on only three studies and further trials with more pragmatic design are needed.

Introduction: Glycemic variability (GV) has been associated with worse prognosis in critically ill patients. We sought to evaluate the potential association between GV indices and clinical outcomes in acute stroke patients. Methods: Consecutive diabetic and nondiabetic, acute ischemic or hemorrhagic stroke patients underwent regular, standard-of-care finger-prick measurements and continuous glucose monitoring (CGM) for up to 96 h. Thirteen GV indices were obtained from CGM data. Clinical outcomes during hospitalization and follow-up period (90 days) were recorded. Hypoglycemic episodes disclosed by CGM but missed by finger-prick measurements were also documented. Results: A total of 62 acute stroke patients [48 ischemic and 14 hemorrhagic, median NIHSS score: 9 (IQR: 3–16) points, mean age: 65 ± 10 years, women: 47%, nondiabetic: 79%] were enrolled. GV expressed by higher mean absolute glucose (MAG) values was associated with a lower likelihood of neurological improvement during hospitalization before and after adjusting for potential confounders (OR: 0.135, 95% CI: 0.024–0.751, p = 0.022). There was no association of GV indices with 3-month clinical outcomes. During CGM recording, 32 hypoglycemic episodes were detected in 17 nondiabetic patients. None of these episodes were identified by the periodic blood glucose measurements and therefore they were not treated. Conclusions: Greater GV of acute stroke patients may be related to lower odds of neurological improvement during hospitalization. No association was disclosed between GV indices and 3-month clinical outcomes. [ABSTRACT FROM AUTHOR]

Background and purpose: In recent years, the use of coiling has gained increased popularity for the treatment of intracranial aneurysms, and stroke physicians are confronted with rare pathologies associated with this relatively new and evolving treatment method, such as embolization of pieces of the polymeric filaments from the coils and a subsequent inflammatory response. In particular, white matter enhancing lesions are a rare complication after aneurysm endovascular therapy (EVT), suggesting a foreign body reaction to shedding of hydrophilic coating from the endovascular devices into the blood stream. The description of such a case aims to raise the clinicians' awareness of the symptomatic delayed and recurring inflammatory changes that may occur after endovascular aneurysmal treatment with the use of coiling devices. Case description: A 64‐year‐old woman underwent coiling of a ruptured right posterior communicating artery aneurysm. She was asymptomatic after EVT. One year later, she presented with headache, acoustic hallucinations, paresthesias and left arm weakness. Brain magnetic resonance imaging (MRI) revealed multiple enhancing white matter lesions in the right hemisphere. She was treated with pulse intravenous methylprednisolone, followed by oral prednisolone; all clinical symptoms resolved and imaging findings improved substantially. Two years after tapering the steroids, follow‐up symptoms recurred and repeat brain MRI revealed new enhancing white matter lesions. Discussion and conclusions: There is an increasing number of similar reports of enhancing white matter lesions after coiling of intracranial aneurysms, with the incidence estimated to be between 0.5% and 2.3% in different cohort studies. Close monitoring for the appearance of new neurologic symptoms that could suggest delayed brain reactivity should be recommended. [ABSTRACT FROM AUTHOR]

Giant cell arteritis (GCA) is a systemic inflammatory arteriopathy of medium and large‐sized arteries, predominantly affecting branches of the external carotid artery. Ischemic stroke has been reported in 2.8–7% of patients diagnosed with GCA. The majority of ischemic strokes may involve the posterior circulation as a result of vertebral and/or, less frequently, of basilar artery vasculitis. Prompt diagnosis is crucial since high‐dose corticosteroid treatment is highly effective in preventing the occurrence or recurrence of ischemic complications, including posterior circulation ischemic stroke in cases with vertebrobasilar involvement. Cervical duplex sonography (CDS) of the temporal arteries is a powerful diagnostic tool with high sensitivity and specificity for the diagnosis of GCA. In cases with clinical suspicion or a temporal artery ultrasonographic confirmation of GCA, a detailed evaluation of the cervical, axillary, and intracranial arteries with CDS and transcranial‐duplex‐sonography, respectively, should be part of the ultrasound examination protocol. Specifically, signs of extracranial vertebral artery wall inflammation ("halo" sign) and focal luminar stenoses may be accurately depicted by ultrasounds in high‐risk patients or individuals with ischemic stroke attributed to GCA. In this review, we present three cases of GCA and posterior circulation ischemic complications that were initially evaluated with comprehensive neurosonology protocol and were promptly diagnosed with GCA based on the characteristic "halo" sign in the temporal and vertebral arteries. In addition, we discuss the relevant literature concerning the utility of CDS for the early diagnosis of GCA, focusing on the subtype with extracranial arterial involvement, particularly that of the vertebral arteries. [ABSTRACT FROM AUTHOR]

Recent randomized controlled clinical trials (RCTs) have revolutionized acute ischemic stroke care by extending the use of intravenous thrombolysis and endovascular reperfusion therapies in time windows that have been originally considered futile or even unsafe. Both systemic and endovascular reperfusion therapies have been shown to improve outcome in patients with wake-up strokes or symptom onset beyond 4.5 h for intravenous thrombolysis and beyond 6 h for endovascular treatment; however, they require advanced neuroimaging to select stroke patients safely. Experts have proposed simpler imaging algorithms but high-quality data on safety and efficacy are currently missing. RCTs used diverse imaging and clinical inclusion criteria for patient selection during the dawn of this novel stroke treatment paradigm. After taking into consideration the dismal prognosis of nonrecanalized ischemic stroke patients and the substantial clinical benefit of reperfusion therapies in selected late presenters, we propose rescue reperfusion therapies for acute ischemic stroke patients not fulfilling all clinical and imaging inclusion criteria as an option in a subgroup of patients with clinical and radiological profiles suggesting low risk for complications, notably hemorrhagic transformation as well as local or remote parenchymal hemorrhage. Incorporating new data to treatment algorithms may seem perplexing to stroke physicians, since treatment and imaging capabilities of each stroke center may dictate diverse treatment pathways. This narrative review will summarize current data that will assist clinicians in the selection of those late presenters that will most likely benefit from acute reperfusion therapies. Different treatment algorithms are provided according to available neuroimaging and endovascular treatment capabilities. [ABSTRACT FROM AUTHOR]

The cerebral cavernous malformations are benign vascular hamartomas, with thin and dilated vascular walls and therefore constantly susceptible to hemorrhage. Clinically, they present with recurrent headaches, acute intracranial hemorrhage and focal neurological deficits. They are considered as angiographically occult vascular malformations and the imaging technique of choice for their diagnosis is magnetic resonance tomography. We present the case of a female patient with acute-onset symptomatology, congruent with a lesion in the basal pons. Her medical history included an intracranial hemorrhage due to a cavernoma, which was surgically removed. The magnetic resonance imaging of the brain revealed two new cavernomas, which were not identified in the imaging conducted in the past. In literature, the cases of de novo appearance of cavernomas are considered highly rare, especially in patients with no consistent family history or medical history of radiation therapy. Resultantly, they should be considered as dynamic lesions, regarding their number, size and behavior. [ABSTRACT FROM AUTHOR]

Carotid cavernous fistulas (CCFs) represent uncommon and anomalous communications between the carotid artery and the cavernous sinus. Case report We present the clinical details and successful management of a previously healthy 44-year-old patient who presented with one-month worsening headache, bilateral abducens palsy and conjunctival injection. Imaging modalities including magnetic resonance imaging (MRI) with contrast and digital subtraction angiography (DSA) facilitated the diagnosis of CCF. The patient underwent endovascular coiling of the CCF, leading to neurological recovery and symptom remission. This case highlights the importance of promptly CCF diagnosis in patients with multiple cranial nerve palsies and conjunctival hyperemia. Moreover, it emphasizes the efficacy of endovascular coiling in achieving symptom remission. [ABSTRACT FROM AUTHOR]

Keywords: Long-COVID; Fatigue; Autonomous nervous system; Vagus nerve; Sympathetic skin response EN Long-COVID Fatigue Autonomous nervous system Vagus nerve Sympathetic skin response 4611 4612 2 08/11/22 20220901 NES 220901 Supplementary Information The online version contains supplementary material available at https://doi.org/10.1007/s00415-022-11172-1. It was demonstrated that LCS patients show vagus atrophy accounting for parasympathetic implication and prolonged SSR latencies, accounting for sympathetic implication. Evaluation of ANS function was performed by Sympathetic Skin Response (SSR) to investigate the Sympathetic Nervous System (SNS), and the cross-sectional area (CSA) of the Vagus Nerve (VN) was assessed by ultrasound to investigate the Parasympathetic Nervous System (PNS) [[4]]. [Extracted from the article]

Severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) causes an infectious disease with the involvement of multiple organ systems widely known as coronavirus disease 2019 (COVID-19) and was first reported in China in December 2019. Thus, stroke care systems should continue optimizing their treatments' workflows in AIS patients in order to minimize the collateral damage of COVID-19 by sustaining optimal patient care. As far as the stroke care concerns, a direct impact has been noticed due to a presumed association between endothelial inflammation and thrombotic diathesis resulting in increase in cryptogenic strokes involving patients with SARS-CoV-2 infection. [Extracted from the article]

Background: Selective serotonin reuptake inhibitors (SSRIs) are the most commonly prescribed antidepressants for the treatment of depression in patients with Parkinson's Disease (PD) but data on their efficacy are controversial. Methods: We conducted a systematic review and meta-analysis of randomized controlled trials to investigate the efficacy and acceptability of SSRIs in the treatment of depression in PD. Results: Ten studies were included. In the comparison between SSRIs and Placebo (n = 6 studies), the combined risk ratio (random effects) was 1.08 (95% confidence interval: 0.77 - 1.55, p = 0.67). In the comparison between SSRIs and Tricyclic Antidepressants (TCAs) (n = 3 studies) the combined risk ratio was 0.75 (0.39 - 1.42, p = 0.37). An acceptability analysis showed that SSRIs were generally well tolerated. Conclusions: These results suggest that there is insufficient evidence to reject the null hypothesis of no differences in efficacy between SSRIs and placebo in the treatment of depression in PD. Due to the limited number of studies and the small sample sizes a type II error (false negative) cannot be excluded. The comparison between SSRIs and TCAs is based on only three studies and further trials with more pragmatic design are needed. [ABSTRACT FROM AUTHOR]

AbstractMotor Neuron Diseases (MNDs), familial and sporadic, are progressive neurodegenerative disorders that, for an extended period in the past, were considered purely motor disorders. During the course of the disease, however, some patients exhibit concomitant non-motor signs; thus, MNDs are currently perceived as multisystem disorders. Assessment of non-motor symptoms is usually performed clinically, although laboratory tests can also be routinely used to objectively evaluate these symptoms. Sympathetic Skin Response (SSR) is an example of a neurophysiological test that has been used in cases of Amyotrophic Lateral Sclerosis, Spinal Muscular Atrophy, and Monomelic Atrophy, mostly to assess Autonomic Nervous System (ANS) disorders. Dysautonomia affects quality of life and life expectancy, as it is involved in cardiovascular events and incidents of sudden death. SSR abnormalities are present even in subclinical involvement of the ANS in MNDs. In this review, we present published research examining SSR findings in various MNDs, and discuss the correlation of SSR findings with clinical symptoms and disease severity, as well as the potential sources of abnormal findings. The aim of this study is to raise clinician awareness of autonomic dysfunction in MNDs and present the benefits of SSR examination in patient care. [ABSTRACT FROM AUTHOR]

Owing to the variety of its clinical presentations, amyotrophic lateral sclerosis (ALS) may mimic several neurological syndromes and even lead to inappropriate surgical procedures. We wished to assess the impact of unnecessary surgical treatments among ALS patients, and therefore we retrospectively reviewed medical records of 164 consecutive ALS patients. We collected data on the clinical presentation of ALS at onset, the initial symptom that led the patients to seek medical care, the timing of diagnosis and surgical procedures attributed to the onset of symptoms. Results showed that among 164 consecutive patients with ALS, 13 (7.9%) were surgically treated as a consequence of false diagnosis. Despite this, these patients showed no statistically significant difference in time of diagnosis compared to non-operated patients. In conclusion, a small but not negligible number of ALS patients are misdiagnosed. The diagnostic pathway of these patients includes often specialists other than neurologists who should be more aware of this disease in order to avoid inappropriate surgical treatments and provide the patients the appropriate diagnostic and therapeutic procedure by referring them promptly to a neurologist. [ABSTRACT FROM AUTHOR]