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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 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: 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]

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]

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]

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]

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]

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]

Abstract: Background: The use of anticonvulsant drugs for the prevention of migraine in children and adolescents has been supported in the past. Aims: To evaluate the available evidence for the efficacy and safety of anticonvulsants drugs in the prevention of migraine attacks in children and adolescents. Methods: Studies were selected through a comprehensive literature search. We included all types of study designs (controlled and uncontrolled) due to the limited evidence. Monthly migraine frequency was used as the primary outcome measure in most of the studies. Studies were classified into levels of evidence according to their design. Results: Fourteen studies were included with a total of 939 patients. Topiramate (4 randomized controlled trials [RCT], two uncontrolled trials), sodium valproate/divalproex sodium (two RCTs, one uncontrolled trial, two retrospective chart reviews) levetiracetam and zonisamide (both only uncontrolled studies) are the anticonvulsants that have been reported in the literature. The findings show that valproate is not different from placebo and topiramate may not be different but further randomized trials are needed. All drugs were well tolerated in this age group with no serious events reported. Conclusions: The use of anticonvulsants in the prevention of migraine in children and adolescents is not adequately supported by methodologically sound RCTs. More research is needed in the future to establish the efficacy and safety of specific agents. [Copyright &y& Elsevier]