Practice recommendations for neurovascular ultrasound investigations of acute stroke patients in the setting of the COVID-19 pandemic: an expert consensus from the European Society of Neurosonology and Cerebral Hemodynamics.

BACKGROUND AND PURPOSE: Patients with acute ischemic stroke are at high-risk for contracting COVID-19 infection. Additionally, healthcare professionals including neurovascular ultrasound providers are also at risk of being infected by SARS-CoV-2 virus. Yet, preparedness to continue to guarantee hyperacute treatment is vital for patients outcome. In light of this situation, the European Society of Neurosonology and Cerebral Hemodynamic (ESNCH) appointed a task force to provide consensus recommendations for the performance of neurovascular ultrasound investigations in acute ischemic stroke during the COVID-19 pandemic with the aim of protecting both patients and ultrasound providers. METHODS: The "ultrasound in acute stroke working group" of the ESNCH examined literature articles and reviews using the following key words: "corona virus" or "COVID-19" or "SARS-CoV-2 virus", and "acute stroke" or "cerebrovascular disease", and "ultrasound". Thereafter, a thorough discussion was conducted with the "education and guidelines working group" of the ESNCH. RESULTS: We propose rapid up-to-date recommendations for healthcare personnel involved in the pre-hospital and intra-hospital assessment of stroke patients, with a particular attention to neurovascular ultrasound performance. CONCLUSION: The ESNCH provides a guidance summary for the performance of neurovascular ultrasound investigations in acute ischemic stroke in the time of COVID-19.

The Posterior Cerebral Artery and its Main Cortical Branches Identified with Noninvasive Transcranial Color-Coded Duplex Sonography.

PURPOSE: To differentiate PCA segments and cortical branches by means of transcranial color-coded duplex sonography (TCCD) and to measure flow parameters at rest and during visual stimulation. MATERIALS AND METHODS: 60 healthy subjects with a good acoustic temporal bone window were examined. The main stem of the PCA (P1, P2 and P3) and 4 main cortical branches - the anterior temporal artery (ATA), the occipital temporal artery (OTA), the parietooccipital artery (POA) and the calcarine artery (CA) - were assessed using an axial transtemporal approach. Systolic and diastolic blood flow velocities (BFVs) were recorded at rest and during visual stimulation. RESULTS: Identification of the P1 segment of the PCA was successful in 97.5% (117/120) of cases. The P2 and P3 segments were visualized in all cases. The 4 main cortical branches could be identified to varying degrees: ATA in 88%, OTA in 96%, POA in 69% and CA in 62%. There was an evoked flow response in the P2 main stem and in all cortical branches. The most pronounced increase in diastolic/systolic BFV after visual stimulation test was seen in the CA (42%/35%), followed by P2 (30%/24%), the POA (27%/27%), the OTA (16%/13%) and the ATA (9%/8%). CONCLUSION: Insonation through the temporal bone window with TCCD confidently allows the assessment of the P1 to P3 segments of the PCA as well as the 2 proximal branches, the ATA and the OTA. An ultrasound-based classification of PCA anatomy and its cortical branches may be used as a noninvasive method for the evaluation of posterior circulation pathology.

Complete basilar artery assessment by transcranial color-coded duplex sonography using the combined transforaminal and transtemporal approach.

PURPOSE: Visualization of the basilar artery (BA) by transcranial color-coded duplex sonography (TCCD) can be challenging. The detection of stenosis or occlusion of the BA often has to rely on indirect signs like a highly pulsatile flow in the vertebral arteries. In case of a distal BA occlusion, however, the flow of the VAs can even be normal. The aim of our TCCD study was to visualize a maximum length of the BA combining the customarily used transforaminal appraoch for the proximal part and the less well known transtemporal coronal approach for the distal BA segment. MATERIALS AND METHODS: TCCD was performed in 60 subjects with a good temporal bone window. Detectable extracranial or intracranial pathology was previously excluded. The BA was insonated using the transforaminal and transtemporal insonation plane and the visible length was measured. In one subject MRA was performed to substantiate our findings. RESULTS: The BA was visualized in all subjects via both approaches. The maximal detectable BA length via the transforaminal and transtemporal approach was 26 ± 8 mm and 18 ± 5 mm, respectively. Assuming a previously reported average anatomical total BA length of 33 ± 6 mm, we supposed complete vessel visualization in 44 subjects (73 %). CONCLUSION: The combined transforaminal and transtemporal insonation approach may permit total BA insonation, thus facilitating the evaluation of BA pathology.

Venous drainage in multiple sclerosis: a combined MRI and ultrasound study.

BACKGROUND: Chronic cerebrospinal venous insufficiency (CCSVI) was proposed as the causal trigger for developing multiple sclerosis (MS). However, current data are contradictory and a gold standard for venous flow assessment is missing. OBJECTIVE: To compare structural magnetic resonance venography (MRV) and dynamic extracranial color-coded duplex sonography (ECCS) in a cohort of patients with MS. METHODS: We enrolled 40 patients (44 ± 10 years). All underwent contrast-enhanced MRV for assessment of internal jugular vein (IJV) and azygos vein (AV) narrowing, graded into 3 groups: 0%-50%, 51%-80%, and >80%. ECCS analysis of blood flow direction, cross-sectional area (CSA), and blood volume flow (BVF) in both IJV and vertebral veins (VV) occurred in the supine and upright body position. RESULTS: MRV identified 1 AV narrowing. IJV analysis yielded 12 patients for group 1 (30%), 19 patients for group 2 (48%), and 9 patients for group 3 (22%). By ECCS criteria, 4 patients (10%) presented with venous drainage abnormalities. Jugular BVF was different only between groups 1 and 3 (616 ± 133 vs. 381 ± 213 mL/min, p = 0.02). No other parameters in supine position and none of the parameters in the upright body position, apart from the IJV-BVF decrease in groups 1 and 3 (479 ± 172 vs. 231 ± 144 mL/min, p = 0.01), were different. CONCLUSIONS: Our ECCS data contradict the postulated 100% prevalence of CCSVI criteria in MS. MRV seems more sensitive to detect IJV narrowing compared to ECCS. A measurable hemodynamic effect only exists in vessel narrowings >80%. Our combined data argue against a causal relationship of venous narrowing and MS, favoring the rejection of the CCSVI hypothesis.

Superior cerebellar artery - a potential pitfall in transcranial posterior cerebral artery insonation.

PURPOSE: Transcranial color-coded duplexsonography (TCCD) of the posterior cerebral artery (PCA) is commonly performed in an axial midbrain insonation plane. The proximal PCA is easily identified as a vessel, originating from the top of the basilar artery (BA). However, the initial segment of the superior cerebellar artery (SCA) also originates from the top segment of the BA and runs in parallel. Therefore, it is possible to confuse the two vessels. The aim of our study is to anatomically differentiate the proximal PCA and SCA by means of TCCD in the coronal plane using a transtemporal approach and to analyze and compare their flow responses during a visual stimulus paradigm. MATERIALS AND METHODS: 60 subjects without a relevant vascular pathology and with a good temporal bone window were examined. The distal BA, the proximal PCA and the SCA were insonated using a coronal insonation plane and identified by their anatomical relationship. The visible length was measured and the blood flow velocities were recorded at rest and during visual stimulation. RESULTS: The identification and differentiation of both vessels was successful in 101 of 120 hemispheres (84.2 %). The PCA systolic flow velocities at rest and during visual stimulation were 58.4 +/- 11 and 71.8 +/- 13 cm/sec (23.6 % increase), while the SCA velocities were 50.7 +/- 12 and 53.5 +/- 13 cm/sec, respectively (5.8 % increase). The difference was significant (p < 0.001). CONCLUSION: Using the coronal transtemporal insonation approach, TCCD makes it possible to identify and distinguish the proximal PCA and SCA. Our study is the first to provide a systematic ultrasound assessment of the SCA and includes data on normal flow velocities and reactivity to a visual stimulus.

Transcranial color-coded sonography successfully visualizes all intracranial parts of the internal carotid artery using the combined transtemporal axial and coronal approach.

BACKGROUND AND PURPOSE: Visualization of the intracranial internal carotid artery (ICA) with transcranial color-coded sonography (TCCS) by using the transtemporal coronal plane has been described previously. Because this approach is limited to the vertical running ICA segments, we investigated the feasibility of using TCCS to visualize all intracranial ICA segments by adding the transtemporal axial approach to the coronal plane. MATERIALS AND METHODS: Subjects with excellent transtemporal acoustic windows were examined by TCCS by using standardized axial and coronal planes. Identification rate, flow velocities, pulsatility and resistance indices, and length (as visible in color-coded power mode) were determined. RESULTS: A total of 120 intracranial ICAs from 60 subjects were investigated. By switching between the axial and coronal insonation planes, all intracranial segments of the ICA could be investigated in 100% of subjects-with the exception of the horizontal part of the petrosal ICA, which was identified in 96.7% of subjects. CONCLUSIONS: TCCS becomes a reliable tool in investigating all parts of the intracranial ICA by adding the transtemporal axial approach to the coronal plane.

Incompetence of internal jugular valve in patients with primary exertional headache: a risk factor?

The pathophysiology of primary exertional headache (EH) is unknown. Physical exertion is associated with Valsalva-like manoeuvres (VM). VM leads to increased intrathoracic pressure and reduces cerebral venous drainage. Internal jugular vein valve incompetence (IJVVI) leads to retrograde venous flow during VM with transient increase of intracranial pressure. We analysed the prevalence of IJVVI in EH patients using duplex ultrasound. Bilateral measurements were performed at rest and during VM in 20 patients and 40 controls. Incompetence was concluded if retrograde venous flow could be seen in the jugular Doppler spectrum during repeated VM. Seventy percent of EH patients and 20% of controls demonstrated IJVVI, yielding a significant difference (P = 0.0004). IJVVI was always observed on the dominant venous drainage side. Our study suggests that intracranial venous congestion caused by retrograde jugular venous flow might play a role in the pathophysiology of EH with IJVVI as a risk factor.

Migraine and delayed ischaemic neurological deficit after subarachnoid haemorrhage in women: a case-control study.

The aim of the present case-control study was to investigate the role of migraine as a potential risk factor for a delayed ischaemic neurological deficit (DIND) after subarachnoid haemorrhage (SAH). A telephone interview was performed in patients or their relatives to determine the prevalence of migraine. Thirty-six women aged <60 years had SAH with Hunt & Hess grade I-III and DIND (group A). This group was compared with an age-matched group of 36 female SAH patients, Hunt & Hess grade I-III without DIND (group B). The two populations were also characterized regarding hypertension, smoking, diabetes mellitus and alcohol use. A significant difference was only found for the prevalence of migraine with 47% in group A and 25% in group B (P < 0.05; odds ratio: 2.68, confidence interval: 0.99-7.29). Migraineurs revealed similar prevalences of risk factors independently of the presence of DINDs. This retrospective study suggests that women with migraine have a higher risk to develop a DIND than women without migraine.

Multiple sclerosis coinciding with Machado-Joseph disease.

Although inflammatory demyelination is considered to be the key feature in multiple sclerosis (MS) pathogenesis, histopathological investigations and MRI studies recently highlighted the extent of neuronal damage that occurs even in the early stages of the disease. We report the unusual case of a patient with Machado-Joseph disease (MJD; spinocerebellar ataxia (SCA) III) and discuss this coincidence in light current pathogenetic paradigms of CNS autoimmunity.

Internal jugular vein valve incompetence and intracranial venous anatomy in transient global amnesia.

BACKGROUND: Recently a causal relation between internal jugular vein valve incompetence (IJVVI) and transient global amnesia (TGA) has been suggested. IJVVI is postulated to provoke a transient mesiotemporal ischaemia by venous congestion. This mechanism requires a patent venous pathway from the affected IJV through the transverse sinus, confluens, straight sinus (SS), vein of Galen into the basal vein of Rosenthal and the internal cerebral veins. OBJECTIVE: To study IJVVI in TGA patients in relation to the intracranial venous anatomy. METHODS: IJVVI was defined if a repeated Valsalva manoeuvre (VM) led to a retrograde jugular flow detected by extracranial duplex ultrasound. Non-contrast venous MR angiography (MRA) was performed to analyse intracranial drainage patterns of the SS in relation to the side of the IJVVI. SS drainage was differentiated into three groups: predominantly right, left, and bilateral drainage. Ultrasound studies were performed in 25 TGA patients and 85 age matched controls. Twenty patients underwent venous MRA. RESULTS: Sixty eight per cent of patients and 33% of controls showed unilateral or bilateral IJVVI (p = 0.0025). In 36% of patients a TGA preceding VM was reported. Drainage pattern of SS and side of IJVVI corresponded in five of eight patients (63%) with VM and four of 12 patients without VM (33%, p = 0.0994). CONCLUSION: Our study confirms the significantly higher prevalence of IJVVI in TGA patients. However, no specific IJVVI related intracranial venous drainage patterns could be found to further support the hypothesis of a direct causal relation between IJVVI and TGA.

A negative personal and family history for venous thrombotic events is not sufficient to exclude thrombophilia in patients with cerebral venous thrombosis.

A hereditary thrombophilia is found in 20-30% of patients with cerebral venous thrombosis (CVT). These patients might have an increased rate of a positive personal or family history of venous thrombotic events. We investigated the diagnostic value of a structured personal and family history for venous thrombotic events in 56 consecutive cases of CVT. Fourteen of 56 patients (25%) had a hereditary thrombophilia, mostly factor V Leiden. Patients with both CVT and hereditary thrombophilia had more frequently a positive family and personal history than patients affected by CVT only but the difference was not strong enough to differ from the 42 CVT patients without thrombophilia (43% vs. 31%; P = 0.52 and 14% vs. 10 %; P = 0.63). We conclude that a negative personal and family history of venous thrombotic events is not sufficient to exclude thrombophilia and patients with CVT should be tested for inherited thrombophilia regardless of the patient's past personal and family history for venous thrombotic events.

Miositis osificante circunscrita axilar / Myositis ossificans circumscripta in the axilla

La miositis osificante circunscrita (MOC) es un proceso benigno raro y hereditario de formación heterotópica de hueso en tejidos blandos y músculo, rara vez encontrada en la infancia. Su localización más frecuente son las extremidades. Se presenta una adolescente de 15 años que sin traumatismo previo presentó una tumoración en axila. Las pruebas de imagen practicadas (radiografía simple, ecografía) no permiten establecer el diagnóstico, ni tampoco excluir malignidad. La biopsia de la tumoración y el estudio histopatológico permitió alcanzar el diagnóstico de miositis osificante circunscrita. En algunos artículos publicados se sugiere que la resonancia magnética (RM) es la primera herramienta en el diagnóstico precoz de esta entidad y evitar la intervención quirúrgica; sin embargo, en muchos casos todavía el diagnóstico es histológico. Debe diferenciarse sobre todo de procesos malignos (sarcomas) y de la fibrodisplasia osificante progresiva. La MOC tiene un buen pronóstico en la infancia (AU)

[Myositis ossificans circumscripta in the axilla]. / Miositis osificante circunscrita axilar.

Myositis ossificans circumscripta (MOC) is a rare benign hereditary disorder characterized by heterotopic bone formation in soft tissue and muscle. This disorder is rarely seen in childhood. MOC is most commonly located in the extremities. We describe a 15-year-old girl with nontraumatic MOC in the axilla. Imaging studies (radiographic, ultrasound) did not provide the correct diagnosis and did not rule out malignancy. Incisional biopsy of the mass and histopathologic examination gave the diagnosis of MOC. Some reports suggest that magnetic resonance imaging may be the first-choice diagnostic option and may avoid unnecessary biopsy. However, in many cases diagnosis is still histological. Differential diagnosis between these lesions and malignant sarcomas and fibrodysplasia ossificans progressiva is required. This entity has a good prognosis in childhood.

Heparin treatment in cerebral sinus and venous thrombosis: patients at risk of fatal outcome.

We performed a retrospective analysis of 79 patients with cerebral sinus venous thrombosis, who were treated with a fixed regimen of dose-adjusted intravenous heparin, to determine predictors of a fatal course. The parameters investigated were the state of consciousness and the presence of intracranial haemorrhage (ICH) at the start of heparin treatment, involvement of the internal venous system, mean delay from initial symptom to stupor or coma and from initial symptom to hospital admission, focal neurological deficits, mean intracranial circulation time (ICT) on conventional angiography, and age and sex distribution. Mortality rate was 10% in this series (8/79). There was a strong link between the outcome and the level of vigilance: 53% of the patients with stupor or coma at the start of the heparin therapy died (8/15), whereas all of the 64 patients with no more than mildly impaired vigilance survived (p < 0.00001). Furthermore, mean age and mean ICT were significantly higher in the group of patients who died. There was a statistical trend (p = 0.056) for ICH to be more frequent in cases with fatal outcome, but there was reason to assume that ICH represented an epiphenomenon of a severe course rather than an independent predictor. Other investigated parameters were not linked with a fatal outcome.