Francesco Causin

No evidence of chronic cerebrospinal venous insufficiency at multiple sclerosis onset.

An impaired cerebrospinal venous drainage, defined as chronic cerebrospinal venous insufficiency (CCSVI), has been recently hypothesized to be the possible cause of multiple sclerosis (MS). We investigated this hypothesis by studying the occurrence of CCSVI in clinically isolated syndromes (CISs) suggestive of MS.

Progressive multiple sclerosis is not associated with chronic cerebrospinal venous insufficiency.

Objective: Chronic cerebrospinal venous insufficiency (CCSVI) had been suggested to play a major pathogenetic role in multiple sclerosis (MS), but recent data on early stages of MS have not confirmed this theory. Nonetheless, CCSVI could represent a late phenomenon of MS or be associated with progression of disability. Thus, we studied CCSVI prevalence in primary progressive (PP) and secondary progressive (SP) MS, to clarify whether CCSVI characterizes the progressive forms of this disease. Methods: A total of 35 patients with SPMS, 25 patients with PPMS, and 60 age- and gender-matched normal controls (NC) were enrolled into a cross-sectional study. Extracranial and transcranial high-resolution venous echo color Doppler sonography (ECDS-TCDS) was performed in all patients and NC. Those patients having any abnormal ultrasound finding were asked to undergo selective venography (VGF). Results: Patients with PPMS (11 women, 14 men; mean age 47 ± 11 years) had a disease duration of 11 ± 7 years and Expanded Disability Status Scale (EDSS) score of 6.0 ± 0.5. Patients with SPMS (22 women, 13 men; mean age 45 ± 14.5 years) had a disease duration of 18 ± 14 years and EDSS score of 6.0 ± 0.8. TCDS was normal in all patients. ECDS showed one or more abnormal findings in 9/60 (15.0%) patients (7/35 [20.0%] SPMS, 2/25 [8.0%] PPMS) and in 14/60 (23.3%) NC (p not significant for all comparisons). CCSVI criteria were fulfilled in 0 NC and 4 (6.7%) patients with MS: 3 SPMS and 1 PPMS. VGF, performed in 6/9 patients, was abnormal only in one case who had bilateral internal jugular vein stenosis. Conclusion: Our findings indicate that CCSVI is not a late secondary phenomenon of MS and is not associated with disability.

Early results of CyberKnife radiosurgery for arteriovenous malformations

OBJECT The authors describe a method that utilizes an image-guided robotic radiosurgical apparatus (the CyberKnife) for treatment of cerebral arteriovenous malformations (AVMs). This procedure required the development of an original technique that allows a high degree of automation. METHODS Angiographic images were imported into the treatment planning software by coregistering CT and 3D rotational angiography. The nidus contour was delineated using the contouring tools of the treatment planning system. Functional MR imaging was employed for contouring critical cortical regions, such as the motor cortex and language areas. Once the radiation dose to be delivered to the target volume and dose constraints to critical structures were prescribed, the inverse treatment planning function determined the optimal treatment plan. RESULTS A series of 279 patients with cerebral AVMs underwent CyberKnife radiosurgery. One transitory adverse effect of the radiation procedure was observed. Eight bleeding occurrences were noted before complete AVM obliteration. Of the 102 patients with follow-up > 36 months, 80 underwent angiographic evaluation. In this group, 65 patients (81.2%) showed complete angiographic obliteration of their AVM. In 8 more patients, complete angiographic obliteration was demonstrated by MR angiography only. CONCLUSIONS This is the first report describing a technique developed for CyberKnife radiosurgery of cerebral AVMs. The use of different imaging modalities for automatic delineation of the target and critical structures combined with the employment of the inverse treatment planning capability is the crucial point of the procedure. The procedure proved to be safe and efficient.

Complications of endovascular treatment of cerebral aneurysms

The number of neuroendovascular treatments of both ruptured and unruptured aneurysms has increased substantially in the last two decades. Complications of endovascular treatments of cerebral aneurysms are rare but can potentially lead to acute worsening of the neurological status, to new neurological deficits or death. Some of the possible complications, such as vascular access site complications or systemic side effects associated with contrast medium (e.g. contrast medium allergy, contrast induced nephropathy) can also be encountered in diagnostic angiography. The most common complications of endovascular treatment of cerebral aneurysms are related to acute thromboembolic events and perforation of the aneurysm. Overall, the reported rate of thromboembolic complications ranges between 4.7% and 12.5% while the rate of intraprocedural rupture of cerebral aneurysms is about 0.7% in patients with unruptured aneurysms and about 4.1% in patients with previously ruptured aneurysms. Thromboembolic and hemorrhagic complications may occur during different phases of endovascular procedures and are related to different technical, clinical and anatomic reasons. A thorough knowledge of the different aspects of these complications can reduce the risk of their occurrence and minimize their clinical sequelae. A deep understanding of complications and of their management is thus part of the best standard of care.

The Parallel Analysis of Phase Sensitive Inversion Recovery (PSIR) and Double Inversion Recovery (DIR) Images Significantly Improves the Detection of Cortical Lesions in Multiple Sclerosis (MS) since Clinical Onset

Background Double inversion recovery (DIR) detects only a minority (<20%) of cortical lesions (CL) in multiple sclerosis (MS). Phase-sensitive inversion recovery (PSIR) was suggested to be substantially superior to DIR in the detection of cortical lesions (CL). These two sequences might be complementary. Objectives To analyze CL frequency and type in MS patients having different disease duration and disability, including patients at clinical onset, and to discern more correctly the artifacts, by combining DIR and PSIR images. Patients and Methods 40 patients were enrolled in the study: 10 clinically isolated syndrome/early relapsing remitting MS (CIS/eRRMS), 24 relapsing remitting MS (RRMS), 6 secondary progressive MS (SPMS). DIR and PSIR images were jointly used to classify lesions as purely intracortical (IC), leukocortical (LC) and juxtacortical (JC). Results PSIR disclosed CL in 100% of the patients and was capable of identifying more than four times lesions (455.5%, p<0.00001), especially IC (mean numbers: 36.5 in CIS/eRRMS, 45.0 in RRMS and 52.3 in SPMS) and LC (mean numbers: 10.9 in CIS/eRRMS, 20.1 in RRMS and 25.3 in SPMS), compared to DIR (p<0.00001). CL number was significantly higher in SPMS compared to RRMS (p<0.0001). Artifacts were more accurately identified by comparing the two sequences. Conclusions Our study confirms the higher ability of PSIR in disclosing and classifying CL. The presence of CL in all CIS patients further points out the relevance of cortical pathology in MS. Whether the parallel analysis of DIR and PSIR images may be useful for diagnostic purposes, especially when a diagnosis of MS is suspected but not confirmed by routine MRI, needs to be evaluated in larger patient series. The analysis of the cortex by DIR and PSIR may also allow a better stratification of the patients for prognostic and counseling purposes, as well as for their inclusion in clinical studies.

Cortical relapses in multiple sclerosis

Background: Multiple sclerosis (MS) is a white and grey matter disease of the central nervous system (CNS). It is recognized that cortical damage (i.e. focal lesions and atrophy) plays a role in determining the accumulation of physical and cognitive disability that is observed in patients with progressive MS. To date, an association of cortical lesions with clinical relapses has not been described. Results: We report clinical and magnetic resonance imaging (MRI) findings of five relapsing–remitting MS (RRMS) patients who had clinical relapses characterized by the acute appearance of cortical symptoms, due to the development of large, snake-like, cortical inflammatory lesions. Symptoms were: acute Wernicke’s aphasia mimicking stroke; agraphia with acalculia, not associated to a motor deficit nor linguistic disturbance; hyposthenia of the left arm, followed by muscle twitching of the hand, spreading to arm and face; acute onset of left lower limb paroxysmal hypertonia; and temporal lobe status epilepticus, with psychotic symptoms. Conclusions: Cortical relapses may occur in MS. MRI examination in MS should include sequences, such as double inversion recovery (DIR) or phase sensitive inversion recovery (PSIR), that are aimed at visualizing cortical lesions, especially in the presence of symptoms of cortical dysfunction. Our observation further stresses and extends the clinical relevance of cortical pathology in MS.

MRI-detectable cortical lesions in the cerebellum and their clinical relevance in multiple sclerosis

Background: The demonstration of cortical lesions (CL) in the cerebellum by magnetic resonance imaging (MRI) is hampered by technical and anatomical constraints. Objective: To investigate the occurrence of cerebellar CL and their correlation with cerebellar-related disability by combining Double Inversion Recovery (DIR) and Phase Sensitive Inversion Recovery (PSIR) MRI images in multiple sclerosis (MS) patients. Material and methods: 40 MS patients (10 CIS/eRRMS, 24 RRMS, 6 SPMS), having a wide range of disability and disease duration, were enrolled. DIR and PSIR images were obtained with a 3T-MRI. Results: Cerebellar white matter lesions (WML) and/or CL were observed in 33/40 patients (82.5%) among which 14/40 had only CL. CL were demonstrated in 26/40 patients by DIR and in 31/40 by PSIR, and their number increased from CIS/eRRMS to SPMS. PSIR disclosed a significantly higher number of CL compared to DIR (RRMS: p=0.0008; SPMS: p=0.002). CL number correlates with the cerebellar Expanded Disability Status Score (EDSS) score (r=0.72, p<0.0001). No correlation was observed between supra-tentorial and cerebellar CL. Conclusions: CL are detected by PSIR in the cerebellum of the majority of MS patients, are more than WML, increase with disease progression and strongly correlate with the cerebellar EDSS. Thus, the observation of CL in the cerebellum of MS at clinical onset might be useful for prognostic and therapeutic aims.

Transdural indocyanine green video-angiography of vascular malformations

BackgroundThe role of indocyanine green video-angiography (ICG-VA) in the surgical resection of vascular malformations has been largely described; conversely, the utility of ICG-VA before dural opening (transdural ICG-VA) in this situation remains unclear. The aim of this study is to present the application of transdural ICG-VA in a consecutive series of patients in order to explore the potential provided by a transdural visualisation of vascular malformations.MethodWe retrospectively analysed the application of intra-operative ICG-VA before dural opening in 15 consecutive patients who underwent surgical resection of vascular malformations. The cases included 12 cerebral arterio-venous malformations (AVMs), 2 cerebral dural arterio-venous fistulas (dAVFs) and 1 spinal arterio-venous fistula (AVF).ResultsICG-VA before dural opening allowed the visualisation of the site and extension of the malformation in 13 out of 15 cases, whilst arterial feeders and venous drainages were identified in 9 out of 15 cases. In two patients with dAVF, the point of fistula could be transdurally identified through ICG-VA. In 14% of cases, the size of bone flap designed on neuronavigation data was then modified according to transdural ICG-VA findings.ConclusionsTransdural ICG-VA proved an efficient tool that allows optimising the exposure of the malformation, performing a safe dural opening and identifying dural vascular connections of the lesion.

Standards of practice in interventional neuroradiology

The growing importance of INR has resulted in the need to define and promote professional standards of clinical practice. Several professional organizations have published guidelines recently for the neurointerventional treatment of cerebrovascular diseases, including technical and personal recommendations, but detailed definitions of technical and organizational conditions needed for the safe and effective performance of such treatments are lacking. To fill this gap ESNR, ESMINT and the UEMS Division for Neuroradiology established a working group, to develop a consensus paper on “Standards of Practice in Interventional Neuroradiology”. This document is the result of the Consensus Working Group and has following review gained approval by the Executive Boards of ESNR and ESMINT and by the members of the UEMS Division for Neuroradiology in 2017.

Rare association between spinal dural arteriovenous fistulas and dysraphisms: Report of two cases and review of the literature with a focus on pitfalls in diagnosis and treatment

Spinal vascular malformations are uncommon yet important spinal pathologies commonly classified in congenital and acquired lesions. Spinal lipomas consist of three subtypes: intramedullary lipomas, lipomyelo(meningo)celes and lipomas of the filum. Although the association of spinal arteriovenous malformations (AVM) with other congenital anomalies is well known, the coexistence of dural arteriovenous fistulas (AVF) and tethered spinal cord is exceptionally rare and only eight cases have been reported. We present two cases from our institution and speculate on the possible origin of such a rare but insidious association. We review the current literature with a focus on possible pitfalls in diagnosis and treatment.