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Over 10 years after European approval, thrombolysis is still limited by a restricted time window and non-optimal territorial coverage. Implementation of telestroke can give a growing number of patients access to treatment. We hereby present the first Italian telemedicine study applied to both the acute and the monitoring phase of stroke care. From January 2011 to December 2013, we tested a web-based, drip, and treat interaction model, connecting the cerebrovascular specialist of one hub center to the Emergency Department of a Spoke center. We then compared thrombolysis delivered using the telestroke model with thrombolysis provided at the Hub Stroke Unit at the time when the telemedicine program was activated. Telethrombolysis data were then compared with data from the two main international telestroke projects (TEMPiS and REACH), and other European telestroke studies performed at the time of writing. We collected a total of 131 thrombolysis procedures (25 telethrombolysis and 106 thrombolysis patients at the Stroke Unit). Statistical analysis with the t test yielded no statistically significant differences between the two populations in door-to-scan, door-to-needle (DTN), and onset-to-treatment times (OTT). Our OTT and DTN pathway times were longer than the TEMPiS and REACH studies but comparable with other European telemedicine trials, despite different models of interaction and number of centers. Our study in a northeastern province of Italy confirms the potential of applying telemedicine to a cerebrovascular pathology.

Background and Purpose— The etiology of spontaneous cervical artery dissection (sCAD) is still unknown, even though an underlying arteriopathy impairing vasomotion has often been suspected. This study was undertaken to investigate: (1) spontaneous, (2) endothelial-dependent, and (3) endothelial-independent vasodilation in patients with multiple sCAD. Methods— In 19 consecutive patients with multiple carotid or vertebral artery dissections high-resolution ultrasound was used to assess spontaneous and endothelial-independent dilations (isosorbide dinitrate-mediated) in the common carotid, vertebral and brachial arteries, and endothelial-dependent dilation (flow-mediated arterial dilation) in the brachial arteries alone. The same parameters were measured in 19 healthy subjects matched for age, sex, and height (controls). Ultrasound studies were performed by one investigator, and off-line analysis by another investigator who was blinded to the clinical data and study status (patient or control). Results— Spontaneous and endothelial-independent dilations were significantly impaired in the carotid ( P =0.0006 and P P =0.0121 and P =0.0047, respectively) of patients as compared with controls, whereas no statistically significant differences were found in the brachial arteries; conversely, endothelial-dependent dilation of the brachial arteries was significantly lower in patients as compared with controls ( P Conclusions— Patients with multiple sCADs have a significantly impaired vasomotion, which may predispose to dissection.

Progressive multifocal leukoencephalopathy (PML) is a serious disorder that primarily affects individuals with a suppressed immune system. Few semiological elements help clearly distinguish PML from other diseases included in the differential diagnosis. Moreover, the clinical course of disease may be chronic or rapidly progressive, with different magnetic resonance imaging (MRI) patterns. The purpose of this study was to confirm the diagnostic value of conventional MRI sequences combined with diffusion-weighted imaging (DWI) in PML to identify those patients with worst prognosis.We used both conventional MRI sequences and DWI to monitor four male patients aged between 40 and 50 years affected by PML. In two patients, the disease rapidly led to death whereas the other two patients presented a chronic course.Conventional MRI sequences enable detection of the extension of white matter lesions. DWI permits more accurate differentiation of the disease progression front, which exhibits low signal intensity in apparent diffusion coefficient (ADC) maps, from the central gliotic area of demyelinisation, characterised by high ADC values. Patients with rapidly evolving PML have a clear progression front on DWI, which seems to be very tenuous, if not absent, during the quiescent phases of the disease.The addition of DWI sequences to conventional MRI seems to be a valid method for accurately diagnosing PML and establishing the degree of disease progression.

Few population studies are available on epidemiological indexes of hereditary ataxias. An investigation on the prevalence rate of these movement disorders is in progress for the Veneto region, the main area of northeast Italy with a population of 4,490,586 inhabitants. The first results of this epidemiological survey concern the province of Padua, which numbers 845,203 residents (January 1, 2002). The prevalence rate of inherited ataxias has been estimated at 93.3 cases per million inhabitants. The most common types appeared to be the autosomal dominant forms, namely spinocerebellar ataxia type 1 and 2, with a prevalence of 24 per 1,000,000. In the same population, with a prevalence rate of 6 per 1,000,000, Friedreich’s ataxia was defined as the prominent recessive autosomal form. There were very rare cases of ataxia telangiectasia, ataxia with vitamin E deficiency and cerebellar ataxia with congenital muscular dystrophy, a recently identified autosomal recessive disease.

Cerebellar hypoplasia may, at neuroimaging studies, be found in association with congenital muscular dystrophy (CMD), although it is an extremely rare occurrence. We here report on three CMD patients who underwent a longitudinal evaluation of clinical and neuroimaging features for a mean period of 18 years. Case 1, a 22-year-old woman, and cases 2 and 3, brothers aged 26 and 20 years, respectively, had presented a mild to moderate muscular weakness and increased serum creatine kinase (CK) levels since birth. All cases were diagnosed in the first years of life, with identification of evident dystrophic changes at muscle biopsy and moderate to severe cerebellar hypoplasia at brain computed tomography (CT) scan. Subsequently, all the patients underwent a second muscle biopsy, with immunostaining and immunoblot analysis, which showed normal values for merosin, dystrophin and dystrophin-related proteins. During the longitudinal study, the patients underwent repeated neurological and psychiatric examinations, serum CK controls, intellectual ability assessments and neuroimaging evaluations (CT and/or magnetic resonance imaging (MRI)). In all cases, these investigations indicated a mild to moderate deficit in the proximal muscles and a clear-cut cerebellar syndrome which, it was assumed, had been present since the first years. The patients also presented some intellectual difficulties, with an IQ of 0.69 in case 1, 0.83 in case 2 and 0.61 in case 3. The clinical course of all the patients was static, and all symptoms of the combined muscle and brain involvement persisted. Nor were any changes in the cerebellar hypoplasia observed at repeat MRIs. Findings obtained by us on the longitudinal study and a review of the literature indicate that cerebellar hypoplasia and merosin-positive CMD constitute a particular clinical phenotype, mainly characterized by an ataxic syndrome associated with a non-severe muscular involvement and a possible mild intellectual impairment.

BACKGROUND AND PURPOSE Internal carotid artery (ICA) elongation (coiling and kinking) has been suggested as a risk factor for carotid dissection. Since vasomotion is known to be impaired in spontaneous cervical vessel dissection, we investigated whether endothelial-dependent vasodilation in subjects with carotid coiling and kinking is compromised. METHODS We undertook a case-control study using high-resolution ultrasound and measured flow-mediated dilation (FMD) of the brachial artery in 80 subjects with carotid elongation and in 80 age- and sex-matched healthy controls (HC). The hemodynamic impact of carotid elongation was taken into consideration subdividing mild/moderate kinking from severe kinking according to a peak systolic blood flow velocity >150 cm/s. RESULTS FMD did not differ among subjects with coiling (14.51 ± 7.86%), mild/moderate kinking (14.38 ± 9.58%) and HC (15.53 ± 8.48%), while subjects with a severe kinking had a significantly lower FMD (8.38 ± 3.26). CONCLUSIONS Among subjects with carotid elongation, those with severe kinking have an impaired endothelial-dependent vasodilation and might be prone to carotid dissection.

Background and Purpose— Transient global amnesia (TGA) has been associated with an increased prevalence of internal jugular valve insufficiency and many patients report Valsalva-associated maneuvers before TGA onset. These findings have led to the assumption of hemodynamic alterations in intracranial veins inducing focal hippocampal ischemia. We investigated this hypothesis in patients with TGA and control subjects. Methods— Seventy-five patients with TGA and 75 age- and sex-matched healthy subjects were enrolled into a cross-sectional study. Extracranial and transcranial high-resolution venous echo-color-Doppler sonography was performed blindly in all patients and control subjects. Blood flow direction and velocities were recorded at the internal jugular veins, basal veins of Rosenthal, and vein of Galen, both at rest and during Valsalva-associated maneuvers. Results— Mean age of patients with TGA was 60.3±8.0 years (median, 60 years; range, 44–78 years); 44 (59%) were female (female/male ratio: 1.42). Internal jugular valve insufficiency (left, right, or bilateral) was found to be more frequent in patients with TGA than in control subjects: 53 (70.7%) versus 22 (29.3%; P Conclusions— This study, although confirming the association between TGA and internal jugular valve insufficiency, challenges the hypothesis that cerebral venous congestion plays a significant role in the pathogenesis of TGA.

To monitor the sonographic course of spontaneous cervical artery dissections (sCADs) and investigate their recanalization and recurrence rates.All consecutive patients with an MRI-proven sCAD were prospectively evaluated by neurovascular ultrasound (nUS) daily while in hospital, then monthly for the first 6 months after discharge and every 6 months thereafter, for a mean follow-up period of 58 months (range, 28-96 months).A total of 105 sCADs were detected in 76 patients: 61 (58.1%) involved the internal carotid artery and 44 (41.9%) the vertebral artery, while multiple sCADs were found in 4 patients (5.3%). Follow-up was obtained in 74 patients (97.3%, 103 vessels). The complete and hemodynamically significant (50% stenosis) recanalization rates were 51.4% (53/103) and 20.4% (21/103). All but one complete recanalization occurred within the first 9 months. There were early recurrences (while in hospital) in 20 previously unaffected arteries (26.3%) and late recurrences in 2 arteries (2.7%), site of a previous sCAD. All patients (n = 6) with a family history of arterial dissection had a sCAD recurrence (4 early and 2 late) as opposed to 16 (22.8%) among those with no known familial disease (p0.001).These results suggest that most lumen changes occur within the first few months after the initial event, but recanalization may occur even after 1 year. Early recurrence is not uncommon and usually involves arteries previously unaffected by dissection, while the risk of late recurrence is low. A family history of arterial dissection is strongly associated with sCAD recurrence.

WCN 2013 No: 2165 Topic: 3 — Stroke Functional condition of central and cerebral hemodynamic in patients with coma caused by acute ischemic stroke O.O. Ivanov, M.G. Zhestikova, Y.A. Churliaev, K.V. Lukashev, O.V. Rudenkova, D.G. Dantsiger, A.G. Chechenin, V.M. Aliferova, N.G. Zhukova, I.A. Zhukova. Neurology, City Clinical Hospital Q 1, Russia; Neurology, State Educational Establishment of Additional Medical Education 'Novokuznetsk Post-Graduate-Medical Institute' Russian Health Care, Russia; Reanimatology, City Clinical Hospital Q 1, Novokuznetsk, Russia; Manegment, City Clinical Hospital Q 1, Novokuznetsk, Russia; Neurology, State Educational Establishment of High Professional Education Siberian Medical University, Tomsk