S. Bahar

Masked assessment of MRI findings: is it possible to differentiate neuro-Behçet's disease from other central nervous system

Abstract Two neuroradiologists reviewed MRI studies of 34 patients with neuro-Behçets disease (NBD), 22 with multiple sclerosis (MS) and 7 with systemic lupus erythematosus (SLE) with central nervous system involvement, masked to the clinical diagnosis, age and sex of the patients. Of the patients with NBD 12 were in an acute attack; the others had chronic disease. MRI was assessed using a set of criteria, looking at atrophy, the site of discrete parenchymal lesions, regions of predominant involvement and the extent of the lesion(s). The observers also made a guess at the clinical diagnosis. The brain stem and/or basal ganglia were the most predominantly involved sites in all patients with acute NBD; 75 % of these lesions were large and confluent, mainly extending from the brain stem to the diencephalon and basal ganglia. However, in chronic cases, the predominant involvement was in the brain stem and/or basal ganglia in only 36 %, and in cerebral hemisphere white matter in another 36 %; 27 % of these patients showed no parenchymal lesion. Hemisphere white-matter lesions were equally distributed between periventricular and other areas in NBD, while in MS more were periventricular, and in SLE more were nonperiventricular. Brain-stem atrophy was seen in 21 % of patients with NBD, with a specificity of 96.5 %. In the absence of cortical atrophy, its specificity was 100 %. The attempt at making a radiological diagnosis was successful in all cases of acute NBD and 95.5 % of patients with MS, but in only 40 % of patients with chronic NBD. Most of this latter groups MRI studies were interpreted as MS. An extensive lesion involving the brain stem and basal ganglia seemed to be diagnostic of acute NBD. However, hemisphere white-matter lesions could not be differentiated from those in MS.

Etiology of first‐ever ischaemic stroke in European young adults: the 15 cities young stroke study

Risk factors for IS in young adults differ between genders and evolve with age, but data on the age‐ and gender‐specific differences by stroke etiology are scare. These features were compared based on individual patient data from 15 European stroke centers.

Spontaneous dissection of the extracranial vertebral artery with spinal subarachnoid haemorrhage in a patient with Behçet's disease

A 40-year-old man with known definite Behçets disease (BD) was admitted with confusional state which had started 4 days before admission with an acute headache and vomiting. Neurological examination revealed confusion, stiff neck, right facial weakness, left hemiparesis, dysartria and truncal ataxia. CSF was haemorrhagic and xanthochromic. Cranial CT scans were negative, but MRI showed a right pontine hyperintense lesion on T2-weighted images. Bilateral carotid angiograms were normal. Right vertebral angiogram showed findings consistent with a dissection at the V2 segment of the artery. At the level of the fifth cervical vertebra, a radiculomedullary branch of the vertebral artery with an ancurysmal dilatation in its intradural portion was notable. This case shows that, in BD, aneurysn formation can also occur in a spinal artery and spontaneous vertebral artery dissection can be seen.

Demographic and geographic vascular risk factor differences in european young adults with ischemic stroke: The 15 cities young stroke study

Background and Purpose— We compared among young patients with ischemic stroke the distribution of vascular risk factors among sex, age groups, and 3 distinct geographic regions in Europe. Methods— We included patients with first-ever ischemic stroke aged 15 to 49 years from existing hospital- or population-based prospective or consecutive young stroke registries involving 15 cities in 12 countries. Geographic regions were defined as northern (Finland, Norway), central (Austria, Belgium, France, Germany, Hungary, The Netherlands, Switzerland), and southern (Greece, Italy, Turkey) Europe. Hierarchical regression models were used for comparisons. Results— In the study cohort (n=3944), the 3 most frequent risk factors were current smoking (48.7%), dyslipidemia (45.8%), and hypertension (35.9%). Compared with central (n=1868; median age, 43 years) and northern (n=1330; median age, 44 years) European patients, southern Europeans (n=746; median age, 41 years) were younger. No sex difference emerged between the regions, male:female ratio being 0.7 in those aged <34 years and reaching 1.7 in those aged 45 to 49 years. After accounting for confounders, no risk-factor differences emerged at the region level. Compared with females, males were older and they more frequently had dyslipidemia or coronary heart disease, or were smokers, irrespective of region. In both sexes, prevalence of family history of stroke, dyslipidemia, smoking, hypertension, diabetes mellitus, coronary heart disease, peripheral arterial disease, and atrial fibrillation positively correlated with age across all regions. Conclusions— Primary preventive strategies for ischemic stroke in young adults—having high rate of modifiable risk factors—should be targeted according to sex and age at continental level.

Spontaneous vertebro-vertebral arterio-venous fistula associated with fibro-muscular dysplasia

SummaryExtracranial vertebral arteriovenous fistulae of non traumatic origin are extremly rare. The authors report two cases of spontaneous vertebro-vertebral arteriovenous fistulae associated with fibromuscular dysplasia. They discuss the relation-ship between arteriovenous fistulae and fibro-muscular dysplasia, and the specific problems of treatment in these two cases.

Cerebral vasculitis and ischaemic stroke in Behçet's disease: report of one case and review of the literature.

Introduction. Behçet’s disease (BD) is a multisystemic, recurrent, inflammatory disorder. Neurological involvement is well‐known but cerebral vasculitis and ischaemic stroke are unusual.

Stroke unit versus neurology ward--a before and after study.

Few studies have tested the hypothesis of whether the beneficial effect of Stroke Units (SUs) can be reproduced in routine clinical practice and whether SU are also superior to neurological wards [NWs]. We aimed to compare the outcomes of patients of a newly implemented SU to the outcomes of patients hospitalized in a NW. We made a before-after comparison of 352 SUs and 352 NWs patients after adjusting for case-mixes by the multivariate method. Subgroup analyses were also performed to evaluate which patient groups benefit the most. In-hospital case-fatality, proportion of independent patients at discharge, length of hospital stay (LOHS), medical complication rate were the main outcome measures. Adjusted in-hospital case fatality was significantly reduced in the SUs (OR: 0.44, 95 % CI: 0.26–0.76; p = 0.003). The proportion of independent patients at discharge and patients having medical complications was not different. Length of hospital stay was shorter in SU patients (13.76 days vs. 16.72 days, p = 0.003). Treatment in the SUs decreased case fatality in many subgroups [men, elderly, early admitted, severe stroke, co-morbidity present and ischemic stroke groups]. The results of randomized trials in favor of SUs can be reproduced in routine clinical practice. The benefit of SU care seems to be more apparent with advancing age and increasing stroke severity. Stroke Unit seems to be a better alternative to an experienced NW.

Intracranial hypertension in Behçet's Disease*

Among 103 neuro‐Behçets disease (NBD) patients seen in an 8‐year period, 16 patients with intracranial hypertension (IH) are presented. The main underlying cause of IH was cerebral venous thrombosis (10 patients), and one patient showed vena cava superior thrombosis, while in three patients angiographies and/or MRI studies remained negative. In the remaining two patients the investigations were not adequate to exclude cerebral venous thrombosis. In a mean follow‐up period of 5 years, three patients had one recurrence of IH, and the three cases in whom cerebral venous thrombosis could not be documented despite adequate investigation had frequent relapses. On follow‐up, none of the patients had any other types of neurologic involvement. Among the other manifestations of Behçets disease thrombophlebitis was significantly higher, whereas uveitis was significantly lower in IH patients when compared with our remaining NBD patients. Intracranial hypertension is one of the major types of neurologic involvement in Behçets disease, and seems to have different pathogenetic implications, usually with a better outcome than other types of neurologic involvement.

A controlled study of reliability and validity of MRI findings in neuro-Behçet's disease

Our aim was to test the reliability of interpreting MRI studies in neuro-Behçets disease (NBD) and to determine the sensitivity and specificity of different MRI findings. We prospectively studied 50 patients: 24 had chronic NBD, 12 multiple sclerosis, 5 vasculitis other than Behçets disease (BD) and 9 patients had BD without neurological involvement. MRI studies were performed according to a standard protocol with a 0.2 T imager. Two neuroradiologists, blinded to the diagnosis, age and sex of the subjects, reviewed the films independently, twice. Separate assessments were made for a set of items: dural sinus pathology, widening of ventricles and sulci, brain stem atrophy, lesions of the cerebral cortex, discrete lesions of deep white matter, basal ganglia, brain stem and cerebellum and the presence of smooth periventricular high-signal foci. Intraobserver agreement was substantial or better, and interobserver agreement moderate to substantial for most items. In these patients with chronic NBD we found low sensitivity on all assessed items. Dural sinus pathology or brain stem atrophy were highly specific, but parenchymal lesions in different sites had uniformly low specificity.

Vertebral artery dissection presenting with isolated neck pain.

Unilateral pain in the cervical region and limitation of neck movements are nonspecific symptoms frequently encountered in daily medical practice. Vertebral artery dissection is rarely considered as a diagnostic possibility unless brainstem or cerebellar ischemia follows the acute pain. Three cases of vertebral artery dissection (VAD) having the sole complaint of pain of acute onset in the posterior neck region are presented. None of the patients had ever reported a similar pain, and the neurological examination was unremarkable in all of them. Doppler ultrasonography suggested VAD in 2 cases, and the diagnosis was confirmed with T1 fat‐suppressed magnetic resonance imaging technique in all patients. Severe neck pain and/or occipital headache frequently accompanies ischemic symptoms in cases with VAD. The cases in this report emphasize that spontaneous and often unilateral posterior cervical pain of acute onset can be the only manifestation of a VAD. A high degree of suspicion especially in young patients with no past history of a similar pain can help to establish the diagnosis, thereby preventing erroneous and potentially hazardous therapeutic interventions such as physiotherapy or neck manipulation.