The SEPIVAC study (Italian initials for "epidemiologic study of incidence of acute cerebrovascular disease") is a community-based epidemiologic survey of incidence and outcome of cerebrovascular disease in the territory of the 6th Local Health Unit, Umbria, Italy, where 49,101 people live. All cases were registered with the study either by notification from general practitioners or by check of hospital admission within the study area and in the two hospitals of Perugia. Death certificates were looked at as well. Patients were registered with the study when the clinical picture fulfilled the definition of stroke and transient ischemic attack (TLA) adopted for this study. Patients were followed up at approximately 30 days and 6 months. During the Arst year of the study (September 1, 1986 to August 31, 1987), 189 cases were registered: 108 suffered a "first ever in a lifetime" stroke, 30 a recurrent stroke, and 51 a "first ever in a lifetime" transient ischemic attack. Sixty-one percent of patients (71% of first strokes) had a computed tomography scan. For our study, the crude annual incidence rate of first stroke was 2.2 per 1,000 (confidence intervals 1.81-2.66); the standardized rate to the European population was 1.36 (confidence intervals 1.06-1.74). At least 83% of first strokes were due to cerebral ischemia; in 26 cases a clinical diagnosis of lacunar ischemia was made. The 30-day case fatality rate was 21%; 25% of our patients had recovered completely or almost completely after 1 month. (Stroke 1989;20:853-857)

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Background: In patients with acute stroke, the hyperdense middle cerebral artery (MCA) and internal carotid artery (ICA) signs on CT scans are markers of early ischemia, but their prognostic implications remain unclear.The aims of this prospective study were to assess: (1) the occurrence rate of hyperdense MCA and/or ICA in patients admitted for acute ischemic stroke; (2) the risk factors for hyperdense MCA and/or ICA; (3) the correlation between hyperdense MCA and/or ICA and functional outcome at 3 months. Methods: Consecutive patients admitted with ischemic stroke between 1 January 2006 and 30 June 2010 were included in this prospective single-centre cohort study. Results: 1,010 patients (mean age 71.9 years; 56.7% males) were included in the study. Among these patients, 148 (14.7%; mean age 71.2 years; 52% males) had hyperdense MCA and/or ICA. Overall, 163 patients (16.1%) had a final infarct covering more than one third of the MCA territory. Seventy-eight of 148 patients (52.7%) with hyperdense MCA and/or ICA had an infarct involving more than one third of the MCA territory compared to 85 of the 862 patients without artery hyperdensity (9.9%). At 3 months, 18 patients were lost to follow-up, 325 patients (32.8%) were disabled and 165 died (16.5%). Age (OR 1.06 for 1 added year; 95% CI 1.04–1.08), National Institute of Health Stroke Scale score for 1 added point on admission (OR 1.2; 95% CI 1.2–1.3), stroke due to atherosclerosis (OR 2.3; 95% CI 1.0–5.4), hemorrhagic transformation of the ischemic lesion (OR 2.2; 95% CI 1.0–4.9), and hyperdense MCA and/or ICA (OR 2.0; 95% CI 1.0–4.0) were associated with adverse outcome. Conclusions: In this prospective cohort of patients with acute ischemic stroke, we observed an incidence of hyperdense MCA and/or ICA arteries of about 15%; hyperdense MCA and/or ICA were associated with a final infarct involving more than one third of the MCA territory and poor functional outcome at 3 months.

DESCRIPTIONPituitary apoplexy (PA) is a clinical syndrome caused by acute haemorrhage or infarction of the pituitary gland, generally within a pituitary adenoma, and manifests as visual impairment, severe headache, meningeal irritation, consciousness disturbance and hormonal dysfunction. 1Cerebral infarction secondary to arterial vasospasm is an extremely rare complication of PA, and only a few cases have been reported. A 55-year-old postmenopausal woman was admitted to the emergency department, with worsening headache and decreased visual acuity. An unenhanced CT scan detected a 3 cm pituitary mass without signs of subarachnoid haemorrhage (SAH). Two days later, she developed fever (39.1°C), and a second CT showed a larger hyperdense sellar mass, suggesting intralesional bleeding of a pituitary adenoma with secondary hypothalamus compression. Laboratory analyses revealed increased erythrocyte sedimentation rate (64 mm/hour) and pituitary hypofunction (table 1). The patient became lethargic, and a subsequent brain MRI examination confirmed the presence of a giant haemorrhagic sellar mass (figure 1) with mild meningeal enhancement, indicative of meningeal irritation. A focal cerebral oedema without signs of restriction was also noted in the left mesial temporal region on diffusion-weighted imaging (DWI). Trans-sphenoidal decompression and tumour excision were performed 3 days after the diagnosis of PA. A second MRI scan (completed the same day for difficult post-operative awakening) detected bilateral extensive ischaemic lesions with DWI restriction affecting the territory of the basilar artery (right cerebellum) and middle-anterior cerebral artery (figure 2), and associated with arterial vasospasm (figure 3) without signs of SAH.

Moyamoya angiopathy (MA) is a rare cerebrovascular disorder characterised by the progressive occlusion of the internal carotid artery. Its aetiology is uncertain, but a genetic background seems likely, given the high MA familial rate. To investigate the aetiology of craniosynostosis and juvenile moyamoya in a 14-year-old male patient, we performed an array-comparative genomic hybridisation revealing a de novo interstitial deletion of 8.5 Mb in chromosome region 1p32p31. The deletion involved 34 protein coding genes, including NF1A, whose haploinsufficiency is indicated as being mainly responsible for the 1p32-p31 chromosome deletion syndrome phenotype (OMIM 613735). Our patient also has a deleted FOXD3 of the FOX gene family of transcription factors, which plays an important role in neural crest cell growth and differentiation. As the murine FOXD3−/− model shows craniofacial anomalies and abnormal common carotid artery morphology, it can be hypothesised that FOXD3 is involved in the pathogenesis of the craniofacial and vascular defects observed in our patient. In support of our assumption, we found in the literature another patient with a syndromic form of MA who had a deletion involving another FOX gene (FOXC1). In addition to describing the clinical history of our patient, we have reviewed all of the available literature concerning other patients with a 1p32p31 deletion, including cases from the Decipher database, and we have also reviewed the genetic disorders associated with MA, which is a useful guide for the diagnosis of syndromic form of MA.