Reversible cerebral vasoconstriction syndrome with involvement of external carotid artery branches.

A 44-year-old woman presented with recurrent episodes of thunderclap headache. Neurological examination and computed tomography brain imaging were unremarkable. Cerebrospinal fluid findings were consistent with subarachnoid hemorrhage. Computed tomography angiography of the circle of Willis showed multiple areas of segmental vasoconstriction. This finding was confirmed on cerebral catheter angiography, with segmental vasoconstriction involving bilateral internal carotid, posterior cerebral, and external carotid branches. No aneurysm or other vascular abnormality was identified. She received treatment with nimodipine. A selective serotonin reuptake inhibitor, started 4 weeks earlier, was discontinued. Follow-up angiography after 3 months demonstrated complete resolution of the segmental vasoconstriction, confirming the diagnosis of reversible cerebral vasoconstriction syndrome (RCVS). She remained headache free at follow-up. To our knowledge, external carotid artery branch involvement in RCVS has been described only in one previous occasion.

Intact neurological outcome after neurointerventional treatment for intracranial venous thrombosis with straight sinus involvement.

The role of neurointerventional treatment, including local thrombolysis, for intracranial venous thrombosis (ICVT) currently remains uncertain. We describe a case of postpartum ICVT involving the straight sinus, with rapid neurological deterioration and progressive thrombosis, despite anticoagulation. She underwent multimodality neurointerventional treatment involving attempted mechanical clot disruption, microcatheter suction, intrathrombus thrombolysis and microballoon disruption and clot retrieval. The patient achieved a full clinical recovery, with radiological evidence of recanalisation at follow-up.

Recurrent stereotyped episodes in cerebral amyloid angiopathy: response to migraine prophylaxis in two patients.

BACKGROUND: Cerebral amyloid angiopathy (CAA) typically presents with cognitive decline or symptomatic intracerebral hemorrhage, but episodes of recurrent stereotyped limb attacks have also been reported. METHODS: Retrospective review of the medical records of 4 patients referred to the general neurology services and a specialist stroke center with clinically probable CAA. RESULTS: Four subjects, all Caucasian, mean age 74 years, were followed up over a mean duration of 20 months. They all experienced recurrent prolonged stereotyped attacks of sensory symptoms, lasting 5-30 min, that resolved completely between attacks. Three subjects developed intracerebral hemorrhage, and 2 had an irreversible rapid cognitive decline. Two patients experienced symptomatic improvement with migraine prophylaxis (verapamil or topiramate). CONCLUSIONS: Recurrent stereotyped prolonged attacks with sensory and motor elements can predate the development of intracerebral hemorrhage in individuals with clinically probable CAA. When evaluating patients with such attacks, neurologists need to consider CAA as a possible mimic of transient ischemic attacks. We suggest a trial of migraine prophylaxis for symptomatic management.

Prevalence and subtypes of radiological cerebrovascular disease in late-onset isolated seizures and epilepsy.

BACKGROUND: Late-onset epilepsy (LOE), onset over 60, is often attributed to cerebrovascular disease (CVD), and is associated with increased stroke risk. We investigated the radiological prevalence of CVD in LOE. METHODS: We undertook a retrospective case-control study of patients with LOE and age and sex-matched controls, also matched for imaging modality. Radiological CVD was recorded, with radiological findings by an experienced consultant neuroradiologist usi a structured proforma. RESULTS: 105 cases and 105 controls were studied, comprising 61 (58.1%) males, mean (±SD) age (years) 72.7±7.48 (cases), 72.4±7.02 (controls). 9 cases had isolated seizures rather than LOE. Imaging modality (in cases and controls) was CT in 59 and MRI in 46. Radiological CVD was more prevalent amongst cases (65.7%) than controls (33.3%) (p<0.0001, Chi-square), odds ratio 3.83 (95% CI 2.16-6.79). Large vessel disease (LVD) (single or multiple cortical or subcortical infarcts>1.5 cm) was present in 23 (21.9%) cases and 2 (1.9%) (p<0.001) controls, with small vessel disease (SVD) (periventricular or subcortical white matter lesions (WMLs), including leukoaraiosis (LA)) present in 52 (49.5%) cases (LA in 4) and 34 (32.3%) controls (LA in 0) (p<0.05). When WMLs were rated using a semiquantitative visual rating scale, a trend towards greater severity was observed amongst cases compared to controls. CONCLUSIONS: Radiological CVD is significantly more prevalent in patients with LOE than controls, including signs of both LVD and SVD. SVD also appears to be of greater severity. Further studies are needed in this area.

Prevalence of radiological and clinical cerebrovascular disease in idiopathic Parkinson's disease.

Comorbid cerebrovascular disease (CVD) can occur in idiopathic Parkinson's disease (IPD) but its reported prevalence varies considerably. CVD may alter the clinical presentation, course and prognosis in IPD. We aimed to determine the prevalence of radiological and clinical CVD in a neurology clinic IPD population. We undertook a retrospective case-control study of neurology clinic patients with IPD for whom cerebral imaging was available, and excluding probable vascular parkinsonism. IPD diagnosis was validated against UK PDS brain bank criteria. Age and sex-matched controls were identified from patients attending neurology clinics with headache. The presence of radiological cerebrovascular disease, symptomatic CVD (stroke and TIA), and CVD risk factors was recorded for cases and controls. Radiological findings were validated by an experienced consultant neuroradiologist using a structured proforma. Eighty-five cases and 85 controls were studied, based on the number of cases for whom brain imaging existed (CT in 50, MRI in 35) and the number of cases for whom suitable controls could be identified. Indications for brain imaging amongst cases were varied. Cases and controls comprised 55(65%) males, mean (± SD) age (years) 67.4 ± 10.1 (cases), 66.6 ± 9.9 (controls). Radiological CVD was significantly commoner amongst cases (39%) than controls (22%) (p=0.02, chi-square; odds ratio 2.2, 95% CI: 1.1-4.6). Cases also had significantly more symptomatic CVD, but not CVD risk factors, than controls. Our findings suggest a higher prevalence of radiological and clinical CVD in patients with IPD compared to controls.

A randomised phase II study of interleukin-1 receptor antagonist in acute stroke patients.

OBJECTIVES: The cytokine interleukin (IL)-1 mediates ischaemic brain damage in rodents. The endogenous, highly selective, IL-1 receptor antagonist (IL-1ra) protects against ischaemic cerebral injury in a range of experimental settings, and IL-1ra causes a marked reduction of cell death when administered peripherally or at a delay in transient cerebral ischaemia. We report here the first randomised, double blind, placebo controlled trial of recombinant human IL-1ra (rhIL-1ra) in patients with acute stroke. METHODS: Patients within 6 hours of the onset of symptoms of acute stroke were randomised to rhIL-1ra or matching placebo. Test treatment was administered intravenously by a 100 mg loading dose over 60 seconds, followed by a 2 mg/kg/h infusion over 72 h. Adverse events and serious adverse events were recorded for up to 3 months, serial blood samples were collected for biological markers up to 3 months, and 5-7 day brain infarct volume was measured by computed tomography. RESULTS: No adverse events were attributed to study treatment among 34 patients randomised. Markers of biological activity, including neutrophil and total white cell counts, C reactive protein, and IL-6 concentrations, were lower in rhIL-1ra treated patients. Among patients with cortical infarcts, clinical outcomes at 3 months in the rhIL-1ra treated group were better than in placebo treated. CONCLUSIONS: These data suggest that rhIL-1ra is safe and well tolerated in acute stroke. In addition, rhIL-1ra exhibited biological activity that is relevant to the pathophysiology and clinical outcome of ischaemic stroke. Our findings identify rhIL-1ra as a potential new therapeutic agent for acute stroke.

The Oxfordshire Community Stroke Project classification in the early hours of ischemic stroke and relation to infarct site and size on cranial computed tomography.

BACKGROUND AND PURPOSE: The Oxfordshire Community Stroke Project (OCSP) classification clinically subdivides cerebral infarction into total anterior circulation (TACS), partial anterior circulation (PACS), posterior circulation (POCS) and lacunar (LACS) syndromes. We compared the OCSP classification in patients presenting within 12 hours of onset of stroke with infarct site and size on computed tomography (CT) brain scan at 5 to 7 days. METHODS: OCSP classification was prospectively assigned by 1 of 3 observers in 43 patients presenting within 12 hours of stroke. CT brain scan was performed on admission to exclude primary intracerebral hemorrhage. Repeat CT brain scan at 5 to 7 days was used to classify recent visible infarction as total anterior circulation infarction (TACI), partial anterior circulation infarction (PACI), lacunar circulation infarction (LACI), or posterior circulation infarction (POCI). For each OCSP subtype, sensitivity and specificity were calculated by using CT classification as a standard. RESULTS: Median (range) interval from onset of stroke symptoms to OCSP classification was 5.0 (1.5, 11.75) hours. Thirty-seven patients had ischemic stroke, with recent visible infarction in 34 (92%). Sensitivity and specificity of each OCSP subtype was TACS (0.80, 0.82), PACS (0.56, 0.79), LACS (0.33, 0.88), and POCS (1.00, 0.97). Overall, 65% of OCSP subtypes assigned were correct when compared to CT classification. CONCLUSIONS: In this small study, we have shown that the OCSP classification within 12 hours of ischemic stroke onset compares with CT classification at 5 to 7 days. Larger studies are required to evaluate the validity of the OCSP classification in the early hours of ischemic stroke in guiding appropriate patient selection for acute stroke therapy and interventions.

Increased survival and deteriorating developmental outcome in 23 to 25 week old gestation infants, 1990-4 compared with 1984-9.

AIMS: To assess whether changes in survival over time in infants of 23 to 25 weeks of gestational age were accompanied by changes in the incidence of disability in childhood during an 11 year period. METHODS: Obstetric and neonatal variables having the strongest association with both survival to discharge from a regional neonatal medical unit and neurodevelopmental disability in 192 infants of 23 to 25 weeks of gestation, born in 1984 to 1994, were studied as a group and in two cohorts (1984 to 1989 n = 96 and 1990 to 1994 n = 96). The data collected included CRIB (clinical risk index for babies) scores and cranial ultrasound scan findings. The children were followed up at outpatient clinics. RESULTS: Between 1984 and 1989 (cohort 1) and 1990 and 1994 (cohort 2) the rate of survival to discharge increased significantly from 27% to 42% and the rate of disability in survivors increased from 38% to 68%; most of this increase was in mild disability. The proportions of survivors with cerebral palsy did not alter significantly (21% vs 18%), but more survivors with blindness due to retinopathy of prematurity (4% vs 18%), myopia (4% vs 15%) and squints (8% vs 13%) contributed to the increased rate of disability. Clinically significant cranial ultrasound findings and a high CRIB score were strongly associated with death. A high CRIB score was most strongly associated with disability. CONCLUSIONS: The rise in disability with improved survival was not due to cerebral palsy; rather the main contributors were blindness due to retinopathy, myopia, and squint. The causes of these disabilities seem to be linked to high CRIB scores. A system of regular and skilled retinal examination and access to facilities for retinal ablation should be in place in all neonatal units which undertake the care of such extremely preterm infants.