INTRODUCTION: Cholesterol crystal embolism complicating arterial catheterization usually presents as a multiorgan disease with renal failure, abdominal problems, and skin manifestations. METHODS: We present a patient with hypertension and generalized arteriosclerosis who presented with muscle weakness, diffuse pain in the extremities, and renal failure 3 weeks after coronary catheterization and angioplasty of the right coronary artery. Muscle weakness progressed during the following months. RESULTS: Nerve conduction studies and nerve biopsy showed severe axonal nerve injury. Biopsy of the kidney revealed the diagnosis of cholesterol crystal embolism. CONCLUSION: The clinical presentation indicates a direct association of cholesterol crystal embolism and polyneuropathy. Although cholesterol crystal embolism represents a rare cause of polyneuropathy, it should be considered in patients with acute onset polyneuropathy and sudden onset multiorgan disease after arterial catheterization.

Hemiplegic migraine (HM) in the setting of Sturge-Weber syndrome (SWS) has been previously described. Here, we report clinical and multimodal imaging data on a 21-year-old man with SWS and HM, who presented during an acute HM attack with a dense left-hemispheric syndrome (expressive aphasia and right sensorimotor hemiplegia), lasting for more than 10days. Repeated EEGs were without evidence of status epilepticus. Consistent with previous findings in prolonged migraine aura, perfusion computed tomography demonstrated left-hemispheric hyperperfusion on day 7. 18F-FDG positron emission tomography (day 7) revealed left-hemispheric hypermetabolism. After 14days, the patient was symptom-free and discharged home. Follow-up after 30days showed normal neurological status. Our observation confirms and reinforces the comborbidity of SWS and HM and shows that prolonged HM attacks are associated with complex changes of both cerebral perfusion and glucose metabolism. A pathophysiological model explaining both the association between SWS/HM and the observed imaging changes is presented.

BACKGROUND: Treatment strategies for acute basilar artery occlusion (BAO) are based on case series and data that have been extrapolated from stroke intervention trials in other cerebrovascular territories, and information on the efficacy of different treatments in unselected patients with BAO is scarce. We therefore assessed outcomes and differences in treatment response after BAO. METHODS: The Basilar Artery International Cooperation Study (BASICS) is a prospective, observational registry of consecutive patients who presented with an acute symptomatic and radiologically confirmed BAO between November 1, 2002, and October 1, 2007. Stroke severity at time of treatment was dichotomised as severe (coma, locked-in state, or tetraplegia) or mild to moderate (any deficit that was less than severe). Outcome was assessed at 1 month. Poor outcome was defined as a modified Rankin scale score of 4 or 5, or death. Patients were divided into three groups according to the treatment they received: antithrombotic treatment only (AT), which comprised antiplatelet drugs or systemic anticoagulation; primary intravenous thrombolysis (IVT), including subsequent intra-arterial thrombolysis; or intra-arterial therapy (IAT), which comprised thrombolysis, mechanical thrombectomy, stenting, or a combination of these approaches. Risk ratios (RR) for treatment effects were adjusted for age, the severity of neurological deficits at the time of treatment, time to treatment, prodromal minor stroke, location of the occlusion, and diabetes. FINDINGS: 619 patients were entered in the registry. 27 patients were excluded from the analyses because they did not receive AT, IVT, or IAT, and all had a poor outcome. Of the 592 patients who were analysed, 183 were treated with only AT, 121 with IVT, and 288 with IAT. Overall, 402 (68%) of the analysed patients had a poor outcome. No statistically significant superiority was found for any treatment strategy. Compared with outcome after AT, patients with a mild-to-moderate deficit (n=245) had about the same risk of poor outcome after IVT (adjusted RR 0.94, 95% CI 0.60-1.45) or after IAT (adjusted RR 1.29, 0.97-1.72) but had a worse outcome after IAT compared with IVT (adjusted RR 1.49, 1.00-2.23). Compared with AT, patients with a severe deficit (n=347) had a lower risk of poor outcome after IVT (adjusted RR 0.88, 0.76-1.01) or IAT (adjusted RR 0.94, 0.86-1.02), whereas outcomes were similar after treatment with IAT or IVT (adjusted RR 1.06, 0.91-1.22). INTERPRETATION: Most patients in the BASICS registry received IAT. Our results do not support unequivocal superiority of IAT over IVT, and the efficacy of IAT versus IVT in patients with an acute BAO needs to be assessed in a randomised controlled trial. FUNDING: Department of Neurology, University Medical Center Utrecht.

BACKGROUND AND PURPOSE: Suboccipital decompressive craniectomy (SDC) is a life-saving intervention for patients with malignant cerebellar infarction. However, long-term outcome has not been systematically analyzed. METHODS: In this monocentric retrospective study we analyzed mortality, long-term functional outcome, and quality of life of all consecutive patients that were treated by SDC for malignant cerebellar infarction in our institution between 1995 and 2006. RESULTS: A total of 57 patients were identified. All of them were treated by bilateral SDC. An external ventricular drainage was inserted in 82%, necrotic tissue was evacuated in 56% of patients. There were no fatal procedural complications. Five patients were lost for follow-up. In the remaining 52 patients, the mean follow-up interval was 4.7 years (1 to 11 years). Within the first 6 months after surgery 16 of 57 patients (28%) had died. At follow-up, 21 of 52 patients (40%) had died and 4 patients (8%) lived with major disability (mRS 4 or 5). Twenty-one patients (40%) lived functionally independent (mRS 0 to 2). The presence of additional brain stem infarction was associated with poor outcome (mRS >/=4; hazard ratio: 9.1; P=0.001). Quality of life in survivors was moderately lower than in healthy controls. CONCLUSIONS: SDC is a safe procedure in patients with malignant cerebellar infarction. Infarct- but not procedure-related early mortality is substantial. Long-term outcome in survivors is acceptable, particularly in the absence of brain stem infarction.

Deep cerebral venous system thrombosis (DCVST) is a rare variety of cerebral vein and sinus thrombosis (CVST), therefore clinical information regarding presentation, course and outcome are limited. In this two-center study including 32 patients, we tried to better define symptoms, neuroradiological findings, course, and outcome in DCVST. All consecutive patients with DCVST admitted to our two institutions over a period of more than 10 years were identified from prospective registries on CVST and stroke patients. Data from the registries were confirmed and complemented by retrospective analysis of patients' charts and neuroradiological imaging. Only patients with an unequivocal diagnosis of DCVST confirmed by MRI and MRA were included. Information on long-term functional outcome (modified Rankin Scale, mRS; ability to return to work) was obtained by telephone interviews performed between 2006 and 2008. The clinical presentation was highly variable with headache (81%) and reduced consciousness (72%) as the most frequent symptoms. In nine patients (28%) thrombosis was confined to the deep venous system (isolated DCVST). In the remaining patients other sinuses and/or cortical veins were additionally affected (non-isolated DCVST). Diagnosis was made within one to 76 days (mean = 10.0 +/- 14.1 days) but was significantly delayed in patients with isolated compared to non-isolated DCVST (19.1 +/- 23.0 vs. 6.3 +/- 6.5 days, P = 0.02). Thalamic edema was the most frequent parenchymal MRI finding present in 69% of patients, bilateral in 47%. D:-dimer levels were normal in 13% of patients. Most patients (75%) stabilized and later improved on intravenous heparin or subcutaneous low molecular weight heparin. Eight (25%) patients deteriorated with progressing coma; six of them received local endovascular therapy but two died. After a mean follow-up of 3.8 years (range 3 months-13 years), 26 patients (81%) were functionally independent (mRS </= 2) including 24 patients (75%) with a mRS </= 1 of whom 23 (96%) returned to their previous job, activity or education. No patients were severely disabled (mRS 4-5). Extension of thrombosis beyond the deep venous system had no effect on outcome. Due to its variable clinical presentation the diagnosis of DCVST is often difficult and heparin treatment therefore established with substantial delay. While most patients stabilize and have a good recovery, progressing coma associated with poor outcome is seen in a subset of patients who may thus require other treatment options, such as endovascular therapy.

Cortical ischemic stroke affecting the precentral "hand knob" area is a rare but well known stroke entity. To date, little is known about the underlying stroke mechanisms and the prognosis. Twenty-nine patients admitted to our service between 2003 and 2007 were included in the study on the basis of an acute ischemic infarct of the cortical "hand knob" area confirmed by diffusion-weighted magnetic resonance imaging with contralateral hand paresis. For all patients clinical, epidemiological as well as imaging data at the time point of admission were analysed retrospectively and follow-up data on all patients was obtained. The majority (n = 21/72%) had an isolated infarct of the cortical "hand knob" area. In 23 (79%) patients it was a first ever stroke. Ten patients (34%) had ipsilateral extracranial stenosis of the internal carotid artery (ICA), whereas potential cardiac embolic sources were less frequent (n = 4/14%). No patient exhibited ipsilateral MCA stenosis. All but two patients (93%) had marked atherosclerotic alterations of the ICA. Hypertension was the most prevalent vascular risk factor (n = 23/79%). At follow-up (mean 25.0 months, range 0.4-47.4 months) no patient had died and only one (3%) experienced a recurrent stroke. The majority of patients (79%) reported improvement of hand paresis, 17 (59%) were asymptomatic (modified Rankin score = 0). Only one patient was significantly disabled due to a recurrent stroke. In conclusion, ischemic infarcts affecting the cortical "hand knob" area are frequently associated with atherosclerotic changes of the carotid artery, suggesting an arterio-arterial thrombembolic stroke mechanism. It mostly reflects first ever ischemic stroke, and follow-up data suggest a rather benign course.

BACKGROUND AND PURPOSE: With its highly variable clinical presentation, the diagnosis of cerebral venous sinus thrombosis (SVT), and especially of deep venous thrombosis (DVT), as rare but important causes of stroke is challenging. Because noncontrast cranial CT (NCCT) is still the imaging technique of choice in most emergency departments, we aimed to investigate its value in the diagnosis of SVT and DVT. MATERIALS AND METHODS: Screening our patient data base, we identified 8 patients with DVT and 25 patients with SVT. We also included a control group of 36 patients who had presented with clinical signs of DVT or SVT but in whom thrombosis was subsequently excluded. MR imaging, multidetector row CT angiography (MDCTA), and/or digital subtraction angiography (DSA) were used as the reference standard. Three independent readers assessed the NCCTs for the presence of direct and indirect signs of DVT or SVT. Direct signs included the presence of hyperattenuated sinuses (ie, cord sign) or veins (ie, attenuated vein sign), whereas parenchymal edema and hemorrhage were indirect signs. RESULTS: The sensitivity and specificity of the attenuated vein sign for the diagnosis of DVT were 100%, and 99.4%, respectively, whereas the sensitivity and specificity of the cord sign for SVT were 64.6% and 97.2%, respectively. The sensitivity and specificity values of NCCT were 93.7% and 98% for intracerebral edema and 94.8% and 98.7% for intracerebral hemorrhages, respectively. CONCLUSIONS: Although NCCT is insufficient to exclude a SVT, its value in the emergency diagnosis of DVT seems to be very high.

Besides being a treatment option for narcolepsy, gamma-hydroxybutyrate is used as an adjuvant during anesthesia in Europe. In addition, it is illegally used as a recreational drug. Fixed and dilated, asymmetric pupils developed in 2 patients during continuous therapy with intravenous gamma-hydroxybutyrate, which was added to the long-term anesthetics fentanyl and midazolam. Cerebral herniation as an alternative cause for the pupillary changes was ruled out by using continuous intracranial pressure monitoring and computed tomography. In both patients, the pupillary abnormalities resolved after discontinuation of gamma-hydroxybutyrate. Thus, fixed and dilated pupils that are asymmetric seem to be an important side effect of gamma-hydroxybutyrate therapy that may mimic cerebral herniation in deeply anesthetized patients.