Rare clinical findings in a patient with sporadic hemiplegic migraine: FDG-PET provides diminished brain metabolism at 10-year follow-up.

BACKGROUND: Sporadic hemiplegic migraine (SHM) is defined as migraine attacks associated with some degree of motor weakness during the aura phase and where no first-degree relative has identical attacks. SHM has a wide inter- and intraindividual clinical spectrum and, in case of prolonged aura symptoms and disturbed consciousness, can mimic several other acute neurological diseases. CASE: In 1996, during his wedding night, a 28-year-old man developed left face, arm and leg weakness, nausea and a throbbing headache. Neurological examination on presentation revealed stupor, fever, meningism and left hemiplegia. There were no abnormalities on emergency magnetic resonance. Lumbar puncture showed mild lymphocytic pleocytosis and slightly elevated protein. He received symptomatic treatment. Subsequent genetic analysis revealed the T666M mutation in the CACNA1A gene of chromosome 19. He was diagnosed with SHM. In 2005, at the end of another episode of hemiplegic migraine (HM), he for the first time developed an episode of paranoid psychosis with anxiety and visual hallucinations. The psychiatric symptoms resolved within a week. DISCUSSION: All perfusion SPECT and transcranial Doppler studies performed in the first days of HM attacks were consistent with hyperemia of the hemisphere contralateral to the neurological signs. FDG-PET/CT in January 2013 revealed a diffusely reduced glucose metabolism of the supratentorial cortex and marked asymmetric hypometabolism of the left cerebellum. The finding of progressive cortical metabolic dysfunction over years appears as a new finding. Glucose hypometabolism may indicate primary neuronal dysfunction as the cause of the prolonged deficits.

[A biomechanical analysis of cyclical hand motor function: a pilot study in different Parkinsonian syndromes]. / Biomechanische Analyse zyklischer Handfunktionen : Eine Pilotstudie bei unterschiedlichen Parkinson-Syndromen.

INTRODUCTION: Clinical assessment of hand bradykinesia in Parkinson's disease (PD) focuses mainly on the frequency, amplitude and rhythm of movements, thereby subjectively evaluating the correct performance of hand movements. The aim of the study was to quantify hand bradykinesia with kinematic data in different Parkinsonian syndromes. PATIENTS AND METHODS: This retrospective study compared patients with idiopathic PD (IPD, n = 18), atypical Parkinson's syndrome (APS, n = 17), secondary Parkinson's syndrome (SPS, n = 18) and healthy controls (C, n = 18). All patients were receiving the best medical treatment. Hand movements were recorded using an ultrasound-system (Zebris®, Isny, Germany). Subjects were asked to perform pronation/supination of the forearm (diadochokinesis), flexion/extension of the hand (hand tapping) and tapping of the index finger. Mean amplitude, mean frequency and mean variability of movements were determined. RESULTS: APS patients had significant complex hand movement disability with reduced amplitude and frequency in combination with increased motion variability in all movement tasks. The key disturbance in the IPD group concerned the rhythm in hand tapping and index finger tapping in combination with moderately reduced velocity and range of motion in all conditions. The cyclical hand movement characteristics in SPS patients showed movement slowness with normal amplitude and variability in all motor conditions. CONCLUSION: Our results suggest that computerized quantitative analysis of cyclical hand movements can characterize and identify different representations of hand bradykinesia in different Parkinsonian disorders and hence may help clinicians to accurately assess therapeutic targets and outcome of interventions.

Thrombolytic therapy for acute stroke in Austria: data from the Safe Implementation of Thrombolysis in Stroke (SITS) register.

BACKGROUND: We aimed at determining the safety and efficacy of IV alteplase in Austrian versus non-Austrian centres as documented in the Internet-based registers Safe Implementation of Thrombolysis for Stroke - MOnitoring STudy (SITS-MOST) and - International Stroke Thrombolysis Register (SITS-ISTR). METHODS: We analysed patient data entered in the registers SITS-MOST and SITS-ISTR in the period December 2002 to 15 November 2007. RESULTS: Compared to the non-Austrian cohort (n=15153), the Austrian cohort (n=896) was slightly older [median, interquartile range (IQR): 70, 60-77 years vs. 69, 60-76 years, P=0.05] and included more women (44.6% vs. 41.0%, P=0.03). Austrian patients had a significantly shorter stroke onset-to-treatment time (OTT; median, IQR: 135, 105-160 min vs. 145, 115-170 min, P<0.0005). Symptomatic intracerebral haemorrhages were observed in 1.6% of Austrian and 1.7% of non-Austrian patients (P=0.82). At 3 months, 50.8% of Austrian and 53.0% of non-Austrian patients were independent (P=0.23), but death was less frequent in Austrian patients (12.1% vs. 14.9%, P=0.03). Multivariate analyses adjusted for demographic and baseline characteristics confirmed lower mortality at 3 months in the Austrian cohort (odds ratio 0.81, 95% confidence intervals 0.71-0.92, P=0.001). Longer OTT was associated with increased mortality at 3 months, with a hazard ratio of 1.02 (95% CI 1.01-1.03; P=0.005) for each 10-min increase in OTT. CONCLUSIONS: The implementation of intravenous alteplase for acute stroke has been safe and efficacious in Austrian centres. OTT and mortality were significantly lower in Austrian patients compared to non-Austrian SITS centres.

Blood-brain barrier permeability is increased in normal-appearing white matter in patients with lacunar stroke and leucoaraiosis.

BACKGROUND AND AIM: The pathogenesis of cerebral small-vessel disease (SVD) is incompletely understood. Endothelial dysfunction has been implicated and may result in increased blood-brain barrier (BBB) permeability with leakage of blood constituents into the vessel wall and white matter. We used contrast-enhanced MRI to determine whether there was any evidence for BBB permeability in the white matter of patients with SVD, and whether this was present not only in areas of leucoaraiosis (white-matter lesions) but also in normal-appearing white matter (NAWM). METHODS: Subjects underwent T1 volumetric MRI before and after bolus injection of contrast. Scanning was continued for 30 min postinjection to determine the contrast-enhancement time course. The mean signal intensity change was plotted against time to calculate the area under the curve values, a parameter related to BBB permeability. Automated brain segmentation and regions of interest analysis were performed to determine 'permeability' in different brain compartments. RESULTS: Compared with controls (n=15), the SVD patient group (n=24) had signal changes consistent with increased BBB permeability in NAWM (p=0.033). Multivariate regression analyses identified leucoaraiosis grade as an independent predictor of these permeability related signal changes in NAWM after adjustment for age, gender, weight, brain volume, area under the curve in the internal carotid arteries and cardiovascular risk factors. CONCLUSION: This study provides evidence for increased BBB permeability in SVD, and this is particularly seen in SVD with leucoaraiosis. Its presence in NAWM would be consistent with it playing a causal role in disease pathophysiology.

High cardiovascular event rates in patients with asymptomatic carotid stenosis: the REACH Registry.

BACKGROUND AND PURPOSE: Data on current cardiovascular event rates in patients with asymptomatic carotid artery stenosis (ACAS) are sparse. We compared the 1-year outcomes of patients with ACAS > or =70% versus patients without ACAS in an international, prospective cohort of outpatients with or at risk of atherothrombosis. METHODS: The Reduction of Atherothrombosis for Continued Health Registry enrolled patients with either > or =3 atherothrombotic risk factors or established atherothrombotic disease. We investigated the 1-year follow-up data of patients for whom physicians reported presence/absence of ACAS at the time of inclusion. RESULTS: Compared with patients without ACAS (n = 30 329), patients with ACAS (n = 3164) had higher age- and sex-adjusted 1-year rates of transient ischaemic attack (3.51% vs. 1.61%, P < 0.0001), non-fatal stroke (2.65% vs. 1.75%, P = 0.0009), fatal stroke (0.49% vs. 0.26%, P = 0.04), cardiovascular death (2.29% vs. 1.52%, P = 0.002), the composite end-point cardiovascular death/myocardial infarction/stroke (6.03% vs. 4.29%, P < 0.0001) and bleeding events (1.41% vs. 0.81%, P = 0.002). In patients with ACAS, Cox regression analyses identified history of cerebrovascular ischaemic events as most important predictor of future stroke (HR 3.21, 95% CI 1.82-5.65, P < 0.0001). CONCLUSION: Asymptomatic carotid artery stenosis was associated with high 1-year rates of cardiovascular and cerebrovascular ischaemic events. Stroke was powerfully predicted by prior cerebrovascular ischaemic events.

Postprocedural high-density lipoprotein cholesterol predicts carotid stent patency at 1 year.

The durability of carotid artery stenting (CAS) is affected by the occurrence of myointimal proliferation and in-stent restenosis (ISR). We aimed to identify clinical, angiographic, and laboratory predictors of ISR, paying special attention to postprocedural metabolic factors. A total of 102 consecutive patients with successful CAS for > or =70% atherosclerotic internal carotid artery stenosis were followed up with neurological assessment and duplex sonography 1 day, 1 month, and 1 year after CAS. Lipid profile and hemoglobin A(1c) were tested at the 1-month follow-up visit. Ten (10%) patients had ISR > or =50% after 1 year. Compared with patients without ISR (n = 92), patients with ISR were more often current smokers (33% vs. 70%, P = 0.034) and had significantly lower 1-month high-density lipoprotein (HDL) cholesterol: median (range) 47 (24-95) mg/dl vs. 39.5 (25-50) mg/dl, P = 0.031. Multivariate logistic regression analyses identified 1-month HDL cholesterol >45 mg/dl as the only independent predictor of carotid stent patency at 1 year (P = 0.033, OR = 0.09, 95% CI 0.01-0.83). Postprocedural HDL cholesterol levels predict carotid stent patency at 1 year. With the possibility of elevation of HDL cholesterol by lifestyle changes and medication, this finding may have implications for the future management of patients undergoing CAS.

Timing of stenting of symptomatic carotid stenosis is predictive of 30-day outcome.

For patients with symptomatic carotid stenosis, benefit from carotid artery stenting (CAS) highly depends on the 30-day stroke and death rates. Identification of predictors of unfavourable outcome would help guide the patient selection. We analysed the influence of clinical and angiographic factors on the 30-day outcomes of 77 consecutive patients who underwent CAS for > or = 60% symptomatic carotid stenosis within 180 days of transient ischaemic attack or moderate stroke (modified Rankin Scale score < or = 3). The 30-day composite end-point for stroke (7.8%) and death of any cause (1.3%) was 9.1%. Patients with complicated CAS were older than patients with uncomplicated CAS (mean age 75.1 +/- 8.2 vs. 65.9 +/- 9.5 years, P = 0.015) and underwent stenting significantly earlier after the qualifying event: median delay 1.5 weeks (range: 0.2-3.0) vs. 3.2 weeks (range: 0.5-26), P = 0.004. In multivariate logistic regression analyses, age [odds ratio (OR) = 1.148; 95% confidence interval (CI): 1.011-1.304 and P = 0.033] and delay of treatment < 2 weeks (OR = 22.399; 95% CI: 2.245-223.445 and P = 0.008) remained the only variables significantly associated with 30-day outcome. CAS carries a considerable risk in old patients and when performed early (< 2 weeks) after the qualifying event. Future reports should address the timing of CAS.

Ultrasonic plaque echolucency and emboli signals predict stroke in asymptomatic carotid stenosis.

OBJECTIVES: Better methods are required to identify patients with asymptomatic carotid stenosis (ACS) at risk of future stroke. Two potential markers of high risk are echolucent plaque morphology on carotid ultrasound and embolic signals (ES) in the ipsilateral middle cerebral artery on transcranial Doppler ultrasound (TCD). We explored the predictive value of a score based on these 2 measures in the prospective, observational, international multicenter Asymptomatic Carotid Emboli Study. METHODS: A total of 435 recruited subjects with ACS ≥70% had baseline ultrasound images and TCD data available. Subjects were prospectively followed up for 2 years. RESULTS: A total of 164 (37.7%) plaques were graded as echolucent. Plaque echolucency at baseline was associated with an increased risk of ipsilateral stroke alone (hazard ratio [HR] 6.43, 95% confidence interval [CI] 1.36-30.44, p = 0.019). A combined variable of plaque echolucency and ES positivity at baseline was associated with a markedly increased risk of ipsilateral stroke alone (HR 10.61, 95% CI 2.98-37.82, p = 0.0003). This association remained significant after controlling for risk factors, degree of carotid stenosis, and antiplatelet medication. CONCLUSIONS: Plaque morphology assessed using a simple, and clinically applicable, visual rating scale predicts ipsilateral stroke risk in ACS. The combination of ES detection and plaque morphology allows a greater prediction than either measure alone and identifies a high-risk group with an annual stroke risk of 8%, and a low-risk group with a risk of <1% per annum. This risk stratification may prove useful in the selection of patients with ACS for endarterectomy.