Non-convulsive status epilepticus associated with Whipple's disease.

A 69-year-old woman developed non-convulsive status epilepticus during inpatient investigation for abdominal pain. Initial detailed investigations did not identify the cause of seizures, but a jejunal biopsy and PCR testing in various fluids led to the diagnosis of Whipple's disease with neurological involvement. The seizures were controlled but she subsequently had moderate cognitive impairment. Whipple's disease is an important diagnosis, being treatable with antibiotics. Testing for Whipple's disease is not part of the recommended workup in for status epilepticus, but this case highlights the importance of considering this condition.

Morphological active contour model for automatic brain tumor extraction from multimodal magnetic resonance images.

BACKGROUND: Brain tumor extraction from magnetic resonance (MR) images is challenging due to variations in the location, shape, size and intensity of tumors. Manual delineation of brain tumors from MR images is time-consuming and prone to human errors. METHOD: In this paper, we present a method for automatic tumor extraction from multimodal MR images. Brain tumors are first detected using k-means clustering. A morphological region-based active contour model is then used for tumor extraction using an initial contour defined based on the boundary of the detected brain tumor regions. The contour evolution for tumor extraction was performed using successive application of morphological operators. In our model, a Gaussian distribution was used to model local image intensities. The spatial correlation between neighboring voxels was also modeled using Markov random field. RESULTS: The proposed method was evaluated on BraTS 2013 dataset including patients with high-grade and low-grade tumors. In comparison with other active contour based methods, the proposed method yielded better performance on tumor segmentation with mean Dice similarity coefficients of 0.9179 ( ±â€¯0.025) and 0.8910 ( ±â€¯0.042) obtained on high-grade and low-grade tumors, respectively. CONCLUSION: The proposed method achieved higher accuracies for brain tumor extraction in comparison to other contour-based methods.

Investigating the association between cancer and the risk of dementia: Results from the Memento cohort.

INTRODUCTION: Studies on the association of cancer and risk of dementia are inconclusive due to result heterogeneity and concerns of survivor bias and unmeasured confounding. METHODS: This study uses data from the Memento cohort, a French multicenter cohort following persons with either mild or isolated cognitive complaints for a median of 5 years. Illness-death models (IDMs) were used to estimate transition-specific hazard ratios (HRs) and 95% confidence intervals (CIs) for incident cancer in relation to dementia from time since study entry. RESULTS: The analytical sample (N = 2258) excluded 65 individuals without follow-up information. At the end of follow-up, 286 individuals were diagnosed with dementia, 166 with incident cancer, and 95 died. Incident cancer was associated with a reduced risk of dementia (HR = 0.58, 95% CI = 0.35-0.97), with a corresponding E-value of 2.84 (lower CI = 1.21). DISCUSSION: This study supports a protective relationship between incident cancer and dementia, encouraging further investigations to understand potential underlying mechanisms.

A pain like no other: From cluster to headache to Eagle's syndrome, a case report.

BACKGROUND: Eagle's syndrome, also called stylohyoid complex syndrome, is a rare syndrome pathology characterised by latero-cervical pain radiating to the face, linked to an abnormal enlargement of the styloid or calcification of the stylo-hyoid ligament. CASE: We report here the case of a young man of 25 suffering from cluster headache resistant to treatments, revealing Eagle's syndrome. CONCLUSION: Only surgery led to a real improvement of his condition.

Sensitivity of the Montreal Cognitive Assessment in screening for cognitive impairment in patients with newly diagnosed high-grade glioma.

INTRODUCTION: Cognitive impairment is frequent in patients with high-grade glioma and requires cognitive follow-up. Cognitive screening tools such as the Montreal Cognitive Assessment (MoCA) have been used to assess cognition in these patients. Here we assessed the sensitivity of the MoCA in screening for cognitive impairment in a cohort of 156 patients with newly-diagnosed high-grade glioma, after surgery and before radiochemotherapy. METHODS: We assessed cognitive performance with the MoCA and a neuropsychological battery. Cognitive scores were analyzed in terms of a previously validated framework designed to control false positives and data for 1003 control participants from the GRECOGVASC study. After comparison of performance on the tests, we used stepwise logistic regression to produce a cognitive summary score from the neuropsychological battery. Then we analyzed sensitivity and specificity of the MoCA with receiver operator characteristic (ROC) curve analysis. RESULTS: Both raw and adjusted MoCA scores showed only moderate sensitivity. The area under the ROC curve was 0.759 (95% CI 0.703-0.815) for the raw score and 0.788 (95% CI 0.734-0.842) for the adjusted score. Optimal discrimination was obtained with a raw score ≤ 25 (sensitivity: 0.526; specificity: 0.832; positive predictive value: 0.2; negative predictive value: 0.96) and an adjusted score - 0.603 (sensitivity: 0.716; specificity: 0.768; positive predictive value: 0.24; negative predictive value: 0.96). CONCLUSION: The moderate sensitivity of MoCA indicates that it is not a suitable screening tool for detecting cognitive impairment in patients with newly-diagnosed high-grade glioma.

Profile of and risk factors for poststroke cognitive impairment in diverse ethnoregional groups.

OBJECTIVE: To address the variability in prevalence estimates and inconsistencies in potential risk factors for poststroke cognitive impairment (PSCI) using a standardized approach and individual participant data (IPD) from international cohorts in the Stroke and Cognition Consortium (STROKOG) consortium. METHODS: We harmonized data from 13 studies based in 8 countries. Neuropsychological test scores 2 to 6 months after stroke or TIA and appropriate normative data were used to calculate standardized cognitive domain scores. Domain-specific impairment was based on percentile cutoffs from normative groups, and associations between domain scores and risk factors were examined with 1-stage IPD meta-analysis. RESULTS: In a combined sample of 3,146 participants admitted to hospital for stroke (97%) or TIA (3%), 44% were impaired in global cognition and 30% to 35% were impaired in individual domains 2 to 6 months after the index event. Diabetes mellitus and a history of stroke were strongly associated with poorer cognitive function after covariate adjustments; hypertension, smoking, and atrial fibrillation had weaker domain-specific associations. While there were no significant differences in domain impairment among ethnoracial groups, some interethnic differences were found in the effects of risk factors on cognition. CONCLUSIONS: This study confirms the high prevalence of PSCI in diverse populations, highlights common risk factors, in particular diabetes mellitus, and points to ethnoracial differences that warrant attention in the development of prevention strategies.

Asleep Robot-Assisted Surgery for the Implantation of Subthalamic Electrodes Provides the Same Clinical Improvement and Therapeutic Window as Awake Surgery.

OBJECTIVE: To study the impact of not performing awake clinical evaluation during the robot-assisted implantation of subthalamic nucleus deep brain stimulation (STN-DBS) electrodes on the stimulation parameters and clinical outcomes in patients with Parkinson disease (PD). METHODS: A total of 23 patients with PD underwent robot-assisted surgery for the bilateral implantation of STN-DBS electrodes. Thirteen patients received general anesthesia (GA) and a limited intraoperative evaluation (side effects only), and the other 10 patients received local anesthesia (LA) and a full evaluation. The primary endpoint was the therapeutic window (TW), defined as the difference between the mean voltage threshold for motor improvement and the mean voltage threshold for side effects in the active contacts at 12 months after surgery. Motor scores were measured as well. RESULTS: The TW was similar in the LA and GA groups, with mean ± standard deviation values of 2.06 ± 0.53 V and 2.28 ± 0.99 V, respectively (P = 0.32). In the short term, the Unified Parkinson Disease Rating Scale (UPDRS) III score in the "off-drug, on-stim" condition fell to a similar extent in the LA and GA groups (by 40.3% and 49%, respectively; P = 0.336), as did the UPDRS III score in the "on-stim, on-drug" condition (by 57% and 70.7%, respectively; P = 0.36). CONCLUSIONS: Asleep, robot-assisted implantation of STN-DBS electrodes (with accurate identification of the STN and positioning of the DBS lead) produced the same motor results and TW as awake surgery.

Prevalence and profile of cognitive impairment in adult glioma: a sensitivity analysis.

Cognitive impairment has been reported in 27-83 % of adults with World Health Organization (WHO) grade I-III glioma. However, the few studies in this field used different methods for cognitive assessment. The objective of the present study was to establish the prevalence of cognitive impairment in patients with WHO grade I-III primary brain tumors and determine the effect sizes of a comprehensive battery of tests. This study used a comprehensive neuropsychological battery to examine 27 patients. To control for false positives, prevalence was estimated from the overall neuropsychological score. Size effects were determined using Cohen's d. Cognitive impairment was observed in 51.9 % (95 % CI 33-70.7 %) of the patients; the impairment affected action speed (38.5 %), cognitive (33 %) and behavioral (21.7 %) executive functions, oral expression (29.6 %), episodic memory (29.6 %) and visuoconstructive abilities (19.2 %). The largest effect sizes (d ≥ 1.645) were observed for the Digit Symbol Substitution test, global hypoactivity, free recall, Stroop time, the Boston Naming test (BNT), the Trail Making test B (TMTB), verbal fluency and the Rey-Osterrieth Complex Figure Test. Four of these scores (global hypoactivity, the Digit Symbol Substitution test, the TMTB perseveration, and the BNT) were combined to make a shortened battery (AUC 0.872; 95 % CI 0.795-0.949). The overall neuropsychological score was the sole factor associated with the functional outcome. Our results suggest that about half of survivors with a grade I-III primary brain tumor suffer from cognitive impairment. Tests with a large effect size should be included in future large-scale studies.

Association between caffeine intake and age at onset in Huntington's disease.

Habitual consumption of caffeine, a non-selective adenosine receptor (AR) antagonist, has been suggested to be beneficial in Parkinson's and Alzheimer's diseases. Experimental evidence support that ARs play a role in Huntington's disease (HD) raising the hypothesis that caffeine may be a life-style modifier in HD. To determine a possible relationship between caffeine consumption and age at onset (AAO) in HD, we retrospectively assessed caffeine consumption in 80 HD patients using a dietary survey and determined relationship with AAO. Following adjustment for gender, smoking status and CAG repeat length, caffeine consumption greater than 190mg/day was significantly associated with an earlier AAO. These data support an association between habitual caffeine intake and AAO in HD patients, but further studies are warranted to understand the link between these variables.

Functional consequences of a section of the anterior part of the body of the corpus callosum: evidence from an interhemispheric transcallosal approach.

The aim of this study was to determine the neuropsychological consequences of a middle interhemispheric approach for the removal of tumors of the third or lateral ventricles. A retrospective analysis of eight callosotomized patients for ventricular tumors (three males/five females; mean age: 48.7 ± 11.2 years; education level: 11.9 ± 2.9 years) and eight healthy subjects was performed. An extensive neuropsychological test battery was used to evaluate global intellectual efficiency, memory capacities, executive functions, and interhemispheric transfer of a procedural learning task (serial reaction time task/SRTT). Neuropsychological results showed that: (1) five of eight patients operated through a middle transcallosal approach had disturbances of verbal or visual memory; (2) three of eight patients displayed a dysexecutive cognitive syndrome(two of eight of whom presenting with a deficit of verbal fluency); (3) two of eight patients presented a dysexecutive behavior syndrome; and (4) with regard to the SRTT, although all participants learned the task, in contrast to controls, the callosotomized patients showed an increase in reaction times and an absence of interhemispheric transfer of learning from one hand to the other. The transcallosal approach transects a large number of callosal fibers. This damage accounts for the deficits of memory, the dysexecutive cognitive and behavioral syndrome, and disturbances in interhemispheric transfer of learning.

Presence of intracranial artery calcification is associated with mortality and vascular events in patients with ischemic stroke after hospital discharge: a cohort study.

BACKGROUND AND PURPOSE: Although intracranial artery calcification (IAC) has been reported to be a risk factor for ischemic stroke, the prognostic implications of IAC in stroke outcome are unknown. The purpose of this study was to determine the association between IAC and risk of vascular events and death in patients with stroke after hospital discharge. METHODS: All patients with ischemic stroke over a 1-year period were included (n=302). IAC, assessed by multidetector CT, was defined as hyperdense foci (peak density>130 Hounsfield units) and assessed in the 7 major cerebral arteries. The IAC scores ranged from 0 (no calcification) to 7. Follow-up information on major clinical events (including fatal or nonfatal ischemic stroke, cardiac and peripheral artery events, and all-cause death) was obtained by means of a structured phone interview. RESULTS: IAC was present in 260 patients (83%). With a mean follow-up of 773±223 days, 88 major clinical events occurred in 67 patients (22%): 45 new ischemic vascular events (ischemic stroke: n=22; cardiac event: n=15; peripheral artery event: n=8) and 43 deaths from any cause. Patients with the highest IAC scores had significantly higher rates of death and vascular events than those with the lowest IAC scores (log rank test, P=0.029). In the Cox proportional hazards regression model, the IAC score was significantly associated with major clinical events (hazard ratio, 1.34; 95% CI, 1.11-1.61; P=0.002). CONCLUSIONS: In patients with ischemic stroke, IAC detection may constitute a simple marker of a high risk of future major clinical events.

Reduced CSF turnover and decreased ventricular Aß42 levels are related.

BACKGROUND: The appearance of Aß42 peptide deposits is admitted to be a key event in the pathogenesis of Alzheimer's disease, although amyloid deposits also occur in aged non-demented subjects. Aß42 is a degradation product of the amyloid protein precursor (APP). It can be catabolized by several enzymes, reabsorbed by capillaries or cleared into cerebrospinal fluid (CSF). The possible involvement of a decrease in CSF turnover in A4ß2 deposit formation is up to now poorly known. We therefore investigated a possible relationship between a reduced CSF turnover and the CSF levels of the A4ß2 peptide.To this aim, CSF of 31 patients with decreased CSF turnover were studied. These patients presented chronic hydrocephalus communicating or obstructive, which required surgery (ventriculostomy or ventriculo-peritoneal shunt). Nine subjects had idiopathic normal pressure hydrocephalus (iNPH), and the other 22 chronic hydrocephalus from other origins (oCH).The Aß42 peptide concentration was measured by an ELISA test in 31 ventricular CSF samples and in 5 lumbar CSF samples from patients with communicating hydrocephalus. RESULTS: The 5 patients with lumbar CSF analysis had similar levels of lumbar and ventricular Aß42. A significant reduction in Aß42 ventricular levels was observed in 24 / 31 patients with hydrocephalus. The values were lower than 300 pg/ml in 5 out of 9 subjects with iNPH, and in 15 out of 22 subjects with oCH. CONCLUSION: The decrease of CSF Aß42 seems to occur independently of the surgical hydrocephalus aetiology. This suggests that a CSF reduced turnover may play an important role in the decrease of CSF Aß42 concentration.

Aspirin non-responder status and early neurological deterioration: a prospective study.

OBJECTIVES: In acute ischemic stroke, early neurological deterioration (END) has a severe impact on patient outcome. We tested the hypothesis that initial biological aspirin non-responder status (ANRS) helps predict END. METHODS: A total of 85 patients with acute ischemic stroke on 160mg aspirin daily were prospectively included. END was defined as an increase in the National Institutes of Health Stroke Scale (NIHSS) ≥4 points in the first 72h after admission. Platelet responsiveness to aspirin was assessed using the PFA-100 system, and ANRS was defined as a collagen/epinephrine closure time <165ms. RESULTS: END was observed in 10 patients (11.8%). The presumed reasons for END were progressive stroke (40%), recurrent cerebral ischemia (30%), malignant middle cerebral artery infarction (20%) and secondary acute hydrocephalus (10%). Patients with END had a non-significant worse neurological status on the NIHSS at hospital admission (8.4 vs. 4.2; p=0.15). Initial impaired consciousness (30% vs. 3%), visual disturbance (60% vs. 23%) and ANRS (60% vs. 20%) were observed more frequently in patients with END. In multivariate analysis, impaired consciousness (OR: 17.3; 95% CI: 2.0-149.5; p=0.01) and ANRS (OR: 6.4; 95% CI: 1.4-29.6; p=0.017) were found to be independently associated with END. CONCLUSION: ANRS is common in acute ischemic stroke patients and is predictive of END. The clinical significance of these findings requires further evaluation in larger longitudinal studies.

Relation between intracranial artery calcifications and aortic atherosclerosis in ischemic stroke patients.

We previously demonstrated a strong relation between carotid atherosclerosis (defined as carotid artery stenosis > or =50%) and intracranial artery calcification (IAC) in ischemic stroke patients. The purpose of this study was to evaluate the relation between aortic atherosclerosis and IAC. Four hundred fifty-four patients with ischemic stroke were included. Complex aortic plaques (CAP) were assessed by transesophageal echocardiography (TEE) and defined as plaques > or =4 mm thick or with mobile components in the proximal aorta. IAC were assessed in the seven major cerebral arteries and a semiquantitative score system was applied, ranging from 0 (no calcification) to 7. Forty-two patients (9.3%) had CAP. Patients with CAP were older compared with patients without CAP (73.6 vs. 63.6 years, p < 0.001), had more vascular risk factors, more significant carotid artery atherosclerosis (p < 0.001), more chronic kidney disease (p < 0.001), and a higher IAC score (3.0 vs. 1.8; p < 0.001). Stepwise logistic regression selected the following independent factors for CAP: previous stroke or TIA (OR 3.3; 95%CI 1.5-7.0; p = 0.002), carotid artery stenosis > or =50% (OR 3.7; 95%CI 1.7-8.0; p = 0.001), chronic kidney disease (OR 3.8; 95%CI 1.9-7.8; p < 0.001), and IAC score (OR 1.5; 95%CI 1.2-1.9; p = 0.002). IAC was present in 100% of patients with CAP. Moreover, IAC had a high sensitivity (100%) and negative predictive value (100%) for the presence of CAP. In ischemic stroke patients, the absence of IAC strongly points to the lack of CAP. However, these results warrant confirmation in prospective studies before concluding the non-utility of the use of TEE to exclude CAP as a potential source of cerebral embolism in patients without IAC.

Troponin levels help predict new-onset atrial fibrillation in ischaemic stroke patients: a retrospective study.

BACKGROUND: Elevated levels of cardiac troponin I (cTnI) have been reported in acute stroke and atrial fibrillation (AF). We tested a hypothesis in which the result of an initial cTnI assay helps predict new-onset AF (NAF) early in the course of ischaemic stroke in patients with sinus rhythm on admission. METHODS: This retrospective study included all patients admitted to our unit for acute ischaemic stroke between January 2006 and December 2007 (n = 402). NAF was defined as AF discovered during cardiac monitoring in patients with sinus rhythm on admission. Elevated cTnI was defined as a value > or =0.03 microg/l. RESULTS: 27 (6.7%) patients had NAF. In a univariate analysis, age (70.8 in patients with NAF vs. 64.6 years in patients without NAF, respectively; p = 0.024), smoking status (3.5 vs. 25.3%; p = 0.009), total anterior circulation infarcts (18.5 vs. 5.9%; p = 0.03) and elevated cTnI levels (63.0 vs. 29.9%; p = 0.001) were associated with NAF. Stepwise logistic regression identified elevated cTnI levels (OR 4.1; 95% CI 1.81-9.33; p = 0.001)and active smoking (OR 0.11; 95% CI 0.01-0.82; p = 0.03) as independent factors for NAF. CONCLUSION: Moderately elevated troponin levels in acute ischaemic stroke are independently associated with NAF.

Microsurgical anatomy of the temporal stem: clinical relevance and correlations with diffusion tensor imaging fiber tracking.

OBJECT: The authors used a fiber dissection technique to describe the temporal stem and explain the tendency of malignant tumors to spread within both the frontal and temporal lobes. The authors focused on the morphological characteristics and course of various fasciculi of the temporal stem, including the uncinate fascicle, occipitofrontal fascicle, anterior commissure, loop of the optic radiations (Meyer loop), and the ansa peduncularis. METHODS: Eight previously frozen, formalin-fixed human brains were dissected under an operating microscope using the fiber dissection technique described by Klingler. Lateral, inferior, and medial approaches were made. Cross-sectional 3D MR images obtained in 10 patients without brain lesions demonstrated that fibers of the temporal stem, which were intermingled together in various ways, curved laterally within the basal forebrain. Various pathological entities affecting the temporal stem are described and discussed. RESULTS: The uncinate fascicle has 3 portions: a ventral extension, an intermediary segment called the isthmus, and a dorsal segment. The inferior occipitofrontal fasciculus is a layer of more superficial white matter that appeared to be superior to the uncinate fasciculus. A short ventral portion of the radiations of the corpus callosum was sometimes noted to run ventrally to enter the temporal stem and to reach both temporal lobes. CONCLUSIONS: To the authors' knowledge, a detailed anatomy of the temporal stem has not been previously described in the literature. The unique anatomy of the temporal stem provides a route for tumor spread between the frontal and temporal lobes.

High prevalence of intracranial artery calcification in stroke patients with CKD: a retrospective study.

BACKGROUND AND OBJECTIVES: Intracranial artery calcification (IAC) is frequently observed on brain computed tomography (CT) scans in stroke patients. This retrospective study was designed to determine the prevalence, risk factors, and clinical relevance of IAC in a cohort of patients with ischemic stroke. DESIGN, SETTING, PARTICIPANTS, & MEASUREMENTS: We included all eligible patients admitted to Amiens University Hospital for acute ischemic stroke between January and December 2006 and assessed using 64-slice multidetector-row CT (n = 340). Patients were classified according to the presence or absence of IAC in the internal carotid arteries, middle cerebral arteries, vertebral arteries, and basilar artery. GFR was estimated using the MDRD equation. Chronic kidney disease (CKD) was defined as a GFR < 60 ml/min/1.73 m(2). We also studied a control group of patients admitted for neurologic diseases other than stroke. RESULTS: Two hundred fifty-nine stroke patients (76.2%) displayed IAC, which was independently associated with carotid atherosclerosis > 50%, age, and GFR. One hundred three nonstroke patients (60.2%) had IAC, with age, arterial hypertension, and GFR as independently associated factors. For all patients taken together, age, arterial hypertension, stroke, and GFR were independently associated with IAC. CONCLUSION: These results confirm the high prevalence of IAC in patients with and without ischemic stroke and show for the first time that IAC is associated with the presence of CKD in these patients. The frequency of IAC was significantly higher in stroke patients than in nonstroke patients. The association between IAC and stroke outcome requires further investigation.