Le développement au cours des deniers années des interventions neurochirurgicales sous anesthésie locale, en particulier dans le champ neuro-oncologique, rend possible la question d’une éventuelle révocation per opératoire du consentement du patient à la procédure chirurgicale.

Les auteurs décrivent la révocation de son consentement par un patient au cours de l’exérèse chirurgicale d’un gliome de bas grade fronto-temporo-insulaire droit.

Les aspects spécifiques aux conditions de révocation du consentement dans le contexte particulier de la chirurgie sous anesthésie locale sont discutés. La possibilité d’un pacte d’Ulysse est proposée et discutée.

Les interventions chirurgicales en condition éveillée créent un contexte particulier en ce qu’une révocation du consentement per opératoire devient possible. Dans ces conditions, l’information préopératoire pourrait aussi aborder avec le malade la question de la conduite à tenir au cas où il serait amené à solliciter un retrait de consentement lors de son réveil per opératoire.

We review clinical, neuroimaging, and genetic information on six individuals with isolated sulfite oxidase deficiency (ISOD).

All patients were examined, and clinical records, biochemistry, neuroimaging, and sulfite oxidase gene (SUOX) sequencing were reviewed.

Data was available on six individuals from four nuclear families affected by ISOD. Each individual began to seize within the first week of life. neurologic development was arrested at brainstem reflexes, and severe microcephaly developed rapidly. neuroimaging within days of birth revealed hypoplasia of the cerebellum and corpus callosum and damage to the supratentorial brain looking like severe hypoxic-ischemic injury that evolved into cystic hemispheric white matter changes. Affected individuals all had elevated urinary S-sulfocysteine and normal urinary xanthine and hypoxanthine levels diagnostic of ISOD. Genetic studies confirmed SUOX mutations in four patients.

ISOD impairs systemic sulfite metabolism, and yet this genetic disease affects only the brain with damage that is commonly confused with the clinical and radiologic features of severe hypoxic-ischemic encephalopathy.

Incidental findings arising from imaging research have important implications for patient safety. Magnetic resonance imaging is widespread in multiple sclerosis (MS) studies and care, yet the prevalence rate of incidental findings in MS is poorly defined. The absence of such reports in the MS literature suggests that such findings may be deemed inappropriate for documentation in research publications, or possibly, not fully reported at all.

We sought to document incidental findings from a study designed to detect features of chronic cerebrospinal venous insufficiency (CCSVI) in MS patients and control subjects.

Magnetic resonance images were obtained as part of a prospective study conducted between October 2010 and September 2012. Patients with MS (relapsing-remitting, primary progressive, secondary progressive), clinically isolated syndromes, and neuromyelitis optica and age/sex-matched healthy controls were included. All images were reviewed by neuro-radiologists for quality-control purposes.

Magnetic resonance imaging was successfully obtained in 166 participants (110 patients, 56 controls). Incidental abnormalities (n = 33) were detected in 15% of patients (n = 17) and 27% of controls (n = 15), comprising 19% overall (n = 32).

The prevalence of incidental findings from the MS population was not significantly different from the control population. However, the overall prevalence was high and warrants a careful management strategy for future imaging studies.

We studied the safety of use of acute reperfusion therapies in patients with stroke- on- awakening using a computed tomographic angiography (Cta) based large vessel occlusion-good scan paradigm in clinical routine.

the Cta database of the Calgary stroke program was reviewed for the period January 2003-March 2010. patients with stroke-on-awakening with large artery occlusions on Cta, who received conservative, iV thrombolytic and/or endovascular treatment at discretion of the attending stroke neurologist were analyzed. time of onset was defined by the time last seen or known to be normal. Baseline non-contrast Ct scan (nCCt) alberta Stroke program early Ct Score (aSpeCtS) > 7 was considered a good scan. hemorrhage was defined on follow-up brain imaging using eCaSS 3 criteria. independence (mrS≤2) at three months was considered a good clinical outcome. Standard descriptive statistics and multivariable analysis were done.

among 532 patients with large artery occlusions, 70 patients with stroke-on-awakening (13.1%) were identified. the median age was 69.5 (iQr 24) and 41 (58.6%) were female; 41 (58.6%) received anti-platelets only and 29 (41.4%) received thrombolytic treatment [iV-12 (17.1%), iV/ia-12 (17.1%) and ia-5(7.1%)]. unadjusted analysis showed that baseline nCCt aSpeCtS ≤ 7 (p=0.002) and higher nihSS scores (p=0.018) were associated with worse outcomes. there were no ph2 hemorrhages in the iV thrombolytic or endovascular treated group. functional outcome was not different by treatment.

When carefully selected using Ct –Cta, by a good scan (aSpeCtS > 7) occlusion paradigm, acute reperfusion therapies in patients with stroke-on-awakening can be performed safely in clinical routine.

Cognitive impairments are common in Parkinson’s disease (PD). Despite its clinical importance, the development of dementia is still difficult to predict. In this study, we investigated the possible associations between non-motor symptoms and the risk of developing dementia within a 2-year observation period in PD.

A total of 80 patients with PD participated in this study. Nonmotor symptoms (the Nonmotor Symptoms Questionnaire), PD status (Unified Parkinson’s Disease Rating Scale), depression (Geriatric d Depression Scale or Montgomery-Asberg Depression Scale), stereopsis and severity of nonmotor symptoms (Non-motor symptoms scale) were assessed. Global cognitive function (Mini-Mental State Examination) were evaluated at baseline and 2 years later.

Presence of depression, vivid dreaming, REM sleep behavior disorders, hyposmia, abnormal stereopsis, non-smoking and postural instability/ gait disturbance phenotype were associated with a significantly more rapid decline of Mini-Mental State Examination. Logistic regression analyses demonstrated that depression (odds ratio=13.895), abnormal stereopsis (odds ratio=10.729), vivid dreaming (odds ratio=4.16), REM sleep behavior disorders (odds ratio=5.353) and hyposmia (odds ratio=4.911) were significant independent predictors of dementia risk within 2 years. Postural instability/ gait disturbance phenotype and age >62 years were also independent predictors of dementia risk (odd ratio=38.333, odds ratio=10.625).

We suggest that depression, vivid dreaming, REM sleep behavior disorders, hyposmia and abnormal stereopsis are closely associated with cognitive decline, and that presence of these nonmotor symptoms predict the subsequent development of Parkinson’s disease dementia.

To prospectively investigate the incidence and prevalence of Wilson disease (WD) in Chinese Han population in Anhui Province, to analyze the genetic mutations in individuals with WD, and to provide basic epidemiological data regarding WD in this Chinese Han population.

Between November 2008 and June 2010, individuals aged from 7 to 75 years were screened for the cornea K-F ring in both eyes using slit lamp examination and random sampling methods based on age stratification and cluster level 1. The participants were from Anhui Province's Hanshan County, Jinzhai County, and Lixin County. The clinical manifestations of the brain, liver, kidney, skin, and other organs in each individual were also determined. Individuals with positive K-F rings and clinical manifestations indicative of WD underwent copper biochemistry evaluations, abdominal ultrasound testing, and ATP7B gene mutation screening to confirm or exclude the diagnosis of WD.

Of 153,370 individuals investigated in this study, nine were diagnosed with WD. In these WD individuals, three cases had neurological symptoms, one has hepatic symptoms, one was hepatic and neurological combined, and the other four cases were presymptomatic. Of the eight individuals in whom genetic mutations were detected, seven individuals had mutations in the ATP7B gene. The other individual had no ATP7B gene mutations but her copper biochemical test results met the diagnostic criteria for WD. The incidence and prevalence of WD in this population were approximately 1.96/100,000 and 5.87/100,000 respectively.

The Chinese Han population had a higher average prevalence of WD than the populations of the United States or Europe.

Since the beginning of the new millennium, prevalence of syphilis has re-increased and is once again, a major public health problem. Neurosyphilis is the extension of syphilitic infection to the nervous system. It is considered by many as a cause of reversible dementia, when treated early. However, scarce data exist on the evolution of cognitive and behavioral impairments in patients affected by tertiary neurosyphilis.

The aim of this study was to explore the cognitive and behavioral changes in a cohort of patients diagnosed with neurosyphilis.

A retrospective study based on systematized chart review between 2000 and 2012 in a large neurological tertiary care facility.

Clinical evaluations by treating physicians.

Eighteen patients were identified with tertiary neurosyphilis. Out of this group, only two had systematic neuropsychological follow-up despite physician reports of significant and persistent cognitive and psychiatric changes. For these two cases, only slight improvements were noted in memory and executive skills while improvements in attention were marked. None of our patients had previous psychiatric history yet a large proportion developed symptoms after the infection.

Although neurosyphilis is traditionally considered a reversible form of dementia, we found limited support for this claim in our two patients with close follow-up. Quality data on the cognitive and psychiatric changes in the rest of our cohort was dramatically lacking, and this could not be explained by absence of symptoms at presentation. Given the recrudescence of syphilis, we propose a systematic approach to the evaluation and follow-up of this disorder.

Acromegaly has important effects on quality of life (QOL). This is the first study to measure QOL in acromegalic patients after endoscopic transsphenoidal surgery (ETSS).

We prospectively collected the RAND-36, Center for Epidemiologic Studies Depression (CES-D), and Pituitary QOL validated questionnaires and patients’ demographics, clinical presentation, endocrine laboratory results, radiological studies, development of complications and remission rates from 20 consecutive acromegalic patients who had undergone endoscopic transphenoidal surgery.

The eleven females and nine males had an average age of 42 years; 90 percent had macroadenomas and 70% had cavernous sinus invasion on their preoperative imaging. Ninety percent had improved symptoms post-operatively and 80% stated that treatment improved their QOL. Biochemically, 35% were cured, 35% had discordant results and 30% were not cured, while pan-hypopituitarism occurred in 4 patients. Physical health subscales and pituitary-related symptoms were similar to norms. “Social,” “emotional health,” and “energy levels” were significantly lower than norms. Seventy percent stated that their relationship with their physician “very much so” affected their quality of life. Pan hypopituitarism and adjuvant therapy were the most significant predictors of lower QOL subscale scores.

Transsphenoidal surgery improves QOL in acromegaly. Attempts to achieve a cure, avoidance of surgically induced pan-hypotpituitarism and adjuvant therapy, will improve quality of life. Our study demonstrates the important role of the patient-physician relationship to QOL and the need to measure QOL in addition to the traditional measures of outcome.

The primary objective was to evaluate the effects of pregabalin relative to placebo in patients with chronic unilateral cervicogenic headache.

To assess the change from baseline in the frequency of cervicogenic headache days per 28-day period between placebo and treatment group. To assess the change from baseline in the intensity of headache, and health outcome measures.

This was a double-blind, randomized, placebo-controlled, parallel-group study, evaluating the efficacy and safety of pregabalin in patients with cervicogenic headache.

The study consisted of two phases. A baseline of -28 days and a double-blind placebo-controlled phase: with an escalation and maintenance phase, during which patients remained at their highest dose until the end of the study, at Day 86.

Forty one patients were randomized, predominantly females, with a mean age of 52 years old. At screening, both groups had, on average, 26 headache-days per month. By the final phase of the study, the number of headache days dropped to 16 per month for the pregabalin group while remaining stable for the placebo group (p=0.037). No serious adverse events were reported during the study.

In this study, primary objectives were achieved with a statistically significant change of ten days in frequency of headache days; with minor side effects that were well tolerated.

There is currently no definite neuroimaging test to detect amyotrophic lateral sclerosis (ALS), which leads to significant delay in diagnosis, particularly if one takes into account the rapidity of disease evolution. Hyperintensity of the corticospinal tracts (CST) on T2 or fluid-attenuated inversion recovery (FLAIR) weighted magnetic resonance imaging (MRI) has been well described, but data on sensitivity and specificity in larger series is lacking to help guide its application to clinical care.

We analyzed clinical and MRI data from 64 patients with a definite retrospective diagnosis of ALS. In this case-control study, two experienced blinded neuroradiologists systematically assessed defined rostrocaudal segments of the intracranial course of the CST.

The overall sensitivity and specificity of conventional MRI for the diagnosis of ALS were 48% and 76% respectively. Highest specificities for CST hyperintensity were noted for the subcortical white matter (92%), centrum semiovale (88%) and medullary pyramids (92%). The lowest specificities were found for the cerebral peduncle (36%) and internal capsule (32%). We did not find a correlation with the rate of clinical progression, age of onset or the presence of upper motor neuron signs on examination.

Conventional MRI was not found to be a reliable diagnostic tool for ALS and it did not help predict clinical characteristics such as speed of evolution or prominence of upper motor neuron signs. Its main role in the setting of ALS should remain to help exclude alternative diagnostic considerations. A multimodal approach relying on newer functional and structural MRI techniques still needs to be developed and validated.

To identify predictors of good outcome in acute basilar artery occlusion (BAO).

Acute ischemic stroke (AIS) caused by BAO is often associated with a severe and persistent neurological deficit and a high mortality rate.

The set consisted of 70 consecutive AIS patients (51 males; mean age 64.5±14.5 years) with BAO. The role of the following factors was assessed: baseline characteristics, stroke risk factors, pre-event antithrombotic treatment, neurological deficit at time of treatment, estimated time to therapy procedure initiation, treatment method, recanalization rate, change in neurological deficit, post-treatment imaging findings. 30- and 90-day outcome was assessed using the modified Rankin scale with a good outcome defined as a score of 0–3.

The following statistically significant differences were found between patients with good versus poor outcomes: mean age (54.2 vs. 68.9 years; p=0.0001), presence of arterial hypertension (52.4% vs. 83.7%; p=0.015), diabetes mellitus (9.5% vs. 55.1%; p=0.0004) and severe stroke (14.3% vs. 65.3%; p=0.0002), neurological deficit at time of treatment (14.0 vs. 24.0 median of National Institutes of Health Stroke Scale [NIHSS] points; p=0.001), successful recanalization (90.0% vs. 54.2%; p=0.005), change in neurological deficit (12.0 vs. 1.0 median difference of NIHSS points; p=0.005). Stepwise binary logistic regression analysis identified age (OR=0.932, 95% CI=0.882–0.984; p=0.012), presence of diabetes mellitus (OR=0.105, 95% CI=0.018–0.618; p=0.013) and severe stroke (OR=0.071, 95% CI=0.013–0.383; p=0.002) as significant independent negative predictors of good outcome.

In the present study, higher age, presence of diabetes mellitus and severe stroke were identified as significant independent negative predictors of good outcome.

few studies have addressed the association between the characteristics of ischemic lesions detected by diffusion-weighted imaging (dWi) and the clinical outcome in patients with hyperacute posterior circulation ischemic stroke. this study demonstrates a relationship between the findings assessed by dWi and the outcome in patients with hyperacute posterior circulation ischemic stroke.

We reviewed data from 118 patients who had posterior circulation ischemic stroke within six hours from the onset of their symptoms. the clinical outcome included early neurological deterioration (end) and a favorable outcome at three months after the onset of symptoms. using dWi, the lesion volume and the number and location of injured anatomical regions were analyzed to evaluate whether the results correlated with the clinical outcome measures.

the number of injured anatomical regions assessed by dWi was associated with the initial and delayed neurological status. Both the total volume and the number of injured anatomical regions associated with end and a favorable outcome. analysis of the location of the injured regions determined that only a pontine lesion independently associated with end. interestingly, four out of five patients who underwent decompressive craniectomy exhibited a large infarction volume but minor symptoms.

in patients with hyperacute posterior circulation ischemic strokes, the lesions assessed by dWi were associated with the clinical outcome, regardless of the initial neurological status. dWi is an effective initial imaging tool for assessing the extent of lesions and clinical outcomes in patients with hyperacute posterior circulation ischemic stroke.

Vitamin D3 (cholecalciferol) deficiency has been associated with dementia and cognitive decline. Which cognitive domains are most associated with D3 levels and how seasonal fluctuations in levels relate to cognition is unclear. We addressed these questions using a prospective observational study examining associations between D3 levels and cognition among individuals living in northern latitudes (54°N) in summer and winter.

Healthy adult participants underwent testing in summer and winter of D3 levels and cognition, using the Symbol digit Modalities test, phonemic fluency, digit Span and CANTAB® battery.

Of 32 participants tested in the summer, 46% were D3 insufficient (<75 nmol/L) and performed worse on digit Span Backward (DS-B) (μ=5.8, SD=2) than those who were sufficient (μ=7.9, SD=2), p=0.018. In multivariate analyses, sufficiency status was an independent predictor of dS-B, (b=0.41, p=0.02). The majority (63%) of 19 participants tested in winter were D3 insufficient, with levels declining by a median of 15 nmol/L overall. Those with insufficient levels performed worse (i.e., higher scores) on the CANTAB® Spatial Working Memory (SWM) task (μ=36.1, SD=6 versus μ=29.3, SD=8), p=0.05). Those with larger drops in levels (≥15 nmol/L) showed decline/less improvement on the CANTAB® one touch Stockings of Cambridge (OTS) task, (μ=0.50, SD=1.9 versus μ=-2.11, SD=2.6, p=0.01), a test of working memory/executive functioning.

Vitamin D3 insufficiency and seasonal declines ≥15 nmol/L were associated with inferior working memory/executive functioning. While our findings require confirmation, they suggest that sufficient D3 levels should be maintained year-round, likely necessitating supplementation, at least during winter at higher latitudes.

To investigate whether repeat saccadic reaction time (SRT) measurements using a portable saccadometer is useful to monitor patients with mild traumatic brain injury (mTBI).

Seven patients with newly-diagnosed mTBI and five agematched controls were prospectively recruited from an emergency Department. Saccadic eye movements, symptom self-reporting and neuropsychological tests were performed within one week of injury and again at follow-up three weeks post-injury. Control patients underwent saccade recordings at similar intervals.

Median saccade reaction times were significantly prolonged within one week post-injury in mTBI compared to controls. At follow-up assessment there was no significant between-groups difference. Changes in median SRT between the two assessments were not statistically significant. Four of the seven mTBI patients showed significantly increased SRT at follow-up; three of the mTBI patients and all controls showed no significant change. Among the three mTBI patients with persistent decreased SRT, two experienced loss of consciousness and reported the greatest symptoms, while the third was the only subject with significant decrease in neuropsychological testing scores at both assessments.

In three of seven mTBI patients, saccadic eye movements remained delayed within three weeks post-injury. These three patients also showed persistent symptoms or no improvement on neuropsychological testing. This pilot study using a portable saccadometer suggests that comparing SRT from three weeks post-injury to that within one week of injury may be useful for early detection of a subpopulation at risk of persistent disability from mTBI. This finding suggests that further investigation in a large study population is warranted.

We aimed to examine changes in serum bilirubin and uric acid (UA) levels in subcortical ischemic vascular disease (SIVD). In addition, we investigated if altered serum bilirubin and UA levels correlate with the subtypes of SIVD as well as the severity of leukoaraiosis (LA).

This cross-sectional study included 1098 consecutive patients with slight symptoms, such as dizziness, vertigo etc. According to magnetic resonance imaging (MRI) appearances, they were divided into either SIVD group or controls (CN), and the SIVD group was further grouped in lacunar infarction (LI) and LA subtypes, as well as different grades. Serum bilirubin and UA levels were determined by the vanadate oxidase method and enzymatic method respectively, after at least an eight hour overnight fasting, in all subjects.

The bilirubin level was obviously lower while the UA level was significantly higher in the SIVD group when compared with the controls. Moreover, the LA subgroup presented more significant changes in bilirubin and UA when compared to the LI subgroup in both males and females. The correlation was positive between the UA levels and the LA severity (r=0.134, p=0.006). Multivariate regression analysis revealed that the odds ratio (95% CI) for SIVD in the lowest tertile of total bilirubin (Tbil<9.58μmol/L) and highest tertile of UA (UA>339μmol/L) were 2.702(1.936–3.770) and 2.135(1.521–2.996) respectively after adjusting for confounding variables.

Serum bilirubin levels were lower, whereas UA levels were higher in SIVD patients when compared with controls in both males and females, especially in LA patients. Moreover, serum UA levels positively correlated to LA severity.