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Sommaires des Revues - Neurology

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Discovering the patient within  Voir?

The gently winding road from Jackson Hole to Yellowstone National Park follows the imposing Grand Teton mountains, which rise abruptly out of the flat plains of northwestern Wyoming. In May, the heavily snow-laden peaks contrasted dramatically with the skies above and lakes below. To the east lay sparse forestland within which I would soon make an unexpected discovery.

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Clinical Reasoning: A man with rapidly ascending paralysis  Voir?

A 38-year-old construction worker with no medical history presented with back pain, urinary retention, and flaccid lower extremity paralysis. Three weeks prior to presentation, he fell from a ladder with no immediate injury. Two weeks after the fall, he presented to another hospital for back pain and urinary retention. MRI of the lumbar, cervical, and thoracic spine without contrast were reportedly normal, and his back pain improved with an oral methylprednisolone dose pack (figure). The urinary retention remained, and he was discharged with an indwelling catheter. Within a week of the initial urinary symptoms, he developed ascending lower extremity numbness and paralysis, and was seen emergently at our hospital.

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Mystery Case: CNS posttransplant lymphoproliferative disorder  Voir?

A 74-year-old right-handed Hispanic woman with history of renal transplant 19 years prior presented at an away institution with episodic confusion and gait ataxia progressing over 2 weeks. She was alert and oriented on initial presentation but with fluctuation in mental status and without obvious cranial nerve, motor, or sensory deficits. The patient received transplant immunosuppression therapy with mycophenolate mofetil (Cellcept; Roche, Basel, Switzerland) and had a history of hypertension and chronic kidney disease. CT of the head showed a hypodense lesion in the right temporal lobe. MRI of the brain demonstrated multifocal, infiltrative T2-hyperintense white matter lesions involving the right temporo-parietal region, right frontal lobe, left parietal lobe, and left thalamus (figure, A–C). Gadolinium was not administered due to renal dysfunction. Lumbar puncture (LP) showed 63 leukocytes with lymphocytic pleocytosis (88%), elevated protein (80 mg/dL), and normal glucose (71 mg/dL). CSF Gram stain and culture were negative. She was treated with corticosteroids and acyclovir for presumed autoimmune/demyelinating vs infectious etiologies. She developed multiple witnessed periods of behavioral arrest with eye blinking and confusion concerning for seizures. She was transferred to our institution.

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Teaching NeuroImages: The Charcot shoulder: Chiari malformation with syringomyelia associated with arthropathy  Voir?

A 39-year-old man presented with 1-year history of shoulder pain, swelling, and limitation of movement. There was a 20-year history of left shoulder and arm numbness. Examination showed decreased vibratory sensitivity on left shoulder, glenohumeral instability, and dislocation. Radiography showed humeral head destruction (figure 1); a spine MRI showed tonsillar herniation and cervical syringomyelia (figure 2). Arthropathy related to syringomyelia was diagnosed.

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Teaching NeuroImages: Three complications of small-cell lung cancer involving the brain  Voir?

A 53-year-old man with small-cell lung cancer (SCLC) with brain metastases presented with acute hyperthermia (up to 39°C) without evidence of infection, aphasia, and 2 generalized epileptic seizures. MRI made 3 brain complications of SCLC visible. First, multiple brain metastases (figure, A and B) were noted. Second, hyperintense areas at the limbic system (figure, C) were present, favoring a diagnosis of paraneoplastic limbic encephalitis, although associated antibodies were negative. Limbic encephalitis can cause both hyperthermia and seizures.1 Third, aphasia was explained by a region of reduced diffusion at the left insula (figure, D). Consistently, CSF analysis confirmed leptomeningeal carcinomatosis.

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Body mass index in early life and stroke in adult life: What is the risk?  Voir?

The article "BMI increase through puberty and adolescence is associated with risk of adult stroke" by Ohlsson et al.1 explores the relationship between body mass index (BMI) when a person is young (adolescence) and risk of stroke when the person is older (adults). Previous studies2,3 have shown that rate of stroke is decreasing in older adults but increasing in younger adults. This finding coincides with an increasing BMI in children and young adults. The researchers wanted to ask: Does an increased BMI during puberty and adolescence increase the risk for stroke later in life? The authors also explored whether the change in BMI throughout puberty and adolescence is associated with increased risk for stroke. Since extensive literature has shown the relationship between elevated weight/BMI and cardiovascular disease, the authors hypothesized that increasing BMI through puberty and adolescence would increase stroke risk.

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Spotlight on the July 25 issue  Voir?

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Dementia with Lewy bodies: Diagnosis is only skin deep?  Voir?

Dementia with Lewy bodies (DLB) is currently diagnosed clinically by identifying dementia in combination with a number of hallmark features: REM sleep behavior disorder (RBD), prominent visual hallucinations, parkinsonism, and marked fluctuations of cognition and alertness.1 DLB is often underdiagnosed, suggesting a role for biomarkers. The main differential diagnosis of DLB is Alzheimer disease (AD); whereas both AD and DLB have amyloid deposition, DLB also has additional α-synuclein deposition (Lewy bodies and Lewy neurites). So, one way to establish DLB diagnosis might be to detect the α-synucleinopathy itself.

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Adolescent weight gain confers long-term increased stroke risk: Weighty matters  Voir?

Adult obesity has been consistently associated with risk of total, ischemic, and hemorrhagic stroke1–3; however, the degree to which change in weight and body mass index (BMI) during late childhood and adolescence affects adult risk of stroke has remained uncertain. Understanding this relationship is of particular importance given the rising obesity epidemic during childhood and adolescence.

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Missed diagnosis of prehospital status epilepticus: Is it serious, doctor?  Voir?

Status epilepticus (SE) represents one of the most frequent neurologic emergencies, with potentially severe morbidity and mortality.1 Considerable attention has been directed towards implementation of treatment protocols,2 and recently of anesthetic treatment,3,4 but prognostic studies focused on early recognition of SE by emergency medical services (EMS) are lacking.

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Improving practice through neurovascular board  Voir?

Management of cervical internal carotid artery (ICA) stenosis remains contentious despite numerous randomized trials and observational studies over the decades since the first report of carotid endarterectomy. Rimmele et al.1 in this issue of Neurology® summarize the 53-month experience of deliberations by a Neurovascular Board at the University Medical Center, Hamburg-Eppendorf (UKE), on management of ICA disease. The multidisciplinary board of senior vascular specialists reviewed 614 cases. The board recommended revascularization in 76% of symptomatic cases and in 27% of asymptomatic cases and encouraged trial participation for a subset of asymptomatic cases. Recommendations appeared well-supported by evidence-based practice guidelines. The authors conclude that the board recommendations provided a helpful and transparent tool to assure adherence to guidelines. However, the study lacked a control group; therefore it is not possible to determine if the board actions truly helped promote evidence-based practice or trial participation. Cluster randomization design might have provided robust evidence for neurovascular boards in a sufficiently brief time frame to avoid the challenge of guideline drift during the study.

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A new potential biomarker for dementia with Lewy bodies: Skin nerve {alpha}-synuclein deposits  Voir?

Objective:

To investigate whether (1) phosphorylated α-synuclein (p-syn) deposits in skin nerves could be useful in differentiating dementia with Lewy bodies (DLB) from different forms of dementia and (2) small fiber neuropathy (SFN) is associated with DLB.

Methods:

We studied 18 well-characterized patients with DLB (11 with autonomic dysfunction), 23 patients with nonsynucleinopathy dementia (NSD; 13 with young-onset Alzheimer disease dementia, 6 frontotemporal dementia, and 4 vascular dementia), and 25 healthy controls. All participants underwent skin biopsies from proximal (i.e., cervical) and distal (i.e., thigh and distal leg) sites to study small nerve fibers and deposits of p-syn, considered the pathologic form of α-synuclein.

Results:

No p-syn was detected in any skin sample in patients with NSD and controls but was found in all patients with DLB. SFN was found in patients with DLB and the autonomic denervation of skin was more severe in patients with autonomic dysfunctions.

Conclusions:

(1) In autonomic skin nerves, p-syn is a sensitive biomarker for DLB diagnosis, helping to differentiate DLB from other forms of dementia, although this needs to be confirmed in a larger, more representative sample; and (2) skin autonomic neuropathy is part of the DLB pathology and may contribute to autonomic symptoms.

Classification of evidence:

This study provides Class III evidence that p-syn in skin nerve fibers on skin biopsy accurately distinguishes DLB from other forms of dementia.

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Odor identification as a biomarker of preclinical AD in older adults at risk  Voir?

Objective:

To assess odor identification (OI) as an indicator of presymptomatic Alzheimer disease (AD) pathogenesis in cognitively normal aging individuals at increased risk of AD dementia.

Methods:

In 274 members of the PREVENT-AD cohort of healthy aging persons with a parental or multiple-sibling history of AD dementia, we assessed the cross-sectional association of OI with potential indicators of presymptomatic AD. Some 101 participants donated CSF, thus enabling assessment of AD pathology with the biomarkers total tau (t-tau), phospho-tau (P181-tau), and their ratios with β-amyloid (Aβ1-42). Adjusted analyses considered age, cognition, APOE 4 status, education, and sex as covariates. We measured OI using the University of Pennsylvania Smell Identification Test and cognitive performance using the Repeatable Battery for Assessment of Neuropsychological Status. Standard kits provided assays of the AD biomarkers. Analyses used robust-fit linear regression models.

Results:

Reduced OI was associated with lower cognitive score and older age, as well as increased ratios of CSF t-tau and P181-tau to Aβ1-42 (all p < 0.02). However, the observed associations of OI with age and cognition were unapparent in adjusted models that restricted observations to CSF donors and included AD biomarkers. OI showed little association with CSF Aβ1-42 alone except in APOE 4 carriers having lowest-quartile Aβ1-42 levels.

Conclusions:

These findings from healthy high-risk older individuals suggest that OI reflects degree of preclinical AD pathology, while its relationships with age and cognition result from the association of these latter variables with such pathology. Diminished OI may be a practical and affordable biomarker of AD pathology.

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Slowing gait and risk for cognitive impairment: The hippocampus as a shared neural substrate  Voir?

Objective:

To identify the shared neuroimaging signature of gait slowing and cognitive impairment.

Methods:

We assessed a cohort of older adults (n = 175, mean age 73 years, 57% female, 65% white) with repeated measures of gait speed over 14 years, MRI for gray matter volume (GMV) at year 10 or 11, and adjudicated cognitive status at year 14. Gait slowing was calculated by bayesian slopes corrected for intercepts, with higher values indicating faster decline. GMV was normalized to intracranial volume, with lower values indicating greater atrophy for 10 regions of interest (hippocampus, anterior and posterior cingulate, primary and supplementary motor cortices, posterior parietal lobe, middle frontal lobe, caudate, putamen, pallidum). Nonparametric correlations adjusted for demographics, comorbidities, muscle strength, and knee pain assessed associations of time to walk with GMV. Logistic regression models calculated odds ratios (ORs) of gait slowing with dementia or mild cognitive impairment with and without adjustment for GMV.

Results:

Gait slowing was associated with cognitive impairment at year 14 (OR per 0.1 s/y slowing 1.47; 95% confidence interval 1.04–2.07). The right hippocampus was the only region that was related to both gait slowing ( = –0.16, p = 0.03) and cognitive impairment (OR 0.17, p = 0.009). Adjustment for right hippocampal volume attenuated the association of gait slowing with cognitive impairment by 23%.

Conclusions:

The association between gait slowing and cognitive impairment is supported by a shared neural substrate that includes a smaller right hippocampus. This finding underscores the value of long-term gait slowing as an early indicator of dementia risk.

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Effects of increasing IV tPA-treated stroke mimic rates at CT-based centers on clinical outcomes  Voir?

Objective:

To determine to what degree stroke mimics skew clinical outcomes and the potential effects of incorrect stroke diagnosis.

Methods:

This retrospective analysis of data from 2005 to 2014 included IV tissue plasminogen activator (tPA)–treated adults with clinical suspicion for acute ischemic stroke who were transferred or admitted directly to our 2 hub hospitals. Primary outcome measures compared CT-based spoke hospitals' and MRI-based hub hospitals' mimic rates, hemorrhagic transformation, follow-up modified Rankin Scale (mRS), and discharge disposition. Secondary outcomes were compared over time.

Results:

Of the 725 thrombolysis-treated patients, 29% were at spoke hospitals and 71% at hubs. Spoke hospital patients differed from hubs by age (mean 62 ± 15 vs 72 ± 15 years, p < 0.0001), risk factors (atrial fibrillation, 17% vs 32%, p < 0.0001; alcohol consumption, 9% vs 4%, p = 0.007; smoking, 23% vs 13%, p = 0.001), and mimics (16% vs 0.6%, p < 0.0001). Inclusion of mimics resulted in better outcomes for spokes vs hubs by mRS ≤1 (40% vs 27%, p = 0.002), parenchymal hematoma type 2 (3% vs 7%, p = 0.037), and discharge home (47% vs 37%, p = 0.01). Excluding mimics, there were no significant differences. Comparing epochs, spoke stroke mimic rate doubled (9%–20%, p = 0.03); hub rate was unchanged (0%–1%, p = 0.175).

Conclusions:

Thrombolysis of stroke mimics is increasing at our CT-based spoke hospitals and not at our MRI-based hub hospitals. Caution should be used in interpreting clinical outcomes based on large stroke databases when stroke diagnosis at discharge is unclear. Inadvertent reporting of treated stroke mimics as strokes will artificially elevate overall favorable clinical outcomes with additional downstream costs to patients and the health care system.

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Aggressiveness of care following intracerebral hemorrhage in women and men  Voir?

Objective:

To compare comorbidities and use of surgery and palliative care between men and women with intracerebral hemorrhage (ICH).

Methods:

The Ethnic/Racial Variations of Intracerebral Hemorrhage (ERICH) study is a prospective, multicenter, case-control study of ICH risk factors and outcomes. We compared comorbidities, treatments, and use of do-not-resuscitate (DNR) orders in men vs women. Multivariate analysis was used to assess the likelihood of ICH surgery and palliative care after adjustment for variables that were p < 0.1 in univariate analyses and backward elimination to retain those that were significant (p < 0.05).

Results:

Women were older on average (65.0 vs 59.9, p < 0.0001), and higher proportions of women had previous stroke (24.1% vs 19.3%, p = 0.002), had dementia (6.1% vs 3.4%, p = 0.0007), lived alone (23.1% vs 18.0%, p = 0.0005), and took anticoagulants (12.8% vs 10.1% p = 0.02), compared with men. Men had higher rates of alcohol and cocaine use. After adjusting for age, hematoma volume, and ICH location, there was no difference in rates of surgical treatment by sex (odds ratio [OR] 0.93 for men vs women, 95% confidence interval [CI] 0.68–1.28, p = 0.67), and there was no difference in DNR/comfort care decisions after adjustment for ICH score, prior stroke, and dementia (OR 0.96, CI 0.77–1.22, p = 0.76).

Conclusions:

After ICH, women do not receive less aggressive care than men after controlling for the substantial comorbidity differences. Future studies on sex bias should include the presence of comorbidities, prestroke disability, and other factors that may influence management.

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Comment: Does patient sex affect decision-making after hemorrhagic stroke?  Voir?

Women do worse than men after both ischemic and hemorrhagic stroke, with evidence that sex bias in physician decision-making may contribute to this increased morbidity and mortality.1 Intracerebral hemorrhage (ICH) poses additional challenges, as the etiologies—amyloid angiopathy associated with prior cognitive decline, illicit drug use—carry their own assumptions. Guha et al.2 report findings from the Ethnic/Racial Variations of Intracerebral Hemorrhage (ERICH) study, a prospective, multicenter, triethnic, case-control study of ICH risk factors and outcomes. In almost 3,000 people, they examined comorbidities, treatments, and use of do not resuscitate (DNR) orders, comparing men vs women (n = 1,220). The outcome measure was aggressiveness of care, measured via use of surgical therapies for hematoma treatment and avoidance of DNR orders.

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Permanent CSF shunting after intraventricular hemorrhage in the CLEAR III trial  Voir?

Objective:

To study factors associated with permanent CSF diversion and the relationship between shunting and functional outcomes in spontaneous intraventricular hemorrhage (IVH).

Methods:

Clot Lysis Evaluation of Accelerated Resolution of Intraventricular Hemorrhage (CLEAR III), a randomized, multicenter, double-blind, placebo-controlled trial, was conducted to determine if pragmatically employed external ventricular drainage (EVD) plus intraventricular alteplase improved outcome, in comparison to EVD plus saline. Outcome measures were predictors of shunting and blinded assessment of mortality and modified Rankin Scale at 180 days.

Results:

Among the 500 patients with IVH, CSF shunting was performed in 90 (18%) patients at a median of 18 (interquartile range [IQR] 13–30) days. Patient demographics and IVH characteristics were similar among patients with and without shunts. In the multivariate analysis, black race (odds ratio [OR] 1.98; 95% confidence interval [CI] 1.18–3.34), duration of EVD (OR 1.10; CI 1.05–1.15), placement of more than one EVD (OR 1.93; CI 1.13–3.31), daily drainage CSF per 10 mL (OR 1.07; CI 1.04–1.10), and intracranial pressure >30 mm Hg (OR 1.70; CI 1.09–2.88) were associated with higher odds of permanent CSF shunting. Patients who had CSF shunts had similar odds of 180-day mortality, while survivors with shunts had increased odds of poor functional outcome, compared to survivors without shunts.

Conclusions:

Among patients with spontaneous IVH requiring emergency CSF diversion, those with early elevated intracranial pressure, high CSF output, and placement of more than one EVD are at increased odds of permanent ventricular shunting. Administration of intraventricular alteplase, early radiographic findings, and CSF measures were not useful predictors of permanent CSF diversion.

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BMI increase through puberty and adolescence is associated with risk of adult stroke  Voir?

Objective:

To evaluate the contribution of prepubertal childhood body mass index (BMI) and BMI change through puberty and adolescence, 2 distinct developmental BMI parameters, for risk of adult stroke in men.

Methods:

In this population-based study in Gothenburg, Sweden, men born in 1945–1961 with information on both childhood BMI at age 8 and BMI change through puberty and adolescence (BMI at age 20–BMI at age 8) were followed until December 2013 (n = 37,669). Information on stroke events was retrieved from high-quality national registers (918 first stroke events, 672 ischemic stroke events [IS], 207 intracerebral hemorrhage events [ICH]).

Results:

BMI increase through puberty and adolescence (hazard ratio [HR] 1.21 per SD increase; 95% confidence interval [CI] 1.14–1.28), but not childhood BMI, was independently associated with risk of adult stroke. Subanalyses revealed that BMI increase through puberty and adolescence was associated with both IS (HR per SD increase 1.19; 95% CI 1.11–1.28) and ICH (HR per SD increase 1.29; 95% CI 1.15–1.46). High BMI increase during puberty was strongly associated with increased risk of adult hypertension (odds ratio per SD increase 1.35; 95% CI 1.32–1.39).

Conclusions:

BMI increase through puberty and adolescence is associated with risk of adult IS and ICH in men. We propose that greater BMI increases during puberty contribute to increased risk of adult stroke at least partly via increased blood pressure.

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Upgoing thumb sign: A sensitive indicator of brain involvement?  Voir?

Objective:

To assess the frequency of this finding in patients with minor stroke and TIAs compared to those with stroke mimics and to evaluate the level of agreement between examiners to detect an upgoing thumb sign.

Methods:

We previously reported an upgoing thumb sign as a subtle clinical finding in patients with transient ischemic attacks or minor stroke. In this study conducted between March 2016 and October 2016 at the Stroke Prevention Clinic at University Hospital, University of Western Ontario, London, Canada, participants were examined independently by stroke faculty and fellows who were blinded to each other's findings. The frequency of the upgoing thumb sign in patients with minor or threatened stroke was compared to that in patients with stroke mimics, and the level of agreement between examiners and clinical findings was assessed with the Cohen test.

Results:

The upgoing thumb sign was observed more frequently in those with minor stroke/TIA than in those with stroke mimics (p = 0.001). A substantial level of agreement between examiners was recorded in the detection of the thumb sign (: right 0.71, left 0.78). In addition, an upgoing thumb sign showed a substantial level of agreement with the patient's symptoms (examiner 1: = 0.65, p < 0.01; examiner 2: = 0.64; p < 0.01).

Conclusions:

An upgoing thumb sign is a sensitive and reliable indicator of brain involvement. This examination is noninvasive, easy, reliable, and highly compatible with and confirmatory of the patient's symptoms.

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Emergency response to out-of-hospital status epilepticus: A 10-year observational cohort study  Voir?

Objective:

To determine the implications of first responses of emergency medical services (EMS) to out-of-hospital status epilepticus (SE) on outcome.

Methods:

From 2005 to 2014, prehospital and in-hospital data were assessed in consecutive adults admitted to an academic medical center with out-of-hospital SE. Logistic regression was performed to identify variables with a robust association between missed epileptic events by the EMS and no recovery to functional baseline in survivors.

Results:

Among 213 SE patients, 150 were admitted via EMS. While nonconvulsive SE (NCSE) was missed by the EMS in 63.7%, convulsive SE (CSE) was not missed except in 4 patients with transformation into subtle SE. Missed NCSE was more likely with older age (odds ratio [OR]per year 1.06, 95% confidence interval [CI] 1.02–1.10, p = 0.003) and no seizure history (OR 6.64, 95% CI 2.43–18.1, p < 0.001). The area under the receiver operating characteristic curve for prediction of missed NCSE by these variables was 0.839. Independent predictors for not receiving benzodiazepines were increasing age (ORper year 1.05, 95% CI 1.01–1.08, p = 0.008) and higher Glasgow Coma Scale score (ORper increasing unit 1.21, 95% CI 1.09–1.36, p = 0.001). Missed NCSE was independently associated with increased odds for no return to functional baseline in survivors (OR 3.83, 95% CI 1.22–11.98, p = 0.021).

Conclusions:

Among patients admitted with out-of-hospital SE, CSE is mostly recognized while NCSE is frequently missed especially in patients with increasing age and no seizure history. This calls for heightened awareness for out-of-hospital NCSE in such patients, as missed NCSE is associated with lack of treatment and less recovery to functional baseline in survivors independent of established outcome predictors.

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DNM1 encephalopathy: A new disease of vesicle fission  Voir?

Objective:

To evaluate the phenotypic spectrum caused by mutations in dynamin 1 (DNM1), encoding the presynaptic protein DNM1, and to investigate possible genotype-phenotype correlations and predicted functional consequences based on structural modeling.

Methods:

We reviewed phenotypic data of 21 patients (7 previously published) with DNM1 mutations. We compared mutation data to known functional data and undertook biomolecular modeling to assess the effect of the mutations on protein function.

Results:

We identified 19 patients with de novo mutations in DNM1 and a sibling pair who had an inherited mutation from a mosaic parent. Seven patients (33.3%) carried the recurrent p.Arg237Trp mutation. A common phenotype emerged that included severe to profound intellectual disability and muscular hypotonia in all patients and an epilepsy characterized by infantile spasms in 16 of 21 patients, frequently evolving into Lennox-Gastaut syndrome. Two patients had profound global developmental delay without seizures. In addition, we describe a single patient with normal development before the onset of a catastrophic epilepsy, consistent with febrile infection-related epilepsy syndrome at 4 years. All mutations cluster within the GTPase or middle domains, and structural modeling and existing functional data suggest a dominant-negative effect on DMN1 function.

Conclusions:

The phenotypic spectrum of DNM1-related encephalopathy is relatively homogeneous, in contrast to many other genetic epilepsies. Up to one-third of patients carry the recurrent p.Arg237Trp variant, which is now one of the most common recurrent variants in epileptic encephalopathies identified to date. Given the predicted dominant-negative mechanism of this mutation, this variant presents a prime target for therapeutic intervention.

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Disruptive technology disorder: A past, present, and future neurologic syndrome  Voir?

Based upon an analysis of 6 major historical technological advances over the last 150 years, a new syndrome, disruptive technology disorder (DTD), is introduced. DTD describes the human health ailments that accompany the implementation of disruptive technologies. Elevator sickness, railway spine, and bicycle face are representative examples. Though the underlying causative disruptive technologies may differ, many neurologic symptoms (headache, dizziness, weakness) are common to multiple DTDs. Born of technology-driven societal change, DTDs manifest as a complex interplay between biological and psychological symptoms.

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Real-world experience of treatment decision-making in carotid stenosis in a neurovascular board  Voir?

Objective:

To describe our experience with consensus-based decision-making for treatment of internal carotid artery (ICA) stenosis by neurologists, interventional neuroradiologists, vascular surgeons, and neurosurgeons in a multidisciplinary neurovascular board and to study adherence to treatment recommendations in the context of uncertainty with respect to the best treatment option.

Methods:

We established a multidisciplinary neurovascular board meeting twice a week with structured documentation of consensus decisions. Over a time period of 53 months, 614 cases with ICA stenosis were discussed, with 285 (46%) symptomatic and 279 (45%) asymptomatic cases.

Results:

Recommendation for symptomatic ICA stenosis was revascularization in 76%, medical management alone in 8%, and further diagnostics in 16%. For asymptomatic ICA stenosis, recommendation was randomization in a clinical trial in 29%, revascularization in 27%, medical management alone in 23%, and further diagnostics in 22%. Treatment recommendations were followed in 94% of symptomatic ICA stenosis and 69% of asymptomatic ICA stenosis. Patients in whom carotid artery stenting was recommended for revascularization were younger and showed a higher rate of severe (≥70%) ICA stenosis.

Conclusions:

Interdisciplinary board decisions are a helpful and transparent tool to assure adherence to guideline recommendations, and to provide consensus-based individualized treatment strategies in clinical practice in the absence of unequivocal evidence.

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Enteroviral T-cell encephalitis related to immunosuppressive therapy including rituximab  Voir?

Enteroviruses are a common cause of aseptic meningitis and mild infections in childhood. However, in immunocompromised patients they may cause severe neurologic conditions such as encephalitis or polio-like paralysis.

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Editors' Note  Voir?

Editors' Note: Comments in WriteClick this week relate to "Neonatal vitamin D status and risk of multiple sclerosis: A population-based case-control study." Dr. Zarghami asks whether sun exposure may be a more cost-effective measure than vitamin D supplementation during pregnancy and suggests randomized clinical trials in the future to compare the 2 interventions. Authors Nielsen et al. respond that a clinical trial of that nature would not be possible due to the relatively low frequency of multiple sclerosis (MS) and the long-time course between intervention and disease onset.

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Letter re: Neonatal vitamin D status and risk of multiple sclerosis: A population-based case-control study  Voir?

In the article by Nielsen et al.,1 the authors supported justification of routine vitamin D supplementation during pregnancy. The region type and ethnic-related characteristics of the study population were well-underlined in the editorial.2 The distribution of multiple sclerosis (MS) might vary in different populations with different geographic characteristics, sociodemographic characteristics, and genetic predisposition.3 Both the current study and similar previous studies were conducted among Scandinavian populations with genetic homogeneity.1,4 Conversely, the geographic placement of these neighboring countries and the resulting lower sun exposures could be a pivotal environmental predisposing factor. The probable independent roles of sun exposure and vitamin D status in the pathophysiology of CNS demyelination of MS were considered.5

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Letter re: Neonatal vitamin D status and risk of multiple sclerosis: A population-based case-control study  Voir?

We read with interest the article by Nielsen et al.,1 which described an inverse association between neonatal levels of 25(OH)D and risk of multiple sclerosis (MS), similar to the recent study by Munger et al.2 However, both articles might only reflect a short-time influence of 25(OH)D, which could be affected by environmental factors (e.g., sun exposure and dietary structure). Two recent studies provided new insights into the effects of latitude and vitamin D supplementation on the disease course in MS,3,4 indicating that a long-time cumulative effect of environmental factors might play an important role in the risk of MS, rather than short-time changes. Furthermore, in the current study, there was a higher rate of parental MS in the MS group,1 which suggests a possible association with genetic variations. These findings support the recommendation of vitamin D supplements to potential beneficiaries who have low serum 25(OH)D levels, have a history of parental MS or are patients with MS, and lack enough sun exposure.

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Author response: Neonatal vitamin D status and risk of multiple sclerosis: A population-based case-control study  Voir?

In response to our article,1 Dr. Zarghami suggests that clinical trials are necessary to compare the efficacy and cost–benefit of vitamin D supplementation vs sun exposure in pregnancy. Such trials are not feasible because of the relatively low frequency of multiple sclerosis (MS) and the long interval between any intervention in pregnancy and the observation of its potential effect on MS risk in offspring. We agree, nevertheless, that judicious sun exposure provides an alternative approach for the correction of vitamin D deficiency in regions with levels of ultraviolet B radiation high enough to support year-round vitamin D production. This is not the case in countries like Denmark, where winter sunlight cannot produce sufficient vitamin D levels.2

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Balo concentric sclerosis evolving from apparent tumefactive demyelination  Voir?

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Dernière mise à jour : 26/07/2017 : 02:03


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