cover image European Journal of Neurology

European Journal of Neurology

2016 - Volume 23
Issue 4 | April 2016
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Editorial

Sirkka‐Liisa Leinonen

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Authors:
A. H. V. Schapira, A. Albanese, M. Brainin, C. Davie, S. Juvela, M. Leone, J. Meschia, P. Saloheimo, E.‐K. Tan and Z. Wszolek
Article first published online:
26 Mar 2015 | DOI: 10.1111/ene.12697

Editorial

The European Association of Young Neurologists and Trainees in 2015: strengthening collaboration with the European Academy of Neurology

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Authors:
P. Balicza, O. Györfi, V. Papp, A. Sauerbier, M. Moarcas, A. Macerollo and J. Sellner
Article first published online:
14 May 2015 | DOI: 10.1111/ene.12721

CME Article

Background and purpose

The temporal course of recanalization and its association with clinical outcome were analysed in our patients with cerebral sinus and/or venous thrombosis (CSVT) and follow‐up magnetic resonance imaging (MRI).

Methods

Between January 1998 and September 2014 all patients from our institutions with CSVT were systematically analysed. Baseline data, treatment characteristics and follow‐up MRI were retrospectively recorded. The status of recanalization was assessed as complete (CRec), partial (PRec) or failed recanalization. Clinical follow‐up was measured with the modified Rankin Scale. Excellent outcome was defined as modified Rankin Scale 0–1.

Results

Ninety‐nine patients were identified; 97% of these patients were treated with oral anticoagulation (OAC) and the median (min–max) time of OAC was 7 months (1–84). CRec was achieved in 57.6% (57/99), PRec in 29.3% (29/99) and only 13 (13.1%) patients did not recanalize. The median (min–max) time to PRec was 4 months (0.25–14) and to CRec 6 months (2–34). Median time to last clinical follow‐up was 8 months (1–88); 91.8% (89/99) had an excellent outcome at last clinical follow‐up and only 2.1% (2/99) died. Only thrombosis of the superior sagittal sinus was independently associated with successful recanalization (odds ratio 16, 95% confidence interval 2–138). No severe haemorrhagic complications and no recurrence of CSVT occurred within clinical follow‐up. No association of outcome and recanalization status was found.

Conclusions

The recanalization rate of CSVT under OAC was high and the median time to CRec was 6 months. Thrombosis of the superior sagittal sinus is a positive predictor of recanalization. Outcome in this cohort was excellent but no significant association of outcome and recanalization status was found.

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Authors:
C. Herweh, M. Griebe, C. Geisbüsch, K. Szabo, E. Neumaier‐Probst, M. G. Hennerici, M. Bendszus, P. A. Ringleb and S. Nagel
Article first published online:
19 Nov 2015 | DOI: 10.1111/ene.12901

Review Article

Abstract

Recently, diagnostic clinical and imaging criteria for primary progressive aphasia (PPA) have been revised by an international consortium (Gorno‐Tempini et al. Neurology 2011;76:1006‐14). The aim of this study was to validate the specificity of the new imaging criteria and investigate whether different imaging modalities [magnetic resonance imaging (MRI) and fluorodeoxyglucose positron emission tomography (FDG‐PET)] require different diagnostic subtype‐specific imaging criteria. Anatomical likelihood estimation meta‐analyses were conducted for PPA subtypes across a large cohort of 396 patients: firstly, across MRI studies for each of the three PPA subtypes followed by conjunction and subtraction analyses to investigate the specificity, and, secondly, by comparing results across MRI vs. FDG‐PET studies in semantic dementia and progressive nonfluent aphasia. Semantic dementia showed atrophy in temporal, fusiform, parahippocampal gyri, hippocampus, and amygdala, progressive nonfluent aphasia in left putamen, insula, middle/superior temporal, precentral, and frontal gyri, logopenic progressive aphasia in middle/superior temporal, supramarginal, and dorsal posterior cingulate gyri. Results of the disease‐specific meta‐analyses across MRI studies were disjunct. Similarly, atrophic and hypometabolic brain networks were regionally dissociated in both semantic dementia and progressive nonfluent aphasia. In conclusion, meta‐analyses support the specificity of new diagnostic imaging criteria for PPA and suggest that they should be specified for each imaging modality separately.

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Authors:
S. Bisenius, J. Neumann and M. L. Schroeter
Article first published online:
22 Feb 2016 | DOI: 10.1111/ene.12902

Letter to the Editor

Beyond spinal muscular atrophy with lower extremity dominance: cerebellar hypoplasia associated with a novel mutation in

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Authors:
C. Fiorillo, F. Moro, G. Brisca, A. Accogli, F. Trucco, R. Trovato, M. Pedemonte, M. Severino, M. Catala, V. Capra, F. M. Santorelli, C. Bruno, A. Rossi and C. Minetti
Article first published online:
18 Mar 2016 | DOI: 10.1111/ene.12914

Letter to the Editor

Stiff leg syndrome after epidural anesthesia

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Authors:
J. R. Pérez‐Sánchez, A. Contreras Chicote, B. Gutiérrez‐Ruano, B. De La Casa‐Fages, A. Traba and F. Grandas
Article first published online:
18 Mar 2016 | DOI: 10.1111/ene.12923

Original Article

Background and purpose

Intrathecal immunoglobulin (Ig) synthesis occurs in various chronic inflammatory neurological diseases. Different formulae have been developed for quantitative determination of Ig synthesis within the cerebrospinal fluid (CSF) compartment. The hyperbolic formula of Reiber is frequently used which, however, returns a considerable number of false positive results in empirical observations.

Methods

A computerized database of more than 19 000 paired CSF and serum samples was screened for patients presumed negative for local Ig synthesis and a new formula characterizing this collective was calculated. The validity of this formula was confirmed by several validation steps.

Results

A cohort of 1173 patients with normal CSF findings was used for quantile regression. The 97.5th quantile of the formula was considered as the cut‐off curve for intrathecal Ig synthesis using different constants and for IgG, IgA and IgM. Compared to the Reiber formula, a lower level of false positive results was produced especially for IgM and IgA which was confirmed in a separate clinically well defined validation cohort. In 77 patients with discrepant findings between Reiber and our formula no specific diagnoses were found confirming the low diagnostic value of borderline Ig synthesis.

Conclusions

A new approximation formula was developed for determination of intrathecal Ig synthesis which produces fewer false positive results without reducing diagnostic sensitivity.

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Authors:
M. Auer, H. Hegen, A. Zeileis and F. Deisenhammer
Article first published online:
25 Jan 2016 | DOI: 10.1111/ene.12924

Original Article

Background and purpose

Sperm‐associated antigen 16 (SPAG16), a sperm protein which is upregulated in reactive astrocytes in multiple sclerosis (MS) lesions, has recently been identified as a novel autoantibody target in MS. The aim of this study was to investigate whether anti‐SPAG16 antibody levels differ between MS subtypes (relapsing−remitting, RR; primary or secondary progressive, PP, SP) and whether antibody positivity is associated with clinical characteristics.

Methods

Plasma anti‐SPAG16 antibody levels were determined by recombinant protein enzyme‐linked immunosorbent assay (ELISA) in 374 MS patients (274 RRMS, 39 SPMS and 61 PPMS) and 106 healthy controls.

Results

Significantly elevated anti‐SPAG16 antibodies were found in 22% of MS patients with 93% specificity. Anti‐SPAG16 seropositivity was associated with an increased Expanded Disability Status Scale (EDSS) in overall MS. A higher proportion of PPMS patients showed anti‐SPAG16 antibody reactivity (34%) compared to RRMS (19%) and SPMS (26%), and presented with higher anti‐SPAG16 antibody levels. Seropositive PPMS patients had a significantly increased progression index compared to seronegative patients.

Conclusions

Anti‐SPAG16 antibodies are associated with an increased EDSS in overall MS, indicating that they are linked to a worse MS disease outcome. Moreover, the presence of anti‐SPAG16 antibodies may be a biomarker for a more severe disease in PPMS patients, as indicated by an increased progression index.

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Authors:
L. de Bock, J. Fraussen, L. M. Villar, J. C. Álvarez‐Cermeño, B. Van Wijmeersch, V. van Pesch, P. Stinissen and V. Somers
Article first published online:
26 Dec 2015 | DOI: 10.1111/ene.12925

Letter to the Editor

Elevated aspartate aminotransferase and lactate dehydrogenase levels are a constant finding in ‐associated neurodegeneration

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Authors:
I. Kraoua, M. Romani, D. Tonduti, H. BenRhouma, G. Zorzi, F. Zibordi, A. Ardissone, N. Gouider‐Khouja, I. Ben Youssef‐Turki, N. Nardocci and E. M. Valente
Article first published online:
18 Mar 2016 | DOI: 10.1111/ene.12927

Letter to the Editor

Response to the letter ‘Type 2 diabetes and amyotrophic lateral sclerosis’

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Authors:
D. Mariosa and F. Fang
Article first published online:
18 Mar 2016 | DOI: 10.1111/ene.12928

Original Article

Background and Purpose

Early relapse outcomes in long‐term stable patients switching from interferon β/glatiramer acetate (IFNβ/GA) to oral therapy are unknown.

Objective

The objective of this study was to compare early relapse and progression in multiple sclerosis (MS) patients switching to oral therapy following a period of stable disease on IFNβ/GA, relative to a propensity‐matched comparator of patients remaining on IFNβ/GA.

Methods

The MSBase cohort study is a global, longitudinal registry for MS. Time to first 6‐month relapse in previously stable MS patients switching from platform injectables (‘switchers’) to oral agents were compared with propensity‐matched patients remaining on IFNβ/GA (‘stayers’) using a Cox marginal model.

Results

Three‐hundred and ninety‐six switchers were successfully matched to 396 stayers on a 1:1 basis. There was no difference in the proportion of patients recording at least one relapse in the first 1−6 months by treatment arm (7.3% switchers, 6.6% stayers; = 0.675). The mean annualized relapse rate ( = 0.493) and the rate of first 6‐month relapse by treatment arm (hazard ratio 1.22, 95% confidence interval 0.70, 2.11) were also comparable. There was no difference in the rate of disability progression by treatment arm (hazard ratio 1.43, 95% confidence interval 0.63, 3.26).

Conclusion

This is the first study to compare early relapse switch probability in the period immediately following switch to oral treatment in a population previously stable on injectable therapy. There was no evidence of disease reactivation within the first 6 months of switching to oral therapy.

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Authors:
T. Spelman, L. Mekhael, T. Burke, H. Butzkueven, S. Hodgkinson, E. Havrdova, D. Horakova, P. Duquette, G. Izquierdo, F. Grand'Maison, P. Grammond, M. Barnett, J. Lechner‐Scott, R. Alroughani, M. Trojano, A. Lugaresi, F. Granella, E. Pucci and S. Vucic
Article first published online:
19 Jan 2016 | DOI: 10.1111/ene.12929

Original Article

Background and purpose

Hemosiderin exhibits a stronger T2 shortening effect than deoxyhemoglobin. The extent of the ‘blooming artifact’ may therefore reflect a composition of different iron forms. Our aim was to investigate the relationship between extent of susceptibility vessel sign (SVS) width beyond the lumen and middle cerebral artery (MCA) recanalization.

Methods

Clinical and imaging data from consecutive acute ischaemic stroke patients with MCA occlusion who underwent susceptibility‐weighted imaging (SWI) before intravenous thrombolysis were examined. The source images of magnitude and angiography were used to obtain the width of SVS and MCA at the interface, respectively.

Results

The presence of MCA SVS was observed in 64 patients on initial SWI scans and recanalization was observed in 30 (46.9%) patients. The overestimation ratio of thrombus width on SWI was an acceptable predictor for no recanalization [odds ratio 1.360 per 0.1; 95% confidence interval (CI) 1.093−1.691; = 0.006]. The optimal cut‐off point was identified at 1.943, and this yielded a sensitivity of 67.6% and a specificity of 86.7%. Extensive blooming artifact, defined as overestimation ratio ≥2, independently predicted no recanalization (odds ratio 9.687, 95% CI 1.974−47.545; = 0.005) and unfavorable outcome (odds ratio 4.916, 95% CI 1.049−23.051; = 0.043).

Conclusions

The extent of SVS width beyond the lumen might reflect the content of hemosiderin. An extreme overestimation ratio might indicate aged thrombus, which may be resistant to thrombolysis.

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Authors:
S. Yan, Q. Chen, X. Zhang, M. Xu, Q. Han, A. Shao, D. S. Liebeskind and M. Lou
Article first published online:
26 Dec 2015 | DOI: 10.1111/ene.12930

Original Article

Background and purpose

Depression is the most common psychiatric disorder in multiple sclerosis (MS). Self‐report depression scales are frequently used as screening, diagnostic and grading instruments. This study investigated the psychometric properties of the Beck Depression Inventory second edition (BDI‐II) for assessing depressive disorders in a sample of Italian MS patients.

Methods

The sample included 141 consecutive non‐demented MS patients who completed the BDI‐II and the Chicago Multiscale Depression Inventory (CMDI). MS patients also completed a clinical interview, a neurological/neuropsychological examination and a Fatigue Severity Score (FSS) questionnaire in order to assess divergent validity.

Results

The BDI‐II showed good internal consistency (Cronbach's alpha 0.89) and good convergent and divergent validity. With respect to CMDI serving as the ‘gold standard’, the receiver operating characteristic curve revealed that BDI‐II is an adequate diagnostic measure and that the optimum total cut‐off score was 18.5. Such score identified clinically relevant depressive symptoms in 25.5% of our MS sample.

Conclusions

The BDI‐II is a simple, reliable and valid tool for detecting and grading depressive symptoms in Italian MS patients.

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Authors:
R. Sacco, G. Santangelo, S. Stamenova, A. Bisecco, S. Bonavita, L. Lavorgna, L. Trojano, A. D'Ambrosio, G. Tedeschi and A. Gallo
Article first published online:
19 Jan 2016 | DOI: 10.1111/ene.12932

Original Article

Background and purpose

It has been postulated that stress is part of the etiological process of Parkinson's disease (PD). The risk of PD was examined in a cohort of patients with adjustment disorders, a diagnosis made in the presence of a severe response to a stressful life event.

Methods

Using Danish medical registries, PD occurrence was examined in a nationwide population‐based cohort of patients with adjustment disorder diagnosed between 1995 and 2011. The standardized incidence ratio of PD was calculated as the ratio of observed to expected cases, stratified by time and potential risk factors, including depression and anxiety.

Results

Our adjustment disorder cohort (67 786 patients) was followed for a median of 8 years (interquartile range 4, 12.6 years). During follow‐up, 119 patients developed PD, versus 64 expected, corresponding to a standardized incidence ratio of 1.84 (95% confidence interval 1.53, 2.20). Consistent results were observed after stratification on potential risk factors, including depression and anxiety.

Conclusion

Adjustment disorder, a diagnosis made in the presence of severe response to stressful life events, was associated with an increased risk of PD.

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Authors:
E. Svensson, D. K. Farkas, J. L. Gradus, T. L. Lash and H. T. Sørensen
Article first published online:
12 Jan 2016 | DOI: 10.1111/ene.12933

Original Article

Background and purpose

Routine screening for asymptomatic cranial‐carotid stenosis (ACCS) is controversial and recommendation in clinical practice is vague. The ankle−brachial index (ABI) is reported as a predictor for cardiovascular disease. However, there is a scarcity of data about the association between abnormal ABI and ACCS. A population‐based cross‐sectional study was conducted to explore the relationship between ABI and ACCS.

Methods

A sample of 5440 Chinese adults aged 40–94 years old was recruited from 2010 to 2011. The ABI was measured using a portable Doppler device and ACCS was evaluated by bilateral carotid duplex ultrasound and portable examination devices. A logistic regression model was used to analyse the association between ABI and ACCS after adjusting for potential confounding factors.

Results

A low ABI was associated with ACCS [odds ratio (OR) 1.95, 95% confidence interval (CI) 1.42–2.67] after adjusting for potential confounders. When the data were stratified by age and sex, the correlation remained statistically significant in the male (OR 2.32, 95% CI 1.60–3.37) and elderly (OR 3.07, 95% CI 1.97–4.78) subgroups compared to the female (OR 1.26, 95% CI 0.67–2.39) and middle‐aged groups (OR 1.27, 95% CI 0.77–2.12), respectively.

Conclusion

This study demonstrated that low ABI is a significant risk factor for ACCS in male and elderly Chinese adults.

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Authors:
J. Qiu, Y. Zhou, X. Yang, Y. Zhang, Z. Li, N. Yan, Y. Wang, S. Ge, S. Wu, X. Zhao and W. Wang
Article first published online:
20 Jan 2016 | DOI: 10.1111/ene.12935

Original Article

Background and purpose

SPG7 is one of the most common forms of autosomal recessive hereditary spastic paraplegia. The phenotype has been shown to be heterogeneous, varying from a complex spastic ataxia to pure spastic paraplegia or pure ataxia. The aim of this study was to clinically and genetically characterize patients with SPG7 in Norway.

Methods

Six Norwegian families with a clinical diagnosis of hereditary spastic paraplegia were diagnosed with SPG7 through Sanger sequencing and whole‐exome sequencing. Haplotypes were established to identify a possible founder mutation. All patients were thoroughly examined and the clinical and molecular findings are described.

Results

The core phenotype was spastic paraparesis with ataxia, bladder disturbances and progressive external ophthalmoplegia. The variant p.H701P was identified in homozygous state in one family and in compound heterozygous state in three families. Haplotype analysis of seven surrounding single nucleotide polymorphisms supports that this variant resides on a founder haplotype. Four of the families were compound heterozygous for the previously well‐described p.A510V variant.

Conclusion

SPG7 is a common subgroup of hereditary spinocerebellar disorders in Norway. The broad phenotype in the Norwegian SPG7 population illustrates the challenges with the traditional dichotomous classification of hereditary spinocerebellar disorders into hereditary spastic paraplegia or hereditary ataxia. A Norwegian founder mutation p.H701P was identified in four out of six families, making it a major cause of SPG7 in Norway.

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Authors:
S. L. Rydning, I. M. Wedding, J. Koht, M. Chawla, A.‐M. Øye, Y. Sheng, M. D. Vigeland, K. K. Selmer and C. M. E. Tallaksen
Article first published online:
12 Jan 2016 | DOI: 10.1111/ene.12937

Letter to the Editor

Low serum levels of vitamin D are associated with post‐stroke depression

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Authors:
A. Kaloglu, B. Canbaz, O. Kara, G. Arik, G. Sengul Aycicek, F. Sumer and Z. Ulger
Article first published online:
18 Mar 2016 | DOI: 10.1111/ene.12938

Original Article

Background and purpose

Dystonia is difficult to recognize due to its large phenomenological complexity. Thus, the use of experts in dystonia is essential for better recognition and management of dystonia syndromes (DS). Our aim was to document managing strategies, facilities and expertise available in various European countries in order to identify which measures should be implemented to improve the management of DS.

Methods

A survey was conducted, funded by the Cooperation in Science and Technology, via the management committee of the European network for the study of DS, which is formed from representatives of the 24 countries involved.

Results

Lack of specific training in dystonia by general neurologists, general practitioners as well as other allied health professionals was universal in all countries surveyed. Genetic testing for rare dystonia mutations is not readily available in a significant number of countries and neurophysiological studies are difficult to perform due to a lack of experts in this field of movement disorders. Tetrabenazine is only readily available for treatment of dystonia in half of the surveyed countries. Deep brain stimulation is available in three‐quarters of the countries, but other surgical procedures are only available in one‐quarter of countries.

Conclusions

Internationally, collaboration in training, advanced diagnosis, treatment and research of DS and, locally, in each country the creation of multidisciplinary teams for the management of dystonia patients could provide the basis for improving all aspects of dystonia management across Europe.

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Authors:
A. Valadas, M‐F. Contarino, A. Albanese, K. P. Bhatia, C. Falup‐Pecurariu, L. Forsgren, A. Friedman, N. Giladi, M. Hutchinson, V. S. Kostic, J. K. Krauss, A. Lokkegaard, M. J. Marti, I. Milanov, Z. Pirtosek, M. Relja, M. Skorvanek, M. Stamelou, A. Stepens, G. Tamás, A. Taravari, C. Tzoulis, W. Vandenberghe, M. Vidailhet, J. J. Ferreira and M. A. Tijssen
Article first published online:
29 Jan 2016 | DOI: 10.1111/ene.12940

Original Article

Background and purpose

Our objective was to evaluate the extent to which the 2005 recommendations of the European Federation of Neurological Sciences (EFNS) on the multidisciplinary management of amyotrophic lateral sclerosis (ALS) are followed in clinical practice.

Methods

This was a multicentre observational study involving six French ALS referral centres receiving prevalent and incident cases. Recommendations were translated into questions referring to key aspects of management, and their application was evaluated by a clinical research assistant who independently examined the medical charts (MCs). When necessary, an independent board‐certified neurologist answered the questions based on examination of the MC and interview of the caring neurologist. Questions regarding diagnosis and communication were put to patients in a self‐administered questionnaire.

Results

In all, 376 patients [176 incident, 200 prevalent cases; median age at diagnosis 62.8 years (interquartile range 55.7–72.3); sex ratio 1.37; 27.3% bulbar onset] were included. All the topics covered in the recommendations were evaluated: diagnostic delay (e.g. mean 13.6 months, associated with age and onset); breaking the news (e.g. criteria for communication quality were satisfactory in more than 90%); multidisciplinary and sustained support (e.g. clinic visits were scheduled every 2–3 months in 90%). Also considered were whether riluzole had been offered, symptom management, genetic testing, ventilation, communication defects, enteral nutrition, palliative and end‐of‐life care. Characteristics associated with poor compliance with some guidelines (schedule of visits, delayed riluzole initiation) were also identified.

Conclusion

This is the first evaluation of the application of the EFNS recommendations for the management of ALS in a nationwide sample. The results allow us to highlight areas for improvement.

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Authors:
B. Marin, E. Beghi, C. Vial, E. Bernard, G. Lautrette, P. Clavelou, N. Guy, G. Lemasson, S. Debruxelles, P. Cintas, J. C. Antoine, J. P. Camdessanche, G. Logroscino, P. M. Preux and P. Couratier
Article first published online:
01 Feb 2016 | DOI: 10.1111/ene.12941

Original Article

Background and purpose

Clinical symptoms and long‐term outcome of autoimmune encephalitis are variable. Diagnosis requires multiple investigations, and treatment strategies must be individually tailored. Better biomarkers are needed for diagnosis, to monitor disease activity and to predict long‐term outcome. The value of cerebrospinal fluid (CSF) markers of neuronal [neurofilament light chain protein (NFL), and total tau protein (T‐tau)] and glial cell [glial fibrillary acidic protein (GFAP)] damage in patients with autoimmune encephalitis was investigated.

Methods

Demographic, clinical, magnetic resonance imaging, CSF and antibody‐related data of 25 patients hospitalized for autoimmune encephalitis and followed for 1 year were retrospectively collected. Correlations between these data and consecutive CSF levels of NFL, T‐tau and GFAP were investigated. Disability, assessed by the modified Rankin scale, was used for evaluation of disease activity and long‐term outcome.

Results

The acute stage of autoimmune encephalitis was accompanied by high CSF levels of NFL and T‐tau, whereas normal or significantly lower levels were observed after clinical improvement 1 year later. NFL and T‐tau reacted in a similar way but at different speeds, with T‐tau reacting faster. CSF levels of GFAP were initially moderately increased but did not change significantly later on. Final outcome (disability at 1 year) directly correlated with CSF‐NFL and CSF‐GFAP levels at all time‐points and with CSF‐T‐tau at 3 ± 1 months. This correlation remained significant after age adjustment for CSF‐NFL and T‐tau but not for GFAP.

Conclusion

In autoimmune encephalitis, CSF levels of neuronal and glial cell damage markers appear to reflect disease activity and long‐term disability.

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Authors:
R. Constantinescu, D. Krýsl, F. Bergquist, K. Andrén, C. Malmeström, F. Asztély, M. Axelsson, E. B. Menachem, K. Blennow, L. Rosengren and H. Zetterberg
Article first published online:
29 Jan 2016 | DOI: 10.1111/ene.12942

Original Article

Background and purpose

In the last few months five multicentre, randomized controlled trials (RCTs) unequivocally showed the superiority of mechanical thrombectomy in large vessel occlusion acute ischaemic stroke compared to systemic thrombolysis. Despite varying inclusion criteria and time intervals from onset to revascularization overall increases of good functional outcome between 55% and 81% were reported. However, only a minority of screened patients (approximately 1%) were eligible for intra‐arterial (IA) therapy.

Methods

An investigator‐initiated, single‐centre, prospective and blinded end‐point analysis was performed of 3123 consecutive patients with acute ischaemic stroke presenting between February 2010 and December 2014.

Results

One hundred and fifty‐four patients [4.9%, age (years) mean (SD), median (interquartile range) 71.2 (±14), 74.7 (65.9–81.4)] met the inclusion criteria of sparse early ischaemic signs on initial standard cranial computed tomography (CT) (ASPECT score ≥7), large vessel occlusion in the anterior circulation on CT angiography and start of treatment within 6 h of onset of symptoms. After consensual interdisciplinary treatment decisions 130 patients (4.2%) received IA treatment – in the majority stent‐assisted thrombectomy in combination with intravenous (IV) recombinant tissue plasminogen activator – and 24 patients (0.7%) standard IV thrombolysis. On 3 months’ follow‐up an overall significant improvement of disability ( = 0.05) as measured by the modified Rankin Scale was shown in favour of the IA treatment group. Good functional outcome was achieved in about twice as many patients (IA vs. IV, 41.2% vs. 21.2%; = 0.078).

Conclusion

By choosing pragmatic inclusion criteria state‐of‐the‐art IA therapy of a specialized tertiary stroke centre can be safely applied under real‐world conditions to a higher percentage of patients with similar success to the recently published RCTs.

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Authors:
O. Nikoubashman, M. Jungbluth, K. Schürmann, M. Müller, B. Falkenburger, S. C. Tauber, M. Wiesmann, J. B. Schulz and A. Reich
Article first published online:
05 Feb 2016 | DOI: 10.1111/ene.12944

Original Article

Background and purpose

Migraine is frequent in patients with systemic lupus erythematosus (SLE), but the pathogenesis and pathophysiology are poorly understood. Migraine is assumed to be a consequence of abnormal neuronal excitability. Based on the hypothesis that the threshold for migraine is lower in SLE patients due to cerebral disturbances, whether structural abnormalities of the brain or relevant biomarkers are associated with headaches in SLE was investigated.

Methods

Sixty‐seven SLE patients and age‐ and gender‐matched healthy subjects participated. Volumes of grey matter (GM) and white matter (WM) were estimated from cerebral magnetic resonance images with SPM8 software. Anti‐NR2 and anti‐P antibodies and protein S100B were measured in cerebrospinal fluid.

Results

In regression analyses, larger GM volumes in SLE patients reduced the odds for headache in general [odds ratio (OR) 0.98, =0.048] and for migraine in particular (OR 0.95, =0.004). No localized loss of GM was observed. Larger WM volumes in patients increased the odds for migraine (OR 1.04, =0.007). These findings could not be confirmed in healthy subjects. Neither anti‐NR2 and anti‐P antibodies nor S100B were associated with headaches in SLE patients.

Conclusions

Systemic lupus erythematosus patients with migraine have a diffuse reduction in GM compared to patients without migraine. This finding was not observed in healthy subjects with migraine, and selected biomarkers did not indicate specific pathophysiological processes in the brain. These findings indicate that unknown pathogenic processes are responsible for the increased frequency of migraine in SLE patients.

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Authors:
A. B. Tjensvoll, M. B. Lauvsnes, S. Hirohata, M. K. Beyer, O. J. Greve, I. Kvivik, J. T. Kvaløy, E. Harboe, L. G. Gøransson and R. Omdal
Article first published online:
20 Jan 2016 | DOI: 10.1111/ene.12946

Original Article

Background and purpose

Hereditary diffuse leukoencephalopathy with spheroids (HDLS) is an autosomal dominant disorder caused by colony‐stimulating factor 1 receptor () gene mutations, resulting in demyelination and axonal degeneration with spheroids. The clinical expression is variable, including behavioral changes, cognitive impairment, motor symptoms and parkinsonism. Magnetic resonance imaging (MRI) reveals white matter (WM) changes and atrophy. The indistinct phenotype has led to misdiagnoses. This study's aim was to compare brain volumetry and radiological ratings in HDLS with multiple sclerosis (MS) patients and controls.

Methods

Five HDLS patients with c.2562T>A p.Asn854Lys mutation, five age‐ and gender‐matched MS patients and five healthy controls were cross‐sectionally studied. All patients were examined neurologically. HDLS patients underwent Mini‐Mental State Examination (MMSE). Brain MRI scans were analyzed volumetrically with FreeSurfer and Lesion Segmentation Toolbox and neuroradiologically with the brain MRI scoring system for HDLS.

Results

Patients with HDLS had lower brain, grey matter and WM fractions (66.3%; 37.9%; 27.6%) compared with controls (78.5%, = 0.008; 44.4%, = 0.008; 32.0%, = 0.008), but not compared with MS patients (65.7%, = 0.7; 36.8%, = 0.4; 27.3%, = 0.7). Cerebellar WM changes and atrophy were not seen in the HDLS group. The HDLS lesion volume fraction correlated with MMSE scores ( = −0.90, = 0.04).

Conclusions

Brain volume fractions in HDLS were lower than in controls and similar to those seen in MS. The cerebellum was relatively spared in HDLS, which may help in differentiating HDLS WM changes from MS. The strong relationship of HDLS lesions with MMSE scores indicates that accumulating WM pathology in HDLS is associated with cognitive decline.

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Authors:
T. Granberg, F. Hashim, O. Andersen, C. Sundal and V. D. Karrenbauer
Article first published online:
12 Jan 2016 | DOI: 10.1111/ene.12948

Original Article

Background and purpose

Numerous studies have shown that repetitive transcranial magnetic stimulation (rTMS) over the primary motor cortex (M1) may improve motor function of the affected hand after stroke. The effects of 1 Hz rTMS applied over the contralesional dorsal premotor cortex (PMd) on hand function and cortical neurophysiology in subacute stroke were examined.

Methods

Ten subacute stroke patients with mild hand motor impairment were enrolled in a prospective, double‐blind, randomized, placebo‐controlled, crossover study with two intervention sessions. 1 Hz rTMS was applied over the contralesional PMd (real rTMS, 900 pulses at 110% of the motor threshold; sham rTMS, 900 pulses at 0% of the motor threshold). Tests of hand function (Jebsen‐Taylor hand function test, box and block test) and neurophysiological evaluations (resting motor threshold, motor evoked potentials, cortical silent period, ipsilateral silent period) were obtained from both hands and hemispheres prior to (baseline) and after each treatment.

Results

Hand function tests revealed significant improvement of motor function of the affected but not of the unaffected hand after real rTMS only. Neither intervention changed the neurophysiological measures in comparison to baseline.

Conclusion

One hertz rTMS over the contralesional PMd improves motor function of the affected hand in subacute stroke. The PMd may be a novel rTMS target to treat motor impairment after stroke.

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Authors:
J. Lüdemann‐Podubecká, K. Bösl and D. A. Nowak
Article first published online:
23 Jan 2016 | DOI: 10.1111/ene.12949

Original Article

Background and purpose

Conjugate eye deviation (CED) and horizontal skew deviation are often seen in patients with intracerebral hemorrhage (ICH), but its prognostic significance is unclear. In this study, the association between brain scan assessed eye position and hospital mortality in patients with supratentorial ICH was tested.

Methods

A retrospective analysis was performed in 316 patients with supratentorial ICH. Eye position was measured on first brain computed tomography or magnetic resonance imaging. Patients with CED, horizontal skew deviation or no deviation were distinguished. The association between eye position and hospital mortality was assessed using logistic regression analysis.

Results

Conjugate eye deviation was present in 96 (30.4%), skew deviation in 44 (13.9%) and no deviation in 176 (55.7%) patients. In patients with CED, 81.3% had an eye position to the ipsilateral side of the hemorrhage. In univariable regression analysis, skew deviation was associated with mortality (odds ratio 3.10, 95% confidence interval 1.57–6.11; = 0.001). In multivariable regression analysis, adjusting for age, ICH volume, intraventricular extension and Glasgow Coma Scale, eye position was not independently associated with mortality.

Conclusion

Horizontal skew eyes were found to be an unfavorable prognostic factor. However, this was not independent of other important predictors of ICH mortality and is most probably explained by its association with worse initial clinical presentation.

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Authors:
K. J. M. Frusch, R. Houben, F. H. B. M. Schreuder, A. A. Postma and J. Staals
Article first published online:
25 Jan 2016 | DOI: 10.1111/ene.12951

Editorial

Abstract

Click to view the accompanying paper in this issue.

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Authors:
G. P. Modolo and G. S. do Olival
Article first published online:
18 Mar 2016 | DOI: 10.1111/ene.12952

Invited Review

Abstract

In this review we discuss the use of conventional (computed tomography, magnetic resonance imaging, ultrasound) and advanced muscle imaging modalities (diffusion tensor imaging, magnetic resonance spectroscopy) in hereditary and acquired myopathies. We summarize the data on specific patterns of muscle involvement in the major categories of muscle disease and provide recommendations on how to use muscle imaging in this field of neuromuscular disorders.

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Authors:
L. ten Dam, A. J. van der Kooi, C. Verhamme, M. P. Wattjes and M. de Visser
Article first published online:
18 Mar 2016 | DOI: 10.1111/ene.12984

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Article first published online:
18 Mar 2016 | DOI: 10.1111/ene.12996