cover image European Journal of Neurology

European Journal of Neurology

2017 - Volume 24
Issue 6 | June 2017
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Original Article

Background and purpose

Pompe disease is a rare inheritable muscle disorder for which enzyme replacement therapy (ERT) has been available since 2006. Uniform criteria for starting and stopping ERT in adult patients were developed and reported here.

Methods

Three consensus meetings were organized through the European Pompe Consortium, a network of experts from 11 European countries in the field of Pompe disease. A systematic review of the literature was undertaken to determine the effectiveness of ERT in adult patients on a range of clinical outcome measures and quality of life. A narrative synthesis is presented.

Results

Consensus was reached on how the diagnosis of Pompe disease should be confirmed, when treatment should be started, reasons for stopping treatment and the use of ERT during pregnancy. This was based on expert opinion and supported by the literature. One clinical trial and 43 observational studies, covering a total of 586 individual adult patients, provided evidence of a beneficial effect of ERT at group level. At individual patient level, the response to treatment varied, but factors associated with a patient's response to ERT were not described in many studies. Eleven observational studies focused on more severely affected patients, suggesting that ERT can also be beneficial in these patients. There are no studies on the effects of ERT in pre‐symptomatic patients.

Conclusions

This is the first European consensus recommendation for starting and stopping ERT in adult patients with Pompe disease, based on the extensive experience of experts from different countries.

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Authors:
A. T. van der Ploeg, M. E. Kruijshaar, A. Toscano, P. Laforêt, C. Angelini, R. H. Lachmann, S. I. Pascual Pascual, M. Roberts, K. Rösler, T. Stulnig, P. A. van Doorn, P. Y. K. Van den Bergh, J. Vissing, B. Schoser, Bruno Bembi, Alexander Broomfield, Matthias Boentert, Claude Desnuelle, Oliver Findling, Andreas Hahn, Jordi Díaz‐Manera, Thomas Hundsberger, Cornelia Kornblum, Franҫois Labarthé, Pascal Laforet, Karl‐Eugen Mengel, Tiziana Mongini, Wolfgang Muller‐Felber, Giancarlo Parenti, W. Pim Pijnappel, Nicolai Preisler, Sabrina Sacconi, Beril Talim, Marine Tardieu, Nadine A.M.E van der Beek and Stephan Wenninger
Article first published online:
06 May 2017 | DOI: 10.1111/ene.13285

Original Article

Background and purpose

Chronic hypoperfusion from athero‐stenotic lesions is thought to lead to better collateral recruitment compared to cardioembolic strokes. It was sought to compare collateral flow in stroke patients with atrial fibrillation (AF) versus stroke patients with cervical atherosclerotic steno‐occlusive disease (CASOD).

Method

This was a retrospective review of a prospectively collected endovascular database. Patients with (i) anterior circulation large vessel occlusion stroke, (ii) pre‐treatment computed tomography angiography (CTA) and (iii) intracranial embolism from AF or CASOD were included. CTA collateral patterns were evaluated and categorized into two groups: absent/poor collaterals (CTA collateral score 0–1) versus moderate/good collaterals (CTA collateral score 2–4). CT perfusion was also utilized for baseline core volume and evaluation of infarct growth.

Results

A total of 122 patients fitted the inclusion criteria, of whom 88 (72%) had AF and 34 (27%) CASOD. Patients with AF were older ( < 0.01) and less often males or smokers ( = 0.04 and < 0.01 respectively). Baseline National Institutes of Health Stroke Scale and Alberta Stroke Program Early CT Score were comparable between groups. Collateral scores were lower in the AF group ( = 0.01) with patients having poor collaterals in 28% of cases versus 9% in the CASOD group ( = 0.03). Mortality rates (20% vs. 0%; = 0.02) were higher in the AF patients whilst rates of any parenchymal hemorrhage (6% vs. 26%; < 0.01) were higher in the CASOD group. On multivariable analysis, CASOD was an independent predictor of moderate/good collaterals (odds ratio 4.70; 95% confidence interval 1.17–18.79; = 0.03).

Conclusions

Atheroembolic strokes seem to be associated with better collateral flow compared to cardioembolic strokes. This may in part explain the worse outcomes of AF‐related stroke.

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Authors:
L. C. Rebello, M. Bouslama, D. C. Haussen, J. A. Grossberg, S. Dehkharghani, A. Anderson, S. R. Belagaje, N. A. Bianchi, M. Grigoryan, M. R. Frankel and R. G. Nogueira
Article first published online:
21 Apr 2017 | DOI: 10.1111/ene.13287

Original Article

Background and purpose

Intravenous thrombolysis (ivT) with recombinant tissue plasminogen activator is established in acute ischaemic stroke. Little is known, however, about its efficacy in different stroke subtypes.

Methods

A retrospective analysis of 128 733 patients from the Austrian Stroke Unit Registry was performed. Patients were classified as lacunar (LacS) or non‐lacunar ischaemic stroke (nonLacS) by use of the clinical syndrome and technical findings. Outcome parameters were the short term improvement in the stroke unit [the difference of the National Institutes of Health Stroke Scale (NIHSS) score at admission and at discharge] and the modified Rankin Scale (mRS) score at 3 months. Patients were assigned to four groups according to thrombolysis and stroke subtype. To control for confounding, patients were matched for variables identified with impact outcome and for variables of general interest (NIHSS at admission, mRS before stroke and general risk factors).

Results

There were four matched groups of 401 cases each. In LacS median short term improvement was 3 [interquartile range (IQR) 2–5] NIHSS points in the thrombolysed patients and 2 (IQR 1–4) in the non‐thrombolysed patients ( < 0.001). In the nonLacS groups median short term improvement was 3 (IQR 1–5) in the thrombolysed and 2 (IQR 0–4) in the non‐thrombolysed patients ( < 0.001). At 3‐month follow‐up, ivT was significantly associated with a better functional outcome in LacS ( < 0.001) and nonLacS patients ( < 0.001). Taking magnetic resonance imaging as a requirement for stroke subtyping led to similar results.

Conclusions

Patients with both lacunar and non‐lacunar stroke benefitted from ivT. The degree of improvement was similar in both groups.

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Authors:
C. C. J. Eggers, C. Bocksrucker and L. Seyfang
Article first published online:
27 Apr 2017 | DOI: 10.1111/ene.13288

Original Article

Background and purpose

New venues are currently being explored to predict disease progression in Parkinson's disease (PD), such as non‐motor subtypes and models merging motor and non‐motor symptoms (NMS). By involving a subgroup of 585 patients from the PRIAMO (Parkinson Disease Non‐motor Symptoms) study, the present 24‐month longitudinal prospective analysis aimed to demonstrate that urinary dysfunction is an early marker of higher motor and non‐motor burden as well as lower health‐related quality of life.

Methods and results

Multivariable mixed‐effect logistic regression models controlling for demographic and clinical variables showed that the following NMS domains were associated with urinary dysfunction: gastrointestinal [odds ratio (OR) 2.57, 95% confidence interval (CI) 1.67–3.97, < 0.001], cardiovascular (OR 2.22, 95% CI 1.18–4.17, = 0.013), skin (OR 1.81, 95% CI 1.06–3.08, = 0.029), sleep (OR 2.06, 95% CI 1.34–3.16, = 0.001), pain (OR 1.85, 95% CI 1.21–2.83, = 0.004), fatigue (OR 2.40, 95% CI 1.56–3.68, < 0.001), apathy (OR 2.79, 95% CI 1.72–4.52, < 0.001) and respiratory (OR 1.82, 95% CI 1.02–3.23, = 0.039). Analysis also demonstrated that urinary dysfunction was associated with higher motor disability (coefficient 1.73, 95% CI 0.68–2.78, = 0.001) and lower health‐related quality of life (coefficient −0.05, 95% CI −0.08 to −0.02, < 0.001, and coefficient −3.49, 95% CI −5.21 to −1.77, < 0.001) but not with more severe cognitive disability (coefficient −0.34, 95% CI −0.92 to 0.24, = 0.251).

Conclusions

This is the first prospective longitudinal study involving a large cohort of PD patients demonstrating the relevance of urinary dysfunction as an early marker of higher motor and non‐motor disability as well as lower health‐related quality of life. These findings support a role for urinary dysfunction as an early marker of more severe disease progression.

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Authors:
M. Picillo, R. Palladino, P. Barone, R. Erro, C. Colosimo, R. Marconi, L. Morgante and A. Antonini
Article first published online:
20 Apr 2017 | DOI: 10.1111/ene.13290

Letter to the Editor

Respiratory function in multiple sclerosis: is there a reliable test?

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Authors:
G. Fiorentino and A. M. Esquinas
Article first published online:
22 May 2017 | DOI: 10.1111/ene.13291

CME Article

Background and purpose

Intravenous alteplase (rtPA) may be associated with seizures and epileptic activity in the electroencephalogram (EEG). The aim of this work was to compare the frequency of seizures and EEG abnormalities between stroke patients treated and not treated with rtPA.

Methods

This was a prospective study of consecutive acute anterior circulation ischaemic stroke patients, with 1‐year follow‐up. Patients were previously independent, had an admission National Institute of Health Stroke Scale score ≥4, an acute ischaemic lesion and no previous seizures. They received standardized diagnostic and medical care. A video‐EEG was performed in 72 h (first EEG); during admission (daily until day 7 and after that if neurological worsening); at discharge and 1 year after stroke.

Results

In all, 151 patients (101 treated with rtPA) were included. The frequency of acute and remote symptomatic seizures was not significantly different between rtPA treated and non‐treated patients ( = 0.726 and = 0.748, respectively). Clinical paroxysmal phenomena during rtPA perfusion were observed in five (5%) patients. In the first EEG, rtPA treated patients more often had background diffuse slowing (43.6% vs. 26.0%, = 0.036). This difference was no longer observed at discharge (24.0% vs. 19.1%, = 0.517) nor 1 year after (11.8% vs. 10.0%, = 0.765). No differences were found in the frequency of epileptiform ( = 0.867) or periodic discharges ( = 0.381).

Conclusions

Intravenous alteplase is not associated with an increased risk of clinical or electroencephalographic epileptic phenomena.

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Authors:
C. Bentes, H. Martins, A. R. Peralta, C. Morgado, C. Casimiro, A. C. Franco, A. C. Fonseca, R. Geraldes, P. Canhão, T. Pinho e Melo, T. Paiva and J. M. Ferro
Article first published online:
18 Apr 2017 | DOI: 10.1111/ene.13292

Original Article

Background and purpose

One of the most widely studied perceptual measures of sensory dysfunction in dystonia is the temporal discrimination threshold (TDT) (the shortest interval at which subjects can perceive that there are two stimuli rather than one). However the elevated thresholds described may be due to a number of potential mechanisms as current paradigms test not only temporal discrimination but also extraneous sensory and decision‐making parameters. In this study two paradigms designed to better quantify temporal processing are presented and a decision‐making model is used to assess the influence of decision strategy.

Methods

22 patients with cervical dystonia and 22 age‐matched controls completed two tasks (i) temporal resolution (a randomized, automated version of existing TDT paradigms) and (ii) interval discrimination (rating the length of two consecutive intervals).

Results

In the temporal resolution task patients had delayed ( = 0.021) and more variable ( = 0.013) response times but equivalent discrimination thresholds. Modelling these effects suggested this was due to an increased perceptual decision boundary in dystonia with patients requiring greater evidence before committing to decisions (= 0.020). Patient performance on the interval discrimination task was normal.

Conclusions

Our work suggests that previously observed abnormalities in TDT may not be due to a selective sensory deficit of temporal processing as decision‐making itself is abnormal in cervical dystonia.

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Authors:
A. Sadnicka, C. Daum, C. Cordivari, K. P. Bhatia, J. C. Rothwell, S. Manohar and M. J. Edwards
Article first published online:
22 May 2017 | DOI: 10.1111/ene.13293

Original Article

Background and purpose

We investigated non‐acute headache patients’ long‐term satisfaction with a telemedicine consultation and consultation preferences in northern Norway. We hypothesized that patients were not less satisfied with telemedicine than traditional consultations. We also examined the influence of gender, age and education on satisfaction.

Methods

For 2.5 years, patients were consecutively screened, recruited and randomly assigned to telemedicine or traditional visits with a consultation at a neurological outpatient department. The primary endpoint was frequency of satisfied patients at 3 and 12 months. Secondary endpoints were satisfaction with consultation, communication, information, diagnosis, advice and prescriptions, and preferred visit form at 12 months.

Results

Of 402 participants, 279 (69.4%) answered questionnaires at both 3 and 12 month, and 291 (72.4%) responded at 12 months. The long‐term satisfaction of telemedicine patients was 124/145 (85.5%) compared with 118/134 (88.1%) in the traditional group ( = 0.653). The groups did not differ with respect to secondary endpoints, but females were more satisfied with telemedicine communication ( = 0.027). In the telemedicine group, 99/147 (67.3%) were indifferent to the type of consultation. Age and education did not alter the primary results.

Conclusions

At 1 year after a specialist evaluation for headache, telemedicine patients did not express less satisfaction than those with traditional consultation. Telemedicine specialist consultations may be a good alternative for headache patients in secondary care.

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Authors:
K. I. Müller, K. B. Alstadhaug and S. I. Bekkelund
Article first published online:
21 Apr 2017 | DOI: 10.1111/ene.13294

Original Article

Background and purpose

Amyotrophic lateral sclerosis (ALS) is characterized by focal disease onset with a predominantly contiguous pattern of disease spread. The pathophysiological mechanisms underlying disease progression in ALS have not been elucidated. Given that cortical hyperexcitability has been identified as an important pathogenic mechanism in ALS, the aim of the present study was to determine whether changes in cortical function could mediate disease spread in ALS.

Methods

Threshold‐tracking transcranial magnetic stimulation was undertaken in 50 patients with sporadic ALS with recording of responses over both abductor pollicis brevis muscles, with results matched to clinical assessments and concurrent neurophysiological investigation of lower motor neuron function. Subsequently, patients were followed longitudinally to map patterns of clinical disease progression.

Results

Cortical dysfunction was evident over both motor cortices, with hyperexcitability more prominent over the dominant motor cortex, contralateral to the site of disease onset, with reduction of resting motor threshold ( = 3.83, < 0.05), short‐interval intracortical inhibition ( = 15.0, < 0.0001) and cortical silent‐period duration ( = 8.01, < 0.01), along with an increase in motor evoked potential amplitude ( = 5.66, < 0.01). In addition, patterns of cortical change were consistent with a contiguous pattern of disease progression.

Conclusions

Cortical hyperexcitability appears to be more prominent over the dominant motor cortex, contralateral to the side of symptom onset, and contributes to a contiguous pattern of spread in sporadic ALS.

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Authors:
P. Menon, N. Geevasinga, M. van den Bos, C. Yiannikas, M. C. Kiernan and S. Vucic
Article first published online:
24 Apr 2017 | DOI: 10.1111/ene.13295

Original Article

Background and purpose

The social and economic consequences of comorbidity in multiple sclerosis (MS) are largely unexplored. Differences were investigated in income and in the rate of broken relationships between cases of MS with and without chronic comorbidity.

Methods

We conducted a nationwide cohort study including all incident cases of MS in Denmark with clinical MS onset between 1980 and 2005. The difference in income was investigated at MS onset and 5 and 10 years after MS onset. The difference in the rate of broken relationships was investigated in subjects who were in a relationship at MS onset or who entered a relationship after MS onset. We used logistic, multiple linear and Poisson regression analyses.

Results

Cases of MS with somatic comorbidity had increased odds of low incomes both 5 years {odds ratio (OR), 1.41 [95% confidence interval (CI), 1.19–1.67; < 0.0005]} and 10 years [OR, 1.37 (95% CI, 1.17–1.60); < 0.0005] after MS onset. The odds of a low income with psychiatric comorbidity was increased 10 years after MS onset [OR, 3.06 (95% CI, 1.47–6.37); = 0.003]. The rate of broken relationships was increased in cases of MS with any somatic comorbidity [incidence rate ratio, 1.46 (95% CI, 1.32–1.61); < 0.0005].

Conclusions

Our results underscore the burden of comorbidity in MS on patients, their partners and society.

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Authors:
A. Thormann, P. S. Sørensen, N. Koch‐Henriksen, L. C. Thygesen, B. Laursen and M. Magyari
Article first published online:
22 May 2017 | DOI: 10.1111/ene.13297

Letter to the Editor

Overestimating the risk of aspiration in acute stroke

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Authors:
M. Trapl, Y. Teuschl, K. Matz, A. Dachenhausen and M. Brainin
Article first published online:
22 May 2017 | DOI: 10.1111/ene.13298

Original Article

Background and purpose

Myasthenia gravis (MG) is an autoimmune disease caused by antibody mediated impairment in the neuromuscular junction. Seronegative MG (SNMG) without antibodies against acetylcholine receptor (AChR) and muscle‐specific kinase (MuSK) by routine assays accounts for about 20% of all MG patients.

Methods

Plasma from 81 Chinese MG patients previously found to be seronegative was tested by routine assays for AChR and MuSK antibodies. These samples were screened by (i) a novel, highly sensitive radioimmunoassay for AChR antibodies; (ii) cell‐based assays for clustered AChR, MuSK and lipoprotein receptor‐related protein 4 (LRP4) antibodies; (iii) a radioimmunoassay for titin antibodies.

Results

Antibodies to AChR, MuSK, LRP4 and titin were found in 25% (20/81), 4% (3/81), 7% (6/81) and 6% (5/78) of SNMG patients, respectively. In total, 37% of SNMG patients were found to be positive for at least one of the tested antibodies. AChR antibody positive patients had more severe disease ( = 0.008) and a trend towards fewer remissions/minimal manifestations than AChR antibody negative patients. The four patients with coexistence of antibodies had more severe disease, whilst the seronegative patients had milder MG ( = 0.015).

Conclusions

Detection of multiple muscle antibodies by more sensitive assays provides additional information in diagnosing and subgrouping of MG and may guide MG treatment.

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Authors:
Y. Hong, P. Zisimopoulou, N. Trakas, K. Karagiorgou, C. Stergiou, G. O. Skeie, H.‐J. Hao, X. Gao, J. F. Owe, X. Zhang, Y.‐X. Yue, F. Romi, Q. Wang, H.‐F. Li, N. E. Gilhus and S. J. Tzartos
Article first published online:
04 May 2017 | DOI: 10.1111/ene.13300

Original Article

Background and purpose

Many patients with multiple sclerosis (MS) report a worsening of symptoms due to high ambient temperatures, but objective data about this association are rare and contradictory. The aim of this study was to investigate the influence of ambient temperature on standard clinical tests.

Methods

We extracted the Symbol Digit Modality Test, Nine Hole Peg Test, Timed 25 Foot Walk (T25FW), Timed Tandem Walk, Expanded Disability Status Scale (EDSS) and quality‐of‐life items on cognition, fatigue and depression from our clinical database and matched them to historical temperatures. We used linear mixed‐effect models to investigate the association between temperature and outcomes.

Results

A total of 1254 patients with MS (mean age, 42.7 years; 69.9% females; 52.1% relapsing–remitting MS, mean EDSS, 3.8) had 5751 assessments between 1996 and 2012. We observed a worsening in the T25FW with higher ambient temperatures in moderately disabled patients (EDSS ≥ 4) but not in less disabled patients. However, an increase of 10°C prolonged the T25FW by just 0.4 s. Other outcomes were not associated with ambient temperatures.

Conclusions

Higher ambient temperature might compromise walking capabilities in patients with MS with a manifest walking impairment. However, effects are small and not detectable in mildly disabled patients. Hand function, cognition, mood and fatigue do not appear to be correlated with ambient temperature.

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Authors:
J.‐P. Stellmann, K. L. Young, E. Vettorazzi, J. Pöttgen and C. Heesen
Article first published online:
22 May 2017 | DOI: 10.1111/ene.13301

Original Article

Background and purpose

In many countries, Alzheimer's disease (AD) has gradually become a common disease in elderly populations. The aim of this study was to analyse trends of mortality caused by AD in the 28 member countries in the European Union (EU) over the last two decades.

Methods

We extracted data for AD deaths for the period 1994–2013 in the EU from the Eurostat and World Health Organization database. Age‐standardized mortality rates per 100 000 were computed. Joinpoint regression was used to analyse the trends and compute the annual percent change in the EU as a whole and by country. Analyses by gender and by European regions were conducted.

Results

Mortality from AD has risen in the EU throughout the study period. Most of the countries showed upward trends, with the sharpest increases in Slovakia, Lithuania and Romania. We recorded statistically significant increases of 4.7% and 6.0% in mortality rates in men and women, respectively, in the whole EU. Several countries showed changing trends during the study period. According to the regional analysis, northern and eastern countries showed the steepest increases, whereas in the latter years mortality has declined in western countries.

Conclusions

Our findings provide evidence that AD mortality has increased in the EU, especially in eastern and northern European countries and in the female population. Our results could be a reference for the development of primary prevention policies.

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Authors:
H. Niu, I. Alvarez‐Alvarez, F. Guillen‐Grima, M. J. Al‐Rahamneh and I. Aguinaga‐Ontoso
Article first published online:
22 May 2017 | DOI: 10.1111/ene.13302

Original Article

Background and purpose

We performed a meta‐analysis of randomized controlled trials to evaluate the efficacy and safety of pramipexole extended‐release (pramipexole ER) versus pramipexole immediate‐release (pramipexole IR) or placebo in Parkinson's disease.

Methods

We performed a systematic online search for clinical trials for pramipexole ER treatment up to 1 August 2016. We assessed differences in Unified Parkinson's Disease Rating Scale (UPDRS) scores, percentage of ‘on’ time or ‘off’ time, withdrawals, adverse events (AEs) and life quality between pramipexole ER and pramipexole IR or placebo. Data analyses were performed by the Cochrane Collaboration's Review Manager 5.3 software.

Results

Six randomized controlled trials were included. Compared with placebo, pramipexole ER achieved a significant improvement in the UPDRS Part II + III score [weighted mean difference, −4.81; 95% confidence interval (CI), −6.40 to −3.23], whereas no significant difference was found in the UPDRS Part III + III score between pramipexole ER and pramipexole IR groups (weighted mean difference, −0.26; 95% CI, −1.15 to 0.64). No differences were found in total AEs (relative risk, 0.97; 95% CI, 0.92 to 1.03), drug‐related AEs (relative risk, 0.97; 95% CI, 0.92 to 1.03) or the commonly reported AEs between pramipexole ER and pramipexole IR.

Conclusions

Pramipexole ER is as safe and effective as pramipexole IR in the treatment of Parkinson's disease.

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Authors:
T. Shen, R. Ye and B. Zhang
Article first published online:
08 May 2017 | DOI: 10.1111/ene.13303

Original Article

Background and purpose

Dysphagia is a well‐known complication of acute stroke. Given the complexity of cerebral swallowing control it is still difficult to predict which patients are likely to develop swallowing dysfunction based on their neuroimaging. In Part 2 of a comprehensive voxel‐based imaging study, whether the location of a stroke lesion can be correlated with further dysfunctional swallowing patterns, pulmonary protective reflexes and pneumonia was evaluated.

Methods

In all, 200 acute stroke cases were investigated applying flexible endoscopic evaluation of swallowing within 96 h from admission. Lesions were mapped using patients’ computed tomography/magnetic resonance images and these were registered to a standard space. The percentage of lesioned volume of 137 anatomically defined brain regions was determined on a voxel basis (FSL5.0). Region‐specific odds ratios (ORs) were calculated with respect to the presence of oropharyngeal residue, delayed swallow response, insufficient cough reflex and occurrence of pneumonia during hospital stay. Colour‐coded lesion location maps of brain regions with significant ORs were created ( < 0.05).

Results

Lesion maps for residue and impaired swallow response depicted parietal‐temporal areas of the right hemisphere. Limbic structures in the right hemisphere and sensory regions on the left were associated with cough reflex disturbance. There was no overlap of lesion maps for impaired swallow response and insufficient cough reflex or pneumonia, but substantial overlap between the last two conditions.

Conclusions

This study gives new insights on the cortical representation of single components of swallowing and airway protection behaviours. The lesion model may help to risk‐stratify patients for dysphagia and pneumonia based on their brain scan.

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Authors:
S. Suntrup‐Krueger, A. Kemmling, T. Warnecke, C. Hamacher, S. Oelenberg, T. Niederstadt, W. Heindel, H. Wiendl and R. Dziewas
Article first published online:
27 Apr 2017 | DOI: 10.1111/ene.13307

Short Communication

Background and purpose

New criteria for the diagnosis of multiple sclerosis (MS) and discovery of myelin oligodendrocyte glycoprotein (MOG) or aquaporin‐4 (AQP4) antibodies (Abs) have changed the management of optic neuritis (ON). Our aim was to specify, in view of these recent advances, the etiologies of acute demyelinating ON for consecutive patients.

Methods

Retrospective database analysis was undertaken of consecutive adult patients with acute ON admitted from 1 December 2014 to 31 January 2016. Diagnosis of MS was made according to the 2010 McDonald criteria. Patients with Abs to AQP4 or MOG were classified as ON‐AQP4 and ON‐MOG, respectively. Patients who did not fulfill the diagnostic criteria and were negative for AQP4 and MOG Ab tests were classified as having idiopathic ON.

Results

Of 110 patients assessed, 78 had ON related to MS (70.9%). All patients without MS were tested for AQP4 and MOG Abs: 11 had MOG Ab (10%), 5 had AQP4 Ab (4.5%) and 16 were considered as having idiopathic ON (14.5%). Presence of intrathecal IgG oligoclonal bands was strongly associated with MS (mean, 88.4% vs. 34.4% in patients without MS; after Bonferroni correction, < 0.0001).

Conclusions

Optic neuritis related to MOG Ab was the second cause identified of demyelinating ON in our center. Idiopathic ON was as frequent as both ON‐AQP4 and ON‐MOG combined.

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Authors:
R. Deschamps, A. Lecler, C. Lamirel, J. Aboab, A. Gueguen, C. Bensa, C. Vignal and O. Gout
Article first published online:
06 May 2017 | DOI: 10.1111/ene.13315

Letter to the Editor

Effectiveness of bilateral deep‐brain stimulation on dystonia: response to the latest meta‐analysis

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Authors:
Y. Ruvalcaba
Article first published online:
22 May 2017 | DOI: 10.1111/ene.13316

Calendar

Calendar

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Article first published online:
22 May 2017 | DOI: 10.1111/ene.13338