European Journal of Neurology 2014, 21: 1425–1434

doi:10.1111/ene.12619

EDITORIAL

Recent advances in neurology 2013–2014

The advent of modern neuro-imaging modalities transformed the practice of neurology and neurosurgery. Advances in this area continue with increased resolution and three-dimensional reconstructions, but perhaps the most exciting area of neuroradiology is the application of radiotracers or receptor ligands to the diagnosis of neurological diseases. The areas of stroke and the neurodegenerative diseases have perhaps been those that have benefited most from the new imaging modalities, but inflammatory disorders and common clinical problems such as headache have also seen novel applications. Probably the next most important area for advance and clinical application has been in the field of molecular neuroscience and genetics in particular. The ability to identify the genetic basis of disease has transformed the way we recognize the spectrum of the clinical phenotype of a particular mutation; indeed this has led us to reconsider the criteria for diagnosis in certain diseases such as Parkinson’s disease. These advances have also provided invaluable insight into the pathogenesis of neurological diseases and potential targets for novel therapeutic interventions. The past year has seen these advances reflected in the number and range of articles published in the European Journal of Neurology. Again, stroke is the most active area for publication followed by Parkinson’s disease and other movement disorders. The pathophysiology of stroke [1–22] has seen advances in the genetics of stroke that include identification of a single gene but more frequently multiple gene associations that confer increased risk. The observation that vitamin D levels can be associated with stroke risk is interesting, bearing in mind that levels have also been associated with the risk for Parkinson’s disease and multiple sclerosis. The management of acute stroke [23–38] is focused on intravascular thrombolysis, now with a longer time window for intervention but with diminishing returns in terms of improved outcome. The management of blood glucose and blood pressure remain of importance in the acute stage as well as in prevention and post-stroke management. The treatment and prevention of stroke [39–47] to improve outcomes [48–72] has as much to do with therapeutic intervention as it has to do with education of the general public as well as physicians. The swift recognition of transient ischaemic attacks and stroke is essential to improve outcome. The identification and correct stratification

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for treatment of patients with atrial fibrillation is a major feature of stroke prevention. Multiple sclerosis [73–101] is considered the prototypic neuro-inflammatory disease. It is now recognized to have a significant genetic component, although no single gene mutations have been identified, so again this is likely to be a complex genetic influence on risk. Other insights into pathogenesis include the role of axonal degeneration and the possible contribution of mitochondrial dysfunction. Disease-modifying therapies have had a significant impact upon relapse rate and outcome but are not without adverse events. Attempts to stratify patients manifesting with a single episode of demyelination into prognostic groups may enable the identification of those patients who might benefit more from earlier as opposed to later intervention with these drugs. Our understanding of the cause and pathophysiology of Parkinson’s disease (PD) [102–123] has benefited considerably from the molecular and imaging advances referred to above. Several monogenetic forms of PD are recognized, and recent genome-wide association studies have confirmed alpha-synuclein, tau, immune pathway and glucocerebrosidase (GBA) genes as playing important roles in determining risk. GBA gene mutations are now recognized as numerically the most important risk factor for PD. Studies have confirmed this in several ethnic groups, although the prevalence of GBA mutations varies between groups, being most common in the Ashkenazi population. Attention is focused on the prodromal period of PD where subtle clinical abnormalities, e.g. hyposmia, rapid eye movement sleep behaviour, can precede the onset of motor problems by several years. It is hoped that a combination of genetic, clinical and biochemical biomarkers may offer clinicians the opportunity to identify those most at risk for PD and therefore those most suitable for early intervention with a diseasemodifying drug, should one become available. Novel imaging modalities to detect microglial activation and alpha-synuclein offer benefits for early detection, but in particular a means potentially to follow the course of the disease and the success or not of therapies to slow progression. Advances in the clinical prodrome and pathogenesis of PD have called into question the traditional clinically based diagnosis of PD. Guidelines on the diagnosis of PD provide a rational basis for diagnosis [102], and for practical clinical practice the clinically

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based diagnosis of PD remains intact. The symptomatic treatment of PD over the past year has been refined rather than revolutionized. Dopamine agonists are now available as once-a-day treatments and have a similar efficacy and adverse event profile to the traditional shorter acting preparations. However, it is clear that patients prefer the convenience of once-aday medication. The European Federation of Neurological Societies guidelines on the treatment of PD provide an excellent evidence-based review [105]. However, the recognition and treatment of non-motor symptoms remain a major unmet need for PD patients [124–132]. Although traditionally associated with features such as cognitive abnormalities, autonomic dysfunction etc., sleep disorders and pain are commonly recognized problems; the latter may be exacerbated by dopaminergic medication. Unfortunately, effective treatments for non-motor symptoms are very limited. Movement disorders other than PD have also benefited from the major developments in genetics and imaging [133–147]. Huntington’s disease is a good example where the use of longitudinal magnetic resonance imaging can follow progression in pre-symptomatic mutation carriers and potentially be used as a marker for efficacy of disease-modifying interventions. Imaging can also be helpful to distinguish some forms of parkinsonism although not necessarily with great sensitivity. Genetics has enabled the identification of several novel parkinsonism dystonia syndromes and has also opened up the essential tremor and dystonia fields [148–153]. The main focus of attention for Alzheimer’s disease and other dementias [154–163] remains the discovery of an intervention that will slow or prevent progression. However, once again, novel imaging techniques have enabled the identification of dementia variants more accurately and the correlation of these with genetics. The burden of vascular disease to cognitive decline can now be more accurately assessed in a longitudinal fashion. Amyotrophic lateral sclerosis (motor neuron disease) genetics have had a prominent advance with the identification of fus gene mutations and these may lead to important insights into pathogenesis and therefore potential targets for intervention. In the interim, the practising neurologist still faces the difficulties of management, particularly of respiratory complications [164–168]. Genetic associations in headache [169–173] and epilepsy [174–183] continue to appear and help define different phenotype correlations. New treatment strategies are becoming available for both, also incorporating lifestyle changes for headache management and new drugs for epilepsy.

The neuromuscular field [184–203] was perhaps the first area of neurology to be transformed by genetics when Duchenne gene mutations were identified. Mutations of primary myopathies including the limb girdle dystrophies and congenital and metabolic myopathies have been identified and have transformed the diagnostic approach to muscle disease. The role of muscle biopsy has changed with this and the European Federation of Neurological Societies guidelines on the use of this investigation in myalgia are welcomed [193]. The genetic discoveries have yet to lead to treatments, but some progress is being made in this field. Neuromuscular disorders and myasthenia gravis are important cases of morbidity and treatment protocols are now refined by autoimmune profiles. Similarly, the diagnosis of immune-based neuropathies and their management has improved outcome in these disorders. Trauma-related neurological dysfunction remains an important cause of long-term disability that encompasses cognitive and physical deficits, epilepsy and spinal cord injury sequelae that are not only of clinical but also of legal relevance [49,56,204–207]. The role of rehabilitation and research into its best delivery and efficacy is an area that deserves greater attention. Inflammatory non-multiple-sclerosis neurological disease [208–216] has traditionally been associated with sarcoid or the vasculopathies, but the discovery of anti-aquaporin antibodies in neuromyelitis optica provided valuable insight into a rather heterogeneous and poorly defined clinical entity. New treatment strategies are now being defined. The same holds for the autoimmune limbic encephalitides. Guidelines [102,105,193,217] remain a vitally important area for education and the development of best medical practice. An enormous amount of work goes into the writing of these and the European Academy of Neurology will be continuing this role through the Scientific Committee. Neurology is a huge area of clinical activity and we have seen publications that cannot easily be incorporated into the major categories above [218–232]. The overlaps with psychiatry, oncology and cardiology in particular are a source of valuable clinical insight. Neurotoxicology is also an important field of development and has the ability to provide valuable insights into potential mechanisms of disease that may in turn have relevance to metabolic pathways involved in genetic neurological disorders.

A. H. V. Schapira Department of Clinical Neurosciences, UCL Institute of Neurology, London, UK

(e-mail: [email protected])

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Editorial

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prospective, non-randomized pilot study. Eur J Neurol 2013; 20: 986–990. 100. Romeo M, Martinelli-Boneschi F, Rodegher M, Esposito F, Martinelli V, Comi G. Clinical and MRI predictors of response to interferon-beta and glatiramer acetate in relapsing remitting multiple sclerosis patients. Eur J Neurol 2013; 20: 1060–1067. 101. Rossi S, Motta C, Studer V, et al. Effect of glatiramer acetate on disease reactivation in MS patients discontinuing natalizumab. Eur J Neurol 2013; 20: 87–94.

Parkinson’s disease – General 102. Berardelli A, Wenning GK, Antonini A, et al. EFNS/ MDS-ES/ENS [corrected] recommendations for the diagnosis of Parkinson’s disease. Eur J Neurol 2013; 20: 16–34. 103. Berg D, Godau J, Seppi K, et al. The PRIPS study: screening battery for subjects at risk for Parkinson’s disease. Eur J Neurol 2013; 20: 102–108. 104. Fanciulli A, Strano S, Colosimo C, Caltagirone C, Spalletta G, Pontieri FE. The potential prognostic role of cardiovascular autonomic failure in alpha-synucleinopathies. Eur J Neurol 2013; 20: 231–235. 105. Ferreira JJ, Katzenschlager R, Bloem BR, et al. Summary of the recommendations of the EFNS/MDS-ES review on therapeutic management of Parkinson’s disease. Eur J Neurol 2013; 20: 5–15. 106. Grosset KA, Malek N, Morgan F, Grosset DG. Phase IIa randomized double-blind, placebo-controlled study of inhaled apomorphine as acute challenge for rescuing ‘off’ periods in patients with established Parkinson’s disease. Eur J Neurol 2013; 20: 1445–1450. 107. Hiorth YH, Lode K, Larsen JP. Frequencies of falls and associated features at different stages of Parkinson’s disease. Eur J Neurol 2013; 20: 160–166. 108. La Morgia C, Barboni P, Rizzo G, et al. Loss of temporal retinal nerve fibers in Parkinson disease: a mitochondrial pattern? Eur J Neurol 2013; 20: 198–201. 109. Lim EC. Dosing issues in Parkinson’s disease – patient preferences and their influence on compliance. Eur J Neurol 2013; 20: 1–2. 110. Liu H, Tao Q, Deng H, et al. Genetic analysis of NR4A2 gene in a large population of Han Chinese patients with Parkinson’s disease. Eur J Neurol 2013; 20: 584–587. 111. Olivares RJ, Arjona PA, Barrero Hernandez FJ, Martin GM, Gil EB. Utility of transcranial sonography in the diagnosis of drug-induced parkinsonism: a prospective study. Eur J Neurol 2013; 20: 1451–1458. 112. Perez-Lloret S. Apomorphine for the treatment of refractory motor fluctuations in late stage Parkinson’s disease: an old drug revisited. Eur J Neurol 2013; 20: 1427–1428. 113. Poewe W, Seppi K. Diagnosis of drug-induced parkinsonism: can transcranial sonography make the difference? Eur J Neurol 2013; 20: 1429–1430. 114. Rocchi L, Conte A, Nardella A, et al. Somatosensory temporal discrimination threshold may help to differentiate patients with multiple system atrophy from patients with Parkinson’s disease. Eur J Neurol 2013; 20: 714–719. 115. Schapira AH, Stocchi F, Borgohain R, et al. Longterm efficacy and safety of safinamide as add-on therapy in early Parkinson’s disease. Eur J Neurol 2013; 20: 271–280.

116. Schapira AH, Barone P, Hauser RA, et al. Patientreported convenience of once-daily versus three-timesdaily dosing during long-term studies of pramipexole in early and advanced Parkinson’s disease. Eur J Neurol 2013; 20: 50–56. 117. Schapira AH, Barone P, Hauser RA, et al. Success rate, efficacy, and safety/tolerability of overnight switching from immediate- to extended-release pramipexole in advanced Parkinson’s disease. Eur J Neurol 2013; 20: 180–187. 118. Stathis P, Smpiliris M, Konitsiotis S, Mitsikostas DD. Nocebo as a potential confounding factor in clinical trials for Parkinson’s disease treatment: a meta-analysis. Eur J Neurol 2013; 20: 527–533. 119. Stocchi F, Bloem BR. Move for Change Part II: a European survey evaluating the impact of the EPDA Charter for people with Parkinson’s disease. Eur J Neurol 2013; 20: 461–472. 120. Zhao YJ, Tan LC, Au WL, et al. Estimating the lifetime economic burden of Parkinson’s disease in Singapore. Eur J Neurol 2013; 20: 368–374.

Parkinson’s disease pathogenesis 121. Jesus S, Perez I, Caceres-Redondo MT, et al. Low serum uric acid concentration in Parkinson’s disease in southern Spain. Eur J Neurol 2013; 20: 208–210. 122. Kumar KR, Ramirez A, Gobel A, et al. Glucocerebrosidase mutations in a Serbian Parkinson’s disease population. Eur J Neurol 2013; 20: 402–405. 123. Mateo I, Gonzalez-Aramburu I, Pozueta A, et al. Reduced serum progranulin level might be associated with Parkinson’s disease risk. Eur J Neurol 2013; 20: 1571–1573.

Parkinson’s disease – non-motor 124. Ceravolo R, Frosini D, Poletti M, et al. Mild affective symptoms in de novo Parkinson’s disease patients: relationship with dopaminergic dysfunction. Eur J Neurol 2013; 20: 480–485. 125. Louter M, van der Marck MA, Pevernagie DA, Munneke M, Bloem BR, Overeem S. Sleep matters in Parkinson’s disease: use of a priority list to assess the presence of sleep disturbances. Eur J Neurol 2013; 20: 259–265. 126. Defazio G, Tinazzi M, Berardelli A. How pain arises in Parkinson’s disease? Eur J Neurol 2013; 20: 1517– 1523. 127. Clark AJ, Ritz B, Prescott E, Rod NH. Psychosocial risk factors, pre-motor symptoms and first-time hospitalization with Parkinson’s disease: a prospective cohort study. Eur J Neurol 2013; 20: 1113–1120. 128. Granovsky Y, Schlesinger I, Fadel S, Erikh I, Sprecher E, Yarnitsky D. Asymmetric pain processing in Parkinson’s disease. Eur J Neurol 2013; 20: 1375–1382. 129. Lin CH, Wu RM, Chang HY, Chiang YT, Lin HH. Preceding pain symptoms and Parkinson’s disease: a nationwide population-based cohort study. Eur J Neurol 2013; 20: 1398–1404. 130. Martinez-Martin P, Rojo-Abuin JM, Dujardin K, et al. Designing a new scale to measure anxiety symptoms in Parkinson’s disease: item selection based on canonical correlation analysis. Eur J Neurol 2013; 20: 1198–1203. 131. Rod NH, Bordelon Y, Thompson A, Marcotte E, Ritz B. Major life events and development of major depression in Parkinson’s disease patients. Eur J Neurol 2013; 20: 663–670.

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132. Saenz A, Doe de Maindreville A, Henry A, de Labbey S, Bakchine S, Ehrle N. Recognition of facial and musical emotions in Parkinson’s disease. Eur J Neurol 2013; 20: 571–577.

Other movement disorders 133. Bohlen S, Ekwall C, Hellstrom K, et al. Physical therapy in Huntington’s disease – toward objective assessments? Eur J Neurol 2013; 20: 389–393. 134. Dostal M, Weber-Schoendorfer C, Sobesky J, Schaefer C. Pregnancy outcome following use of levodopa, pramipexole, ropinirole, and rotigotine for restless legs syndrome during pregnancy: a case series. Eur J Neurol 2013; 20: 1241–1246. 135. Giardino G, Fusco A, Romano R, et al. Betamethasone therapy in ataxia telangiectasia: unraveling the rationale of this serendipitous observation on the basis of the pathogenesis. Eur J Neurol 2013; 20: 740–747. 136. Kostic VS, Mijajlovic M, Smajlovic D, Lukic MJ, Tomic A, Svetel M. Transcranial brain sonography findings in two main variants of progressive supranuclear palsy. Eur J Neurol 2013; 20: 552–557. 137. Lambrecq V, Langbour N, Guehl D, Bioulac B, Burbaud P, Rotge JY. Evolution of brain gray matter loss in Huntington’s disease: a meta-analysis. Eur J Neurol 2013; 20: 315–321. 138. Lin CH, Wu VC, Li WY, et al. Restless legs syndrome in end-stage renal disease: a multicenter study in Taiwan. Eur J Neurol 2013; 20: 1025–1031. 139. Meneret A, Gaudebout C, Riant F, Vidailhet M, Depienne C, Roze E. PRRT2 mutations and paroxysmal disorders. Eur J Neurol 2013; 20: 872–878. 140. Prodi E, Grisoli M, Panzeri M, et al. Supratentorial and pontine MRI abnormalities characterize recessive spastic ataxia of Charlevoix Saguenay. A comprehensive study of an Italian series. Eur J Neurol 2013; 20: 138–146. 141. Saini J, Bagepally BS, Sandhya M, et al. Subcortical structures in progressive supranuclear palsy: vertexbased analysis. Eur J Neurol 2013; 20: 493–501. 142. Sikk K, Haldre S, Aquilonius SM, et al. Manganeseinduced parkinsonism in methcathinone abusers: biomarkers of exposure and follow-up. Eur J Neurol 2013; 20: 915–920. 143. Thompson JA, Cruickshank TM, Penailillo LE, et al. The effects of multidisciplinary rehabilitation in patients with early-to-middle-stage Huntington’s disease: a pilot study. Eur J Neurol 2013; 20: 1325–1329. 144. Trenkwalder C. Emergency call from gynecologists: how to treat restless legs syndrome during pregnancy? Eur J Neurol 2013; 20: 1223–1224. 145. Whitwell JL, Jack CR Jr, Parisi JE, et al. Midbrain atrophy is not a biomarker of progressive supranuclear palsy pathology. Eur J Neurol 2013; 20: 1417–1422. 146. Whitwell JL, Duffy JR, Strand EA, et al. Neuroimaging comparison of primary progressive apraxia of speech and progressive supranuclear palsy. Eur J Neurol 2013; 20: 629–637. 147. Zhang P, Gao Z, Jiang Y, et al. Follow-up study of 25 Chinese children with PLA2G6-associated neurodegeneration. Eur J Neurol 2013; 20: 322–330.

Other movement disorders – essential tremor 148. de Verdal M, Renard D, Collombier L, et al. I123-FPCIT single-photon emission tomography in patients with long-standing mixed tremor. Eur J Neurol 2013; 20: 382–388.

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149. Louis ED, Ottman R. Is there a one-way street from essential tremor to Parkinson’s disease? Possible biological ramifications. Eur J Neurol 2013; 20: 1440–1444. 150. Louis ED. The primary type of tremor in essential tremor is kinetic rather than postural: cross-sectional observation of tremor phenomenology in 369 cases. Eur J Neurol 2013; 20: 725–727. 151. Louis ED, Gerbin M, Galecki M. Essential tremor 10, 20, 30, 40: clinical snapshots of the disease by decade of duration. Eur J Neurol 2013; 20: 949–954.

Other movement disorders – dystonia 152. Loyola DP, Camargos S, Maia D, Cardoso F. Sensory tricks in focal dystonia and hemifacial spasm. Eur J Neurol 2013; 20: 704–707. 153. Lungu C, Considine E, Zahir S, Ponsati B, Arrastia S, Hallett M. Pilot study of topical acetyl hexapeptide-8 in the treatment for blepharospasm in patients receiving botulinum toxin therapy. Eur J Neurol 2013; 20: 515–518.

Alzheimer’s disease and other dementias 154. Andro M, Le Squere P, Estivin S, Gentric A. Anaemia and cognitive performances in the elderly: a systematic review. Eur J Neurol 2013; 20: 1234–1240. 155. Gil-Navarro S, Lomena F, Cot A, et al. Decreased striatal dopamine transporter uptake in the non-fluent/ agrammatic variant of primary progressive aphasia. Eur J Neurol 2013; 20: 1459-e126. 156. Gomez-Tortosa E, Guerrero-Lopez R, Gil-Neciga E, et al. Plasma progranulin levels in cortical dementia phenotypes with asymmetric perisylvian atrophy. Eur J Neurol 2013; 20: 1319–1324. 157. Gurol ME. Cerebral hypoperfusion and white matter disease in healthy elderly and patients with Alzheimer’s disease. Eur J Neurol 2013; 20: 214–215. 158. Haubois G, de Decker L, Annweiler C, et al. Derivation and validation of a Short Form of the Mini-Mental State Examination for the screening of dementia in older adults with a memory complaint. Eur J Neurol 2013; 20: 588–590. 159. Huang CW, Chang WN, Huang SH, et al. Impact of homocysteine on cortical perfusion and cognitive decline in mild Alzheimer’s dementia. Eur J Neurol 2013; 20: 1191–1197. 160. Lahiri DK, Ray B. Abnormal cerebrospinal fluid (CSF) dynamics in Alzheimer’s disease and normal pressure hydrocephalus: CSF-amyloid beta precursor protein metabolites as possible biomarkers. Eur J Neurol 2013; 20: 211–213. 161. Leinonen V, Rinne JO, Virtanen KA, et al. Positron emission tomography with [18F]flutemetamol and [11C] PiB for in vivo detection of cerebral cortical amyloid in normal pressure hydrocephalus patients. Eur J Neurol 2013; 20: 1043–1052. 162. Makedonov I, Black SE, MacIntosh BJ. Cerebral small vessel disease in aging and Alzheimer’s disease: a comparative study using MRI and SPECT. Eur J Neurol 2013; 20: 243–250. 163. Tabara Y, Igase M, Okada Y, et al. Association of Chr17q25 with cerebral white matter hyperintensities and cognitive impairment: the J-SHIPP study. Eur J Neurol 2013; 20: 860–862.

Motor neuron disease, amyotrophic lateral sclerosis 164. Korner S, Kollewe K, Ilsemann J, et al. Prevalence and prognostic impact of comorbidities in amyotrophic lateral sclerosis. Eur J Neurol 2013; 20: 647–654.

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165. Raaphorst J, Tuijp J, Verweij L, et al. Treatment of respiratory impairment in patients with motor neuron disease in the Netherlands: patient preference and timing of referral. Eur J Neurol 2013; 20: 1524– 1530. 166. Roth NM, Saidha S, Zimmermann H, et al. Optical coherence tomography does not support optic nerve involvement in amyotrophic lateral sclerosis. Eur J Neurol 2013; 20: 1170–1176. 167. Swash M. Ventilation in ALS. Eur J Neurol 2013; 20: 1508–1509. 168. Waibel S, Neumann M, Rosenbohm A, et al. Truncating mutations in FUS/TLS give rise to a more aggressive ALS-phenotype than missense mutations: a clinicogenetic study in Germany. Eur J Neurol 2013; 20: 540– 546.

Headache 169. Esserlind AL, Christensen AF, Le H, et al. Replication and meta-analysis of common variants identifies a genome-wide significant locus in migraine. Eur J Neurol 2013; 20: 765–772. 170. Huang Y, Cai X, Song X, et al. Steroids for preventing recurrence of acute severe migraine headaches: a metaanalysis. Eur J Neurol 2013; 20: 1184–1190. 171. Ozge A, Sasmaz T, Bugdayci R, et al. The prevalence of chronic and episodic migraine in children and adolescents. Eur J Neurol 2013; 20: 95–101. 172. Verrotti A, Agostinelli S, D’Egidio C, et al. Impact of a weight loss program on migraine in obese adolescents. Eur J Neurol 2013; 20: 394–397. 173. Wang J, Huang Q, Li N, Tan G, Chen L, Zhou J. Triggers of migraine and tension-type headache in China: a clinic-based survey. Eur J Neurol 2013; 20: 689–696.

Epilepsy 174. Agostinelli S, Traverso M, Accorsi P, et al. Early-onset absence epilepsy: SLC2A1 gene analysis and treatment evolution. Eur J Neurol 2013; 20: 856–859. 175. Balestrini S, Cagnetti C, Foschi N, et al. Emergency room access for recurring seizures: when and why. Eur J Neurol 2013; 20: 1411–1416. 176. Belcastro V, Striano P, Parisi P. Interictal and periictal headache in patients with epilepsy: migraine-triggered seizures or epilepsy-triggered headache? Eur J Neurol 2013; 20: 1333–1334. 177. Duchaczek B, Ghaeni L, Matzen J, Holtkamp M. Interictal and periictal headache in patients with epilepsy. Eur J Neurol 2013; 20: 1360–1366. 178. Hilger E, Zimprich F, Jung R, Pataraia E, Baumgartner C, Bonelli S. Postictal psychosis in temporal lobe epilepsy: a case control study. Eur J Neurol 2013; 20: 955–961. 179. Labate A, Mumoli L, Fratto A, Quattrone A, Gambardella A. Hippocampal sclerosis worsens autosomal dominant nocturnal frontal lobe epilepsy (ADNFLE) phenotype related to CHRNB2 mutation. Eur J Neurol 2013; 20: 591–593. 180. Sellner J, Trinka E. Clinical characteristics, risk factors and pre-surgical evaluation of post-infectious epilepsy. Eur J Neurol 2013; 20: 429–439. 181. Sturiale CL, Rigante L, Puca A, et al. Angioarchitectural features of brain arteriovenous malformations associated with seizures: a single center retrospective series. Eur J Neurol 2013; 20: 849–855.

182. Witt JA, Elger CE, Helmstaedter C. Impaired verbal fluency under topiramate – evidence for synergistic negative effects of epilepsy, topiramate, and polytherapy. Eur J Neurol 2013; 20: 130–137. 183. Zaccara G, Giovannelli F, Cincotta M, Verrotti A, Grillo E. The adverse event profile of perampanel: meta-analysis of randomized controlled trials. Eur J Neurol 2013; 20: 1204–1211.

Neuromuscular 184. Argyriou AA, Briani C, Cavaletti G, et al. Advanced age and liability to oxaliplatin-induced peripheral neuropathy: post hoc analysis of a prospective study. Eur J Neurol 2013; 20: 788–794. 185. Briani C, Campagnolo M, Lucchetta M, et al. Ultrasound assessment of oxaliplatin-induced neuropathy and correlations with neurophysiologic findings. Eur J Neurol 2013; 20: 188–192. 186. Bsteh G, Wanschitz JV, Gruber H, Seppi K, Loscher WN. Prognosis and prognostic factors in non-traumatic acute-onset compressive mononeuropathies – radial and peroneal mononeuropathies. Eur J Neurol 2013; 20: 981–985. 187. Collongues N, Depienne C, Boehm N, et al. Novel SPG10 mutation associated with dysautonomia, spinal cord atrophy, and skin biopsy abnormality. Eur J Neurol 2013; 20: 398–401. 188. Dimberg EL. Grip strength in CIDP: does one function fit all? Eur J Neurol 2013; 20: 733–734. 189. Galban-Horcajo F, Fitzpatrick AM, Hutton AJ, et al. Antibodies to heteromeric glycolipid complexes in multifocal motor neuropathy. Eur J Neurol 2013; 20: 62– 70. 190. Goedee HS, Brekelmans GJ, van Asseldonk JT, Beekman R, Mess WH, Visser LH. High resolution sonography in the evaluation of the peripheral nervous system in polyneuropathy – a review of the literature. Eur J Neurol 2013; 20: 1342–1351. 191. Gregianin E, Vazza G, Scaramel E, et al. A novel SACS mutation results in non-ataxic spastic paraplegia and peripheral neuropathy. Eur J Neurol 2013; 20: 1486–1491. 192. Kamradt T, Rasch C, Schuld C, et al. Spinal cord injury: association with axonal peripheral neuropathy in severely paralysed limbs. Eur J Neurol 2013; 20: 843–848. 193. Kyriakides T, Angelini C, Schaefer J, et al. EFNS review on the role of muscle biopsy in the investigation of myalgia. Eur J Neurol 2013; 20: 997–1005. 194. Liewluck T, Winder TL, Dimberg EL, et al. ANO5muscular dystrophy: clinical, pathological and molecular findings. Eur J Neurol 2013; 20: 1383–1389. 195. Linssen WH, de Voogt WG, Krahn M, et al. Longterm follow-up study on patients with Miyoshi phenotype of distal muscular dystrophy. Eur J Neurol 2013; 20: 968–974. 196. Lozeron P, Theaudin M, Mincheva Z, Ducot B, Lacroix C, Adams D. Effect on disability and safety of tafamidis in late onset of Met30 transthyretin familial amyloid polyneuropathy. Eur J Neurol 2013; 20: 1539– 1545. 197. Maniaol AH, Boldingh M, Brunborg C, Harbo HF, Tallaksen CM. Smoking and socio-economic status may affect myasthenia gravis. Eur J Neurol 2013; 20: 453–460.

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198. Markvardsen LH, Debost JC, Harbo T, et al. Subcutaneous immunoglobulin in responders to intravenous therapy with chronic inflammatory demyelinating polyradiculoneuropathy. Eur J Neurol 2013; 20: 836–842. 199. Nakata R, Motomura M, Masuda T, et al. Thymus histology and concomitant autoimmune diseases in Japanese patients with muscle-specific receptor tyrosine kinase-antibody-positive myasthenia gravis. Eur J Neurol 2013; 20: 1272–1276. 200. Pedersen EG, Pottegard A, Hallas J, et al. Use of azathioprine for non-thymoma myasthenia and risk of cancer: a nationwide case control study in Denmark. Eur J Neurol 2013; 20: 942–948. 201. Sandell SM, Mahjneh I, Palmio J, Tasca G, Ricci E, Udd BA. ‘Pathognomonic’ muscle imaging findings in DNAJB6 mutated LGMD1D. Eur J Neurol 2013; 20: 1553–1559. 202. Vanhoutte EK, Latov N, Deng C, et al. Vigorimeter grip strength in CIDP: a responsive tool that rapidly measures the effect of IVIG – the ICE study. Eur J Neurol 2013; 20: 748–755. 203. Zach H, Cetin H, Hilger E, et al. The effect of early prednisolone treatment on the generalization rate in ocular myasthenia gravis. Eur J Neurol 2013; 20: 708– 713.

Trauma 204. Furlan JC, Fehlings MG. Blood alcohol concentration as a determinant of outcomes after traumatic spinal cord injury. Eur J Neurol 2013; 20: 1101–1106. 205. Lannsjo M, Backheden M, Johansson U, Af Geijerstam JL, Borg J. Does head CT scan pathology predict outcome after mild traumatic brain injury? Eur J Neurol 2013; 20: 124–129. 206. Theadom A, Mahon S, Barker-Collo S, et al. Enzogenol for cognitive functioning in traumatic brain injury: a pilot placebo-controlled RCT. Eur J Neurol 2013; 20: 1135–1144. 207. Sabre L, Hagen EM, Rekand T, Asser T, Korv J. Traumatic spinal cord injury in two European countries: why the differences? Eur J Neurol 2013; 20: 293– 299.

Neuro-inflammatory 208. Banati M, Csecsei P, Koszegi E, et al. Antibody response against gastrointestinal antigens in demyelinating diseases of the central nervous system . Eur J Neurol 2013; 20: 1492–1495. 209. Chanson JB, Lamy J, Rousseau F, et al. White matter volume is decreased in the brain of patients with neuromyelitis optica. Eur J Neurol 2013; 20: 361–367. 210. Frisch C, Malter MP, Elger CE, Helmstaedter C. Neuropsychological course of voltage-gated potassium channel and glutamic acid decarboxylase antibody related limbic encephalitis. Eur J Neurol 2013; 20: 1297–1304. 211. Kim W, Kim SH, Huh SY, et al. Reduced antibody formation after influenza vaccination in patients with neuromyelitis optica spectrum disorder treated with rituximab. Eur J Neurol 2013; 20: 975–980. 212. Kiyat-Atamer A, Ekizoglu E, Tuzun E, et al. Longterm MRI findings in neuromyelitis optica: seropositive versus seronegative patients. Eur J Neurol 2013; 20: 781–787. 213. O’Dwyer JP, Al-Moyeed BA, Farrell MA, et al. Neurosarcoidosis-related intracranial haemorrhage: three

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new cases and a systematic review of the literature. Eur J Neurol 2013; 20: 71–78. 214. Oyanguren B, Sanchez V, Gonzalez FJ, et al. Limbic encephalitis: a clinical-radiological comparison between herpetic and autoimmune etiologies. Eur J Neurol 2013; 20: 1566–1570. 215. Steinbrink F, Evers S, Buerke B, et al. Cognitive impairment in HIV infection is associated with MRI and CSF pattern of neurodegeneration. Eur J Neurol 2013; 20: 420–428. 216. Tjensvoll AB, Harboe E, Goransson LG, et al. Headache in primary Sjogren’s syndrome: a populationbased retrospective cohort study. Eur J Neurol 2013; 20: 558–563.

Guidelines 217. Leone MA, Brainin M, Boon P, Pugliatti M, Keindl M, Bassetti CL. Guidance for the preparation of neurological management guidelines by EFNS scientific task forces – revised recommendations 2012. Eur J Neurol 2013; 20: 410–419.

General 218. Benito-Leon J, Mitchell AJ, Hernandez-Gallego J, Bermejo-Pareja F. Obesity and impaired cognitive functioning in the elderly: a population-based cross-sectional study (NEDICES). Eur J Neurol 2013; 20: 899– 907. 219. Chen C, Xu T, Lu Y, Chen J, Wu S. The efficacy of temozolomide for recurrent glioblastoma multiforme. Eur J Neurol 2013; 20: 223–230. 220. Eddy CM, Cavanna AE. ‘It’s a curse!’: coprolalia in Tourette syndrome. Eur J Neurol 2013; 20: 1467– 1470. 221. Graff-Radford NR, Knopman DS, Penman AD, Coker LH, Mosley TH. Do systolic BP and pulse pressure relate to ventricular enlargement? Eur J Neurol 2013; 20: 720–724. 222. Hammer MB, Eleuch-Fayache G, Gibbs JR, et al. Exome sequencing: an efficient diagnostic tool for complex neurodegenerative disorders. Eur J Neurol 2013; 20: 486–492. 223. Jankelowitz SK, Spies JM, Burke D. Late-onset neurological symptoms in thalidomide-exposed subjects: a study of an Australasian cohort. Eur J Neurol 2013; 20: 509–514. 224. Lee CC, Su YC, Chien SH, et al. Increased stroke risk in Bell’s palsy patients without steroid treatment. Eur J Neurol 2013; 20: 616–622. 225. McColgan P, McKeown PP, Selai C, Doherty-Allan R, McCarron MO. Educational interventions in neurology: a comprehensive systematic review. Eur J Neurol 2013; 20: 1006–1016. 226. Miyajima M, Nakajima M, Ogino I, Miyata H, Motoi Y, Arai H. Soluble amyloid precursor protein alpha in the cerebrospinal fluid as a diagnostic and prognostic biomarker for idiopathic normal pressure hydrocephalus. Eur J Neurol 2013; 20: 236–242. 227. Olesen J. Ten-year anniversary of the European Brain Council: the major achievements. Eur J Neurol 2013; 20: 595–596. 228. Schapira AH. Progress in European neurology 2012– 2013. Eur J Neurol 2013; 20: 1499–1507. 229. Schestatsky P, Dall-Agnol L, Gheller L, et al. Pain autonomic interaction after work-induced sleep restriction. Eur J Neurol 2013; 20: 638–646.

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230. Van ME, Wozniak A, Geussens pseudoprogression in glioblastoma Neurol 2013; 20: 1335–1341. 231. Welschehold S, Kerz T, Boor S, intracranial circulatory arrest in

Y, et al. Defining multiforme. Eur J et al. Detection of brain death using

cranial CT-angiography. Eur J Neurol 2013; 20: 173– 179. 232. Wijnia JW, Oudman E. Biomarkers of delirium as a clue to diagnosis and pathogenesis of Wernicke Korsakoff syndrome. Eur J Neurol 2013; 20: 1531–1538.

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Recent advances in neurology 2013-2014.

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