cover image European Journal of Neurology

European Journal of Neurology

2014 - Volume 21
Issue 6 | June 2014
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Short Communication

Background and purpose

There is a paucity of data available regarding the occurrence of sleep disorders in myotonic dystrophy type 2 (DM2). In this study the sleep–wake cycle and daytime sleepiness were investigated in DM2 patients and compared with results from healthy subjects and myotonic dystrophy type 1 (DM1) patients.

Methods

Twelve DM2 outpatients, 12 age‐ and sex‐matched healthy controls and 18 DM1 patients were recruited. Subjective quality of sleep was assessed by means of the Pittsburgh Sleep Quality Index (PSQI). Both the Epworth Sleepiness Scale and the Daytime Sleepiness Scale were performed in order to evaluate excessive daytime sleepiness (EDS). All participants underwent polysomnographic monitoring over 48 h as well as the Multiple Sleep Latency Test.

Results

Sleep efficiency was < 90% in 12/12 DM2 patients, and significantly reduced when compared with controls or with DM1. Decreased sleep efficiency was associated with sleep‐disordered breathing in seven out of 12 DM2 patients and/or periodic limbs movements of sleep (PLMS) in three out of eight patients. Six DM2 patients showed REM sleep without atonia, whereas none of the controls or DM1 patients showed REM sleep dysregulation. The global PSQI score was higher in DM2 patients than in controls and DM1 patients.

Conclusions

Sleep quality in DM2 patients is poorer than in DM1 patients and controls. Sleep apnea is the most common sleep disorder in DM2 patients. Obstructive sleep apnea and sleep fragmentation may represent the main cause of EDS, whereas PLMS is a frequent finding in DM1.

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Authors:
A. Romigi, M. Albanese, F. Placidi, F. Izzi, C. Liguori, M. G. Marciani, N. B. Mercuri, C. Terracciano, G. Vitrani, A. Petrucci, B. Di Gioia and R. Massa
Article first published online:
10 Jul 2013 | DOI: 10.1111/ene.12226

Short Communication

Background and purpose

The hereditary spastic paraplegias (HSP) are characterized by progressive spasticity of the lower limbs, mostly inherited as an autosomal dominant trait. Analyses of large HSP pedigrees could help to better characterize the phenotype due to a single causative mutation. Patients in a seven‐generation kindred carrying a large deletion in /SPG4 are described.

Methods

Individuals originating from Sardinia were clinically and genetically studied.

Results

Sixty‐seven subjects carried a heterozygous deletion encompassing exons 2–17 of . Fifty patients (53.2 ± 15.4 years) presented a pure form of spastic paraparesis characterized by mild impairment and slow progression. Most patients showed spasticity, increased tendon reflexes in the lower limbs and Babinski sign, whilst weakness was rarely detected and urinary disturbances occasionally reported. Amongst the 17 asymptomatic carriers of the mutation, minimal neurological signs were detected in 11 cases.

Conclusions

A focus on spasticity, increased tendon reflexes and Babinski sign, more than on weakness, could help clinicians to promote early diagnosis in asymptomatic carriers of deletions.

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Authors:
L. Racis, R. Di Fabio, A. Tessa, F. Guillot, E. Storti, F. Piccolo, C. Nesti, A. Tedde, F. Pierelli, V. Agnetti, F. M. Santorelli and C. Casali
Article first published online:
04 Nov 2013 | DOI: 10.1111/ene.12290

Original Article

Background and purpose

In the epilepsy community, there is talk that the number of classical patients with early onset temporal lobe epilepsy (TLE) and Ammon's horn sclerosis (AHS) is decreasing. This is counterintuitive, considering the success story of epilepsy surgery, improved diagnostic methods and the current recommendation of early admission to surgery. In order to recognize trends, the development of temporal lobe surgery over 20 years in three major German epilepsy centers was reviewed.

Methods

Age at surgery and duration of epilepsy, which was differentiated according to histopathology (AHS, developmental, tumor, vascular), year of surgery and center, were evaluated in a cohort of 2812 patients from three German epilepsy centers who underwent temporal lobe surgery between 1988 and 2008. The analysis was carried out for the pooled cohort as well as for each center separately.

Results

Of all patients, 52% showed AHS. Compared with other pathologies, the AHS group had the earliest epilepsy onset and the longest duration of epilepsy. Across five time epochs, the diagnosis of AHS increased in the first epoch, remaining constant thereafter. Contrary to the trends in other pathologies, in the AHS group the mean age of patients at surgery increased by 7 years and the duration of epilepsy until surgery increased by 5 years. This trend could be replicated in all three centers. As initially hypothesized for all groups, age and duration of epilepsy in other pathology groups remained constant or indicated earlier submission to surgery.

Conclusions

During the first few years studied, most probably due to progress in brain imaging, the proportion of patients with AHS increased. However, despite stable numbers over time, and contrary to the trends in other pathology groups, age and duration of epilepsy in mesial TLE with AHS (mTLE + AHS) increased over time. This supports the hypothesis of a decreasing incidence of AHS. This trend is discussed with respect to disease‐modifying factors which have changed the incidence of classical mTLE + AHS or, alternatively, to recent developments in antiepileptic drug treatment, the appraisal of surgery and economic incentives for treatment options other than surgery.

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Authors:
C. Helmstaedter, T. W. May, M. von Lehe, M. Pfaefflin, A. Ebner, H. W. Pannek, C. E. Elger, H. Stefan and J. Schramm
Article first published online:
07 Dec 2013 | DOI: 10.1111/ene.12322

Original Article

Background and purpose

It was recently reported that there was no significant overall association between interferon beta exposure and disability progression in relapsing−remitting multiple sclerosis (RRMS) patients in an observational study from Canada. In the current study, the potential for heterogeneity in the association between exposure to interferon beta and disability progression across patients' baseline characteristics was investigated.

Methods

RRMS patients treated with interferon beta ( = 868) and two cohorts of untreated patients (829 contemporary and 959 historical controls) were included. The main outcome was time from interferon beta treatment eligibility (baseline) to a confirmed and sustained Expanded Disability Status Scale (EDSS) score 6 using a multivariable Cox model, with treatment as a time‐varying predictor, testing interaction effects for five pre‐specified baseline characteristics: sex, age, disease duration, EDSS and annualized relapse rate (ARR) based on the previous 2 years.

Results

Significant heterogeneity was found in the association of interferon beta exposure and disability progression only across ARR, and only when treated patients were compared with historical controls ( = 0.005 at a Bonferroni‐adjusted alpha of 0.01). For patients with ARR>1, treatment‐exposed time was associated with a hazard ratio of 0.38 (95%CI 0.20–0.75) for disability progression compared with the unexposed time.

Conclusions

RRMS patients with more frequent relapses at baseline may be more likely to benefit from interferon beta treatment with respect to long‐term disability progression.

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Authors:
A. Shirani, Y. Zhao, M. E. Karim, J. Petkau, P. Gustafson, C. Evans, E. Kingwell, M. L. van der Kop, J. Oger and H. Tremlett
Article first published online:
18 Dec 2013 | DOI: 10.1111/ene.12324

Editorial

Abstract

Click to view the accompanying paper in this issue.

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Authors:
A. Neligan
Article first published online:
25 Jan 2014 | DOI: 10.1111/ene.12358

Review Article

Abstract

The aim of this narrative review is to evaluate the pathogenesis, clinical features, diagnosis, treatment and prognosis of intracranial artery dissection (IAD). IAD is a rare and often unrecognized cause of stroke or subarachnoid haemorrhage (SAH), especially in young adults. Two types of IAD can be identified: a subintimal or subadventitial dissection. It is suggested that a subintimal dissection results in luminal stenosis, thromboembolism and subsequently cerebral ischaemia, whilst a subadventitial IAD could result in the formation of a pseudo‐aneurysm and compression on brainstem or cranial nerves. Rupture of such a dissecting aneurysm causes SAH. The exact cause of IAD remains unknown but several factors are associated with its development. Diagnosis is based on clinical presentation and specific features seen on multimodal neuroimaging. The management of IAD depends on the clinical presentation. In the case of cerebral ischaemia, anticoagulants or antiplatelet agents are used, whilst in the case of SAH endovascular treatment is primarily advocated. Prognosis depends on clinical presentation. Presentation with SAH has a worse prognosis.

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Authors:
T. Sikkema, M. Uyttenboogaart, O. Eshghi, J. De Keyser, R. Brouns, J. M. C. van Dijk and G. J. Luijckx
Article first published online:
22 Feb 2014 | DOI: 10.1111/ene.12384

Original Article

Background and purpose

The neurological outcome of acute encephalitis can be devastating and early prognosis remains difficult. Biomarkers that quantify the extent of early brain injury are needed to improve the prognostic accuracy and aid patient management. Our objective was to assess whether cerebrospinal fluid (CSF) protein biomarkers of neuroaxonal and glial cell injury are elevated in distinct forms of acute encephalitis and predictive of poor outcome.

Methods

This was a prospective study of patients presenting with acute encephalitis to three teaching hospitals in London, UK. Levels of neurofilament heavy chain (NfH, SMI35) and S100B were quantified in CSF using enzyme‐linked immunosorbent assay. The outcome was assessed by the Glasgow Outcome Scale (GOS).

Results

Fifty‐six patients with acute encephalitis were recruited and classified into the following diagnostic categories: infectious ( = 20), inflammatory ( = 14) and unknown etiology ( = 22). Pathological levels of NfH and S100B were observed in 24/56 (43%) and 54/56 (96%), respectively. Patients with infectious encephalitis had significantly higher NfH levels compared with the other two groups ( < 0.05). A poor outcome (GOS < 5) was associated with significantly higher CSF NfH levels within samples taken 2 weeks after symptom onset.

Conclusions

This study suggests that longitudinal CSF NfH levels are of superior prognostic value compared with CSF S100B levels. Prolonged release of NfH, a marker of neuroaxonal damage, was associated with poor outcome. Potentially there is a window of opportunity for future neuroprotective treatment strategies in encephalitis.

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Authors:
J. Sellner, N. W. Davies, R. S. Howard and A. Petzold
Article first published online:
29 Mar 2014 | DOI: 10.1111/ene.12390

Original Article

Background and purpose

OnabotulinumtoxinA was effective and well tolerated for prophylaxis of headache in patients with chronic migraine (CM) in two randomized, double‐blind, placebo‐controlled, phase 3 trials. To further assess the safety and tolerability of onabotulinumtoxinA in CM prophylaxis in adults, the pooled safety data from four double‐blind, placebo‐controlled trials were analyzed.

Methods

The pooled analysis included two phase 2 and two phase 3 double‐blind, placebo‐controlled trials. The safety population was 2436 patients, 1997 of whom received ≥1 dose of onabotulinumtoxinA. The studies shared similar dosing intervals (approximately 12 weeks) with doses between 75 and 260 U. Safety assessments included adverse events (AEs), physical examination and clinical laboratory tests.

Results

OnabotulinumtoxinA was safe and well tolerated, with a low discontinuation rate (3.4%) due to AEs. The majority of patients in this pooled analysis received doses between 150 and 200 U, with an average of 163 U per treatment cycle. Of the 1997 patients who received any onabotulinumtoxinA injections, 1455 patients (72.9%) reported at least one AE. Neck pain (12.6%) was the most common onabotulinumtoxinA‐associated AE, followed by muscle weakness (8.0%), musculoskeletal stiffness (6.1%) and eyelid ptosis (4.6%). Serious AEs were infrequent, occurring in 5.4% of patients who received any onabotulinumtoxinA treatment and 3.0% of patients receiving placebo. AEs were consistent with the known tolerability profile of onabotulinumtoxinA.

Conclusions

Multiple treatments with onabotulinumtoxinA at doses of 75–260 U administered every 12 weeks, and up to five treatment cycles, were well tolerated for the prophylaxis of headache in adults with CM.

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Authors:
H.‐C. Diener, D. W. Dodick, C. C. Turkel, G. Demos, R. E. DeGryse, N. L. Earl and M. F. Brin
Article first published online:
15 Mar 2014 | DOI: 10.1111/ene.12393

Original Article

Background and purpose

Brain imaging with positron emission tomography using [F]fluorodeoxyglucose (FDG‐PET) and transcranial B‐mode sonography (TCS) improves the differential diagnosis of parkinsonism. The diagnostic merits of these approaches in identifying and differentiating atypical parkinsonian syndromes (APS) are compared.

Methods

Data were included from 36 patients with clinically suspected APS who underwent PET and TCS. FDG‐PET scans were analyzed by visual assessment (including voxel‐based statistical maps) of defined disease‐specific metabolic patterns. Sonographers achieved diagnoses according to pre‐defined criteria for echogenicities of the substantia nigra and lenticular nucleus, and third ventricle diameter. Patients with APS were identified and allocated to the subgroups multiple system atrophy (MSA), progressive supranuclear palsy (PSP) or corticobasal degeneration (CBD).

Results

After a median follow‐up period of 9 months, the final clinical diagnoses (reference standard) were Parkinson's disease,  = 15; MSA, = 9; PSP, = 7; and CBD, = 5 ( = 21 APS in total). Six patients (4 APS) showed an insufficient bone window for TCS. In the remaining 30 patients, sensitivity/specificity for diagnosing APS were 82%/100% and 82%/85% for FDG‐PET and TCS, respectively. Diagnostic accuracies did not differ between FDG‐PET (90%) and TCS (83%;  = 0.69). Likewise, overall accuracy of subgroup classification (non‐APS, MSA, PSP and CBD) did not differ between modalities (FDG‐PET 87% and TCS 83%;  = 1.00).

Conclusions

FDG‐PET and TCS show comparable accuracies for differential diagnosis of neurodegenerative parkinsonism. This preliminary study supports the use of TCS and warrants further prospective validation.

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Authors:
S. Hellwig, M. Reinhard, F. Amtage, B. Guschlbauer, R. Buchert, O. Tüscher, C. Weiller, W. D. Niesen and P. T. Meyer
Article first published online:
06 Mar 2014 | DOI: 10.1111/ene.12394

Original Article

Background and purpose

Several studies have reported moyamoya syndrome associated with thyroid disease, and the mechanism involved in this relationship is unknown. This study aimed to clarify the involvement of thyroid antibodies and thyroid function in intracranial arterial stenosis.

Methods

The study included 30 patients <65 years of age with intracranial arterial steno‐occlusion. Patients with definitive moyamoya disease were excluded. Thyroid function and thyroid antibody levels were evaluated. The steno‐occlusive site and the presence of moyamoya vessels were evaluated using digital subtraction angiography. The characteristics of intracranial arterial lesions were compared between patients with and without elevated thyroid antibody levels, and between patients with increased thyroid function and those with normal thyroid function.

Results

Five patients had increased thyroid function and seven had elevated thyroid antibody levels. Four were diagnosed with Graves' disease, 13 with atherosclerotic intracranial stenosis, two with intracranial arterial dissection, one with vasculitis syndrome and 10 with intracranial stenosis of unknown cause. All patients with Graves' disease and patients with elevated antithyroid peroxidase antibody levels had steno‐occlusion in the terminal portion of the internal carotid arteries, whereas most of the patients with normal thyroid function or without elevated thyroid antibody levels had stenosis in the middle cerebral arteries.

Conclusions

In young and middle‐aged patients, a lesion in the terminal portion of the internal carotid artery was associated with elevated thyroid antibody levels and increased thyroid function. Stenoses found in the terminal portion of the internal carotid artery and immune‐mediated thyroid diseases may share a common background.

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Authors:
M. Tanaka, M. Sakaguchi, Y. Yagita, Y. Gon, K. Yoshikawa, T. Takahashi, R. Fukunaga, H. Mochizuki and K. Kitagawa
Article first published online:
06 Mar 2014 | DOI: 10.1111/ene.12397

Original Article

Background and purpose

Impairment of cognitive functions occurs in essential tremor (ET) although the mechanism is largely unknown. A possible association between cognitive performance and brain atrophy in ET patients was examined using neuropsychological tests and voxel‐based morphometry (VBM).

Methods

Twenty‐five patients with ET and 25 matched healthy controls were evaluated. ET was diagnosed using the National Institutes of Health collaborative genetic criteria. Severity of tremor was assessed using the Fahn−Tolosa−Marin (FTM) tremor rating scale. Subjects were assessed using a structured neuropsychological battery. Brain images were acquired using a 3T magnetic resonance imaging scanner. VBM analysis was performed using Statistical Parametric Mapping 8.

Results

The age of the patients was 45.0 ± 10.7 years and of controls 45.4 ± 10.7 years. Tremor duration was 9.84 ± 6.63 years and total FTM score was 37.34 ± 17.67. Patients were divided into two groups: ETCI with cognitive impairment (three or more abnormal neuropsychological tests, 1.5 standard deviation criterion) and ETNCI without cognitive impairment. Compared with controls, the ETCI group had significantly impaired performance in neuropsychological tests. One‐way analysis of variance was performed between the three groups (ETCI, ETNCI, controls) followed by the two‐sample test. Compared with controls, grey matter volume (GMV) loss was observed in ETCI in the cerebellum (anterior and posterior lobes) and medial frontal gyrus. GMV loss was observed in ETCI compared with ETNCI in the medial frontal gyrus, post central gyrus, anterior cingulate and insula. Impairment in neuropsychological tests significantly correlated with GMV of the medial frontal gyrus, superior parietal lobe, middle temporal gyrus, occipital lobe, lentiform nucleus, insular and cingulate cortices and cerebellum posterior lobe in ETCI.

Conclusions

A correlation between neurocognitive deficits in ETCI and GMV was observed suggesting that grey matter atrophy appears to be a correlate of cognitive impairment in ET.

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Authors:
K. S. Bhalsing, N. Upadhyay, K. J. Kumar, J. Saini, R. Yadav, A. K. Gupta and P. K. Pal
Article first published online:
11 Mar 2014 | DOI: 10.1111/ene.12399

Original Article

Background and purpose

Atrial fibrillation (AF) increases the risk of stroke fourfold and is associated with a poor clinical outcome. Despite work‐up in compliance with guidelines, up to one‐third of patients have cryptogenic stroke (CS). The prevalence of asymptomatic paroxysmal atrial fibrillation (PAF) in CS remains unknown. The SURPRISE project aimed at determining this rate using long‐term cardiac monitoring.

Methods

Patients with CS after protocolled work‐up including electrocardiography (ECG) and telemetry were included after informed consent. An implantable loop recorder (ILR) was implanted subcutaneously. PAF was defined by events of atrial arrhythmia >2 min with a correlating one‐lead ECG confirming the diagnosis.

Results

Eighty‐five patients were monitored for a mean of 569 days (SD ±310). PAF was documented in 18 patients (20.7%) during the study period and detected by ILR in 14 patients (16.1%). In three patients PAF was detected by other methods before or after monitoring and was undiscovered due to device sensitivity in one case. The first event of PAF was documented at a mean of 109 days (SD ±48) after stroke onset. PAF was asymptomatic in all cases and occurred in episodes lasting predominantly between 1 and 4 h. Four recurrent strokes were observed, three in patients with PAF; all three patients were on oral anticoagulation (OAC).

Conclusions

One in five patients with CS had PAF, which occurred at low burden and long after stroke. Future studies should determine the role of implantable cardiac monitors after stroke and determine the potential therapeutic benefit of OAC treatment of patients with PAF.

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Authors:
L. M. Christensen, D. W. Krieger, S. Højberg, O. D. Pedersen, F. M. Karlsen, M. D. Jacobsen, R. Worck, H. Nielsen, K. Ægidius, L. L. Jeppesen, S. Rosenbaum, J. Marstrand and H. Christensen
Article first published online:
15 Mar 2014 | DOI: 10.1111/ene.12400

Original Article

Background and purpose

Median nerve somatosensory evoked potential (SEP) recordings play an important role in outcome algorithms in comatose patients after cardiopulmonary resuscitation. Knowledge of technical difficulties, clinical implications and uniform interpretation of SEP recordings is crucial. The aim of this study was to evaluate the skills of neurologists to interpret SEP recordings in post‐anoxic patients.

Methods

Nationwide Dutch clinical neurophysiology examinations from 2007, 2008 and 2011, containing SEP related questions, were analysed. Participants were classified as neurology residents, neurologists with less than 10 years of experience, neurologists with more than 10 years of experience and clinical neurophysiologists. End‐points were the knowledge of all participants about SEP recordings per year as well as improvement in knowledge over the years, as reflected by the test scores.

Results

A total of 194 participants completed the examination in 2007, 200 in 2008 and 263 in 2011. Between 2007 and 2008, all groups of respondents showed a significant increase in percentage of correct answers to SEP questions. Sixty‐six participants completed all three examinations. The SEP score of this group improved in 2008 [75%, interquartile range (IQR) 50–75,  < 0.001] compared with 2007 (38%, IQR 38−50); there was no further improvement in 2011 (69%, IQR 54−77).

Conclusion

Continuing education about technical knowledge, possible pitfalls and interpretation of SEP recordings remains of utmost importance.

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Authors:
B. Jaeger, A. Bouwes, J. M. Binnekade, A. A. Hilgevoord, J. Horn and A.‐F. van Rootselaar
Article first published online:
17 Mar 2014 | DOI: 10.1111/ene.12405

Original Article

Background and purpose

Hemodialysis (HD) may increase the risk of acute subdural hematoma (SDH) with high fatality, but the extent of this disease in non‐western populations is unclear. The incidence of and fatality from SDH in patients with end‐stage renal disease (ESRD) on HD were examined for an Asian population.

Methods

A cohort of 4709 newly diagnosed ESRD patients on HD from 1998 to 2010 and a control cohort of 18 663 subjects without any kidney disease were identified from a universal insurance claims database in Taiwan. The incidence and hazard of SDH for the two cohorts and 30‐day mortality from SDH were measured by the end of 2010.

Results

The incidence of SDH was 4.47‐fold higher in the HD cohort than in the control cohort (56.3 vs. 12.6 per 10 000 person‐years) with an adjusted hazard ratio (HR) of 3.81 (95% CI 2.77–5.25). HD patients with SDH had a high odds of 30‐day mortality with an adjusted odds ratio of 6.34 (95% CI 2.37–16.9).

Conclusions

ESRD patients with HD were demonstrated to be at high risk of subsequent SDH and to have a high mortality risk from SDH. Proper care for HD patients is necessary to prevent the devastating disorder.

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Authors:
I.‐K. Wang, C.‐L. Lin, Y.‐Y. Wu, H.‐L. Kuo, S.‐Y. Lin, C.‐T. Chang, T.‐H. Yen, F.‐R. Chuang, Y.‐K. Cheng, C.‐C. Huang and F.‐C. Sung
Article first published online:
01 Apr 2014 | DOI: 10.1111/ene.12406

Original Article

Background and purpose

Anti‐β2‐glycoprotein I (anti‐β2‐GPI) antibodies are part of the heterogeneous family of antiphospholipid antibodies and seem to be present in various neurological manifestations in addition to antiphospholipid syndrome (APS). Our objective was to analyse the clinical, radiological and therapeutic characteristics of neurological patients with positive anti‐β2‐GPI antibodies and without the Sapporo criteria for APS.

Methods

The medical records were retrospectively reviewed of 28 consecutive patients hospitalized in the Neurology Department of Strasbourg University Hospital, France, in whom anti‐β2‐GPI antibodies (immunoglobulin G and/or immunoglobulin M) were positive and other antiphospholipid antibodies negative, from November 2005 to July 2011. Clinical, radiological, biological and therapeutic data and clinical course were studied.

Results

Positive anti‐β2‐GPI antibodies were present in 28 patients. The predominant physiopathological process was mainly inflammatory (25% with myelitis, 14.3% with optic neuritis) or vascular (14.3% with cerebral ischaemia, 7.1% with cerebral vasculitis). Brain magnetic resonance imaging was performed in 89.3% of patients: atypical lesions were observed in 44% and typical inflammatory and vascular lesions in 16% and 12%, respectively.

Conclusion

The anti‐β2‐GPI antibody seems to be involved in two types of neurological disease: vascular or inflammatory ‘multiple sclerosis‐like’ disease. These two types of patients frequently develop an autoimmune disease (multiple sclerosis, systemic lupus erythematosus, APS). However, a large proportion of the patients had an undefined profile with aspecific cerebral lesions and required monitoring. This study raises questions about a separate entity at the border between APS and multiple sclerosis which remains to be better defined in a larger cohort.

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Authors:
M. Renaud, J. Aupy, B. Uring‐Lambert, J.‐B. Chanson, N. Collongues, F. Blanc and J. De Sèze
Article first published online:
02 Apr 2014 | DOI: 10.1111/ene.12408

Original Article

Background and purpose

To investigate the spectrum and risks of accidental injuries (AIs) amongst Parkinson disease (PD) patients.

Methods

The participants comprised PD patients aged 50 years and older who were initially diagnosed between 2000 and 2009, and a comparison group of non‐PD patients. The incidence rates of accidental injury types amongst PD and non‐PD patients were calculated; hazard ratios were calculated and adjusted for comorbidities, using 95% confidence intervals (CIs) of developing such outcomes in PD patients.

Results

In total, 4046 PD patients and 16 184 non‐PD patients were followed over time. The PD patients demonstrated the following incidence rates and hazard ratios in comparison to the control cohort for accidental injuries: all injuries, 19.78 per 100 person‐years (100 PYs), adjusted hazard ratio (HR) 1.30 (95% CI 1.24–1.36); head injury, 2.95 per 100 PYs, HR 1.88 (95% CI 1.64–2.15); bone fracture and dislocation, 4.61 per 100 PYs, HR 1.39 (95% CI 1.25–1.54); burns, 0.66 per 100 PYs, HR 1.01 (95% CI 0.78–1.32); injury to spinal cord, plexus and nerves, 0.15 per 100 PYs, HR 1.25 (95% CI 0.72–2.17); superficial injuries and contusions, 11.41 per 100 PYs, HR 1.20 (95% CI 1.12–1.27). The injury risk for the 69–79 years age group in PD compared with controls of the same age (HR 1.38) was significantly higher compared with that of the 50–69 age groups in PD and controls (HR 1.16).

Conclusions

Parkinson disease patients demonstrate a significantly elevated risk of developing all accidental injury types except burn injuries and injuries to spinal cord, plexus and nerves, compared with age‐matched controls. The risk increases as age increases.

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Authors:
H.‐C. Wang, C.‐C. Lin, C.‐I. Lau, A. Chang, F.‐C. Sung and C.‐H. Kao
Article first published online:
17 Mar 2014 | DOI: 10.1111/ene.12410

Original Article

Background and purpose

B‐type natriuretric peptide (BNP) is a marker of cardiac dysfunction that is released from myocytes in response to ventricular wall stress. Previous studies suggested that BNP predicts stroke events in addition to classical risk factors. It was suggested that the BNP‐associated risk results from coronary atherosclerosis or atrial fibrillation.

Methods

Three thousand six hundred and seventy five subjects from the population‐based Heinz Nixdorf Recall study (45–75 years; 47.6% men) without previous stroke, coronary heart disease, myocardial infarcts, open cardiac valve surgery, pacemakers and defibrillators were followed up over 110.1 ± 23.1 months. Cox proportional hazards regressions were used to examine BNP as a stroke predictor in addition to vascular risk factors (age, gender, systolic blood pressure, low‐density lipoprotein, high‐density lipoprotein, diabetes, smoking), renal insufficiency, atrial fibrillation/known heart failure and coronary artery calcification.

Results

Eighty‐nine incident strokes occurred (80 ischaemic, 9 hemorrhagic). Subjects suffering stroke had significantly higher BNP values at baseline than the remaining subjects [26.3 (Q1; Q3 = 12.9; 51.0) vs. 17.4 (9.4; 31.4); <0.001]. In a multivariable regression, logBNP was an independent stroke predictor [hazard ratio 1.96, 95% confidence interval (CI) 1.13–3.41; =0.017] in addition to age (1.24 per 5 years, CI 1.04–1.49; =0.016), systolic blood pressure (1.25 per 10 mmHg, CI 1.14–1.38; <0.001), smoking (2.05, CI 1.24–3.39; =0.005), atrial fibrillation/heart failure (2.25, CI 1.05–4.83; =0.037) and computed‐tomography‐based log(coronary artery calcification + 1) (1.47, CI 1.15–1.88; =0.002). LogBNP predicted stroke in men but not women, both in subjects ≤65 and >65 years. In subsequent analyses, BNP discriminated the incidence of cardioembolic stroke ( for trend = 0.001), but not stroke of macroangiopathic (=0.555), microangiopathic (=0.809) or unknown (=0.367) origin.

Conclusions

BNP predicts presumable cardioembolic stroke independent of coronary calcification.

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Authors:
K. Kara, J. Gronewold, T. Neumann, A. A. Mahabadi, C. Weimar, N. Lehmann, K. Berger, H. I. M. Kälsch, M. Bauer, M. Broecker‐Preuss, S. Möhlenkamp, S. Moebus, K.‐H. Jöckel, R. Erbel and D. M. Hermann
Article first published online:
24 Mar 2014 | DOI: 10.1111/ene.12411

Original Article

Background and purpose

To evaluate whether white matter hyperintensities (WMHs) may act as an independent predictor for progression of cognitive status, the authors analyzed the longitudinal effects of WMHs on cognitive dysfunction in non‐demented patients with Parkinson's disease (PD).

Methods

A total of 111 patients with PD were enrolled, including subjects with mild cognitive impairment (MCI,65) and cognitively normal subjects (CN,46). These individuals were classified as MCI converters (22) or MCI non‐converters (43) and CN converters (18) or CN non‐converters (28) based on whether they were subsequently diagnosed with PD dementia or PD‐MCI during a minimum 24‐month follow‐up. The WMH burden and the Cholinergic Pathway Hyperintensities Scale (CHIPS) and their relationships to longitudinal changes in cognitive performance were examined.

Results

PD‐MCI converters had larger WMH volume (14421.0 vs. 5180.4, < 0.001) and higher CHIPS score (22.6 vs. 11.2, 0.001) compared with PD‐MCI non‐converters. Logistic regression analysis revealed in patients with PD‐MCI that WMH volume (odds ratio 1.616, 0.009) and CHIPS score (odds ratio 1.084, 0.007) were independently associated with PD dementia conversion. However, WMH volume and CHIPS score did not differ between PD‐CN converters and PD‐CN non‐converters. In patients with PD‐MCI, both WMH volume and CHIPS score were closely associated with longitudinal decline in general cognition, semantic fluency and Stroop test scores.

Conclusions

The present study demonstrates that WMH burden is a significant predictor of conversion from PD‐MCI to PD dementia and is related to ongoing decline in frontal‐lobe‐based cognitive performance.

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Authors:
M. K. Sunwoo, S. Jeon, J. H. Ham, J. Y. Hong, J. E. Lee, J.‐M. Lee, Y. H. Sohn and P. H. Lee
Article first published online:
24 Mar 2014 | DOI: 10.1111/ene.12412

CME Article

Background and purpose

Smoking and hypertension are risk factors for aneurysmal subarachnoid hemorrhage (aSAH), whilst excessive alcohol consumption is less consistently linked with aSAH. Perimesencephalic hemorrhage (PMH) is a benign subset of non‐aneurysmal subarachnoid hemorrhage. The exact cause of PMH is unknown, and its risk factor profile may help to elucidate the pathogenesis. The influence of smoking, hypertension and excessive alcohol consumption on the occurrence of PMH was studied.

Methods

Seventy‐nine patients admitted with a PMH to the University Medical Center Utrecht were studied. As controls 574 persons were selected from five different general practices in the referral region of the University Medical Center Utrecht. All participants filled in a questionnaire about smoking habits, the presence of hypertension and alcohol consumption before their hemorrhage. Odds ratios (ORs) with corresponding 95% confidence intervals (CIs) were calculated to assess the association of risk factors and PMH, and multivariable logistic regression was used to adjust for possible confounding by age and sex.

Results

Adjusted ORs for the occurrence of PMH were 1.7 (95% CI 1.0–2.8) for smoking cigarettes, cigars, pipes or any combination of these, 1.1 (95% CI 0.6–2.0) for hypertension and 1.1 (95% CI 0.5–2.1) for excessive alcohol consumption.

Conclusions

Similar to aSAH, smoking is a risk factor for PMH and excessive alcohol consumption is not. In contrast to aSAH, hypertension is not a risk factor for PMH. This implies that the pathophysiological mechanisms causing PMH might be slightly different from those causing aSAH.

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Authors:
L. A. Mensing, Y. M. Ruigrok, P. Greebe, M. H. M. Vlak, A. Algra and G. J. E. Rinkel
Article first published online:
15 Mar 2014 | DOI: 10.1111/ene.12414

Letter to the Editor

Cranial, axial and proximal myopathy and hypertrophic cardiomyopathy caused by a mutation in the globular head region of the gene

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Authors:
J. Díaz‐Manera, A. Alejaldre, J. Llauger, S. Mirabet, R. Rojas‐García, A. Ramos‐Fransi, E. Gallardo and I. Illa
Article first published online:
08 May 2014 | DOI: 10.1111/ene.12416

Letter to the Editor

Carotid web and stroke

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Authors:
P. M. C. Choi, B. K. Menon and A. M. Demchuk
Article first published online:
08 May 2014 | DOI: 10.1111/ene.12418

Editorial

The European Academy of Neurology is founded: a fundamental step linking the glorious past with our future challenges

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Authors:
C. L. Bassetti and R. Hughes
Article first published online:
08 May 2014 | DOI: 10.1111/ene.12454

Calendar

Calendar

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Article first published online:
08 May 2014 | DOI: 10.1111/ene.12455