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Epilepsy treatment priorities: answering the questions that matter
Introduction ‘Nothing about them, without them’ is the clarion call of those who are committed to genuine user participation in research. The James Lind Alliance brings patients, carers and clinicians together as priority sharing partnerships to ensure that research is targeted at questions that matter to these interested parties. In 2010, we contributed to the Database of Uncertainties of the Effects of Treatments (DUETs). Three hundred ninety-eight questions regarding treatments and their effects were produced from focus groups composed of people with epilepsy, their carers and relatives, or epilepsy clinicia...
Source: Journal of Neurology, Neurosurgery and Psychiatry - October 9, 2017 Category: Neurosurgery Authors: Hughes, E. G., Thomas, R. H. Tags: PostScript Source Type: research

The TMEM106B risk allele is associated with lower cortical volumes in a clinically diagnosed frontotemporal dementia cohort
Introduction Frontotemporal dementia (FTD) is a neurodegenerative disease associated with impaired behaviour, language and motor function. Around a third of FTD is familial, with mutations in microtubule-associated protein tau (MAPT), progranulin (GRN) and chromosome 9 open reading frame 72 (C9orf72) being the most common genetic causes. No other risk factors for FTD had been identified until a genome-wide association study of patients with the most common pathological form of FTD, frontotemporal lobar degeneration with TDP-43 inclusions (FTLD-TDP), showed an association with single nucleotide polymorphisms (SNPs) in a reg...
Source: Journal of Neurology, Neurosurgery and Psychiatry - October 9, 2017 Category: Neurosurgery Authors: Harding, S. R., Bocchetta, M., Gordon, E., Cash, D. M., Cardoso, M. J., Druyeh, R., Ourselin, S., Warren, J. D., Mead, S., Rohrer, J. D. Tags: Open access PostScript Source Type: research

Familial primary lateral sclerosis or dementia associated with Arg573Gly TBK1 mutation
Primary lateral sclerosis (PLS) is a rare variant of motor neuron disease (MND) characterised by selective upper motor neuron features whose causes and pathogenic mechanisms remain largely unknown. While some familial cases of childhood to young–adult onset with recessive transmission have been reported in association with mutations in the Alsin, SPG11 and SPG7 genes,1 most adult cases occur sporadically. Recently, Tank-binding kinase 1 (TBK1) mutations have been identified in 1.9% of frontotemporal dementia(FTD) and/or Amyotrophic lateral sclerosis (ALS) cohorts.2 However, pathogenicity of many TBK1 missense mutatio...
Source: Journal of Neurology, Neurosurgery and Psychiatry - October 9, 2017 Category: Neurosurgery Authors: Gomez-Tortosa, E., Van der Zee, J., Ruggiero, M., Gijselinck, I., Esteban-Perez, J., Garcia-Redondo, A., Borrego-Hernandez, D., Navarro, E., Sainz, M. J., Perez-Perez, J., Cruts, M., Van Broeckhoven, C., Guerrero-Lopez, R., on behalf of the EU EOD Consort Tags: PostScript Source Type: research

The increasing impact of cerebral amyloid angiopathy: essential new insights for clinical practice
Cerebral amyloid angiopathy (CAA) has never been more relevant. The last 5 years have seen a rapid increase in publications and research in the field, with the development of new biomarkers for the disease, thanks to advances in MRI, amyloid positron emission tomography and cerebrospinal fluid biomarker analysis. The inadvertent development of CAA-like pathology in patients treated with amyloid-beta immunotherapy for Alzheimer’s disease has highlighted the importance of establishing how and why CAA develops; without this information, the use of these treatments may be unnecessarily restricted. Our understanding of th...
Source: Journal of Neurology, Neurosurgery and Psychiatry - October 9, 2017 Category: Neurosurgery Authors: Banerjee, G., Carare, R., Cordonnier, C., Greenberg, S. M., Schneider, J. A., Smith, E. E., Buchem, M. v., Grond, J. v. d., Verbeek, M. M., Werring, D. J. Tags: Open access, Editor's choice Cerebrovascular disease Source Type: research

The screening and management of pituitary dysfunction following traumatic brain injury in adults: British Neurotrauma Group guidance
This article summarises the current literature on PTHP, and sets out guidance for the screening and management of pituitary dysfunction in adult patients with TBI. It is hoped that future research will lead to more definitive recommendations in the form of guidelines. (Source: Journal of Neurology, Neurosurgery and Psychiatry)
Source: Journal of Neurology, Neurosurgery and Psychiatry - October 9, 2017 Category: Neurosurgery Authors: Tan, C. L., Alavi, S. A., Baldeweg, S. E., Belli, A., Carson, A., Feeney, C., Goldstone, A. P., Greenwood, R., Menon, D. K., Simpson, H. L., Toogood, A. A., Gurnell, M., Hutchinson, P. J. Tags: Open access, Screening (epidemiology) Neurosurgery Source Type: research

Impulsivity in Parkinsons disease is associated with altered subthalamic but not globus pallidus internus activity
Background A significant subset of patients with Parkinson’s disease (PD) suffer from impulse control disorders (ICDs). A hallmark feature of many ICDs is the pursuit of rewarding behaviours despite negative consequences. Recent evidence implicates the subthalamic nucleus (STN) and globus pallidus internus (GPi) in reward and punishment processing, and deep brain stimulation (DBS) of these structures has been associated with changes in ICD symptoms. Methods We tested the hypothesis that in patients with PD diagnosed with ICD, neurons in the STN and GPi would be more responsive to reward-related stimuli and less resp...
Source: Journal of Neurology, Neurosurgery and Psychiatry - October 9, 2017 Category: Neurosurgery Authors: Rossi, P. J., Shute, J. B., Opri, E., Molina, R., Peden, C., Castellanos, O., Foote, K. D., Gunduz, A., Okun, M. S. Tags: Movement disorders Source Type: research

Pallidal deep brain stimulation for dystonia: a long term study
Background Pallidal deep brain stimulation (globus pallidus internus (GPi) DBS) is the best therapeutic option for disabling isolated idiopathic (IID) and inherited (INH) dystonia. Acquired dystonia (AD) may also benefit from GPi DBS. Efficacy and safety in the long-term remained to be established. Objective To retrospectively assess long-term clinical outcomes and safety in dystonic patients who underwent GPi DBS. Methods Patients were videotaped and assessed preoperatively and postoperatively (1-year and at last available follow-up) using the Burke-Fahn-Marsden Dystonia Rating Scale (motor score (BFMDRS-M); disability ...
Source: Journal of Neurology, Neurosurgery and Psychiatry - October 9, 2017 Category: Neurosurgery Authors: Meoni, S., Fraix, V., Castrioto, A., Benabid, A. L., Seigneuret, E., Vercueil, L., Pollak, P., Krack, P., Chevrier, E., Chabardes, S., Moro, E. Tags: Movement disorders Source Type: research

Impact of sleep-related symptoms on clinical motor subtypes and disability in Parkinsons disease: a multicentre cross-sectional study
Conclusion Our study found a significant relationship between sleep disturbances and clinical motor subtypes. An increased number of sleep-related symptoms had an impact on disease-related disability. (Source: Journal of Neurology, Neurosurgery and Psychiatry)
Source: Journal of Neurology, Neurosurgery and Psychiatry - October 9, 2017 Category: Neurosurgery Authors: Suzuki, K., Okuma, Y., Uchiyama, T., Miyamoto, M., Sakakibara, R., Shimo, Y., Hattori, N., Kuwabara, S., Yamamoto, T., Kaji, Y., Hirano, S., Kadowaki, T., Hirata, K., on behalf of the Kanto NMPD investigators Tags: Open access Movement disorders Source Type: research

Biomarkers predict outcome in Charcot-Marie-Tooth disease 1A
Conclusions In summary, we provide evidence that cutaneous transcripts in patients with CMT1A serve as disease severity and progression biomarkers and, if implemented into clinical trials, they could markedly accelerate the development of a therapy for CMT1A. (Source: Journal of Neurology, Neurosurgery and Psychiatry)
Source: Journal of Neurology, Neurosurgery and Psychiatry - October 9, 2017 Category: Neurosurgery Authors: Fledrich, R., Mannil, M., Leha, A., Ehbrecht, C., Solari, A., Pelayo-Negro, A. L., Berciano, J., Schlotter-Weigel, B., Schnizer, T. J., Prukop, T., Garcia-Angarita, N., Czesnik, D., Haberlova, J., Mazanec, R., Paulus, W., Beissbarth, T., Walter, M. C., TR Tags: Neuromuscular Source Type: research

Factors affecting seizure outcome after epilepsy surgery: an observational series
Conclusions and relevance Readily identified clinical features and investigations are associated with reduced probability of good outcome and need consideration when planning presurgical evaluation. (Source: Journal of Neurology, Neurosurgery and Psychiatry)
Source: Journal of Neurology, Neurosurgery and Psychiatry - October 9, 2017 Category: Neurosurgery Authors: Bell, G. S., de Tisi, J., Gonzalez-Fraile, J. C., Peacock, J. L., McEvoy, A. W., Harkness, W. F. J., Foong, J., Pope, R. A., Diehl, B., Sander, J. W., Duncan, J. S. Tags: Epilepsy Source Type: research

Functional connectivity disturbances of the ascending reticular activating system in temporal lobe epilepsy
Objective Seizures in temporal lobe epilepsy (TLE) disturb brain networks and lead to connectivity disturbances. We previously hypothesised that recurrent seizures in TLE may lead to abnormal connections involving subcortical activating structures including the ascending reticular activating system (ARAS), contributing to neocortical dysfunction and neurocognitive impairments. However, no studies of ARAS connectivity have been previously reported in patients with epilepsy. Methods We used resting-state functional MRI recordings in 27 patients with TLE (67% right sided) and 27 matched controls to examine functional connect...
Source: Journal of Neurology, Neurosurgery and Psychiatry - October 9, 2017 Category: Neurosurgery Authors: Englot, D. J., DHaese, P.-F., Konrad, P. E., Jacobs, M. L., Gore, J. C., Abou-Khalil, B. W., Morgan, V. L. Tags: Epilepsy Source Type: research

Cortical influences drive amyotrophic lateral sclerosis
The early motor manifestations of sporadic amyotrophic lateral sclerosis (ALS), while rarely documented, reflect failure of adaptive complex motor skills. The development of these skills correlates with progressive evolution of a direct corticomotoneuronal system that is unique to primates and markedly enhanced in humans. The failure of this system in ALS may translate into the split hand presentation, gait disturbance, split leg syndrome and bulbar symptomatology related to vocalisation and breathing, and possibly diffuse fasciculation, characteristic of ALS. Clinical neurophysiology of the brain employing transcranial ma...
Source: Journal of Neurology, Neurosurgery and Psychiatry - October 9, 2017 Category: Neurosurgery Authors: Eisen, A., Braak, H., Del Tredici, K., Lemon, R., Ludolph, A. C., Kiernan, M. C. Tags: JNNP Patients' choice Neurodegeneration Source Type: research

Patterns of atrophy in pathologically confirmed dementias: a voxelwise analysis
Conclusions Pathologically distinct dementias exhibit characteristic patterns of regional volume loss compared with controls and other dementias. Voxelwise differences identified in these cohorts highlight imaging signatures that may aid in the differentiation of dementia subtypes during life. The results of this study are available for further examination via NeuroVault (http://neurovault.org/collections/ADHMHOPN/). (Source: Journal of Neurology, Neurosurgery and Psychiatry)
Source: Journal of Neurology, Neurosurgery and Psychiatry - October 9, 2017 Category: Neurosurgery Authors: Harper, L., Bouwman, F., Burton, E. J., Barkhof, F., Scheltens, P., OBrien, J. T., Fox, N. C., Ridgway, G. R., Schott, J. M. Tags: Open access Neurodegeneration Source Type: research

Structural MRI correlates of amyotrophic lateral sclerosis progression
Conclusion A more rapid disease progression and poorer functional decline were associated with greater involvement of the extra-motor cortex and basal ganglia, suggesting that the spatial extent of brain involvement can be an indicator of the progression in ALS. (Source: Journal of Neurology, Neurosurgery and Psychiatry)
Source: Journal of Neurology, Neurosurgery and Psychiatry - October 9, 2017 Category: Neurosurgery Authors: Senda, J., Atsuta, N., Watanabe, H., Bagarinao, E., Imai, K., Yokoi, D., Riku, Y., Masuda, M., Nakamura, R., Watanabe, H., Ito, M., Katsuno, M., Naganawa, S., Sobue, G. Tags: Neurodegeneration Source Type: research

The reticular activating system in the wake of temporal lobe epilepsy: dazed and confused
Repeated consciousness-impairing seizures weaken the reticular activating system’s connections, possibly leading to the baseline neurocognitive dysfunction these patients experience How does the localised ictal activity of a temporal lobe seizure generate a global impairment of consciousness? And how do brief temporal lobe seizures beget long-lasting interictal changes in cognition? Dr Englot and his colleagues have pursued these questions over the past decade through several innovative studies, and have convincingly implicated the ascending reticular activating system (ARAS) as an important contributor.1–3 In ...
Source: Journal of Neurology, Neurosurgery and Psychiatry - October 9, 2017 Category: Neurosurgery Authors: Rolston, J. D. Tags: Editorial commentaries Source Type: research

Is pathology-specific diagnosis of the dementia possible in life?
Young-onset and elderly-onset Alzheimer’s produce different patterns of brain atrophy while pathological subtypes of frontotemporal dementia remain largely indistinguishable Establishing whether distinct pathological forms of dementia have anatomical signatures at presentation is monumental work involving the long-term follow-up of patients assessed sufficiently early in their course to allow MRI imaging, who are then enrolled into a brain bank with subsequent successful retrieval of their brains after death. Few places in the world are capable of this feat, and it is no surprise that the article by Harper et al1 rep...
Source: Journal of Neurology, Neurosurgery and Psychiatry - October 9, 2017 Category: Neurosurgery Authors: Hodges, J. R. Tags: Editorial commentaries Source Type: research

'Its the progression, doctor: what patients with motor neurone disease really are interested in
To see a patient and recognise amyotrophic lateral sclerosis as the underlying cause of progressive, painless paralysis without sensory symptoms has been deemed easy, and indeed it is for physicians seeing patients with motor neuron disease on a regular basis. Answering patients’ questions of ‘How bad is it? What will happen to me? How long do I have?’ is however no mean feat; these questions invariably challenge the treating physician to perform a thorough assessment of the extent and the aggressiveness of the disease. While the Amyotrophic lateral sclerosisfunctional rating scale (ALSFRS-R) provides a q...
Source: Journal of Neurology, Neurosurgery and Psychiatry - October 9, 2017 Category: Neurosurgery Authors: Grosskreutz, J. Tags: Open access Neurodegenerative Source Type: research

Bilateral frontal cortex encephalitis and paraparesis in a patient with anti-MOG antibodies
Encephalitis seldom causes paraparesis as the initial symptom. Here, we report a case of steroid-responsive bilateral frontal cortical encephalitis involving leg motor areas in a patient who presented with paraparesis on admission. Interestingly, the initial paraparesis evolved into an acute disseminated encephalomyelitis (ADEM)-like illness and optic neuritis, and the patient was found to be positive for anti-myelin oligodendrocyte glycoprotein (MOG) antibodies. Case report A 46-year-old man experienced transient dizziness in early September 2008. Brain MRI retrospectively showed a slight fluid attenuation inversion recov...
Source: Journal of Neurology, Neurosurgery and Psychiatry - May 23, 2017 Category: Neurosurgery Authors: Fujimori, J., Takai, Y., Nakashima, I., Sato, D. K., Takahashi, T., Kaneko, K., Nishiyama, S., Watanabe, M., Tanji, H., Kobayashi, M., Misu, T., Aoki, M., Fujihara, K. Tags: PostScript Source Type: research

Who thinks functional neurological symptoms are feigned, and what can we do about it?
Introduction Patients with functional neurological symptoms are often suspected of feigning their symptoms. These suspicions have been reported not only in the doctors1 and nurses2 who treat them, but also in the general public who commonly infer that patients described as having functional symptoms are simply pretending to be sick.3 We found that limited education was an important determinant in who made this inference, and that even very simple additional explanation substantially reduced it. Methods We conducted a survey of the people waiting in a general hospital outpatient department on their understanding of a number...
Source: Journal of Neurology, Neurosurgery and Psychiatry - May 23, 2017 Category: Neurosurgery Authors: Kanaan, R. A. A., Ding, J. M. Tags: PostScript Source Type: research

Clinician and patient perceptions of free will in movement disorders: mind the gap
Introduction Most people perceive that their actions arise from their own ‘free will’, commonly defined as the ability to choose how to act.1 Neurological disorders, such as epileptic seizures and movement disorders, are often regarded as conditions in which free will is undermined.2 Clinically, an action is considered involuntary when it is automatically performed and cannot be controlled. Our study aim was to explore patients’ views as well as clinicians’ views on ‘free will’ and voluntariness in three hyperkinetic disorders: tics, functional movement disorders (FMD, previously ‘...
Source: Journal of Neurology, Neurosurgery and Psychiatry - May 23, 2017 Category: Neurosurgery Authors: van der Salm, S. M., Cath, D. C., Rootselaar, A.-F. v., Koelman, J. H., de Haan, R. J., Tijssen, M. A., Meynen, G. Tags: PostScript Source Type: research

Impaired cerebral autoregulation: measurement and application to stroke
The objective of this review is to provide a comprehensive insight about physiology of autoregulation, measurement methodologies and clinical applications in stroke to help build a consensus for what should be included in an internationally agreed protocol for CA testing and monitoring, and to promote its translation into clinical bedside practice for stroke management. (Source: Journal of Neurology, Neurosurgery and Psychiatry)
Source: Journal of Neurology, Neurosurgery and Psychiatry - May 23, 2017 Category: Neurosurgery Authors: Xiong, L., Liu, X., Shang, T., Smielewski, P., Donnelly, J., Guo, Z.-n., Yang, Y., Leung, T., Czosnyka, M., Zhang, R., Liu, J., Wong, K. S. Tags: Cerebrovascular disease Source Type: research

Cognitive and psychosocial function in retired professional hockey players
Conclusions We found only subtle objective cognitive impairment in alumni athletes in the context of high subjective complaints and psychiatric impairment. Apolipoprotein 4 status related to psychiatric, but not cognitive status. These findings provide benchmarks for the degree of cognitive and behavioural impairment in retired professional athletes and a point of comparison for future neuroimaging and longitudinal studies. (Source: Journal of Neurology, Neurosurgery and Psychiatry)
Source: Journal of Neurology, Neurosurgery and Psychiatry - May 23, 2017 Category: Neurosurgery Authors: Esopenko, C., Chow, T. W., Tartaglia, M. C., Bacopulos, A., Kumar, P., Binns, M. A., Kennedy, J. L., Müller, D. J., Levine, B. Tags: Cognition Source Type: research

Long-term health outcomes after exposure to repeated concussion in elite level: rugby union players
Conclusions Despite a high number of concussions in RIRP, differences in mental health, social or work functioning were not found late after injury. Subtle group differences were detected on two cognitive tests, the cause of which is uncertain. Prospective group comparison studies on representative cohorts are required. (Source: Journal of Neurology, Neurosurgery and Psychiatry)
Source: Journal of Neurology, Neurosurgery and Psychiatry - May 23, 2017 Category: Neurosurgery Authors: McMillan, T. M., McSkimming, P., Wainman-Lefley, J., Maclean, L. M., Hay, J., McConnachie, A., Stewart, W. Tags: Editor's choice, Drugs: CNS (not psychiatric), Neurological injury, Trauma CNS / PNS, Trauma, Injury Neuropsychiatry Source Type: research

Rates and correlates of suicidal ideation among stroke survivors: a meta-analysis
Background A better understanding of the epidemiological impact of suicidal ideation after stroke is required to identify subjects needing personalised interventions. Objective The aim of this meta-analysis was to estimate rates and correlates of suicidal ideation among stroke survivors. Methods We searched via Ovid, Medline, Embase and PsycInfo from database inception until August 2016. Predefined outcomes were (1) rates of suicidal ideation based on random-effects pooled proportion and (2) relevant sociodemographic and clinical correlates, using random-effects odds ratio (OR) or standardised mean difference (SMD) for c...
Source: Journal of Neurology, Neurosurgery and Psychiatry - May 23, 2017 Category: Neurosurgery Authors: Bartoli, F., Pompili, M., Lillia, N., Crocamo, C., Salemi, G., Clerici, M., Carra, G. Tags: Neuropsychiatry Source Type: research

Cingulo-insular structural alterations associated with psychogenic symptoms, childhood abuse and PTSD in functional neurological disorders
Conclusions This study reveals distinct cingulo-insular alterations for FND and PTSD symptoms and may advance our understanding of FND. Potential biological convergence between stress-related neuroplasticity, functional neurological symptoms and reduced insular volume was identified. (Source: Journal of Neurology, Neurosurgery and Psychiatry)
Source: Journal of Neurology, Neurosurgery and Psychiatry - May 23, 2017 Category: Neurosurgery Authors: Perez, D. L., Matin, N., Barsky, A., Costumero-Ramos, V., Makaretz, S. J., Young, S. S., Sepulcre, J., LaFrance, W. C., Keshavan, M. S., Dickerson, B. C. Tags: Neuropsychiatry Source Type: research

Randomised feasibility study of physiotherapy for patients with functional motor symptoms
Conclusions This feasibility study demonstrated high rates of recruitment, retention and acceptability. Clinical effect size was moderate to large with high probability of being cost-effective. A randomised controlled trial is needed. Trial registration number NCT02275000; Results. (Source: Journal of Neurology, Neurosurgery and Psychiatry)
Source: Journal of Neurology, Neurosurgery and Psychiatry - May 23, 2017 Category: Neurosurgery Authors: Nielsen, G., Buszewicz, M., Stevenson, F., Hunter, R., Holt, K., Dudziec, M., Ricciardi, L., Marsden, J., Joyce, E., Edwards, M. Tags: JNNP Patients' choice, Physiotherapy Neuropsychiatry Source Type: research

Differentiating lower motor neuron syndromes
Lower motor neuron (LMN) syndromes typically present with muscle wasting and weakness and may arise from pathology affecting the distal motor nerve up to the level of the anterior horn cell. A variety of hereditary causes are recognised, including spinal muscular atrophy, distal hereditary motor neuropathy and LMN variants of familial motor neuron disease. Recent genetic advances have resulted in the identification of a variety of disease-causing mutations. Immune-mediated disorders, including multifocal motor neuropathy and variants of chronic inflammatory demyelinating polyneuropathy, account for a proportion of LMN pres...
Source: Journal of Neurology, Neurosurgery and Psychiatry - May 23, 2017 Category: Neurosurgery Authors: Garg, N., Park, S. B., Vucic, S., Yiannikas, C., Spies, J., Howells, J., Huynh, W., Matamala, J. M., Krishnan, A. V., Pollard, J. D., Cornblath, D. R., Reilly, M. M., Kiernan, M. C. Tags: Open access Neuromuscular Source Type: research

Paranodal dissection in chronic inflammatory demyelinating polyneuropathy with anti-neurofascin-155 and anti-contactin-1 antibodies
Objective To investigate the morphological features of chronic inflammatory demyelinating polyneuropathy (CIDP) with autoantibodies directed against paranodal junctional molecules, particularly focusing on the fine structures of the paranodes. Methods We assessed sural nerve biopsy specimens obtained from 9 patients with CIDP with anti-neurofascin-155 antibodies and 1 patient with anti-contactin-1 antibodies. 13 patients with CIDP without these antibodies were also examined to compare pathological findings. Results Characteristic light and electron microscopy findings in transverse sections from patients with anti-neurof...
Source: Journal of Neurology, Neurosurgery and Psychiatry - May 23, 2017 Category: Neurosurgery Authors: Koike, H., Kadoya, M., Kaida, K.-i., Ikeda, S., Kawagashira, Y., Iijima, M., Kato, D., Ogata, H., Yamasaki, R., Matsukawa, N., Kira, J.-i., Katsuno, M., Sobue, G. Tags: Neuromuscular Source Type: research

Concussion, dementia and CTE: are we getting it very wrong?
A little less conversation, a little more science please There has been a large media interest over the recent years in the long-term health effects of concussive injuries sustained by sportsman, culminating in a Hollywood movie ‘Concussion’ starring Will Smith. Hardly a week goes by without further studies being announced in the media and creating considerable interest in social media. The message is that concussion is a dangerous condition that causes a complex neuropsychiatric disorder, chronic traumatic encephalopathy (CTE), with a neuropsychiatric phenotype of mood change, irritability and suicidal be...
Source: Journal of Neurology, Neurosurgery and Psychiatry - May 23, 2017 Category: Neurosurgery Authors: Carson, A. Tags: Editorial commentaries Source Type: research

Movement retraining for the dysfunctional brain
Physiotherapy for functional disorders is potentially highly effective in selected patients In their JNNP publication, Nielsen et al1 present data from a randomised controlled trial of 57 patients with functional (psychogenic) motor disorders, such as limb weakness, gait disorder, tremor and dystonia. They are a leading cause of neurological disability at all ages. They may be isolated but more commonly co-occur with other physical and psychological comorbidities such as fatigue, pain and anxiety. Randomised trials are scarce. The active group had just five consecutive days of twice daily physiotherapy, designed specifical...
Source: Journal of Neurology, Neurosurgery and Psychiatry - May 23, 2017 Category: Neurosurgery Authors: Stone, J., Carson, A. Tags: Editorial commentaries Source Type: research

Nodopathy: chronic inflammatory demyelinating polyneuropathy with anti-neurofascin 155 antibodies
Anti-neurofascin 155 antibodies cause myelin detachment and ‘nodopathy’ in a subgroup of patients diagnosed with chronic inflammatory demyelinating polyneuropathy While peripheral neuropathies have conventionally been classified into demyelinating or axonal, recent studies have proposed a novel concept, namely a ‘nodopathy’, whereby microstructural changes restricted to the nodal and paranodal regions may induce significant nerve dysfunction.1 The nodes of Ranvier are the sites where sodium channels are clustered at their highest density and therefore remain the critically important region for the g...
Source: Journal of Neurology, Neurosurgery and Psychiatry - May 23, 2017 Category: Neurosurgery Authors: Kuwabara, S., Misawa, S., Mori, M. Tags: Editorial commentaries Source Type: research

Listeria rhomboencephalomyelitis complicated by haemorrhagic transformation
Conclusions Concurrent Listeria myelitis with rhomboencephalitis has not been reported. Furthermore, the corresponding MRI hyperintensities demonstrated haemorrhagic transformation without abscess formation is a novel finding, likely secondary to bacterial invasion or localised necrotic inflammation of blood vessels leading to exudation of blood products. First line treatment of CNS Listeriosis is either ampicillin or benzylpenicillin with sulfamethoxazole/trimethoprim as second line treatment. Consideration of switching antibiotics in the event of inadequate clinical improvement can be trialled as Australian guidelines di...
Source: Journal of Neurology, Neurosurgery and Psychiatry - May 8, 2017 Category: Neurosurgery Authors: Nham, B., Baskin, J., Choong, H. Tags: Abstracts Source Type: research

Ketogenic diet to manage refractory epilepsy in the cdkl5 disorder
Conclusions Benefits of KD in the CDKL5 disorder coincide with findings of previous trials on refractory epilepsy. However, poor long-term efficacy remains as a significant barrier. In view of its side effects, KD should be commenced under the guidance of a paediatric neurologist and/or specialist dietitian. (Source: Journal of Neurology, Neurosurgery and Psychiatry)
Source: Journal of Neurology, Neurosurgery and Psychiatry - May 8, 2017 Category: Neurosurgery Authors: Lim, Z., Downs, J., Wong, K., Leonard, H. Tags: Abstracts Source Type: research

Paramedic validation of an australian large vessel occlusion triage algorithm for stroke
Objectives Endovascular reperfusion is now standard treatment for large vessel occlusion (LVO) but good outcomes depend on timely therapy. Accurate identification of LVO in the pre-hospital setting has therefore become a key priority to allow ambulance bypass to endovascular centres. Several tools such as the Spanish Rapid Arterial Occlusion Evaluation Scale (RACE)1 have been developed, but paramedic studies to date have shown low specificity, despite training.1,2 We aimed to develop an identification algorithm that would provide high accuracy when used by Australian paramedics. Methods A new LVO algorithm was developed f...
Source: Journal of Neurology, Neurosurgery and Psychiatry - May 8, 2017 Category: Neurosurgery Authors: Zhao, H., Pesavento, L., Coote, S., Churilov, L., Smith, K., Bernard, S., Yassi, N., Davis, S. M., Campbell, B. C. Tags: Abstracts Source Type: research

Screening for cognitive and behavioural impairment in patients with amyotrophic lateral sclerosis: frequency of abnormality and effect on survival
Conclusions There was a greater frequency of cognitive impairment in people with ALS than in patients with other neuromuscular diseases. The cognitive and behavioural tests are potential biomarkers of the prognosis of ALS. The results of cognitive tests are stable over 6 months and possibly longer. (Source: Journal of Neurology, Neurosurgery and Psychiatry)
Source: Journal of Neurology, Neurosurgery and Psychiatry - May 8, 2017 Category: Neurosurgery Authors: Xu, Z., Alruwaili, A., Henderson, R., McCombe, P. Tags: Abstracts Source Type: research

A potential 40% cost savings through treating cidp with tpe instead of ivig
Conclusions TPE is cheaper than IVIg for CIDP with respect to what is paid by DHB’s to the NZBS. It is unknown if similar cost savings exist in other healthcare systems. Potentially, IVIg may not need to be continued at full doses for an entire year and similarly TPE may not need to be continued at the same frequency. To address issues such as convenience of therapy and total patient care costs a prospective evaluation is needed. Furthermore, as there are no head-to-head IVIG-to-TPE studies in CIDP, the long-term efficacy of TPE in treating CIDP requires evaluation. (Source: Journal of Neurology, Neurosurgery and Psychiatry)
Source: Journal of Neurology, Neurosurgery and Psychiatry - May 8, 2017 Category: Neurosurgery Authors: Parry, G. J., Buenz, E. J., Ranta, A. Tags: Abstracts Source Type: research

Myasthenia gravis with an unusual pattern of weakness and positive igg anti gm1 antibody
Conclusions This case highlights the varied clinical presentation of NMJ disorder like myasthenia gravis. Whether the positive anti GM1 antibody in this case is a pathological antibody or if its presence is coincidental or an epiphenomenon is unclear. NMJ junction is rich in gangliosides and potential NMJ dysfunction may occur but clinical cases of myasthenia associated with antiGM1 antibodies has not been reported to our knowledge. (Source: Journal of Neurology, Neurosurgery and Psychiatry)
Source: Journal of Neurology, Neurosurgery and Psychiatry - May 8, 2017 Category: Neurosurgery Authors: Ng, J. L., Hooi, W. F., Malhotra, A., Talman, P. Tags: Abstracts Source Type: research

Efficacy and safety of teriflunomide in chinese patients with relapsing forms of ms: a subgroup analysis of the phase 3 tower study
Conclusions In Chinese patients enrolled in TOWER, teriflunomide 14 mg reduced ARR and risk of 12 w-CDW vs placebo; the small number of patients in this subgroup may have contributed to the lack of statistical significance observed for 12 w-CDW. However, there was a consistency of outcomes between the Chinese subpopulation and the overall TOWER population in terms of efficacy and safety. Study supported by Sanofi Genzyme. (Source: Journal of Neurology, Neurosurgery and Psychiatry)
Source: Journal of Neurology, Neurosurgery and Psychiatry - May 8, 2017 Category: Neurosurgery Authors: Macdonell, R., Freedman, M. S., Xu, X., Vucic, S., Truffinet, P., Benamor, M., Thangavelu, K., Miller, A. E. Tags: Abstracts Source Type: research

A better mbs for all australians
Conclusions We will report progress of the neurology clinical committee establishment. (Source: Journal of Neurology, Neurosurgery and Psychiatry)
Source: Journal of Neurology, Neurosurgery and Psychiatry - May 8, 2017 Category: Neurosurgery Authors: Robinson, B. Tags: Abstracts Source Type: research

Psychological interventions for psychogenic non-epileptic seizures: a meta-analysis
Conclusions The studies identified for this analysis were diverse in nature and quality. The findings highlight the potential for psychological interventions as a favourable alternative to the limited treatment options currently offered to people with PNES. (Source: Journal of Neurology, Neurosurgery and Psychiatry)
Source: Journal of Neurology, Neurosurgery and Psychiatry - May 8, 2017 Category: Neurosurgery Authors: Carlson, P., Perry, K. N. Tags: Abstracts Source Type: research

The hummingbird identifies psp among patients with non-fluent primary progressive aphasia
Objectives Non-fluent primary progressive aphasias include progressive non-fluent aphasia (PNFA), a subtype of frontotemporal dementia,1 and logopenic progressive aphasia (LPA), an atypical form of Alzheimer’s disease.1–3 Progressive supranuclear palsy (PSP) overlaps with PNFA at both clinical and pathological levels; cognitive deficits are well recognised and may precede the typical motor syndrome.4 The hummingbird sign, reflected in a reduced midbrain to pons ratio, distinguishes PSP from other parkinsonian disorders. Our objective was to determine if the midbrain to pons ratio (M/P) could be used as a biomar...
Source: Journal of Neurology, Neurosurgery and Psychiatry - May 8, 2017 Category: Neurosurgery Authors: Silsby, M., Tweedie-Cullen, R., Murray, C. R., Halliday, G. M., Hodges, J. R., Burrell, J. R. Tags: Abstracts Source Type: research

Energy expenditure in frontotemporal dementia: a behavioural and imaging study
Conclusions These neural correlates overlap the network involved in eating behaviour in FTD suggesting, a complex interaction between eating behaviour, autonomic function and energy homeostasis. The present study suggests that increased heart rate and autonomic changes are prevalent in bvFTD, and are associated with changes in energy expenditure. An understanding of these changes and neural correlates may have potential relevance to disease progression and prognosis. (Source: Journal of Neurology, Neurosurgery and Psychiatry)
Source: Journal of Neurology, Neurosurgery and Psychiatry - May 8, 2017 Category: Neurosurgery Authors: Ahmed, R. M., Landin-romero, R., Collette, T.-H., Klaauw, A. V. d., Devenney, E., Henning, E., Piguet, O., Farooqi, S., Hodges, J. R., Kiernan, M. C. Tags: Abstracts Source Type: research

Functional and structural connectivity in als: insights from mri connectome analyses and tms
Objectives Functional abnormalities of motor and extra-motor cortical regions are an intrinsic feature of amyotrophic lateral sclerosis (ALS). Connectomics is a novel field utilising whole brain network analysis, providing an in depth understanding of cortical networks. The present study utilised a multimodality approach encompassing connectome network and brain volume analysis, combined with clinical functional measures and cortical excitability studies to gather novel insights in ALS pathogenesis. Methods Resting state functional MRI was used to map functional connectomes in 20 ALS patients and 20 age-matched controls. ...
Source: Journal of Neurology, Neurosurgery and Psychiatry - May 8, 2017 Category: Neurosurgery Authors: Geevasinga, N., korgaonkar, M., Menon, P., Bos, M. V. D., Gomes, L., Forster, S., Kiernan, M., Vucic, S. Tags: Abstracts Source Type: research

Cortical hyperexcitability may contribute to disease spread in als
Objectives Amyotrophic lateral sclerosis (ALS) is characterised 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 (TMS) was undertaken in 50 sporadic ALS patients with recording of responses over both abductor polli...
Source: Journal of Neurology, Neurosurgery and Psychiatry - May 8, 2017 Category: Neurosurgery Authors: Menon, P., Geevasinga, N., Bos, M. V. d., Yiannikas, C., Kiernan, M. C., Vucic, S. Tags: Abstracts Source Type: research

JC virus conversion rates in natalizumab treated patients: the melbourne longitudinal cohort study
Conclusions In contrast to recently published data from colleagues in France and Germany, in our longitudinal cohort study rates of durable positive JCV seroconversion correlate closely with expected background rates of JCV seroconversion in the general population. In addition, JCV seroconversion and positive serostatus is not increased in patients with prior exposure to immunosuppressive agents. The low seroconversion rates seen after the first year of exposure suggests that less frequent JCV testing of natalizumab treated patients may be safe. (Source: Journal of Neurology, Neurosurgery and Psychiatry)
Source: Journal of Neurology, Neurosurgery and Psychiatry - May 8, 2017 Category: Neurosurgery Authors: Dwyer, C., Jokubaitis, V., Baker, J., Haartsen, J., Rath, L., Coleman, J., Fryer, K., Macintyre, J., Cartwright, A., Skibina, O., Macdonell, R., Butler, E., Shuey, N., Butzkueven, H., Kilpatrick, T., Walt, A. v. d. Tags: Abstracts Source Type: research

Imbalance in cortical inhibition-excitation networks underlies als
Objectives Recent cellular work in TDP-43 models of ALS convincingly demonstrated the importance of somatostatin interneuronal circuits in mediating corticomotoneuronal hyperexcitability, previously indirectly measured by short interval intracortical inhibition (SICI). Somatostatin interneurons reside in layers 2–3 of the primary motor cortex, with their output projecting directly onto and modulating pyramidal tract neurons. These circuits can be non-invasively interrogated using TMS. As such, the present study developed a novel threshold tracking TMS technique of this excitatory output, termed short interval intraco...
Source: Journal of Neurology, Neurosurgery and Psychiatry - May 8, 2017 Category: Neurosurgery Authors: Bos, M. A. V. d., Geevasinga, N., Menon, P., Kiernan, M. C., Vucic, S. Tags: Abstracts Source Type: research

Hypermetabolism in motor neurone disease is associated with a greater functional decline but not weight loss
Objectives Motor Neurone Disease (MND) is fatal neurological disease. Hypermetabolism (increased resting energy expenditure (REE)) and loss of body weight occur in MND. We aimed to determine whether hypermetabolism correlates with disease severity and weight loss. Methods Patients (n=44) and control participants (n=45) presented at the MND clinic at the CCR following an overnight fast. Body composition was assessed using air displacement plethysmography (BodPod; COSMED). REE was assessed by indirect calorimetry (Quark RMR, COSMED) and a metabolic index (MI;% change in measured REE versus predicted REE) determined. Hyperme...
Source: Journal of Neurology, Neurosurgery and Psychiatry - May 8, 2017 Category: Neurosurgery Authors: Ioannides, Z. A., Ngo, S. T., Henderson, R. D., McCombe, P. A., Steyn, F. J. Tags: Abstracts Source Type: research

Comparing magnetic resonance imaging brain atrophy measurement techniques in multiple sclerosis clinical practice: longitudinal assessment
Objectives To determine the annualised percentage whole brain volume change (PBVC) between two time-points using Structural Image Evaluation using Normalisation of Atrophy (SIENA), SIENA Cross-sectional (SIENAX), and MSmetrixTM-longitudinal (MSmetrix-long) in a real-world multiple sclerosis (MS) cohort. To compare the whole brain atrophy (WBA) measurements derived by two registration-based longitudinal software methods (SIENA, MSmetrix-long), and a cross-sectional, segmentation-based method (SIENAX). Methods Clinical MRI scans (1 mm3 3DT1 IR-FSPGR; GE 3.0T scanner) were acquired from 102 MS patients at baseline and approx...
Source: Journal of Neurology, Neurosurgery and Psychiatry - May 8, 2017 Category: Neurosurgery Authors: Beadnall, H. N., Wang, C., Hecke, W. V., Ribbens, A., Barnett, M. H. Tags: Abstracts Source Type: research

timing of high-efficacy disease modifying therapies for relapsing-remitting multiple sclerosis
Conclusions Early commencement of high-efficacy DMTs in relapsing-remitting multiple sclerosis provides superior control over relapse activity than their delayed commencement. However, disability outcomes on high-efficacy DMTs are independent of the timing of therapy. (Source: Journal of Neurology, Neurosurgery and Psychiatry)
Source: Journal of Neurology, Neurosurgery and Psychiatry - May 8, 2017 Category: Neurosurgery Authors: Merkel, B., Jokubaitis, V., Spelman, T., Lechner-Scott, J., McCombe, P., Slee, M., Vucic, S., Skibina, O., Barnett, M., Hodgkinson, S., Butzkueven, H., Kalincik, T. Tags: Abstracts Source Type: research

The many faces of anti-glycine receptor related disease: a case series and literature review
Conclusions Anti-glycine receptor antibody related neurological disease encompasses a broader clinical spectrum than originally reported with a greater association with epileptic seizures demonstrated both in the case series presented and in a review of the literature. (Source: Journal of Neurology, Neurosurgery and Psychiatry)
Source: Journal of Neurology, Neurosurgery and Psychiatry - May 8, 2017 Category: Neurosurgery Authors: Swayne, A., Tjoa, L., Wong, R., Gillis, D., Schweitzer, D., Tsang, B., McNabb, A., Broadley, S., Blum, S. Tags: Abstracts Source Type: research