bluuebunny/crossref_metadata_2025_split huggingface dataset:
041 Predictors of clinical outcomes in patients with brain abscess
Authors: Amornpojnimman Thanyalak , Korathanakhun Pat 
Published: May 2018

IntroductionBrain abscess is an intra-parenchymal suppurative infectious condition of the brain caused by various microorganisms and potentially resulting in a life-threatening condition. Despite Thailand being located in a tropical region, studies on brain abscess are quite limited and outdated. Most of the published data in the Thai population were collected during a period when advanced neuroimaging techniques were not widely available. This issue contributed to late diagnosis and treatment that impacted the clinical outcomes. Therefore, our study aims to identify predictors of mortality among patients with brain abscess in Thailand.MethodsWe retrospectively studied patients diagnosed with brain abscess admitted to Songklanagarind hospital in Songkhla, Thailand between 2002 and 2017. Demographic data, neurological status, clinical presentations, predisposing factors, microbiological profiles, neuroimaging findings, treatments, and outcomes were collected from electrical medical records. Predictors of death outcome were analysed by univariate and multivariate logistic regression analysis.Results81 patients were enrolled in the study. Forty-seven patients (58%) were male and 34 patients (42%) were female. The overall mean age was 47.68±16.92 years. The common risk factors of brain abscess were immunocompromised state (38.3%) and the extension of a peri-cranial infection (24.7%). The common clinical presentations were headache (61.7%), fever (50.6%), and hemiplegia (34.6%). Eleven patients (13.6%) were dead at hospital discharge. The independent factor associated with death outcome identified by multivariate analysis was confusion (odds ratio 7.67, 95% CI 1.95–30.14; p=0.003).ConclusionThe current study shows that increased immunocompromised state is a predisposing factor of brain abscess. The independent factor associated with death was confusion, which correlated with septic encephalopathy.
046 Efficacy and safety of intravenous immunoglobulin (IVIG) IGPRO10 in chronic inflammatory demyelinating polyneuropathy (CIDP)
Authors: Sabet Arman , Mielke Orell , Schaik Ivo N van , Leger Jean-Marc , Bril Vera , Gloven Nan van , Hartung Hans Peter , Lewis Richard A , Sobue Gen , Lawo John-Philip , Durn Billie L , Cornblath David R , Bleecker Jan LDe , Sommer Claudia , Robberecht Wim , Saarela Mika , Kamienowski Jerzy , Stelmasiak Zbigniew , Tackenberg Bjorn , Merkies Ingemar SJ 
Published: May 2018

IntroductionWe analysed the efficacy and safety of IVIG IgPro10 (CSL Behring) in two CIDP studies: PRIMA and PATH.MethodsPRIMA was a prospective, open-label, single-arm study in 28 CIDP patients (n=13 IVIG-pretreated; n=15 untreated) investigating efficacy and safety of IgPro10 for induction (2 g/kg) and maintenance therapy (1 g/kg every 3 weeks for 21 weeks). This regimen was also used for 207 IVIG-pretreated patients during the 10–13 week pre-randomization phase of the PATH study (before randomization to subcutaneous immunoglobulin maintenance therapy or placebo). Both studies investigated a 1-point decrease in adjusted Inflammatory Neuropathy Cause and Treatment (INCAT) disability score as a response parameter, and evaluated changes in mean grip strength and Medical Research Council (MRC) score. Treatment-emergent adverse events (AEs) were assessed.ResultsResponse rate was 76.9% (95% confidence interval [CI] 49.7–91.8) in PRIMA (IVIG-pretreated patients) at week 21% and 72.9% (95%CI 66.5–78.5) in PATH at week 13; median time to first INCAT response was 3.0 and 3.7 weeks, respectively. Median (Q1;Q3) improvements in outcome measures (baseline to last observation) for PRIMA pre-treated patients and PATH, respectively, were: INCAT, −2.0 (-3.0;−1.0) and −1.0 (-2.0;0.0) points; grip strength, 5.0 (-9.0;22.0) and 9.4 (1.3;18.8) kPa; and MRC score, 5.0 (3.0;10.0) and 3.0 (0.0;6.0) points. In the PRIMA safety population (n=28), 108 AEs occurred in 22 (78.6%) patients (0.417/infusion); 284 AEs in 100 (48.3%) patients (0.175/infusion) were reported in the PATH safety population (n=207). Headache was the most frequent AE. Causally related serious AEs in PRIMA and PATH occurred in 2 and 7 patients, respectively.ConclusionSimilar efficacy results of IgPro10 in CIDP were observed in PRIMA and the PATH pre-randomization period. IgPro10 is well tolerated, with clinically meaningful improvement in disability in CIDP patients.Study SupportCSL Behring
056 Perampanel and secondarily generalised seizures in a pooled analysis of phase III studies and their open-label extension: effect of enzyme-inducing antiepileptic drugs
Authors: Ko David , Williams Betsy , Patten Anna , Laurenza Antonio 
Published: May 2018

IntroductionPerampanel is approved for adjunctive treatment of partial seizures, with or without secondarily generalised seizures (SGS), and primary generalised tonic-clonic seizures in epilepsy patients aged ≥12 years. Approval of perampanel for partial seizures was based on three randomised, double-blind, placebo-controlled, Phase III Studies 304 (NCT00699972), 305 (NCT00699582) and 306 (NCT00700310); patients completing these could enter open-label extension (OLEx) Study 307 (NCT00735397). Here, we report efficacy of perampanel as adjunctive treatment of SGS by co-administration of enzyme-inducing antiepileptic drugs (EIAEDs) versus non-EIAEDs in both the Phase III and OLEx studies.MethodsIn the double-blind studies, patients (≥12 years) with partial seizures, with or without SGS, receiving 1–3 AEDs at Baseline were randomised to placebo or 2–12 mg/day perampanel for 19 weeks. In the OLEx, patients received ≤12 mg/day perampanel for ≤272 weeks. Efficacy assessments included median percent change in SGS frequency/28 days, SGS 50% and 75% responder and seizure-freedom rates.ResultsFor patients with SGS at pre-perampanel Baseline, 564 were in the double-blind studies and 388 received perampanel for ≥1 year in the OLEx. In the double-blind studies, perampanel co-administered with an EIAED (carbamazepine, eslicarbazepine, oxcarbazepine, phenytoin) had reduced efficacy compared with non-EIAEDs due to increased clearance; this was particularly evident at higher doses, although these differences were still greater than placebo. In the OLEx, concomitant administration of both non-EIAEDs and EIAEDs was associated with sustained efficacy, with slightly better efficacy during the first, second and third years of perampanel exposure for non-EIAEDs compared with EIAEDs.ConclusionPerampanel demonstrated good and sustained long-term efficacy against SGS. With the recent FDA approval of perampanel for monotherapy use for partial seizures, non-EIAED data may be more relevant for consideration if perampanel is used as a single agent (no other AED) while real-world data and experience are accumulated.Study supportEisai Inc.
058 ANTI-CASPR2-ANTIBODY associated encephalitis in a 63-year old male with chronic lymphocytic leukaemia
Authors: Lilley Kate , Swayne Andrew , Watson Emily , Kee Rachel , Wong David , Lehn Alexander , Brown Helen , O’Gorman Cullen , Sheikh Nabeel , Bird Robert , Prain Kerri , Gillis David , Wong Richard C , Blum Stefan 
Published: May 2018

IntroductionThis case from a tertiary neurology centre reports a novel association between the emerging clinical entity of anti-contactin associated protein-2 (CASPR-2) antibody encephalitis and chronic lymphocytic leukaemia (CLL).CaseWe describe a 63 year old Maori male truck-driver who presented with progressive altered personality, speech, cognition and perception over 9 months. The patient also developed choreiform movements, broad-based gait, incontinence, sleep apnoea with type 2 respiratory failure and episodic loss of consciousness. 12 months prior, he had been diagnosed with low-risk CLL, for which he remained untreated. MRI of the brain revealed mid-sagittal bilateral mid-temporal T2/FLAIR hyperintensities. Cerebrospinal fluid examination showed a mononuclear pleocytosis (WCC 270×10^6/L) with 15% of these CD5/CD19/CD23 positive and 92% CD3/CD5 positive CD 19 negative on flow cytometry, protein was also elevated at 2600 mg/L. The interplay between CLL and inflammation is uncertain. Anti-Caspr2-antibody was identified in CSF and serum. The patient was treated with a combination of fludarabine, cyclophosphamide, rituximab, dexamethasone and intravenous immunoglobulin (IVIG). Clinical status improved and antibody titre decreased from 580 to 241 pM in three weeks and to 55pM (negative <85 pM) at five months. Symptoms worsened when IVIG doses were missed. He returned home after inpatient rehabilitation, showed striking clinical improvement at 12 month follow-up and continues on maintenance IVIG therapy.ConclusionWhilst paraneoplastic VGKC encephalitis has been described associated with a number of malignancies, this is the first reported case of CASPR-2 antibody present in association with CLL.References. Van Sonderen A, Petit-Pedrol M, Dalmau J, Titulaer MJ. The value of LGI1, Caspr2 and voltage-gated potassium channel antibodies in encephalitis. Nature Reviews Neurology2017;13(5):290–301.. Nogai H, Israel-Willner H, Zschenderlein R, Pezzutto A. Improvement in Paraneoplastic Limbic Encephalitis after Systemic Treatment with Rituximab in a Patient with B-Cell Chronic Lymphocytic Leukaemia. Case Reports in Haematology2013;2013:Article ID958704.. Van Sonderen A, Ariño H, Petit-Pedrol M, et al. The Clinical Spectrum of Caspr2 antibody-associated disease. Neurology2016;87:521–528.. Gultekin SH, Rosenfeld MR, Voltz R, Eichen J, et al. Paraneoplastic limbic encephalitis: Neurological symptoms, immunological findings and tumour association in 50 patients. Brain2000;123:1481–1494.
060 Association of brain volume loss and neda outcomes in patients with relapsing multiple sclerosis in the opera i and opera ii studies (ENCORE)
Authors: Traboulsee Anthony , Arnold Douglas , Klawiter Eric C , Havrdova Eva , Fiore Damian , Zheng Ray , Julian Laura , Giovannoni Gavin 
Published: May 2018

IntroductionBrain volume loss (BVL) occurs in multiple sclerosis (MS) patients, reflecting irreversible tissue damage. No evidence of disease activity (NEDA), a composite measure assessing absence of clinical and magnetic resonance imaging (MRI) disease activity has emerged as important treatment goal in MS and may be associated with preservation of brain tissue and BVL prevention.MethodsPatients in OPERA-I/II (NCT01247324/NCT01412333) received 600 mg ocrelizumab (intravenous) very 24 weeks or 44 µg subcutaneous interferon beta-1a (IFNβ-1a) 3x-weekly for 96 weeks. Brain MRI assessments were completed at baseline and 24/48/96 Weeks. Brain volume normalised for head size was measured using SIENAX software. Percent change in whole brain volume (WBV) was determined using SIENA software, changes in cortical grey (GMV) and white (WMV) matter volumes were measured using validated, locally developed Jacobian integrator atrophy software. NEDA was defined as absence of relapses, 12-week confirmed disability progression, T1 Gd–enhancing lesions and new and/or enlarging T2-lesions. Changes from baseline in brain volume were examined in NEDA patients and those with evidence of disease activity (EDA), using the mixed-effects model for repeated measures method.ResultsThe analysis included 1520 patients (ocrelizumab-761; IFNβ-1a-759). Over 96 weeks, 569 (37%) patients [ocrelizumab-363 (48%); IFNβ-1a-206(27%); p<0.001] had NEDA. Compared with EDA patients, NEDA patients had significantly less WBV loss from baseline (30%-reduction; p<0.001). In the NEDA group, ocrelizumab patients had significantly less WBV loss (32%-reduction; p<0.001), WMV loss (34%-reduction; p=0.044) and GMV loss (30%-reduction; p<0.001) from baseline than IFNβ-1a patients. In the EDA group, ocrelizumab patients had significantly less WBV loss (11%-reduction; p=0.047) and GMV loss (21%-reduction; p<0.001) but not WMV loss (1.03%-increase; p=0.90) from baseline than IFNβ-1a patients.ConclusionThese findings highlight the importance of NEDA as treatment goal with respect to brain tissue preservation regardless of treatment choice. Ocrelizumab may confer additional benefits in NEDA patients NEDA beyond what is observed with IFNβ-1a.
074 First seizures in emergency department vs ward: study in an elderly population
Authors: Foster Emma , Holper Sarah , Kwan Patrick 
Published: May 2018

IntroductionSeizures are common in hospitals, both as presentations to Emergency Departments (ED) and as hospital onset seizures (HOS), occurring in ward patients hospitalised for non-seizure reasons. Prompt identification of seizure aetiology is important, as it affects prognosis and management choices. Acute symptomatic seizures due to acute disturbance of brain function have a far lower risk of recurrence compared to unprovoked seizures. Timely investigations and specialist review assesses individual risk for seizure recurrence, which then guides therapeutic decisions including antiepileptic drug (AED) use. This study includes a larger proportion of older patients than usually reported, and as such, provides important insights into seizure aetiology and management strategies in this demographic.MethodsThis retrospective survey of medical charts reviewed patients aged 18 or over with a hospital separation coded as ICD-10 G40 (Epilepsy), G41 (Status epilepticus), or R56.9 (convulsions not otherwise specified), presenting between 1 January 2008 through 30 November 2016, to a large metropolitan private hospital. 97 episodes of ED attendance for first seizure and 54 episodes of HOS were identified.ResultsMedian age was 70 years in ED-cohort and 80.5 years in HOS-cohort. Symptomatic seizure risk factors were identified in 62.89% of ED-cohort and 83.33% of HOS-cohort, including exposure to known epileptogenic drugs in 38.89% of HOS-cohort. Antiepileptic drugs (AEDs) were prescribed on discharge to 74.23% of ED-cohort and 81.48% of HOS-cohort, but far fewer had scheduled Neurologist review (58.76% of ED- and 35.19% of HOS-cohorts).ConclusionThis study includes a larger proportion of older patients than usually reported, and as such, provides important insights into seizure aetiology and management strategies in this demographic. This includes caution when prescribing known epileptogenic drugs; mindful prescription of AED on discharge; and ensuring adequate Neurologist follow-up to monitor further seizure activity, addressing seizure risk factors, and ongoing need for AED.
076 The expanding clinical phenotype of NMDAR encephalitis
Authors: Qiu Jessica , Helou Jacob , Urriola Nicholas , Welgampola Miriam , Parratt John , Wong Toh Hock , Parratt Kaitlyn 
Published: May 2018

IntroductionThe classic phenotype of anti-N-methyl-d-aspartate receptor (NMDA-R) encephalitis is a constellation of prodromal psychiatric symptoms, seizures, movement disorder and progressive autonomic dysfunction, frequently associated with ovarian teratoma. We describe four cases illustrating the expanding phenotype of NMDA-R encephalitis. We present two cases from two tertiary hospitalsCase 1 A 31 year old woman presented with focal onset seizures. She developed epilepsia partialis continua. CSF and serum NMDA-R antibodies were detected and PET of the brain showed bi-frontal hyper-metabolism. The seizures resolved with immunotherapy.Case 2 A 26 year old woman presented with vomiting and dysequilibrium. She was found to have torsional downbeat nystagmus with otherwise normal eye movements and vestibulo-ocular reflexes. She developed bilateral appendicular and truncal ataxia, head bobbing and ocular flutter. PET demonstrated diffuse cerebellar hyper-metabolism. CSF NMDA-R antibodies were detected and an ovarian teratoma was identified.Case 3 A 67 year old right-handed woman was treated for HSV encephalitis. MRI of the brain demonstrated typical changes in the right anteromedial temporal lobe, inferior frontal lobe and insular cortex. Five months later, she deteriorated with progressive cognitive dysfunction and prominent orofacial dyskinesia. Initially both CSF and serum were negative for NMDAR antibodies, but repeat CSF and serum were positive.Case 4 A 61 year old woman presented with progressive cognitive impairment over eight months initially characterised by difficulty with calculations and making mistakes at work but subsequent severe impairment of language, executive function and memory and the development of facile and disinhibited behaviour. PET demonstrated mesial bi-temporal hyopo-metabolism and NMDA-R antibodies were detected in serum. She recovered with immunotherapy.ConclusionNMDA-R encephalitis should be considered in various neurological presentations including explosive onset focal epilepsy, rapidly progressive dementia, post HSV encephalitis and rhombencephalitis. Early detection is important given the frequent response to immunotherapy.
077 Recurrent guillain-barre syndrome
Authors: Hooi Wai Foong , Hutton Elspeth , Day Bruce 
Published: May 2018

IntroductionRecurrent Guillain-Barre syndrome (GBS) is very rare. It occurs in 2% to 5% of GBS patients. It is unclear as to why some patients have a recurrence and whether this occurs more frequently in a distinct group of patients. We report a case of a 75 year old man who had five discreet attacks of GBS in 30 years. He had an excellent response to intravenous immunoglobulin (IVIG) and achieved full clinical recovery after each attack.CaseA 75 year old man presented to the hospital with progressive ascending pattern of numbness and weakness in upper and lower limbs. This was in the setting of 10 days history of upper respiratory tract infective (URTI) symptoms. Neurological examination revealed distal sensory loss and distal weakness in upper and lower limbs. Deep tendon reflexes were absent. Nerve conduction studies showed features in keeping with GBS. He was treated with 5 days of intravenous immunoglobulin (IVIG) with significant improvement. Interestingly, this was on the background of having had 4 previous attacks of GBS in 1986 (preceded by acute gout), 1997 (preceded by the flu), 2010 (preceded by small bowel obstruction) and 2016 (preceded by viral gastroenteritis). He responded to IVIG each time and returned to his premorbid level function promptly after each attack.ConclusionRecurrent GBS is rare. It is important for clinician to be able to distinguish between recurrent GBS, treatment related fluctuations (GBS-TRF) and chronic inflammatory demyelinating polyneuropathy as the treatment and outcomes are different for each of these conditions.References. Kuitwaard K, Van Koningsveld R, Ruts L, Jacobs B, Van Doorn P. Recurrent Guillain Barre Syndrome. J Neurol Neurosurg Psychiatry2009;80:56–59.. Pyun SY, Jeong JH, Bae JS. Recurrent Guillain-Barré syndrome presenting stereotypic manifestations, positive antiganglioside antibodies, and rapid recovery. Clin Neurol Neurosurg2015Dec;139:230–3.. Mossberg N, Nordin M, Movitz C, Nilsson S, Hellstrand K, Bergström T, Andersson B, Andersen O. The recurrent Guillain-Barré syndrome: A long-term population-based study. Acta Neurol Scand2012Sep;126(3):154–61.
078 Deep brain stimulation (DBS) for dyskinetic cerebral palsy: a pilot study
Authors: Duma Stephen R , Mahant Neil , Ha Ainhi , Kim Samuel , Phu Amy , Stewart Kirsty , Waugh Mary-Clare , Wolfe Nigel , Russell Dale C , Owler Brian , Krause Martin , Fung Victor SC 
Published: May 2018

IntroductionDeep brain stimulation (DBS) targeting the internal segment of the globus pallidus (GPi) is effective for several forms of dystonia, particularly idiopathic isolated dystonia. DBS may also be helpful for some causes of chorea and other hyperkinetic disorders. A minority of people with cerebral palsy (CP) have dystonia or choreoathetoid movements (labelled dyskinetic CP). Treatment options to improve function for this group are limited. We aimed to investigate whether DBS is effective in reducing symptoms and improving function in dyskinetic CP.MethodsThis study was a randomised, placebo-controlled, double-blinded, crossover trial. Four participants (2M:2F, aged 11–48 year) with dyskinetic CP were included between 2010–2011. Participants underwent GPi DBS implantation and were randomised to active or sham stimulation for 3 months, following which their DBS stimulation was switched for a further 3 months. The Bourke-Fahn-Marsden Dystonia Rating Scale (BFMDRS) was used to rate the severity of dystonia at baseline, 3 months after initial treatment; and 3 months after crossover treatment. The study was terminated early due to slow recruitment.ResultsOne participant had a reduction in BFMDRS score with active stimulation; this participant was the oldest and had the mildest BFMDRS score. The remainder of the participants had either no change or a slight increase in BFMDRS score. Despite this, in longer-term follow-up, 3 participants reported symptomatic improvement and continue active DBS treatment 7–8 years post-surgery.ConclusionWe did not identify a benefit of GPi DBS for dyskinetic CP in our randomised controlled trial. However, 3 participants have had symptomatic improvement on long-term follow-up, consistent with other reports of benefit with GPi DBS. Limiting factors of the study include small sample size, participant heterogeneity and study design. It was noted that the final (efficacious) stimulation parameters required open label programming to achieve and were outside of those permitted by the protocol.
080 Electrodiagnostic and ultrasound correlates of clinical severity in ulnar neuropathy
Authors: Pelosi Luciana , Mulroy Eoin 
Published: May 2018

IntroductionThis is a retrospective review of 135 consecutive patients (M:F=71:64, mean age 52.6 years; 141 arms) referred to our institution with ulnar neuropathy over a two year period. We analysed electrodiagnostic and ultrasound findings in relation to clinical severity.MethodsAll patients underwent electrodiagnostic (AAN) and ultrasound examination of the symptomatic ulnar nerve(s). Clinical severity was graded on a 4 point scale from ‘very mild’ (symptoms only) to ‘severe’ (sensory loss plus marked atrophy of ulnar-innervated hand muscles).ResultsIn ‘very mild’ neuropathies, the number of abnormal electrodiagnostic and ultrasound studies was 2 and 11 respectively, out of 54; in ‘mild’ neuropathies 19 and 25 out of 40; in ‘moderate’, 23 and 24 out of 24; and in ‘severe’, 23 and 23 out of 23. In 25 arms, (18 of which were clinically ‘severe’ or ‘moderate’), electrophysiology was abnormal but non-localising. Ultrasound showed abnormally large cross-sectional area at the elbow in 22 and diffuse nerve enlargement in three. Ultrasound identified nerve subluxation in 24 (17%) neuropathies, 58% of which were ‘very mild’, 25% ‘mild’ and 17% ‘moderate’ or ‘severe’.ConclusionIn patients with symptoms but no clinical signs, electrophysiological evidence of ulnar neuropathy was present in 3.7%, whereas abnormal nerve ultrasound, often associated with subluxation, was demonstrated in 20%. Ultrasound increased the diagnostic yield of electrophysiology in the ‘very mild’ and, to a lesser extent, the ‘mild’ neuropathies by a combined 11%, and localised the lesion in all ulnar neuropathies with abnormal but non-localising electrophysiology. Nerve subluxation was disproportionately represented amongst the clinically ‘very mild’ neuropathies with abnormal ultrasound.