bluuebunny/crossref_metadata_2025_split huggingface dataset:
130 Continuous EEG monitoring in australia: s first year audit of practice 2018–2019 of a combined hospital epilepsy program in melbourne
Authors: Laing Joshua , Grant Georgia , Harb Cecilia , White Elise , O’Brien Terence , Kwan Patrick 
Published: July 2019

IntroductionContinuous EEG (cEEG) monitoring is essential for the optimal diagnosis and management of non-convulsive seizures. We audited the use of cEEG across two major metropolitan hospitals in Melbourne, Australia as part of a new combined epilepsy program. There are no reported case series of cEEG in Australia.MethodsData was collected retrospectively from consecutive patients who underwent cEEG monitoring as part of their acute inpatient care between Jan 2018 to Dec 2018 at the Alfred and Royal Melbourne Hospitals. All inpatient EEG studies over 1 hr were included. Elective cases from the epilepsy monitoring unit were excluded. Demographic and clinical information regarding their admission was collected. Descriptive statistics, and comparative analysis was performed.ResultsThere were 94 patients identified that underwent cEEG. 50% were male and 50% female, with an average age of 51.3 yrs. 45% were performed in the ICU, and 55% on the acute medical ward. The average duration of recording per patient was 86.2 hrs or 3.6 days. Of 89 of 94 available cEEG reports, seizures were seen in 55%, the majority being non-convulsive. Interictal discharges were seen in 60%. 19% (13/67 of available records) were dead at the time of the audit.ConclusionNon-convulsive seizures and non-convulsive status epilepticus is common and widely underrecognized without cEEG. We present 94 cases as part of our newly expanding cEEG program across two major Australian hospitals over a one year period, 55% with seizures which were predominantly non-convulsive. A prospective database will be designed for further quality improvement and future research.
022 Patient-initiated event monitoring for acute vertigo
Authors: Young Allison S , Lechner Corinna , Bradshaw Andrew P , MacDougall Hamish G , Black Deborah A , Halmagyi Michael G , Welgampola Miriam S 
Published: July 2019

IntroductionThe diagnosis of vestibular disorders may be facilitated by analysing patient-initiated capture of ictal nystagmus.MethodsAdults with a history of recurrent vertigo were taught to self-record spontaneous and positional-nystagmus at home while symptomatic, using video-goggles. Patients with final diagnoses of disorders presenting with recurrent vertigo were analysed: 121 patients with Ménière’s Disease (MD), Vestibular Migraine (VM), Benign Positional Vertigo (BPV), Episodic Ataxia Type II (EAII), Vestibular Paroxysmia (VP) or Superior Semicircular Canal Dehiscence (SSCD) were included.ResultsOf 43 MD patients, 40 showed high-velocity spontaneous horizontal-nystagmus (median slow-phase velocity (SPV) 39.7 degrees/second (°/s); Twenty-one showed horizontal-nystagmus reversing direction within 12-hours (24 on separate days). In 44 of 67 patients with VM, low velocity spontaneous horizontal (n=28, 4.9°/s), up-beating (n=6, 15.5°/s) or down-beating-nystagmus (n=10, 5.1°/s) was observed; Sixteen showed positional-nystagmus only, and seven had no nystagmus. Spontaneous horizontal-nystagmus with SPV >12.05°/s had a sensitivity and specificity of 95.3% and 82.1% for MD. Nystagmus direction-change within 12-hours was highly specific (95.7%) for MD. Spontaneous vertical-nystagmus was highly specific (93.0%) for VM. In the seven BPV patients, spontaneous-nystagmus was absent or <3°/s, and characteristic paroxysmal positional nystagmus was observed in all cases. Patients with central and MD-related positional vertigo demonstrated persistent nystagmus. Two patients with EAII showed spontaneous vertical nystagmus, one patient with VP showed short bursts of horizontal-torsional nystagmus lasting 5–10s, and one patient with SSCD demonstrated paroxysmal torsional down-beating nystagmus when supine.ConclusionsPatient-initiated vestibular event-monitoring is feasible and could facilitate rapid and accurate diagnosis of episodic vestibular disorders.
026 Variation in the anatomy of the normal human optic chiasm: an MRI study
Authors: Lueck Christian J , Bosler Nicholas , Neely Andrew , Ashton David 
Published: July 2019

IntroductionCompression of the optic chiasm gives rise to bitemporal hemianopia. The reason for this is unclear, but one hypothesis suggests it relates to the fact that nasal retinal fibres cross each other while temporal fibres do not. This ‘crossing’ hypothesis has been investigated using finite element modelling but this requires accurate anatomical data. The precise shape of the chiasm is not clear: nasal fibres may not decussate centrally (as if the chiasm were ‘X’-shaped) but, instead, decussate paracentrally and run parallel to each other in the central arm of an ‘H’. This study aimed to determine the population variance in chiasmal shape.Methods68 MRI scans of healthy individuals without visual abnormality were randomly selected. A 2D image was created and images were analysed using AutoCAD software to determine the offset between lines drawn down the centres of the optic nerves and contralateral optic tracts. A positive offset would suggest an ‘H’ shape while an ‘X’-shaped chiasm would have a offset of 0.ResultsThe mean width of the chiasm was 12.0 mm, and the mean offset was 4.7 mm generating a mean offset:width ratio of 0.38. No chiasm had an offset of zero. Fibre crossings occurred approximately 2.35 mm lateral to the midline, and nasal (crossing) fibres travelled an average of 4.7 mm in the mediolateral plane before entering the contralateral optic tract.ConclusionsThe human optic chiasm is H-shaped, not X-shaped. This information will inform future models of chiasmal compression.
035 Valproate-induced parkinsonism ‘an early warning’: case reports and review of literature
Authors: Dal Shoaib , Whyte Scott 
Published: July 2019

IntroductionWe describe two cases of valproate-induced parkinsonism, where the parkinsonian features develop after commencing valproate (VPA), in a time frame and manner well described by previous publications.1 In contrast to the published literature, that has short duration of follow-up, we have had a prolonged follow-up of these two cases, who initially improved on ceasing valproate, but then after a period progressed again, with the development of levodopa-responsive asymmetrical Parkinson’s disease.2Cases67 years old man, on VPA 500 mg twice a day for seizures since 2008, developed new rest, re-emergent left hand tremor and hypomimia in 2012. Within months, the parkinsonism progressed to decremental bradykinesia, cog-wheel rigidity and hypophonia. The symptoms initially improved with changing VPA to levetiracetam; however, the disease reappeared after 6 months and although levodopa-responsive, it has been progressive since then.88 years old man, on VPA 700 mg twice a day for seizures since 2010, developed upper limb decremental bradykinesia, cog-wheel rigidity, camptocormia and rest, re-emergent tremor in hands in 2011. The parkinsonism improved significantly with cessation of VPA; however, the disease re-emerged after 2 years and has been progressive since then.ConclusionVPA may be responsible for unmasking underlying subclinical parkinsonism and these patients represent an earlier onset of Parkinson’s disease related to VPA exposure, rather than a purely drug related parkinsonian dysfunction. These cases also highlight the importance of long-term follow up in VPA-induced parkinsonism. These observations are an important consideration for clinicians treating patients with parkinsonian features secondary to VPA exposure.ReferencesBrugger F, Bhatia KP, Besag FM. Valproate-associated parkinsonism: a critical review of the literature. CNS Drugs 2016;30(6):527–540.Mahmoud F, Tampi RR. Valproic acid-induced parkinsonism in the elderly: a comprehensive review of the literature. Am J Geriatr Pharmacother 2011;9(6):405–412.
040 Acute cerebellar ataxia following epstein-barr virus infection
Authors: Barnes Stephanie L , Brew Bruce J 
Published: July 2019

IntroductionInfectious aetiologies such as acute Epstein-Barr virus (EBV) infection are in the differential diagnosis for acute cerebellar ataxia (ACA). This syndrome remains exceptionally rare and not well characterised in adults.e.g. 1 2MethodsA retrospective case review of a patient diagnosed with ACA following EBV infection with implications for pathogenesis and treatment.ResultsA 29-year-old Caucasian male presented with a three day history of ACA. Seven days prior he was diagnosed with infectious mononucleosis; bloodwork was consistent with acute EBV infection. These symptoms improved rapidly with oral prednisolone. He took no regular medications, drank alcohol moderately and had no significant family history.On examination, he was afebrile, ataxic and mildly dysarthric. Sensory examination was normal, particularly proprioception. Romberg’s test was negative. Remaining neurological and general examination was normal.Bloodwork showed mild liver dysfunction and positive ANA (titre 1/320, homogenous and speckled patterns). Immune screen was otherwise negative. Antineuronal antibody panel was negative in serum and CSF. CSF glucose was 3.1 mmol/L, protein 751 mg/L, albumin 523 mg/L, neopterin 24 nmol/L and B2 microglobulin 1.1 mg/L. The sample was acellular with negative EBV PCR (<500 copies/mL). Other infective serology and PCRs were also negative. MRI brain with gadolinium showed no abnormality.The patient received supportive care and was neurologically normal within three months.ConclusionsACA related to EBV is rare in adults. This report is important because it documents an adult case, other ACA causes have been rigorously excluded, resolution without antiviral therapy is detailed, and investigations support an immune-mediated pathogenesis.ReferencesMcCarthy CL, McColgan P, Martin P. Acute cerebellar ataxia due to Epstein-Barr virus. Pract Neurol 2012;12:238–240.Lascelles RG, Longson M, Johnson PJ, Chiang A. Infectious mononucleosis presenting as acute cerebellar syndrome. Lancet 1973;2:707.
044 Dramatic delayed recovery following severe anti-NMDAR encephalitis
Authors: MacDonald Allycia , Jordan Nerissa , Leong Wai 
Published: July 2019

IntroductionLong term outcomes in anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis are reported to be favourable in 80% of cases, but there is little information regarding recovery following prolonged severe neurological disability.Case 1A 16-year-old female presented with headache, fever and acute encephalopathy. Anti-NMDAR antibodies were positive, with an ovarian teratoma. Treatment included oophorectomy, plasmapheresis, corticosteroids, cyclophosphamide, intravenous and intrathecal rituximab, and alemtuzumab. After 15 months hospitalisation, she remained in a vegetative state, with tracheostomy and percutaneous gastrostomy tubes, on multiple anticonvulsants. She received no further immunotherapy. MRI brain 2 years after illness onset demonstrated severe generalised cerebral atrophy. Three years after onset, she began to improve. At 5+ years she was seizure-free without anticonvulsants, fully independent and ambulatory, without overt cognitive deficits or personality change. MRI brain at 5 1/2 years demonstrated recovery of cerebral volume to within normal limits, but residual hippocampal atrophy.Case 2A 21-year-old female presented with headache, fever and personality change. NMDAR-antibodies were positive. A malignant ovarian teratoma was treated with salpingoophorectomy and chemotherapy (bleomycin/etoposide/cyclophosphamide). She received plasmapheresis, immunoglobulins, corticosteroids, intravenous and intrathecal rituximab, and bortezomib. She was finally discharged after 21 months hospitalisation with tracheostomy and gastrostomy tubes, severe behavioural disturbance, minimal functional limb movement, and fully dependent care needs. Clinical recovery began 2 1/2 years after disease onset, and at 4 years she is fully independent with residual cognitive deficits, engaging in volunteer work.ConclusionsThese cases highlight the potential for dramatic delayed clinical improvement despite prolonged severe neurological disability in anti-NMDAR encephalitis.
055 An unusual presentation of SOD1-ALS: a case report
Authors: MacDonald Allycia , Dyke Jason , Khangure Simon , Kelly Andrew 
Published: July 2019

IntroductionApproximately 10% of amyotrophic lateral sclerosis (ALS) cases are inherited, of which 20% are due to mutations in the superoxide dismutase-1 gene (SOD1). MRI abnormalities are not uncommon in ALS, and there have been previous case reports of peripheral nerve enhancement in patients with SOD1 mutations, typically attributed to rapid neuronal degeneration.CaseA 31-year-old previously well Malaysian woman presented with a 3 month history of progressive lower limb weakness, initially involving the right lower limb but progressing to involve the left, requiring the use of a walking aid. Initial examination demonstrated asymmetric upper and lower motor neuron signs in bilateral upper and lower limbs. EMG findings were of a severe pure motor axonal process. CSF examination revealed elevated protein without significant elevation of white cells. MRI brain and spine demonstrated smooth cauda-equina ventral nerve root thickening and enhancement. Treatment with intravenous immunoglobulin and high dose corticosteroid was commenced for a presumed inflammatory process, with no clinical improvement. A cauda-equina nerve root biopsy was performed, demonstrating features consistent with an immune-mediated demyelinating neuropathy. The patient continued to deteriorate, developing flaccid upper limb weakness and facial involvement. Plasma exchange, azathioprine, cyclophosphamide, and rituximab were sequentially administered over the following two months without altering the rate of disease progression. Genetic testing returned a positive SOD1 heterozygous gene mutation, confirming the diagnosis of ALS.ConclusionsWe present a case of SOD1-ALS with atypical features on imaging and histopathology suggesting an underlying demyelinating process, expanding the known clinical spectrum of this mutation.
056 Efficacy and safety of the Bruton’s tyrosine kinase inhibitor evobrutinib (M2951) in patients with relapsing multiple sclerosis over 48 weeks: a randomized, placebo-controlled, phase 2 study
Authors: Montalban Xavier , Arnold Douglas L , Weber Martin S , Staikov Ivan , Piasecka-Stryczynska Karolina , Gillett Alan , Martin Emily C , Syed Sana , Dangond Fernando , Wolinsky Jerry S 
Published: July 2019

IntroductionEvobrutinib (M2951) is a highly specific oral inhibitor of Bruton’s tyrosine kinase, a key regulator of B cell and macrophage functions implicated in MS.MethodsIn this double-blind, phase 2 study (NCT02975349), adult patients (≤65 years) with relapsing MS (RMS) were randomized to evobrutinib 25 mgQD, 75 mgQD, 75 mgBID, placebo, or open-label dimethyl fumarate (240 mgBID; reference arm) for 48 weeks; placebo-treated patients switched to evobrutinib 25 mgQD after 24 weeks. The primary endpoint was the total number of T1 gadolinium-enhancing (T1Gd+) lesions at Weeks 12, 16, 20, and 24. Secondary endpoints included annualized relapse rate (ARR), MRI measures at Weeks 24 and 48, and safety.ResultsAmong 261 patients, the sum of T1Gd+ lesions over Weeks 12–24 was reduced with evobrutinib 75 mgQD (p=0.002) and 75 mgBID (p=0.03); a dose response was observed (p=0.001). There was no evidence of change in effect on T1Gd+ lesions (mean±SD; Wilcoxon signed-rank test) between Weeks 24 and 48 with evobrutinib 75 mgQD (0.28±0.91 to 0.85±2.87; p=0.57) or 75 mgBID (0.24±0.88 to 0.49±1.22; p=0.23). ARR (unadjusted [95%CI]) was 0.25 (0.12–0.44) for evobrutinib 75 mgQD and 0.11 (0.04–0.25) for 75 mgBID over 48 weeks, and 0.37 (0.17–0.70) for placebo over 24 weeks. Evobrutinib appeared well-tolerated. Shifts to Grade 3–4 ALT and AST elevations from normal (grade 0) occurred in 8 (5.4%) and 6 (3.9%) evobrutinib-treated patients respectively, driven by events with onset within the first 24 weeks.ConclusionsEvobrutinib is the first BTK inhibitor to demonstrate disease activity reduction in RMS. The observed benefit-risk profile supports further clinical development.
060 Impaired color discrimination is associated with hallucinations in dementia with lewy bodies
Authors: Matar Elie , Phillips Joseph R , Ehgoetz Martens Kaylena A , Halliday Glenda M , Lewis Simon JG 
Published: July 2019

IntroductionEmerging evidence indicates that color discrimination impairments can predict the development of dementia across a range of prodromal Lewy body conditions. However, color vision deficits are not seen uniformly in patients with Dementia with Lewy Bodies (DLB), suggesting a more nuanced association. Visual hallucinations(VH) represent a discriminating feature of DLB, and recent evidence implicates visual pathway dysfunction as a significant contributor to this phenomenon. We therefore hypothesized that color impairment will more closely associate with VH in DLB rather than general cognition.MethodsIn this study, we examined the relationship between color vision impairment and VH, along with other clinical and neuropsychological features in 24 patients with DLB alongside 25 age-matched controls. Color discrimination was assessed using the Farnsworth-Munsell-100 Hue (FM-100) test.ResultsColor discrimination impairment was seen in 16/24 DLB participants (67%) with a higher error score relative to controls(p=0.001). We demonstrate for the first time a strong association between color discrimination errors and both the presence and severity of VH in DLB based on clinician-derived(p=0.008) and questionnaire-derived(p=0.03) measures. Correlation with clinical and neuropsychological variables revealed that color discrimination is significantly related to visuospatial impairment(p=0.02) but not to global measures of cognition, motor severity, age or disease duration. Factor analysis confirmed a unique relationship between color discrimination, visual hallucinations and visuospatial function.ConclusionOur results suggest that color impairments may be a specific biomarker of visual hallucinations and associated visuoperceptual deficits in evolving Lewy body disorders rather than dementia per se and thus providing insight into a shared pathophysiological substrate.
061 Training in headache in australia, new zealand and asia
Authors: Zhang Lin , Jenkins Bronwyn , Stark Richard , Hutton Elspeth 
Published: July 2019

IntroductionMigraine is the leading cause of age-adjusted neurological disability in Australia, but little is known about headache training in our region. We aimed to assess the quantity of teaching in headache subjects during undergraduate and postgraduate years.MethodThis is a cross-sectional survey study where questionnaires were sent to 137 delegates from Australia, New Zealand and Asia, prior to the Headache Master School in Sydney in August 2018. The Main outcome measured are recalled number of hours of teaching in undergraduate year and postgraduate years in: 1) Migraine; 2) Trigeminal autonomic cephalalgias (TACs); 3) Asthma; 4) Myasthenia gravis (MG).ResultsThe questionnaire response rate was 73% (100 of 137), of which 29 delegates were within 10 years of completing their undergraduate degree and 98 were neurologists. In undergraduate training, there was much greater quantity of teaching in asthma than migraine (Z=5.007, p<0.000) despite both being high-prevalent (asthma 11%, migraine 15–20%) conditions. Similarly, for diseases of medium-to-low prevalence, there was less training in TACs (1/1000), compared to MG (1.2/10,000) (Z=6.196, p<0.000). These major differences in training were also seen in postgraduate years even though overall headache teaching was greater in postgraduate than undergraduate training (p<0.000).ConclusionsDespite the high prevalence and morbidity of headache disorders, they receive less attention in training than conditions with similar prevalence. We propose that headache training opportunities should be improved in our region, particularly in the undergraduate course and preceptorships or fellowships in postgraduate years.
065 Presence of anti-myelin oligodendrocyte glycoprotein antibodies in the serum of two patients following alemtuzumab therapy for suspected multiple sclerosis
Authors: Seneviratne Sinali , Marriott Mark , Monif Mastura 
Published: July 2019

IntroductionMyelin oligodendrocyte glycoprotein (MOG) antibody mediated disease is an autoimmune demyelinating disorder which can resemble multiple sclerosis (MS).1 2 Thus, this condition can be misdiagnosed and treated as MS.3 We present the clinical trajectory of two cases initially diagnosed as MS, treated with Alemtuzumab followed by clinical and radiological deterioration. Both were subsequently found to have anti MOG antibody in their serum.MethodsThis is a retrospective case study based on a medical record search of neuroimmunology clinics in two teaching hospitals in Victoria. We searched for patients treated with Alemtuzumab who subsequently tested positive for MOG antibody.ResultsWe found two young women who fulfilled the eligibility criteria. One patient presented with dizziness and vertigo, the other with unilateral optic neuritis. Both had supratentorial MRI lesions and were both diagnosed as having MS. Both patients experienced multiple relapses while on treatment for MS. Hence, they were commenced on Alemtuzumab therapy. Unexpectedly, both patients experienced a decline in their clinical status with worsening of expanded disability status scale (EDSS) and an increasing lesion load on MRI brain. Their serum anti MOG antibodies were then found to be positive. Subsequently, patients were treated with rituximab and plasma exchange with a favorable response.ConclusionsThese two cases demonstrate that Alemtuzumab is ineffective and in fact can worsen cases of anti-MOG antibody associated encephalomyelitis. This highlights the importance of anti MOG antibody testing when patients diagnosed with MS do not respond to Alemtuzumab and in those patients presenting with atypical features of MS.ReferencesWeber MS, Derfuss T, Metz I, Bruck W. Defining distinct features of anti-MOG antibody associated central nervous system demyelination. Ther Adv Neurol Disord. 2018;11:1756286418762083.Narayan R, Simpson A, Fritsche K, Salama S, Pardo S, Mealy M, et al. MOG antibody disease: A review of MOG antibody seropositive neuromyelitis optica spectrum disorder. Mult Scler Relat Disord. 2018;25:66–72.Wildemann B, Jarius S, Schwarz A, Diem R, Viehover A, Hahnel S, et al. Failure of alemtuzumab therapy to control MOG encephalomyelitis. Neurology 2017;89(2):207–9.