bluuebunny/crossref_metadata_2025_split huggingface dataset:
282 Skull base dural AVF mistaken as cervical myelitis: a series
Authors: Dan Whittam , Saif Huda , Richard Pulicino , Arun Chandran , Mani Puthuran , Anu Jacob 
Published: October 2018

BackgroundCervico-medullary spinal cord lesions are usually inflammatory (or rarely neoplastic). Dural arteriovenous fistula (DAVF) is considered very unlikely in the cervical cord, particularly if the history is of an acute myelopathy.MethodCase note review of five patients with skull base DAVF.ResultsAll were men aged 60–69 years. Four were symptomatic for a month or less, followed by acute deterioration over hours-7days, mimicking myelitis. MRIs showed extensive T2-hyperintensity and swelling in the cervico-medullary region. Worsening after corticosteroids occurred in 3/4 patients and one needed ventilation. Conspicuous and unambiguous cord-surface vessels were present in only one patient. Clinical suspicion prompted digital subtraction angiography (DSA) in the other four patients. Median time from initial MRI to diagnostic DSA and embolization was 28 days (7–91 days). All patients survived with good clinical recovery.Systematic retrospective review of MRIs showed lack of gadolinium enhancement in 4/5. CSF examination (n=4) showed normal leucocyte count in all cases and elevated protein concentration in 3/4 (0.6–1.2 g/L).ConclusionSkull base DAVF can mimic acute cervical myelitis. Steroids may prompt life-threatening clinical deterioration mistaken for ascending myelitis. A low threshold for angiography in the context of non-enhancing lesions and acellular CSF will facilitate earlier diagnosis and improve outcomes.
WED 026 ‘creating a neurological exam template for acute medical admissions’
Authors: Kieran McMullan , Janice Redmond 
Published: October 2018

IntroductionThere is sub optimal documentation of neurological exam findings in patient’s hospital records. Documented Neurological findings are often incomplete, omitted or scattered in various places in the clinical notes. This is important in the setting of acute neurological emergencies, where accurate documentation is vital in gauging potential deterioration/improvement in a patient’s condition.MethodA chart review was performed on 80 patients referred to the Neurology consult service in St James’s Hospital between January-February 2018. All aspects of the Neurological exam findings documented in these charts were noted. A gold standard template for accurate recording of Neurological exam findings was then created and circulated to all Hospital NCHD’s. A repeat chart review was performed on 80 patients referred to Neurology Consults between March-May 2018 (following the circulation of this template) and results were compared.DiscussionRecord keeping varied according to different clinical parameters, being lowest for speech at just over 20% and highest for muscle power at over 70%. Globally there was a small but significant improvement (P Value<0.001) in the documentation rates of neurological exam parameters following circulation of the template. We plan to apply this template to all acute medical admissions in St James’s Hospital.
297 Genetic associations of ICD in parkinson’s disease
Authors: Richard Rees , Leon Hubbard , Yoav Ben-Shlomo , Donald Grosset , Nigel Williams , Huw Morris 
Published: October 2018

IntroductionImpuse Control Disorders (ICD) are a potentially devastating side-effect of dopaminergic therapy in Parkinson’s disease (PD). We explore the genetic factors associated with ICD in Tracking Parkinson’s/PRoBaND – a UK-wide cohort of early-stage PD.MethodsParticipants were diagnosed with PD within 3 years and had longitudinal assessment including the Questionnaire for ICD in Parkinson’s (QUIP) for up to 5 years. We defined cases as having any positive response to the QUIP (lax criteria) or 2 positive responses in any domain (strict criteria). We performed a candidate-gene analysis based on systematic review, followed by a genome-wide association study. We used age at onset, gender, and three significant principle components as covariates.ResultsAfter clinical and genetic quality control steps, we analysed 1602 participants. Prevalence was significantly affected by classification criteria (strict/lax): ICD – 26.8%/11.1%, IRB 29.3%/27.2%, any 31.7%/41.9%. Six SNPs in dopamine, glutamate and adreno- receptor genes achieved nominal significance (p<0.05) in the candidate study. We have identified several SNPs in the GWAS that approach genome wide significance (p<5 × 10–7).ConclusionsThis work is the first genome-wide study of genetic determinants of ICD. Our findings support the hypothesis of genetic determinants of ICD in Parkinson’s and further work will allow understanding of the biology of ICD.
299 Immune-dysregulation in late onset myasthenia gravis
Authors: Girija Sadalage , Saiju Jacob , Paul Maddison 
Published: October 2018

IntroductionThe aim of our study was to establish if there were any clinical and immunological differences between early (EOMG) and late onset myasthenia gravis (LOMG).Methods and resultsWe recruited 150 patients with myasthenia gravis making this the largest prospective cohort study in MG to date. Most (74%) patients have LOMG. Of these, 8 (5.33%) are seronegative. Most (88%) are positive for AChR Abs, 4% have MuSK, 2% LRP4 and 10% are double positive to AChR and MuSK Ab on CBA.The incidence of LOMG is higher than EOMG. There is a statistically significant fall in AChR titre levels on serial measurement (p <0.0001). LOMG pts have higher AChR titres at recruitment compared to EOMG (p=0.0498) with higher MG composite scores (p=0.0287). Significantly higher numbers of LOMG pts are positive for AChR Ab on RIA in generalised MG compared to EOMG. A third of the pts with Early Onset Generalised MG are positive for MuSK Abs on CBA. There is no significant difference in clinical presentation between single seropositive pts Vs double seropositive pts. AChR Ab titres are lower in ocular EOMG versus ocular LOMG and titres in ocular patients are lower than in generalised patients.Flow cytometric analysis of PBMCs showed a significantly reduced percentage of Treg cells in our patient cohort compared to healthy controls (p<0.0001) and a significant difference in the levels of TNFa, IL10 and IL17 produced.ConclusionOur study has shown that there are clinical and immunological differences between early onset and late onset myasthenia gravis, both respond well to treatment with an improvement in composite scores and a fall in antibody titres. We are continuing to study this cohort of MG patients with extended follow-up.
WED 031 Neuro hot clinics: direct access clinic for acute medical patients
Authors: Z Clifford , P Morrish , C Strait , S Hinze 
Published: October 2018

20% of acute medical patients present with neurological symptoms, yet are often managed by non-specialist acute physicians. This district general hospital (DGH) introduced a direct access neurology clinic, to reduce hospital admissions and improve access to neurological expertise for vulnerable patients. Patients would otherwise have been admitted to await ward consultation. 20 ‘Hot’ clinic appointments each week were allocated by administration prior to discharge from the medical admissions unit. All appointments were within 48 hours. Common diagnoses were migraine, first seizure, and non-epileptic attack disorder and rarer presentations included 5 with transverse myelitis, 1 with cerebral vasculitis, and 1 with Hepatitis E related encephalomyelopathy.243 patients were seen by a Consultant Neurologist in 9 months in this hot clinic, thus saving at least 243 bed days and £73 000. Only 4 of these patients were readmitted. The hot clinic required 4PAs of consultant time split across weekdays, at an estimated cost of £30 000 per annum.This neurology acute clinic successfully provided front door neurological input, a vital service for GPs and patients, and made approximate annual saving of £70 000. Evidently, every DGH should consider implementing neurology ‘hot’ clinics.
THUR 056 Juvenile myoclonic epilepsy in a tertiary centre – a database review
Authors: Marc Edwards , PEM Smith , Khalid Hamandi 
Published: October 2018

BackgroundJuvenile myoclonic epilepsy (JME) is generally thought to respond well to first line treatment but many patients continue to suffer with seizure activity. This study reviews JME patients managed in a tertiary epilepsy centre.MethodsA JME patient cohort with data on medication and seizure frequency was sourced from an epilepsy database at University Hospital of Wales, Cardiff.Results168 patients (18–84 years old) were identified with a diagnosis of JME and a clinic appointment in the past 5 years. There was a female predominance of 2.8:1. The average age at onset was 13.56% patients were free of generalised tonic-clonic seizures with 40% free of all seizure types. Of those with frequent seizure activity, 66% were on at least 2 anti-epileptic drugs (AEDs). Levetiracetam was the most common therapy (48%). 32 women of childbearing age (32%) were taking Valproate with 71% under regular follow-up.ConclusionWhilst JME is considered to respond well to AEDs a significant proportion in our tertiary cohort were deemed drug refractory. However, this may show bias when compared to community samples. The number of women taking Valproate highlights the benefit of the drug and the need for careful monitoring and shared clinical decision making.
THUR 004 Foramen magnum decompression for tonsillar herniation secondary to meningoencephalitis
Authors: Barbara Wysota , Fardad Afshari , Michelle Oswall , Gordon Mazibrada , Edward White 
Published: October 2018

Tonsillar herniation and coning is a rare complication of meningoencephalitis and is associated with poor neurological outcome. Our patient presented unresponsive with radiological evidence of tonsillar herniation secondary to meningoencephalitis. She underwent an emergency foramen magnum decompression and C1 laminectomy with full recovery.16 years old female presented to her local ED with headache, vomiting, hallucinations, photophobia and neck stiffness. Her CT head was normal. She was commenced on acyclovir and ceftriaxone on the same day and clinically improved. LP performed 24 hours later showed WBC of 226 (200 lymphocytes, 6 polymorphs), protein 1.2 g/L, negative MC and S, negative viral PCR, negative TB PCR. CSF glucose was not sent. In light of a predominantly lymphocytic CSF and presumed viral meningitis antibiotics were stopped. Three days later acutely deteriorated. MRI head showed evidence of tonsillar herniation. She was restarted on antibiotics and was transferred to tertiary centre. She underwent emergency insertion of an EVD, foramen magnum decompression and C1 arch laminectomy. She made a full recovery with no residual neurological deficit.The mainstay treatment of meningoenecephalitis is intravenous antibiotics. However, in cases complicated by tonsillar herniation with reactive pupils, foramen magnum decompression should be considered.
WED 094 Diagnostic error rates in diagnosing idiopathic intracranial hypertension
Authors: Sangeeta Scotton , Anthony Liczkowski , P Mollan Susan , J Sinclair Alexandra 
Published: October 2018

ObjectiveTo quantify the rate of diagnostic error amongst patients with IIH. Additionally to identify factors contributing to diagnostic error.MethodsSequential patients referred with a diagnosis of IIH to the Birmingham tertiary neuro-ophthalmology IIH clinic were prospectively included (October 2013- February 2017) A diagnostic error taxonomy tool was applied to cases referred as ‘definite’ or ‘possible’ IIH. Discrepancy between referred and final diagnosis were recorded. Results212 patients were referred, (96.2% female), 138/212 (65%) with definite IIH and 74/212 (35%) with possible IIH. Of those diagnosed with definite IIH 25% were not IIH and out of those diagnosed with possible IIH 57% were not IIH. Reasons for diagnostic error included incorrectly identifying papilloedema where in fact pseudopapilloedema existed and diagnosing IIH following an isolated lumbar puncture (LP) pressure >25 cmCSF (but in the absence of other diagnostic criteria for IIH). Misdiagnosis lead to 43% receiving unnecessary acetazolamide (or other diuretics) and 14% having multiple LPs.ConclusionsWe noted a high diagnostic error rate amongst IIH patients referred to a tertiary centre for ongoing management. Where there is doubt about the presence of true papilloedema early specialist review may reduce unnecessary treatment and LP’s.
WED 106 Unilateral abnormal dat scan: early indicator of parkinson’s disease
Authors: Nicholas Heng , Ying Chin Mei , Clare Monaghan , John Davidson , Tom Gilbertson , Miratul Muqit 
Published: October 2018

IntroductionDopamine transporter (DaT) scans are used in the investigation of clinically uncertain Parkinsonian syndromes (CUPS). Presently, bilateral abnormal DaT scans are diagnostic of Parkinson’s disease and degenerative Parkinsonism, while normal scans suggest non-degenerative causes. However, the clinical significance of unilateral abnormal scans remains ambiguous. Therefore, we aimed to determine their relevance in CUPS, and hypothesised that they may presage Parkinson’s disease and degenerative Parkinsonism.MethodsA retrospective analysis was performed for patients with CUPS within a tertiary institution who had undergone DaT scans. Demographic data, clinical features, imaging and diagnoses were collected. In patients with unilateral scans, follow-up assessments, further imaging and diagnostic changes were also recorded.ResultsA total of 200 DaT scans were performed from 1 January 2008 to 1 January 2017. There were 100 (50.0%) with normal scans, 77 (38.5%) with bilateral scans, and 23 (11.5%) with unilateral scans. Of the latter group (n=23), 8 (34.8%) underwent follow-up DaT imaging in a mean period of 31.9±12.6 months from baseline, with 4 (17.4%) now reported as bilateral, although 2 (8.7%) remained unilateral and 2 (8.7%) were reported as normal.ConclusionUnilateral DaT scans can serve as a predictive factor for development of Parkinson’s disease and degenerative Parkinsonism.
THUR 117 The L-DOPA response in pathologically confirmed parkinson’s disease
Authors: Pitz Vanessa , Malek Naveed , Grosset Katherine A , Grosset Donald G 
Published: October 2018

Backgroundl-dopa is the standard treatment for Parkinson’s disease, but the response is variable.AimSystematic review of papers reporting the l-dopa response (motor response and/or complications) in pathologically confirmed Parkinson’s disease.Results467 cases of pathologically confirmed Parkinson’s were identified: 60.2% male, age at disease onset 63.3 years (SD 10.3), age at death 76.7 years (SD 7.8). Data on a graded l-dopa response were available in 411 cases (88.0% of 467). The motor response was excellent in 148/411 cases (36.0%), good in 179/411 (43.6%), moderate in 51/411 (12.4%) and poor/absent in 33/411 (8.0%). Data about motor complications were available for 161 patients: 71/161 (44.1%) had motor fluctuations and 89/161 (55.3%) had dyskinesia. Comorbid brain pathology was evaluated in 251/411 cases (61.1%), and was present in 148/251 (59.0%): cerebrovascular in 65/148 (43.9%), Alzheimer’s in 55/148 (37.2%), amyloid angiopathy in 18/148 (12.2%), and diffuse Lewy body disease in 10/148 (6.8%). Data linking the graded l-dopa response to comorbid pathologies were available in only 17 cases, of whom 8/17 (47.1%) had a good/excellent response.ConclusionThere is variation in the l-dopa response in pathologically confirmed Parkinson’s disease. The limited available information suggests a possible association of motor response to comorbid brain pathology.
WED 130 Designing a care pathway for high risk parkinson’s patients
Authors: Chloe Tam Lok Yee , Dominey Thea , Pearson Emma , Murphy Fiona , Carroll Camille 
Published: October 2018

ObjectiveTo inform pathway design for patients at high risk of an adverse outcome at 5 years post-Parkinson’s disease (PD) diagnosis.BackgroundIn our service, all newly diagnosed patients follow a 12 month new patient pathway, which includes assessment with PDQ-39, NMS Quest and MOCA. Use of a prognostic calculator1 allows for refinement of the pathway according to patient need. Patients are classified as low (0–0.3), medium (0.31–0.79) or high (0.8–1) risk of an adverse outcome at 5 years (postural instability, dementia, death).AimWe aim to evaluate whether assessments in the newly diagnosed pathway identify additional care needs in higher risk patients.MethodsAn evaluation was carried out of our new patient database. Individuals who had the prognostic indicator performed (n=52) were included in the evaluation.ResultsPatients in the high risk groups were found to be older and more cognitively impaired than lower risk patients (p<0.05). MOCA scores negatively correlated with NMS Quest (r=−0.44, p<0.001) and PDQ-39 (r=−0.51, p<0.001) suggesting as cognition becomes impaired, NMS burden increases, and quality of life is reduced.ConclusionsAdditional care needs were identified in high-risk patients. These findings highlight additional resources are required in this patient cohort to ensure needs are met.Reference. Velseboer DC, et al. Development and external validation of a prognostic model in newly diagnosed Parkinson disease.
THUR 134 Isolated CNS histoplasmosis in an immunocompetent patient
Authors: Fatima Jaffer , Stephen Glass , Eli Silber , Naomi Sibtain , Tihana Bicanic , Lina Nashef 
Published: October 2018

A 26-year-old Bolivian man presented with a 3 year history of occipital headaches and bilateral papilloedema. MRI showed communicating hydrocephalus and leptomeningeal enhancement. CSF analysis revealed an opening pressure of 32cmH2O, lymphocytic pleocytosis, protein 1.9 g/dL and CSF glucose 0.4 mmol/L. CSF AFB, bacterial and fungal culture, TB-PCR, 18S rDNA PCR, serum b-d glucan, and HIV were all negative. Serum ACE was normal. Steroids and methotrexate were commenced for probable neurosarcoidosis.He had readmissions with recurrent headache, meningism, symptoms of raised intracranial pressure and development of limb spasticity and brisk reflexes. Repeat imaging demonstrated stable hydrocephalus. Despite immunosuppression and empirical TB treatment, he remained symptomatic with a persistently active CSF: maximum opening pressure 36cmH2O, protein 3.9 g/dL, WCC 205 lymphocytes and low CSF-to-serum glucose ratio on repeated therapeutic LPs.After re-evaluation and positive CSF b-d glucan, a diagnosis of Histoplasma meningitis, without systemic dissemination, was confirmed by positive serological and CSF tests: positive immunodiffusion; yeast CFT 1:16; CSF EIA antigen 0.85 ng/ml.He was treated with AmBisome, followed by oral itraconazole, with complete symptom resolution and improvement on interval imaging.We discuss challenges in reaching a diagnosis of isolated histoplasmosis, its neurological manifestations and diagnostic pathway in evaluating patients with suspected CNS fungal infection.
WED 141 Pathological correlates of age at death in multiple sclerosis
Authors: Ruth Geraldes , Margaret Esiri , Jacqueline Palace , Gabriele DeLuca 
Published: October 2018

IntroductionFew studies have investigated which pathological features, including systemic vascular disease (VD), associate with younger age at death in multiple sclerosis (MS) and this constitutes the aim of the present study.MethodsA post mortem MS brain autopsy tissue was studied for 1)systemic VD scores 2)% brain plaque area and activity from a)frontal plus occipital white matter(WM), b)pons, and c)basal ganglia(BG), 3)frontal plus occipital cortex. These pathology measures, sex, disease duration (DD), and cause of death, were fitted into a regression model to explain age at death.Results34 MS cases (mean age 61.6±13.05 years, 58.8% females, DD 20.2±13.45 years) were included. Age at death decreased with increasing WM+pons+ BG (r=−0.382, r=0.02) and cortical (r=−0.299, p=0.03) demyelination, and WM+pons+ BG active plaques (r=−0.326, p=0.011) but increased with DD(r=0.298, p=0.016) and VD(r=0.329, p<0.0001). In the regression model only cortical demyelination (b=−0.39, p=0.014), DD (b=0.36, p=0.009) and VD (b=0.64, p=0.001) persisted.ConclusionHigher cortical demyelination associated with younger age at death. Systemic VD did not associate with younger age at death in this MS cohort.
WED 143 Chronic relapsing inflammatory optic neuritis (CRION) in the neuroinflammatory clinic
Authors: Oliver Cousins , Hina Khan , Sreedharan Harikrishnan 
Published: October 2018

CRION is a rare cause of optic neuritis. It is usually bilateral, painful and associated with profound visual loss. Significant response to corticosteroid treatment is typical but relapse is common when treatment is withdrawn. We present 2 cases of possible CRION and discuss the diagnostic and management considerations.Case 1: 50-year-old woman presented with right optic neuritis which spontaneous recovered. A year later she had left painful visual loss which improved with a short course of corticosteroids. MRI neuroaxis revealed left optic nerve enhancement. Non-specific, faint unmatched OCBs were detected. AQP4-IgG was negative. Nine months later she had further visual loss in her left eye. Prednisolone and azathioprine were commenced.Case 2: 55-year-old woman with bilateral, painless visual acuity deterioration over two weeks. Investigations revealed negative anti-aquaporin 4 antibodies (AQP4-IgG), normal MRI of the neuroaxis, negative oligoclonal bands (OCBs) and visual evoked potentials showed bilateral delay. Serum ACE was slightly elevated. She was started on a tapering course of steroids and had significant visual acuity improvement.The diagnosis of CRION involves the exclusion of other causes of optic neuritis, particularly multiple sclerosis (MS), Neuromyelitis Optica (NMO) and sarcoidosis. Correct diagnosis is important as aggressive and long-term immunosuppression is required.
THUR 149 ANCA/MPO vasculitis presenting with bilateral ischaemic optic neuropathy
Authors: Dwij Mehta , Charles Wade 
Published: October 2018

An 80 year old gentleman presented with bilateral, sequential ischaemic optic neuropathy. He initially developed progressive loss of vision in left eye with loss of colour vision and subsequently developed similar symptoms in the right eye with headaches, weight loss, malaise and lethargy. His visual acuity dropped to 6/12 on the right and counting fingers on the left. He had a dense central scotoma in left eye with left-sided RAPD but no other focal neurological deficits.Blood tests revealed an ESR of 107 with an MPO ANCA titre of 19. MRI brain with contrast showed prominent meningeal enhancement and infiltration with ischaemic changes in the brain. CSF analysis revealed WCC of 24 (95% lymphocytes), RCC 22 and protein 0.4 g/L with negative bacterial culture. Temporal artery biopsy was normal.He was treated initially with IV methylprednisolone and 6 cycles of IV cyclophosphamide and subsequently put on methotrexate. His systemic symptoms have resolved completely and his visual acuity continues to gradually improve.MPO-ANCA vasculitis can mimic temporal arteritis and should be considered in patients presenting with an ischaemic optic neuropathy. It is also a treatable cause of meningeal disease.