bluuebunny/crossref_metadata_2025_split huggingface dataset:
P71 Neurosurgical presentation of hereditary transthyretin (ATTR) amyloidosis: early recognition for earlier genetic therapies
Authors: Kapoor M , Quaegebeur Annelies , Shah Sachit , Carr AS , Reilly MM 
Published: February 2019

ObjectivesHereditary Transthyretin (ATTR) amyloidosis is a genetic disorder where insoluble amyloid fibrils deposit in heart, nerves and various musculoskeletal tissues. Early recognition of TTR-FAP is important as new genetic therapies become available.DesignWe present one confirmed and one clinical case of ATTR deposition in ligamentum flavum causing lumbar canal stenosis.SubjectsThe first case is a 44-year-old female with TTR 184S heterozygous mutation who developed urinary urgency and bilateral leg pain exacerbated by walking. Neurological examination was normal. MRI lumbar spine showed a disc bulge in combination with ligamentum flavum thickening at L2/3 causing stenosis of the vertebral canal. She had a laminectomy of the L2/3 level. Histopathological analysis of the surgical tissue was positive on Congo red staining for amyloid and immunohistochemically identified as TTR type. The second case is a 68-year-old man with TTR V122I heterozygous mutation. He presented with paraesthesia from the knees to dorsum of the feet. On examination, power was normal and he had a length dependent neuropathy. A CT myelogram demonstrated spondylosis at L4/5 and L3/4 from disc bulge and marked flaval hypertrophy causing canal stenosis. He was placed on the waitlist for L4-5 decompression but unfortunately died prior to surgery.ConclusionsIdentifying ATTR in surgical specimens may diagnose hereditary amyloidosis in otherwise asymptomatic patients and in the future, provide early access to effective therapies.
P93 Randomised controlled trial to determine the role of endoscopic clot lavage after intraventricular haemorrhage in neonates ENLIVEN
Authors: Dawes W , James G , Aquilina K 
Published: February 2019

ObjectivesClot lavage following intraventricular haemorrhage (IVH) in premature neonates has been shown to reduce rates of hydrocephalus1and improve neurodevelopmental outcome at 2 years and beyond.2The aim of the ENLIVEN trial (IRAS: 198936 Rec Ref: 16/LO/1792) is to determine the efficacy of Endoscopic clot lavage after intraventricular haemorrhage in Neonates.DesignSingle centre prospective randomised controlled clinical trial comparing standard treatment with subgaleal shunt (control arm) to endoscopic washout plus standard treatment (intervention arm).SubjectsPower calculation based on published literature1 2: 50 neonates recruited over 4 years – enrolment commenced January 2018 – current recruitment 3 neonates.MethodsNeonates with radiological evidence of IVH (ventricular index >99 th centile +4 mm). Primary outcome is the rate of permanent shunting, secondary outcomes include advanced MRI at term and six months, behavioural assessment at early middle and late infancy, biochemical analysis of CSF and blood.ResultsPreliminary data from the ENLIVEN trial is presented including a full description of the radiological analysis and behavioural assessment.ConclusionsDetermining how endoscopic clot lavage impacts on clinical outcome is of paramount importance in the management of neonatal IVH, the ENLIVEN trial will afford us the possibility of objectively assessing its safety and efficacy.ReferencesSchulz M, Buhrer C, Pohl-Schickinger A, et al. Neuroendoscopic lavage for the treatment of intraventricular hemorrhage and hydrocephalus in neonates. J Neurosurg Pediatr2014;13(6):626–35.Whitelaw A, Jary S, Kmita G, et al. Randomized trial of drainage, irrigation and fibrinolytic therapy for premature infants with posthemorrhagic ventricular dilatation: developmental outcome at 2 years. Pediatrics2010;125(4):e852–e58.
P95 Surgical approach and morbidity of paediatric fourth ventricular tumours: analysis of a large institutional series
Authors: Toescu SM , Samarth G , Horsfall HL , Phipps K , Jeelani O , Thompson D , Aquilina K 
Published: February 2019

ObjectivesTo characterise the surgical management of paediatric 4th ventricular tumours and their attendant neurological complications.DesignRetrospective cohort study.SubjectsChildren referred to our institution with tumours of the 4th ventricle between 2008–2017 inclusive.MethodsClinical notes and imaging review. Two-tailed Fisher’s test used to determine differences between proportions.Results95 patients were seen (53 males, mean 5.81y). The commonest presenting symptom was vomiting (63.3%). The commonest tumour type was medulloblastoma (54 cases)>pilocytic astrocytoma (20)>anaplastic ependymoma (12)>ATRT(4)>Teratoma (2), with 3 miscellaneous lesions. 55 pts presented with hydrocephalus. 27.4% of patients had an EVD (30.8% of these prior to tumour surgery), and 23.3% a VP shunt sited. The surgical approach was either via telovelar (50%) or transvermian (50%) routes; 29.5% were done in the sitting position. In the first 5y of the series, 7/31 cases used the telovelar approach, whilst in the latter 5y, this proportion was 30/51 (p=0.0015). New post-operative deficit was evident in 68% of cases (42.4% gait abnormality, 23.9% cranial neuropathy, 16.3% diplopia). There was no significant difference in the rates of cerebellar mutism syndrome between telovelar or transvermian approaches (p=0.62). There was 1 mortality within 30d of operation.ConclusionsResection of paediatric 4th ventricular tumours is increasingly performed by the telovelar route, and carries significant morbidity, although surgical mortality remains low.
P110 External ventricular drain-related infections: a single centre study
Authors: Duddy JC , O’Sullivan MGJ , Lim C , Kaar GF 
Published: February 2019

ObjectivesTo quantify the External Ventricular Drain-related infection (ERI) rate in a one year period at Cork University Hospital, and to identify any risk factors for infection.DesignA retrospective audit of all External Ventricular Drains (EVDs) inserted in a one year period.SubjectsAll patients who had an EVD inserted between February 2017 and February 2018.MethodsPatients were identified from operating theatre logbooks. All relevant data was obtained from a retrospective review of medical and operative records. ERI was defined as evidence of positive CSF culture.Results41 EVDs were inserted in a total of 30 patients during the study period. The average age was 52.9 years. 46.6% of patients were female. The average length of EVD insertion was 8.85 days. The most common reason for EVD insertion was subarachnoid haemorrhage (31.7%) followed by supratentorial tumour (24.4%). 78% of EVDs were antibiotic-impregnated. Average EVD sampling rate was 0.7. ERI rate was 1/41 (2.4%). The infection occurred in a patient who had an EVD inserted for haemorrhage secondary to an AVM which had remainedin situfor 13 days. The patient subsequently developed problems with repeated shunt infections resulting in a nine-month hospital stay.ConclusionsThe ERI rate in our patient cohort was 2.4%. We recommend using a strict EVD sampling protocol to minimise manipulation of EVDs and where possible to limit the length of time an EVD remainsin situ. EVD-related infections can result in lengthy hospital stays and increased healthcare costs.
P113 Efficacy, safety and long-term durability of endovascular treatment of MCA aneuryms: a review of 161 treated aneurysms
Authors: Hannan C , Sunderland G , Zakaria R , Niven S , Nahser H , Putharan M , Chandran A , Javadpour M 
Published: February 2019

ObjectivesThe aim of this study was to assess the long-term outcomes of endovascular treatment of MCA aneurysms and identify factors associated with recurrence.Subjects161 aneurysms in 147 patients treated between January 2008 and August 2011.MethodsA retrospective case note review was performed. Aneurysm occlusion was assessed using the Raymond-Roy classification. Clinical outcome was graded using the mRS.ResultsThe minimum period of follow up was 65 months, with a maximum follow up period of 120 months. 49% of aneurysms were ruptured at the time of treatment. Morbidity was observed in 15% of the unruptured aneurysms (6% permanent neurological deficit), and in 33% of ruptured aneurysms (15% permanent neurological deficit). 84% of aneurysms were fully occluded following first treatment. Of these, 34% demonstrated angiographic recurrence and 10% required re-intervention. 25% of aneurysms≤7 mm in maximal diameter demonstrated angiographic recurrence, as compared with 50% of aneurysms>7 mm. Aneurysm size >7 mm was associated with a significantly shorter time to recurrence (log rank=9.655, p=0.002).ConclusionsThis is a large series of MCA aneurysms with a long period of follow up. Our results demonstrate that endovascular treatment of MCA aneurysms is associated with a low morbidity and mortality. Given the increasing use of adjunctive devices (stents, web devices) full occlusion of ruptured aneurysms in the acute phase is not necessary, and deliberate staged treatment a reasonable strategy.
P117 Predictive factors of outcome in poor grade subarachnoid haemorrhage (SAH)
Authors: Fahmy J , Boumrah T , Trippier S , Hainsworth A , Madigan J , Pereira E , Shtaya A , Minhas P 
Published: February 2019

ObjectivesTo study the factors associated with outcome in poor grade SAH in a busy tertiary centre.DesignRetrospective records review.SubjectsAll Patients with SAH WFNS grades IV and V admitted Jan 2016-Dec 2017.MethodsWe admitted 379 SAH patients, 84 (22%) were poor grade (n=84, 33M/51F mean age 60.7±1.4 y). Outcome was assessed by Modified Rankin Scale (mRS) scores before surgery and at latest follow up (3–6 months). mRS was dichotomised as good (0–3) and poor (4–6).Spearman’s rank-order test evaluated correlation between latest mRS and all other variables (WFNS grade, GCS, Motor score of GCS, age, sex, smoking, hypertension, intraventricular haemorrhage (IVH) and intracerebral haemorrhages (ICH)).Results63 patients (75%) had poor outcome, of which 46 (55%) died (44 with 30 days), versus 21 (25%) had good outcome. Spearman’s correlation analysis revealed that patients with smaller aneurysms (3.3±0.4 mm in good outcome patients vs 11.3±1.2 mm in poor outcome) (rs=0.37, p=0.009), who are younger (rs=0.24, p=0.03), have higher GCS (rs=−0.24, p=0.03), higher motor score (rs=0.25, p=0.02), lower WFNS grade (rs=0.3, p=0.007) and received coiling of aneurysms vs no treatment (rs=−0.39, p<0.0001) had better outcome. There was no significant correlation in outcome with ICH, IVH, external ventricular drain insertion, location of aneurysms, smoking, hypertension, other co-morbidities or sex.ConclusionsIn poor grade SAH, younger patients with smaller aneurysms, higher GCS and higher motor score who received endovascular coiling had better outcome.
WP1-17 Risk factors and patterns of progression in a surgical cohort of low grade glioma patients
Authors: Acharya S , Lavrador J , Visagan R , Narbad V , Jung J , Vergani F , Bhangoo R , Ashkan K 
Published: February 2019

ObjectivesTo understand the risk factors for and patterns of progression of low grade glioma (LGG).DesignSingle centre retrospective cohort study.SubjectsPatients undergoing at least two neurosurgical procedures for LGG, the first being for diffuse LGG (WHO 2). 22 patients included (14M; 8F); mean age at time of first operation 37.7±2.7 years. 20 patients had a Performance Status (PS) 0–1 and 2 patients had a PS 2.MethodsAll patients with LGG diagnosed in between 2009–2018 were retrospectively evaluated. Variables of interest included demographics, staging, performance status, time to re-operation (TTR), extent of resection, molecular genetics (1p19q co-deletion, IDH status). Tumour volumes were estimated from MRI images by the validated ABC/2 equation. Statistical analyses were performed by Stata13.0.ResultsThe tumour progressed in WHO grade in 18 patients (WHO grade 3 (n=15); WHO grade 4 (n=3). Mean time to re-operation after the first surgery was 7.0±1.2 years following gross total resection (GTR) and 3.2±0.7 years following subtotal resection (STR). Non-adjusted analysis of risk factors for time to re-operation (TTR) showed absence of 1p19q co-deletion as a risk factor (p=0.021). Adjusted analysis revealed that GTR, 1p19q mutation, PS 0 at 1 st surgery and tumour volumetric change decrease the risk for re-intervention (p<0.05). Chemo-radiotherapy was not associated with TTR.ConclusionsIn our cohort, TTR in LGG was influenced by the amount of initial resection, 1p19q deletion, PS and post-operative volumetric change.
WP1-25 Improving neurological examination in acute admission: the INSPECT method
Authors: Islam M 
Published: February 2019

ObjectivesClerking proforma in the trust includes a detailed neurology examination which is mandatory to fill up according to the trust guideline. Incomplete neurological examination at the time of admission significantly increases length of hospital stay and hamper the overall prognosis.DesignA retrospective study was conducted for 2 weeks on 60 patients clerked in AMU. A general survey was conducted among 26 doctors to detect the possible reasons and suggestions.To ensure every patient gets a quick and accurate neurological examination during clerking, we suggested a new mnemonic method called ‘INSPECT’: I – Inspection ( GCS, GAIT), N- Neck Rigidity, S –Speech, sensory P- Pupil E- Eye movement, C –Cerebellar and Cranial Nerves, T- (Tone, Power, Reflexes).ResultsThe primary audit revealed that the neurology proforma completed in 18.3% notes, Partially completed – 43.2% Not completed – 38.5% and frequently missed examinations were Cranial Nerve examination: 68.3%, Sensory: 56.2% Cerebellar: 42.1%, Motor power: 21% Doctor’s survey revealed reasons behind examinations not being carried out: Time consuming: 46.1%, Not relevant: 23.07%. Not enough training 15.38%.ConclusionsResults showed that only 18.3% patients had complete neurological examination, mostly missed cranial nerves examination ( 68.3% ) Survey suggested time constraint is a major issue ( 46.1% ) and quicker assessment and concise proforma will help. The suggested method will be re-audited in 3 months time.
TM2-2 Analysis of adverse events in the management of chronic headache by occipital nerve stimulation
Authors: Miller S , Watkins L , Matharu M 
Published: February 2019

ObjectivesTo analyse long-term adverse events of occipital nerve stimulation (ONS).DesignProspective open-label observational study.Subjects134 patients with refractory headaches implanted between 2007–2014 in a single specialised centre.MethodsInformation was collected on ONS device, implantable pulse generator (IPG) site and adverse event rates. The impact of implanter experience and the association between IPG site and adverse event rates was also explored.ResultsMean follow up was 46 months (6–108 months). A total of 139 adverse events were recorded in 75 patients (56%). A total of 59 additional surgeries were needed in 39 patients. A significant difference was seen in the rates of adverse events recorded between 2007–2010 and 2011–2014 (60.7% vs 42.6%, p=0.002). A significant reduction in ONS revision was seen over time (25.7% vs 5.9%, p=0.002). Analysis of adverse events with IPG site showed those with abdominal implants recorded higher adverse event rate that those with an IPG in the chest (65.8% vs 40.3%, p=0.004).ConclusionsIn specialist centres, adverse event rates of ONS can be much lower than reported in the literature. Our results suggest implanter experience and IPG site both have an effect on adverse event rate.
TM2-5 Percutaneous electrical nerve stimulation (PENS) therapy for refractory primary headache disorders: a pilot study
Authors: Weatherall MW , Nandi D 
Published: February 2019

ObjectivesPrimary headache disorders are common, but many patients are refractory to medical treatment. PENS therapy involves the stimulation of one or more individual nerves or dermatomes using needle probes. We assessed whether a ‘single shot with single probe’ strategy would benefit patients with refractory headache disorders, including chronic migraine (CM), and chronic cluster headache (CCH).DesignService evaluation of 36 patients treated with PENS therapy between September 2012 and June 2016. Follow-up data was available for 33 patients.Subjects16 patients with CM, nine with CCH, and one with hemicrania continua. Secondary headaches comprised occipital neuralgia, cervicogenic headache, and trigeminal neuropathy.MethodsPENS was given using Algotec® disposable 21 gauge PENS therapy probes (8 cm) to the occipital nerve ipsilateral to the pain (or bilaterally in cases of bilateral pain). Stimulation was delivered at 2 Hz/100 Hz, at 3 cycles/second, between 1.2–2.5 V depending on patient tolerability, for 25–28 min.Results6/9 patients with CCH improved significantly after the first session. In all patients with CCH, PENS therapy was well tolerated, with no significant adverse events reported. One patient with CCH reverted to episodic cluster. Only four patients with CM experienced any benefit.ConclusionsPENS therapy shows potential as a relatively non-invasive, low-risk, and inexpensive component of the treatment options for refractory primary headache disorders, particularly chronic cluster headache.
WP1-2 Labial gland biopsy for the investigation of neurological sjogrens
Authors: Jebril A , Lavin T 
Published: February 2019

ObjectivesNervous System involvement in Sjogren’s Syndrome (SS) commonly affects the peripheral nerves, though central nervous manifestations such as myelitis can occur. Peripheral Neuropathy has of 2%–10% of SS cohorts.1Involvement of different segments of the peripheral nervous system leads to a widespread spectrum of neuropathic manifestations including dorsal root gangliopathy (DRG), small fibre neuropathy (SFN), sensorimotor neuropathy (SMN), trigeminal neuropathy (TN) and vasculitic mononeuropathy (VMN). Less commonly, the central nervous system can also be affected, causing various focal or multifocal CNS disorders. In the absence of antibodies, LGB remains central to validated diagnostic criteria2We aimed to ascertain the yield of labial gland biopsy (LGB) for suspected Neurological Sjogrens and identify predictive factors for positive and negative biopsies.Methods10 year retrospective case note review of patients referred for LGB for suspected Neurological Sjogrens, from during a period of October 2007 to 2017. Demographics, Clinical syndrome, Investigations and Biopsy Results were obtained.Results107 patients (73.8% female, mean age 55 years) had LGB for suspected Neurological Sjögren’s. Phenotypes were DRG 60/107 (56.1%), SFN 37/107 (34.6%), TM 4/107 (3.7%), TN 2/107 (1.9%), VMN 2/107 (1.9%) and SMN 1/107 (0.9%). Overall positive biopsy yield was 32/107 (29.9%). Yield in different phenotypes; DRG 11/60 (18.3%) and SFN 20/37 (54.1%). The presence or absences of SSA/SSB antibodies (p=0.35, chi) or Sicca symptoms (p=0.1, chi) were not significant in predicting a positive biopsy.ConclusionsLGB remains a vital investigation in suspected neurological Sjogrens including small fibre neuropathy. Prior investigations and different phenotype did not predict positive biopsy. Our practice and biopsy yield is similar to other published series.3
TM3-9 Neurosarcoidosis presenting with normal pressure hydrocephalus: case series
Authors: Golahmadi AK , Craven CL , Thorne LT , Toma AK , Watkins LD 
Published: February 2019

ObjectivesNeurosarcoidosis is a rare condition with a high mortality. Early recognition of symptoms is important to enable prompt interventions. We report a case series of NS with ventriculomegaly that presented with symptoms resembling normal pressure hydrocephalus (NPH).DesignCase series.SubjectsPatients with ventriculomegaly on MR imaging and diagnosis of confirmed or probable NS.MethodsAnalysis of medical records for presenting clinical features, neuroradiology, intervention and clinical outcomes in patients with ventriculomegaly and confirmed or probable NS.ResultsFour patients (2M:2F) aged 49.0±3.01 years (mean ±SD) were identified. Three had definite NS and one probable. Three presented with gait disturbance and one with memory impairment. MR Imaging (with gadolinium) demonstrated ventriculomegaly with leptomeningeal enhancement. One patient underwent 24 hour ICPM, with a median ICP of 3.47 mmHg and pulse amplitude of 4.35 mmHg. CSF showed mild increase in chronic inflammatory cells in the absence of infection. Patients underwent medical management plus ventriculoperionteal shunt insertion (with adjustable valves set to 5 cm H2O and anti-siphon devices). There was a propensity for transient development of slit-like ventricles but without other features of over-drainage. All patients showed symptom improvement following CSF diversion at mean follow-up of 27 months.ConclusionsNS can present similarly to NPH. Early combined treatment of CSF diversion and medical management is effective for symptom management.
TP3-1 Memory impairment in epilepsy
Authors: Wieshmann UC , Chawira A , Keller S , Baker G 
Published: February 2019

ObjectivesMemory impairment is an extremely common complaint in people with epilepsy (PWE). The purpose of our cross sectional study was to identify factors associated with self-reported memory impairment in PWE.Methods514 PWE and 52 controls completed the Liverpool Adverse Event Profile, a 19 item questionnaire which includes items on memory and depression and provided clinical data on age, sex, medication, seizure onset, frequency and severity. For memory impairment, sleep disturbance, depression and seizure type scores were dichotomised into minor deficits (Likert scores 1 and 2), and major deficits (Likert scores 3 and 4).ResultsIn univariate analysis we found significant associations for mono vs polytherapy (χ2=14.85, p<0.001); minor/major depression (χ2=29.41, p<0.001); minor/major sleep disturbance (χ2=65.34, p<0.001); seizure freedom/persistence (χ2=14.40, p<0.001), the number of seizures in the last four weeks (p<0.001, U=17530.50, z=−5.11, but not sex, mean age of onset, mean duration of epilepsy or seizure type. Multinomial logistic regression revealed that sleep disturbance (p<0.001), AED poly therapy (p=0.004), depression (p<0.001) all retained significance whereas seizure persistence did not (p=0.377). Applying ANOVA to examine mean age with memory deficit outcomes, older age was significantly linked to poorer memory (F=3.34, p=0.002).ConclusionsSleep disturbance, poly therapy and depression are potentially treatable factors associated with self-reported memory impairment in PWE.
TP3-7 Sleep-disordered breathing as a consequence of vagal nerve stimulation
Authors: Pérez-Carbonell L , Higgins S , Koutroumanidis M , Leschziner G 
Published: February 2019

ObjectivesVagal nerve stimulation (VNS) is a neuromodulatory therapy indicated in drug-resistant epilepsy (DRE). Its side effects are frequently minor, however, sleep-disordered breathing (SDB) has been previously reported.1Obstructive sleep apnoea (OSA) is highly prevalent in individuals with refractory epilepsy, and may be a cause of poor control of seizures.2MethodsThree DRE patients with active VNS underwent a video-polysomnography with 21-channel montage electroencephalography in our centre.ResultsFirst and second patients showed OSA at the time of VNS activation. In the first patient, the apnoeic-induced arousals triggered VNS auto-firing and consequent respiratory events, perpetuating the SDB. The third patient had episodes of stridor, and an increased respiratory rate, coinciding with VNS activation. Our cases are representative of different forms of SDB that occurred as a consequence of the switch-on phase of the VNS device.ConclusionsSleep-related breathing disturbances should be considered before VNS implantation, and should be routinely assessed after having started the therapy. Changes in stimulation parameters, and positive airway pressure therapy, may be required to treat the SDB.