bluuebunny/crossref_metadata_2025 huggingface dataset:
P.022 Hemifacial Spasm due to dolichoectatic vertebrobasilar artery compression
Authors: Honey CM , Almojuela A , Hasen M , Kaufmann AM
Published: June 2018

Background: Hemifacial Spasm (HFS) is rarely caused by a dolichoectatic vertebrobasilar artery (eVB) compression of the Facial Nerve. This can pose a surgical challenge when performing microvascular decompression as vessel mobilization is often difficult due to atherosclerosis, tethering from brainstem perforators, and large size. These patients are often not considered for surgery. Methods: A retrospective chart review of patients who were surgically treated by the senior author between 2003 and 2017 with an admitting diagnosis of HFS was performed. Patients with preoperative neuroimaging demonstrating eVB compression of their facial nerve/root were included. Results: During the 15-year review, 315 patients underwent microvascular decompression for HFS and 21 (6.7%) had dolichoectactic vertebrobasilar compressions. At final followup (>3 months) 19 patients (90.4%) experienced reduction in symptoms with 15 (71.4%) having complete resolution. One patient required re-operation and benefitted from subsequent symptom relief. The majority of culprit compression was found proximally on the pontine surface. Mobilization of the culprit vessel was achieved successfully in the majority of cases with Teflon pledgets. There were no perioperative strokes or death. Complications are presented Conclusions: Microvascular Decompression for Hemifacial Spasm caused by dolichoectatic vertebrobasilar artery compression can be performed with a high rate of safety and success in the setting of a high case volume centre.
P.023 Clinical and patient satisfaction outcomes after partial sensory rhizotomy for refractory trigeminal neuralgia among MS patients
Authors: Bigder M , Krishnan S , Cook EF , Kaufmann AM
Published: June 2018

Background: MS related trigeminal neuralgia (MS-TN) is associated with high recurrence and retreatment rates. Optimal treatment and role for partial sensory rhizotomy (PSR) for MS-TN remains to be determined. Methods: We analyzed time to treatment failure (TTF) after PSR (n=14) versus other prior procedures (n=53) among 12 consecutively treated MS-TN patients. Kaplan-Meier curves and Log-Rank tests were utilized to compare BNI pain scores and TTF after PSR vs prior procedures using the same patient cohort as their own control group. Subsequent analysis compared TTF after PSR to other procedures (n=93) among a second cohort of 18 MS-TN patients not undergoing PSR. Results: TTF was significantly longer after PSR compared to prior procedures among the PSR cohort (p<0.0001) with median TTF of 79 vs 10 months respectively. Similarly, there was a longer TTF after PSR compared to prior procedures among the MS-TN cohort with median TTF 79 vs 16 months respectively (p<0.001). PSR resulted in a higher proportion of excellent pain scores when compared to prior procedures in the MS-TN cohort (77% vs 29%, p<0.001). Conclusions: TTF was significantly longer following partial sensory rhizotomy compared to other prior procedures in MS-TN patients. A higher proportion of patients achieved excellent BNI pain scores after PSR.
P.026 Rare association in childhood vasculitis: unique case of pituitary involvement in a child with GPA
Authors: Yaworski A , Srivastava R , Al Qarni M , Yager J , Rumsey D , Kassiri J
Published: June 2018

Background: Granulomatosis with polyangitis (GPA) is a rare systemic vasculitis with a prevalence of 0.6 per million in the pediatric population. CNS involvement occurs in 7-18% of cases. Pituitary involvement is only noted in 1% of cases. Methods: A 16-year-old girl with newly-diagnosed GPA presented to our hospital with progressive debilitating headaches, polyuria, and polydipsia. Results: Initial MRI showed changes to the pituitary. Lumbar puncture (LP) revealed opening pressure of 26. She developed central diabetes insipidus (DI) and visual changes. Repeat head imaging showed adenohypophysitis. The GPA was previously treated with steroids and cyclophosphamide, followed by Cellcept. Once the pituitary involvement was discovered, she was given re-induction therapy with Rituximab and steroid dose was increased. DI is being treated with DDAVP. Her headaches are improving. Conclusions: CNS inflammatory diseases are rare in childhood. Pituitary involvement is extremely rare in GPA. Induction therapy for adults with GPA and pituitary involvement includes glucocorticoids and cyclophosphamide, which often leads to improvement of MRI abnormalities but is not effective in resolving pituitary dysfunction. Our patient had already received this treatment when she developed the CNS findings. This case demonstrates that cerebral involvement is often resistant to classic therapy, and one should be vigilant in looking for CNS inflammation in these patients.
P.031 A qualitative study of patient perspectives regarding the role of the neurologist in advanced Multiple Sclerosis
Authors: Falet JR , Deshmukh S , Babinski M , Sigler G , Al-Jassim A , Moore F
Published: June 2018

Background: With few evidence-based disease-modifying therapies being available for patients with progressive multiple sclerosis (PMS), how can neurologists best care for their patients? Little is known about the perspectives of patients with respect to the role they would like their neurologist to play in their care. We hereby report an update to our abstract presented at the Canadian Neurological Sciences Federation’s annual congress in 2016. Methods: Patients with PMS having an Expanded Disability Status Scale (EDSS) score of 6 or more were invited to participate. Semi-structured interviews were conducted with patients and their caregivers, and written questionnaires were completed by all participants. Collected data was subjected to thematic coding. Results: We have now interviewed a total of 18 patients (compared to 10 in 2016) and have reached thematic saturation. The majority of patients identified the neurologist as a useful figure in their care. Three main reasons were identified: (1) The neurologist provides information about new research and therapies (2) The neurologist educates patients about their disease and available services (3) The neurologist is viewed as an important supportive figure. Conclusions: Despite a lack of disease-modifying treatments for progressive multiple sclerosis, patients with PMS view the neurologist as an essential provider of care.
P.037 Disseminated leptomeningeal hemangioblastoma in a case of Von Hippel Lindau
Authors: Maroun FB , Hache N , Bartlett P , Galway B , Engelbrecht A , Snow P , Jacob JC
Published: June 2018

Background: Leptomeningeal dissemination of hemangioblastomas (HB), whether sporadic or associated with Von Hippel Lindau (VHL), are extremely rare. Very scanty literature is available. Methods: A 36 year old female with VHL and stable pancreatic, adrenal and renal lesions was operated upon 4 years ago for a large symptomatic cervicomedullary cystic and solid tumor. 2 years after surgery the tumour recurred and further removal was unsuccessful due to medullary adhesions. Radiation was given to the posterior fossa area and to several small nodules over the cauda equina resulting in severe pain. Serial follow up imaging revealed diffuse leptomeningeal dissemination increasing in size of the suprasellar region, ambient cistern and Sylvian fissures. Clinically, she has been stable with small dose of steroids and VP shunt insertion for papilledema. Results: Review of the literature consists of 2 series of 7 and 21 patients each with leptomeningeal dissemination involving sporadic HB and VHL associated HB. Leptomeningeal dissemination is estimated at about 4.3%. It is postulated that the tumour starts in the Pia and spreads in an extra medullary fashion throughout the subarachnoid spaces. Conclusions: Long term recurrence has been noted raising the question of aggressive treatment with some drug therapy related to angiogenesis is postulated.
P.050 NICO-assisted neuroendoscopic management of enlarging subependymal giant cell astrocytoma in tuberous sclerosis complex: a case report
Authors: Dakson A , McNeely P
Published: June 2018

Background: Tuberous sclerosis complex (TSC) is an autosomal dominant neurocutaneous syndrome classically associated with mental disability, seizure disorder and adenoma sebaceum, among other anomalies. One of the major causes of mortality and morbidity in adults is the exclusive occurrence of subependymal giant cell astrocytoma (SEGA) which responds in at least 35% of cases to everolimus, mTOR inhibitor. However, drug treatment is associated with 33% rate of adverse events and requires long-term treatment Methods: In this report, we present a case of 49-year old female with TSC and a left enlarging SEGA that was approached endoscopically in order to minimize morbidity associated with open surgical approaches. Results: The use of NICO Myriad system is described in this case to achieve successful tumor debulking without post-operative neurologic morbidity. Conclusions: This report reveals the value of minimally invasive neuroendoscopic techniques in the management of challenging intraventricular tumors while avoiding injury to crucial deep venous structures.
P.053 Expanded endoscopic endonasal approach for petrous apex lesions: our clinical experience and surgical techniques
Authors: Alkherayf F , Kilty S
Published: June 2018

Background: Traditionally petrous apex lesions surgical approach is associated with multiple complications including brain injury secondary to brain retraction. Expanded endoscopic endonasal trans-clival (EEET) can be used in selected patients with minimal complications. Methods: We are presenting our experience over the last three years in patients who underwent EEET resection of petrous apex lesions: 8 patients underwent such procedure. All patients underwent extensive workup including MRI and CTA to identify the relation of the carotid to the lesion. All surgeries were done with neuro-physiological monitoring. Intraoperative neuronavigation and endoscopic Doppler were used to identify the petrous segment of the internal carotid artery. Our follow up ranged from 6 months to 2.5 years. Results: All patients presented with severe neurologic symptoms related to either fifth cranial nerve, sixth cranial nerve or brain stem compression. Pathologies included chondrosarcoma, cholesterol granulomas and lymphangioma. All patients demonstrated improvement in their symptoms. None of our patients had intra-operative vascular injury. There was no post-operative CSF leak or infection. Postoperative imaging demonstrated excellent resection with no clear residual. Three patient required adjuvant stereotactic radiosurgery because of their underlying pathology. Conclusions: The expanded endoscopic endonasal approach for petrous apex lesion should be considered as a minimally invasive approach in selected cases.
P.056 Predictability of pituitary tumor resection and recurrence following endoscopic endonasal trans-sphenoidal surgery
Authors: Alkherayf F , Alshardan M , Lasso A , Mohajeri S , Masoudian P , Lamothe A , Agbi C , Caulley L , Banaz F , Kilty S
Published: June 2018

Background: The surgical treatment of pituitary tumour has undergone substantial changes over time. In this study we evaluated our institutional results for pituitary tumour surgery using the endoscopic endonasal trans-sphenoidal (EETS) approach. Methods: Patient demographic, clinical and surgical data were extracted from medical records. Preoperative MRI images were reviewed. The SIPAP classification was applied to the pituitary tumors. Chi2 test and t test were used for statistical analysis. Results: 202 cases were identified. Functional tumors were present in 29% of the cohort. Patients with a suprasellar or parasellar SIPAP score of 0 or 1 had complete resection of their tumor in 66.6% of cases, compared to 29% with a suprasellar or parasellar SIPAP score ≥ 2 (Risk Ratio 2.3 CI 1.58-3.39, p=0.0005). When the tumor was completely resected radiologically, the mean time to recurrence was not different for the SIPAP 0 or 1 group which was 27 months in comparison to 34 months for the group with a SIPAP score 2 (p=0.13). Conclusions: Our study results showed that the preoperative MRI SIPAP score can be used to better inform patients about their expected outcomes of EETS.
P.057 A systematic review of the prophylactic antibiotic use in endoscopic endonasal transsphenoidal surgery for pituitary lesions
Authors: Moldovan ID , Agbi C , Kilty S , Alkherayf F
Published: June 2018

Background: The benefit of prophylactic antibiotic use in endoscopic endonasal transsphenoidal surgery (EETS) for pituitary lesions is controversial. Many surgeons administer antibiotics perioperatively not based on clear guidelines but “to be safe”. Methods: A systematic review using PRISMA guidelines was performed to assess the efficacy of perioperative antibiotic use to prevent infectious complications in patients undergoing EETS. Inclusion criteria: randomized controlled trials, systematic reviews, observational studies, and case series. Data extracted: study design, year of publication, sample size, surgery type, perioperative antibacterial treatment (antibiotic, dose, and duration), number of patients with 30-days post-operative meningitis and/or sinusitis. End points: rates of meningitis and sinusitis post-EETS. Results: A total of 280 articles were identified. Four observational studies met inclusion criteria. Based on GRADE score these studies were considered low in quality. 633 patients were -included in those studies. The most common antibiotics used were cefazolin and ceftazidime. The rate of infection ranged from 0.5% to 3.1 % for meningitis as the most common infection. Conclusions: The need to use antibiotic(s) perioperatively is not clear in patients with pituitary lesions undergoing EETS. Randomized control trials are needed to evaluate the efficacy of prophylactic antibiotic use in patients with pituitary lesions undergoing EETS.
P.062 MR Venography predicts increased intracranial hypertension in children with hydrocephalus
Authors: Rohr AC , Knerlich-Lukoschus F , Heran M , Shewchuk J , Margraf N , van Baalen A , Jansen O
Published: June 2018

Background: We investigated whether the presence of dural sinus narrowing is a more reliable marker of intracranial hypertension / shunt failure in children than the imaging finding of hydrocephalus. -Methods: Cranial MRIs of n=12 children were included when being well and when there was definitive intracranial hypertension as per follow-up and intraoperative results (gold standard). Images werde assessed for hydrocephalus on T2w images and narrowing of dural sinuses on MR vengraphy (diameter of <50%). Results: Dural sinuses narrowing was detected with a sensitivity of 0.67, a specificity of 1.0, PPV of 1.0 and NPV of 0.75 (Table 1). Hydrocephalus was detected with a sensitivity of 0.5, a specificity of 0.83, PPV of 0.75 and NPV of 0.63. Results differed between the test methods (p = 0.01, Cochrane Q test). Conclusions: Dural sinus narrowing more reliably predicted intracranial hypertension, a sign which might significantly improve care in critically ill children.Age at MRIShuntfailure as per clinical follow-up
(Goldstandard)Hydro
cephalusDural Sinus
NarrowingPatient #Years1 = yes
2 = no1 = yes
2 = no1 = yes
2 = no111104000261116000312101120004181011900050110101060010011071710117000810111100009011110001081018000111410114000121810018000Shuntfailure(Goldstandard)AffectedNonaffectedtotalHydrocephalusPositive618Negative61016121224Shuntfailure(Goldstandard)AffectedNonaffectedtotalDural Sinus NarrowingPositive808Negative412161212
P.066 Cortical autonomic patterns in Neurogenic Orthostatic Hypotension
Authors: Baker J , Paturel JR , Kimpinski K
Published: June 2018

Background: Neurogenic orthostatic hypotension (NOH), defined as a drop in systolic blood pressure (SBP) ≥30mmHg on standing or head-up tilt, is associated with autonomic dysfunction. The cortical autonomic network (CAN) is a network of brain regions associated with autonomic function. Our aim was to investigate CAN activation patterns in NOH patients during autonomic testing. Methods: Fifteen controls (61±14 years) and 13 NOH patients (68±6 years;p=0.1) completed: 1)Deep Breathing (DB), 2)Valsalva maneuver (VM) and 3)Lower-body negative pressure (LBNP) during a functional MRI. Blood-oxygen level dependent (BOLD) contrasts were obtained and contrasted. Results: Compared to controls (C), patients (NOH) had significantly smaller heart rate (HR) responses to DB (C:15.3±9.6 vs.NOH:6.0±2.2) and VR’s (C:2.1±0.47 vs.NOH:1.2±0.1;p<0.001). Patients had larger SBP drops during LBNP (C: -22.3±6 vs.NOH: -61±22) with -significantly smaller compensatory tachycardias (19±8.5 vs.7.6±4.3)(p<0.001). BOLD response: During VM, controls had greater activation in the right (R) hippocampus (T-value:7.34), left (L) posterior cingulate (T-value:7.22) bilateral mid-cingulate (TR-value:5.76; TL-value:6.84) and bilateral thalamus (TR-value:7.23, TL-value:8.16)(pFWE<0.001). Following subtraction analysis, brain activation patterns showed no significant differences in the regions of interest in response to DB and LBNP. Conclusions: During tests of autonomic function, NOH patient had different cortical activation patterns during VM only. Cortical activation pattern during DB and LBNP showed similar patterns to that of controls.
P.067 Phosphoserine aminotransferase (PSAT) deficiency: Imaging findings in a child with congenital microcephaly
Authors: Shapira Zaltsberg G , McMillan H , Miller E
Published: June 2018

Background: Serine deficiency disorders can result from deficiency in one of three enzymes. Deficiency of the second enzyme in the serine biosynthesis pathway, 3-phosphoserine aminotransferase (PSAT), has been reported in two siblings when the eldest was investigated for acquired microcephaly, progressive spasticity and intractable epilepsy. Methods: Our patient had neurological symptoms apparent at birth. Fetal magnetic resonance imaging (MRI) at 35 weeks gestation demonstrated microencephaly and simplification of the the gyration (anterior>posterior) which was confirmed upon subsequent post-natal MRI. Congenital microcephaly was apparent at birth. Results: PSAT deficiency was confirmed when exome sequencing identified biallelic mutations in PSAT1; c.44C>T, p.Ala15Val and; c.432delA, p.Pro144fs and biochemical testing noted low plasma serine 22 mcmol/L (normal 83-212 mcmol/L) and low CSF serine 10 mcmol/L (normal 22-61 mcmol/L). Despite oral serine and glycine supplementation at 4 months old the patient showed little neurodevelopmental progress and developed epileptic spasms at 10 months old. Serological testing for TORCH infections was negative. Conclusions: PSAT deficiency should be considered for patients with congenital microcephaly. Although further characterization of MRI findings in other patients is required, microencephaly with simplified gyral pattern could provide imaging clues for this rare metabolic disorder.
P.080 Neurosurgery Residency Program at the Faculty of Medicine, Universitas Gadjah Mada, Indonesia: a unique approach and strategy for an archipelago country
Authors: Manusubroto W , Ekong C
Published: June 2018

Background: Indonesia is a vast archipelago country with over 17,000 islands. Many of the islands are in underdeveloped provinces with no neurosurgeon. Neurosurgery is often considered an expensive and sophisticated field to fund. Our neurosurgery department used the vision and mission of the medical faculty, which is “globally respected and locally rooted” to make a difference in many of the islands. Methods: Careful selection of provinces and candidates that involve local governments and hospitals within the province. This includes resident recruitment, planning and developing neurosurgical infrastructure in the province. Our program uses innovative neurosurgical techniques that are standardized to be applicable in underdeveloped areas. The residents are exposed to their province and hospital during the training. We optimize IT interaction, including teleconference, videoconference and telemedicine Results: At the fifth year of our program, we have sixteen residents from 8 underdeveloped provinces and have established MoU with 4 local hospitals around Indonesia. We have also sent residents to rural areas. We routinely participate in international teleconferences and videoconferences, including those with the Saskatchewan team. Conclusions: A well planned and structured neurosurgical program, with standardized processes and involvement of local officials, combined with extensive use of IT, is effective in preparing neurosurgeons who can provide quality care in underdeveloped regions.
P.089 AMPA receptor modulation as a therapeutic strategy to enhance survival of spinal cord neural stem cells
Authors: Hachem LD , Mothe AJ , Tator CH
Published: June 2018

Background: Transplantation of neural stem/progenitor cells (NSPCs) following spinal cord injury (SCI) is a promising strategy to enhance regeneration but is limited by poor survival of grafted cells. Recently, we demonstrated for the first time that the excitatory neurotransmitter glutamate, which is released after SCI, promotes survival/proliferation of spinal cord NSPCs via the AMPA subtype of glutamate receptors. Here, we examine the therapeutic potential of selective AMPA receptor modulation on NSPC survival using allosteric AMPA receptor modulators known as ampakines. Methods: NSPCs from the periventricular region of the adult rat spinal cord were treated with ampakines CX614 and CX546 for 72h either alone or in the presence of low-dose glutamate (50uM). Results: Treatment with CX-546 or CX-614 in the presence of glutamate led to a significant increase in live cell numbers. This was due to both a reduction in cell death and increase in cellular proliferation. Ampakine/glutamate treatment led to a significant increase in cell survival compared to controls in the setting of oxidative stress. Conclusions: We present the first examination of the effect of allosteric AMPA receptor modulators on adult spinal cord--derived NSPCs. Positive modulation of AMPA receptors may be a promising therapeutic strategy in the subacute/chronic phases after SCI to increase survival of endogenous or transplanted NSPCs.
P.097 Subarachnoid hemorrhage associated with a thromboembolic ischemic stroke- an unexpected observation
Authors: Almutlaq A , Alhusaini S , Chalk C , Cote R
Published: June 2018

Atherosclerosis is a significant risk factor for ischemic stroke, and is a frequent cause for extra- and intra-cranial vessels stenosis. Here, we present an unusual case of ischemic stroke associated with intra-cranial vessel stenosis and subarachnoid hemorrhage (SAH) secondary to carotid artery atheroma. A 64-year old female known for hypertension and dyslipidemia presented with a three-day history of three transient episodes (< 30 minutes) of dysarthria and right hand weakness. An initial brain CT scan revealed left frontal SAH. She was admitted to our Stroke Unit for observation and management. CT-angiogram revealed 90% ICAs stenosis bilaterally with several short focal stenotic lesions, involving several left MCA branches. Brain MRI revealed acute infarcts in the left insula, external capsule and inferior frontal gyrus. The clinical picture was attributed to a thromboembolic left MCA ischemic stroke. She was managed with maximum medical therapy, and later underwent successful left carotid endarterectomy. The presence of SAH on our patient’s presenting CT scan lead to an initial radiological diagnosis of RCVS. However, subsequent imaging studies indicated that SAH had occurred in association with a thromboembolic ischemic stroke. Despite its rarity, this clinical association is important to recognize to avoid diagnostic confusion and guide appropriate management.
P.100 Endovascular Thrombectomy (EVT) for stroke: experience in a Canadian teaching hospital
Authors: Hu S , Virani K , Phillips S , Shankar J
Published: June 2018

Background: EVT is now recommended as standard of care for stroke in Canada, but its implementation still poses challenges. We studied the delivery of EVT in our hospital, a participanting site in the ESCAPE trial, which serves the province of Nova Scotia. Methods: Patients who underwent EVT December 2011 – December 2016 were identified prospectively. Demographics, process measures, imaging characteristics (Alberta Stroke Program Early CT Score [ASPECTS], collateral score, Thrombolysis in Cerebral Infarction [TICI] score), and outcomes, including modified Rankin score [mRS] ~ 90 days post-EVT, were collected retrospectively. Effectiveness was assessed by comparison with outcomes in the ESCAPE trial. Results: 91 patients (M:F= 48:43; mean age 64 years) presented to hospital after 194 min ± 230 min from last seen normal. In 58%, the ASPECTS was >7. 80% had good/intermediate collaterals. Alteplase was administered to 72% (75% in ESCAPE, p=0.97). EVT mean duration was 70 min ± 62 min. Successful recanalization (≥TICI 2b) was achieved in 76% (vs 72.4% in ESCAPE, p= 0.97). Among the 54 patients recanalized, mRS scores of 0-2, 3-5 and 6 were seen in 57.4, 24.1 and 14.8% respectively; ESCAPE comparators 53, 37 and 10%, p=0.96, 0.86 and 0.91. Conclusions: EVT at our hospital yielded results similar to the ESCAPE trial.
P.102 Endoscopic harvesting of a saphenous vein graft for EC-IC bypass followed by proximal artery occlusion of a pediatric giant fusiform MCA aneurysm
Authors: Wolf AM , Swinamer S , Van Adel B , Lownie S
Published: June 2018

Background: Minimally invasive techniques for graft procurement are the norm in cardiac surgery and yet their use in neurosurgery is only in its infancy. We present the case of a 10-year-old boy presenting with fluctuating right facial and upper extremity weakness who was found to have a giant, partially thrombosed, fusiform aneurysm of the M1 segment of the left MCA. Methods: Endoscopic harvesting of the saphenous vein was performed with a procedure time of 30 minutes. The graft was used as an interposition graft between the common carotid artery and the superior M2 division of the MCA, which was tunneled subcutaneously. Once Doppler ultrasound confirmed good flow through the graft, an aneurysm clip was then secured on the M1, proximal to the saccular component of the fusiform aneurysm and just distal to the anterior temporal branch. Results: Intraoperative 2D and 3D angiogram confirmed a patent extracranial to intracranial bypass with thrombosis of the giant fusiform M1 aneurysm. By 1-month post-operatively, he had returned to school and routine activities. He continues to do well 6 months post-operatively with a minimal and well-healed donor site scar. Conclusions: Endoscopic graft harvesting is an emerging option in the pediatric population undergoing extracranial to intracranial bypass, associated with lower wound complications and improved cosmesis.
P.103 Persistent primitive hypoglossal artery with an associated posterior circulation aneurysm
Authors: McGregor S , Chalil A , Boulton M
Published: June 2018

Background: Persistent fetal carotid-vertebrobasilar anastomoses are rare, with an incidence of <1%. The most common anomaly seen in this group is a persistent primitive trigeminal artery; others such as a persistent hypoglossal artery account for less than 15% of all persistent fetal anastomoses, making this finding exceedingly rare. Methods: We present the case of a 32-year-old-female with Poland syndrome (right-sided), who presented with thunderclap headache and reduced level of consciousness secondary to diffuse subarachnoid hemorrhage and hydrocephalus. CT and catheter angiography demonstrated an aneurysm of the V4 segment of the right vertebral artery arising from a persistent right hypoglossal artery, with an absent ipsilateral vertebral artery proximal to the anomaly. Results: Hydrocephalus was treated with an EVD, followed by a successful embolization of the V4 aneurysm with Axium coils. Subsequent MR studies demonstrated minimal recanalization of the aneurysm, and small foci of possible infarcts in the hippocampi. Four months later, the patient has some persistent short term memory difficulties but is otherwise neurologically intact. Conclusions: We present a rare finding of a persistent fetal hypoglossal artery with an associated vertebral aneurysm. The aneurysm was successfully treated endovascularly through coil embolization with minimal residual -neurological deficit. This vascular anomaly was ipsilateral to her Poland Syndrome defects.
P.105 Missed vertebral artery dissection: a case series
Authors: Persad A , Stewart B
Published: June 2018

Background: Vertebral artery dissections are the second most common cause of posterior circulation stroke. Particularly in young people, they must be considered as causes of acute infarction, especially with a history of cervical trauma. Here, we present three cases of vertebral artery dissection that were initially not diagnosed as such. All were caused by uncommon mechanisms; one by self-inflicted neck manipulation, and one as a sequela of falling from a trampoline, and one from minor trauma to the head while standing. Methods: This is a series of three cases seen by the authors of posterior circulation stroke secondary to vertebral artery dissection caused by uncommon mechanisms. Results: N/A Conclusions: Vertebral artery dissection should be considered as a differential diagnosis in patients presenting with acute head and/or neck pain and any neurological findings in relation to acute neck trauma.
P.108 Case report: listeria rhombencephalitis in a healthy 64 year old woman
Authors: Kirk A , Omene E
Published: June 2018

Background: Listeria rhombencephalitis is a rare and serious complication of Listeria monocytogenes infection. We present a case of presumed Listeria rhombencephalitis with dramatic recovery from a highly morbid state. Methods: A previously healthy 64 year old woman with a remote and stable history of a major depressive episode and no history to suggest immune compromise presented with nausea and vomiting followed by the acute onset of diplopia and gait disturbance 28 days after exposure to an identified infectious source of spring rolls and 21 days after a severe diarrheal illness from that exposure. Our patient was evaluated by emergency physicians and general internists over a period of 1 week after the onset of diplopia and gait disturbance and given a diagnosis of serotonin syndrome before receiving a consultation from Neurology. Her presentation featured a deep encephalopathy and an unusual hyperkinetic movement disorder with startle myoclonus, palatal myoclonus and diffuse tremor. Results: Her MRI scan showed FLAIR hyperintensities in the bilateral cerebellum and pons with adjacent pial enhancement, characteristic of Listeria rhombencephalitis. Her CSF showed a lymphocytic pleocytosis with normal chemistry. Conclusions: She recovered dramatically to treatment with IV ampicillin. This case report illustrates the importance of considering Listeria rhombencephalitis in immunocompetent patients with brainstem symptoms following a diarrheal illness.
P.110 Effects of hypoglycemia on sensitive brain structures in a patient with an insulinoma
Authors: Khayambashi S , Ng M , Katz P , Udow S
Published: June 2018

Background: A previously healthy 26 year-old male presented with confusion and recurrent hypoglycemia (blood glucose lows of 2.5 mmol/L) while on vacation in Las Vegas. He denied substance or heavy alcohol use and the toxicology screen was negative. He was transferred home to Winnipeg for further care and was found to have only patchy memories of his trip and the days leading up to the trip, consistent with mixed anterograde and retrograde amnesia. MoCA score at presentation was 16/30 with points lost on orientation, delayed recall and visuospatial-executive tasks. MRI revealed T2 hyperintensities and diffusion abnormalities in bilateral hippocampi and globus pallidi. Electroencephalography showed triphasic waves. The patient was found to have a pancreatic insulinoma, which was surgically resected. In follow-up nine weeks later he was near his cognitive baseline, though he had ongoing difficulties with delayed recall. Repeat MRI showed improvement but not resolution of hippocampal and pallidal signal change, with mild hippocampal atrophy.

Neuropathological and animal studies have shown that structures most sensitive to hypoglycemic neural injury include the hippocampus, basal ganglia, and neocortex. The clinical and radiographic findings in this case illustrate an unusual presentation of insulinoma and the effects of hypoglycemia on the brain. Methods: N/A Results: N/A Conclusions: N/A
P.116 Orbital lymphaticovenous malformation with intradural extension: a rare case
Authors: Abbass M , Tso MK , Weis E , Mitha AP
Published: June 2018

Orbital lymphaticovenous malformations (LVM) are congenital vascular lesions that are typically infiltrative in nature. There have been reports of orbital LVMs extending intracranially through orbital fissures, but there have been no reports of intradural extension that we are aware of. We present the case of an otherwise healthy 25-year-old female with an orbital LVM extending intradurally. Imaging revealed an intraorbital lesion extending through a bony defect in the medial orbital roof to the orbitofrontal cortex. A modified orbitozygomatic approach was used to obliterate this lesion. A durotomy was created to examine the intradural extension of the lesion, which appeared as a lobulated red vascular structure emanating from the dura along the roof of the orbit. This was gradually and comprehensively bipolar coagulated and subsequently obliterated. Neurosurgical and ophthalmological collaboration was used in the surgical management of this case. In summary, we report the first case of an orbital LVM extending intradurally, and provide pre and post-operative imaging as well as images captured through the intraoperative microscope. Through this case we highlight the importance of an interdisciplinary approach when managing orbital LVMs, as both ophthalmological and neurosurgical expertise were critical in the success of the surgery.
P.119 Industry relationships with neurological surgery in the 2015 Open Payments Database
Authors: de Lotbiniere-Bassett MP , McDonald PJ
Published: June 2018

Background: The 2013 Physician Payments Sunshine Act mandates that all US drug and device manufacturers disclose payments to physicians annually in the Open Payments Database (OPD). We aimed to determine the prevalence, magnitude and nature of these payments to neurological surgery in 2015. Methods: Records of payments to physicians identified by the ‘neurological surgery’ taxonomy code in 2015 were accessed via the OPD. The data were analyzed in terms of the type and amounts of payments, companies making payments, and in comparison to previous studies. Results: In 2015, 330 companies made 83,690 payments ($99,048,607) to 7,613 physicians. The mean payment ($13,010) was substantially greater than the median ($114). Royalties and licensing accounted for the largest proportion of total payment value (74.2%), but only 1.7% of the total number. Food and beverage payments were the most commonly reported transaction (75%), but only 2.5% of the total value. Neurological surgery had the second highest average total payment per physician of any specialty. Conclusions: The overall value of payments to the neurological surgery specialty is driven by a small number of payments that may represent appropriate compensation for novel device development. The OPD provides an opportunity for increased transparency and for the interpretation of research in light of potential conflicts of interest.
P.125 Is idiopathic normal pressure hydrocephalus familial—what do we know thus far? Case report and critique of the literature
Authors: Li H , Meguro K
Published: June 2018

Background: One aspect of idiopathic normal pressure hydrocephalus (iNPH) that has garnered interest is whether it can be familial. Thus far, the literature consists of several case reports, and two larger pedigree cohorts. Our objective is to highlight key deficiencies in such studies so far, illustrating them through a family case study of our own, and to propose a set of criteria that studies on familial iNPH should incorporate. Methods: Our case study is a retrospective chart review of three siblings, two male and one female, who were diagnosed with iNPH after the age of 60, and whose symptoms improved with cerebrospinal fluid (CSF) shunting. An interview with them revealed that their mother also exhibited symptoms of iNPH, but was never treated with a shunt. Results: Our family case is reflective of several deficiencies of familial iNPH research as a whole—unconfirmed diagnosis, especially confirmation with shunt responsiveness, and lack of measures of symptom improvement. Conclusions: Research on familial iNPH should focus on patients whose diagnosis is confirmed by shunt responsiveness, and should involve a system to objectively measure signs of NPH. Studies should also compare the prevalence of iNPH among first degree relatives of NPH patients to that in the general population.
P.129 Worster-Drought syndrome caused by LINS mutations
Authors: McMillan HJ , Holahan A , Richer J
Published: June 2018

Background: Worster-Drought syndrome (WDS) is a congenital, pseudobulbar paresis. Patients show oromotor apraxia causing impaired speech, drooling, dysphagia and varying degrees of cognitive impairment. Familial cases are reported although causative genes have not been identified. LINS mutations have recently been reported in patients with severe cognitive and language impairment. Methods: The proband was diagnosed with WDS at 8 years old because of longstanding drooling, dysphagia and impaired tongue movement. At 14 years old, he remains aphonic, using sign language and typing on a smart-tablet to communicate. Neurological examination including facial and extraocular movement was otherwise unremarkable. MRI brain revealed no heterotopia or atrophy. Results: An expanded intellectual disability panel at GeneDx identified nonsense mutations in LINS alleles: c.1096; p.Glu366X and c.1178 T>G, p.Lys393X. Neuropsychological testing at 14 years old noted nonverbal reasoning skills at 5 year old level with relative sparing of his receptive vocabulary and visual attention. Compared to prior testing at 9 years his receptive language improved from a 6 year old to an 8.5 year old level. Conclusions: Nonsense mutations of LINS have been identified in a patient with WDS. Despite his severe and persistent aphonia, improvements in receptive language were observed with global intellectual functioning better than expected.
P.132 Redesign of a neuropsychology service in a tertiary pediatric hospital (CHEO)
Authors: Holahan A , Irwin J , Honeywell C , Kortstee S , Anderson P
Published: June 2018

Background: Neuropsychological assessments are used in hospitals to examine brain-behaviour relationships, and are an integral part of care for medically complex patients. Unfortunately, waitlists can be lengthy. We gathered information regarding best-practice guidelines and physician referral patterns in an effort to better manage the neuropsychology waitlist at a pediatric hospital. Methods: We conducted: 1) A semi-structured telephone survey with 4 Canadian, pediatric, hospital-based neuropsychology services; 2) An electronic survey distributed to referring physicians at CHEO; 3) A focus group for CHEO neurologists and neurosurgeons. Results: The telephone survey indicated that there are no clear, best-practice guidelines for pediatric neuropsychologists working in a tertiary, pediatric hospital. The electronic survey revealed some confusion about neuropsychology services and indicated the need for better communication between neuropsychology and referral sources. The focus group revealed that demand for neuropsychology services far outstrips supply and confirmed the need for better communication. Conclusions: The results confirmed the need for best-practice guidelines to be developed around delivering neuropsychology services within a pediatric tertiary care setting, as well as continuing to work closely with neurology and neurosurgery to ensure that the neuropsychological needs of their patients are met.
P.133 Expanding the phenotype of TRNT1 mutations to include Leigh syndrome
Authors: Gorodetsky C , Morel CF , Tein I
Published: June 2018

Background: Children with biallelic mutations in TRNT1 have multi-organ involvement with congenital sideroblastic anemia, -B-cell immunodeficiency, periodic fevers, and developmental delay (SIFD) as well as seizures, ataxia and sensorineural hearing loss. The TRNT1 gene encodes the CCA-adding enzyme essential for maturation of both nuclear and mitochondrial transfer RNAs accounting for phenotypic pleitropy. Neurodegenerative Leigh syndrome has not been previously reported. Methods:Case summary: A Portuguese boy presented with global developmental delay, 2 episodes of infantile Leigh encephalopathy at 8 mo and 4 yr responsive to high-dose steroids, slow neurodegeneration of cognitive, language and motor functions with optic atrophy, pigmentary retinopathy, spasticity, dystonia, and focal dyscognitive seizures, pancytopenia, transfusion dependent sideroblastic anemia, recurrent febrile infections (pulmonary, gastrointestinal), hypernatremia, with tracheostomy dependence at age 5 yr, malabsorption and TPN dependence at 9 yr, and survival to early adulthood. Neuroimaging showed symmetric hemorrhagic lesions in the thalamus, brain stem (periaqueductal grey) and cerebellum consistent with Leigh syndrome but no lactate peak on MRS. Results: Whole exome sequencing identified a homozygous missense pathogenic variant in TRNT1, c.668T>C (p.I223T) in the affected individual. Conclusions: This report expands the neurological phenotype of TRNT1 mutations and highlights the importance of considering this gene in the evaluation of Leigh syndrome.
P.141 Is occipito-condylar screw a better alternative fixation point for occipitocervical stabilization? 1. Review of the literature
Authors: Alshafai Ns , Dibenedetto M
Published: June 2018

Background: Occipitocervical fusion (OCF) using screws and rods offers immediate stability and an high fusion rates. However, multiple cranial fixation points are required in order to compensate for the poor bony purchase. Methods: The aim of this study was to compare the occipital condyle screw with the standard OCF techniques as well as to compare available techniques of the occipital condyle screw insertion. Materials and Methods: A comprehensive “Medline” and “Web of science” database search was performed. Cadaveric, radiographic and case studies were included. Results: The occipital condyle screw in comparison to the occipital plate enables an increased screw length, greater screw pullout strength, lower profile of the hardware and extended grafting surface. Both constructs have similar biomechanical properties (range of motion restriction, stiffness). Proximity of the vertebral artery and hypoglossal canal presents the greatest technical challenge of occipital condyle screw. Four surgical techniques with different entry points, cranial-caudal and medial angulations were described. None of these techniques is superior to the other. Conclusions: Occipital condyle screw is a viable alternative to standard OCF techniques. Challenges exist due to the proximity of the vital anatomical structures. Choice between four available techniques depends on unique patient’s anatomy
P.142 The effect of modern technology on cervical spine biomechanics. Literature review
Authors: Alshafai Ns , Aldhafeeri W
Published: June 2018

Background: The use of smartphones has increased drastically over the last decade. Improper posture, and excessive use have raised concerns about their effect on cervical spine health. Methods: -MEDLINE database was searched for articles using the keywords: neck pain, musculoskeletal symptoms, cervical spine, cervical -biomechanics, mobile phone, cell phone, smart phone, smartphone, mobile device, touchscreen phone. Full-text Articles from 1990 to 2017 were included. Statistical comparisons and tables are provided when appropriate. Results: 43 articles were included for review. First article was published in 2002. Majority of studies were published between 2010 – 2017 (36 vs. 5 in 2000-2010). Studies included were of cross-sectional, experimental, or systemic review design. No longitudinal studies were identified. We categorized articles into 5 subgroups; we found 14 biomechanical studies, 10 electromyographic studies, 5 ergonomical studies, 14 clinical studies, and no surgical studies. Conclusions: Text-neck posture leads to significant changes in cervical spine biomechanics. Increased compressive load, antero-posterior shear load, and high cervical extensor muscles activity were associated with forward flexed neck posture adapted by smartphones users. Neurosurgeons need to take the abnormal posture and load distribution into consideration when planning for surgical interventions, especially in young adults with history of excessive use of smartphones.
P.143 Spinal cord intramedullary malignant peripheral nerve sheath tumour: case report and review of literature
Authors: Karapetyan V , Staudt MD , McGregor SM , AlYamany B , Haji FA , Ang LC , Siddiqi F
Published: June 2018

Background: Malignant peripheral nerve sheath tumours (MPNST) are uncommon but aggressive neoplasia associated with radiation exposure and neurofibromatosis (NF). These tumours are often found in the trunk, extremities, head and neck, with capacity to metastasize. Only a handful of case reports have described intramedullary spinal cord MPNST. Methods: We report the case of a 35 year-old female who presented with progressive gait disturbance and paraparesis. MRI of the spine demonstrated an enhancing intramedullary mass at the C7 vertebra. Laminectomy with expansile duraplasty, and extended surgical biopsy were performed for cord decompression and tissue diagnosis. Results: Pathological sections demonstrated a spindle cell neoplasm with nuclear atypia, frequent mitotic figures, focal necrosis, and infiltration into adjacent neurological tissue. It was positive for S100, SOX10, p53 with partial loss of INI-1. Diagnosis of spinal intramedullary MPNST was confirmed, however given the prior history of remote trigeminal MPNST, it was unclear whether the mass represented a delayed metastasis, or a de novo neoplasm. Conclusions: Typically relegated to the periphery, our case represents a rare spinal medullary presentation of MPNST. While the differential for tumours in this location typically includes diffuse astrocytomas, ependymomas, and rarely schwannomas, we move that consideration of MPNST in select high risk cases advise surgical planning and subsequent therapy.
GP.02 A population-based study of “no evident disease activity” (NEDA) in multiple sclerosis
Authors: Parks NE , Pittock SJ , Mandrekar J , Kantarci OH , Lucchinetti CF , Weinshenker BG , Keegan M , Tobin O , Tillema J , Toledano M , Flanagan EP
Published: June 2018

Background: NEDA is a composite measure that may ultimately influence clinical decisions concerning switches of disease modifying therapy (DMT) for relapsing remitting multiple sclerosis (RRMS) patients. Cohort studies from MS clinics suggest NEDA is not sustained over time in most patients despite DMT but may be limited by referral bias. We investigated NEDA in a population-based RRMS cohort. Methods: We identified all incident cases of RRMS in Olmsted County from 01/01/2000-12/31/2011. Retrospective chart review was conducted to determine persistence of NEDA -following RRMS diagnosis. NEDA failure was defined as new MRI activity, relapse, or expanded disability status scale (EDSS) -worsening. Results: There were 93 incident cases of RRMS with 82 individuals having sufficient follow-up to determine persistence of NEDA. Prior to NEDA failure 44 were not on DMT, 37 were on first-tier, injectable DMT, and 1 received mitoxantrone. NEDA was maintained by 63% at 1 year, 38% at 2 years, 19% at 5 years, and 12% at 10 years. Disability measured by EDSS was no different at 10 years in patients maintaining NEDA versus those that failed NEDA at one year (p=0.3). Conclusions: Maintenance of NEDA beyond 2 years is infrequent among a population-based cohort of newly diagnosed RRMS patients and similar to prior clinic-based cohorts.