Background: The aim of this study is comparing the waiting time and patient’s satisfaction of microvascular decompression (MVD) surgery between local Manitoba (MB) and out of province (OOP) patients, treated at our Centre for Cranial Nerve Disorder (CCND). Methods: Data from 100 consecutive patients (average age: 56.8±10.6 years), undergoing MVD surgery for Trigeminal Neuralgia (TN) and Hemifacial Spasm (HFS) were reviewed. The outcome measures included the time intervals between disease onset, diagnosis and referral to CCND, postoperative discharge, satisfaction with surgical outcome and referral process. Results: The preoperative time leading to CCND referral were longer for OOP patients, (onset to diagnosis/diagnosis to referral: 2.6±3.8/4.2±4.7 (OOP) versus 1.2±2.1/2.5±4.1 (MB) years; p=0.04/0.04), and referrals were more likely self-directed in OOP patients (62% (OOP), 21% (MB); p=0.007). Postoperative satisfaction with MVD outcome were 8.6/10 for OOP and 8.3/10 for MB patients. There was no significant difference in postoperative length of stay (38±50 (OOP)/43±42 (MB) hours); however, OOP patients were more likely discharged on the first postoperative day (58% (OOP), 31% (MB); p=0.17). Conclusions: Delays in diagnosis and surgical referral of TN/HFS are common, and many patients seek specialist’s opinion in high volume surgical centers. For those OOP patients, travelling for treatment, MVD outcome were at least as good as for local patients.

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.

Background: Wallenberg’s syndrome (WS), or lateral medullary syndrome is rare in pediatrics, but is not uncommon in adults. It is characterized by neurological deficits due to an ischemic lesion in the lateral medulla. Methods: Case report Results: We describe a 17-year-old boy who developed WS in the context of hyperflexion injury to the neck while diving in shallow water with vertebral dissection as a presumed etiology. He had ‘crossed’ neurological deficits above and below the neck. His MRA showed intra and extracranial left vertebral artery occlusion and his MRI showed T2W/FLAIR signal abnormality involving the left lateral medulla and inferomedial aspect of the cerebellum in keeping with infarcts secondary to the left vertebral artery thrombosis and occlusion of the left posterior inferior cerebellar artery. He was started on anti-coagulation after spinal surgery. On discharge, he had persistent dysphagia which prompt a gastrostomy tube placement prior to transfer to a rehabilitation center. Conclusions: Our case demonstrates that WS can occur post flexion injury in the pediatric population. The presence of crossed neurological findings above and below the neck in the context of neck injury is an important diagnostic clue that should prompt imaging study focusing on the brain stem and the posterior fossa vascular structures.

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.

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.

Background: Intracerebral hemorrhage (ICH) remains a significant cause of morbidity and mortality. While traditional surgical techniques have shown marginal clinical benefit of ICH evacuation, minimally invasive techniques have shown some promise. Endoscopic evacuation of the hemorrhage may reduce the peri-hematoma edema and subsequent atrophy around the hemorrhage cavity. This study aims to quantify the changes in cavity volume following hematoma evacuation. Methods: Patients from the INVEST registry of minimally invasive ICH evacuation were included retrospectively if follow-up computed tomography (CT) scans were available for analysis. Hematoma cavity volumes were calculated from the immediate post-procedural and three-month follow-up CT scans using the Analyze Pro software. Results: Twenty patients had follow-up CT scans at a mean time of 93 days from hematoma evacuation. The average cavity size at follow-up was 11938.12 mm3 (SD: 6996.49). The change in cavity size compared to the prior CT was 6396.74 mm3 (median 2542; range: -1030-27543; SD: 8472.45). This represented mean growth in cavity volume of 54%. Conclusions: This study provides preliminary data describing increase in cavity size after endoscopic minimally invasive evacuation of ICH. Comparison to atrophy in conservatively-managed patients is a further planned avenue of research.

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.

Background: Surgical resection of arteriovenous malformations (AVMs) in eloquent areas is significantly associated with greater surgical morbidity. We describe a functional approach for surgical treatment of these lesions Methods: A total of 20 patients with AVMs in eloquent areas were surgically treated and retrospectively analyzed. Individualized functional approach, using brain mapping and/or neurophysiological monitoring was performed in each case according to every case specific features and location. Seventeen patients underwent surgery under assleep conditions and 3 -patients underwent awake intraoperative mapping Results: There was no mortality. Four patients had hemorrhagic complications (20%). Ten (50%) presented neurological immediate postoperative worsening. Eight of them achieved complete recovery in follow up and 2 showed a permanent deficit. At 6 months follow up all the patients (100%) had good clinical outcome (mRS less than 2). There were no intraoperative seizures but 5 patients (26.3%) developed postoperative seizures. Fifteen patients (75%) had total AVM resection. Language and/or motor function were identified in all but one patient (95%). Each case required changes in surgical strategy to preserve the motor and/or language functions during surgery. Conclusions: Intraoperative monitoring and brain mapping are valuable and safe for the treatment of eloquent AVMs by indentifying and protecting motor and language function during resection.

Background: The safety and clinical outcomes of thrombectomy in the 80 years or older age group are not yet clear. Our aim is to provide data from clinical practice to assess the safety and efficacy of endovascular thrombectomy in this age group. Methods: We retrospectively reviewed consecutive patients of age ≥80 referred for thrombectomy procedures at our institutions from 01/01/2015 to 01/09/2015. We collected demographic data, risk factors, clinical and radiological findings, treatment details, clinical and radiological outcomes. Results: Data for 75 patients was included. Baseline clinical characteristics are similar to previous trials. There were MCA occlusions in 49% (37/75) and tandem occlusions in 15% (11/75) patients. 67% (50/75) patients received IVtPA. Good reperfusion (mTICI 2b/3) was achieved in 60% (45/75) patients within 224 minutes. Good clinical outcome (mRS 0-2) at 90 days was achieved in 31% (23/75) patients. Results are similar to HERMES data on patients of age ≥80 and differences will be discussed. Conclusions: Our study adds valuable evidence to the limited data on safety and clinical outcomes in patients 80 years of age and older who undergo thrombectomy. Our findings support the data from clinical trials and confirm that mechanical thrombectomy can be performed safely and in a timely fashion outside of trials with similar results.

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.

Background: We set out to test the discriminative power of an age-adjusted upper reference limit (URL) for CSF total protein (CSF-TP) in identifying pathological causes of albuminocytologic dissociation (ACD). Methods: We reviewed the charts of 2,627 adult patients who underwent a lumbar puncture at a tertiary care center over a 20-year period. Samples with CSF-TP above 45 mg/dL (0.45 g/L) were included. Samples with white blood cell count > 5×109/L, red blood cell count > 50×109/L, and glucose < 2.5 mmol/L (45 mg/dL) were excluded. Patients with CSF-TP elevated above 45 mg/dL were considered to have ‘pseudo’ albuminocytologic dissociation (ACD) or ‘true’ ACD if their CSF-TP was in excess of age-adjusted norms. Results: Among all patients with ACD, a pathological source of CSF-TP elevation was identified in 57% (1490/2627) of cases, 51% of those with ‘pseudo’ ACD, and 75% with ‘true’ ACD (p< 0.001). Use of an age-adjusted upper reference limit favored the detection of polyneuropathy patients (13.5% proportionate increase) and excluded a larger number of patients with isolated headache (10.7% proportionate decrease; p < 0.0001). Conclusions: Elevated CSF-TP is a relatively common finding. Use of age-adjusted upper reference limits for CSF-TP values improve diagnostic specificity and help to avoid over-diagnosis of ACD.

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

Background: To develop a scale to quantify and discriminate orthostatic from non-orthostatic symptoms. We present initial validation and reliability of orthostatic and non-orthostatic symptom scores taken from the Orthostatic Discriminate and Severity Scale (ODSS). Methods: Validity and reliability were assessed in participants with and without orthostatic intolerance. Convergent validity was assessed by correlating symptoms scores with previously validated tools (Autonomic Symptom Profile (ASP) and the Orthostatic Hypotension Questionnaire (OHQ)). Clinical validity was assessed by correlating scores against standardized autonomic testing. Test-retest reliability was calculated using an intra-class correlation coefficient. Results:Convergent Validity: Orthostatic (OS) and Non-Orthostatic (NS) Symptom Scores from 77 controls and 67 patients with orthostatic intolerance were highly correlated with both the Orthostatic Intolerance index of the ASP (OS:r=0.903;NS:r=0.651; p<0.001) and the OHQ: (OS:r=0.800;NS:r=0.574; p<0.001). Clinical Validity: Symptom Scores were significantly correlated with the blood pressure change during head-up tilt (OS:r=-0.445;NS:r=-0.354; p<0.001). Patients with orthostatic intolerance had significantly higher symptom scores compared to controls (OS:66.5±18.1 vs. 17.4±12.9; NS:19.9±11.3 vs. 10.2±6.8; p<0.001, respectively). Test-retest reliability: Both symptom scores were highly reliable (OS:r=0.956;NS:r=0.574, respectively; p<0.001) with an internal consistency of 0.978 and 0.729, respectively. Conclusions: Our initial results demonstrate that the ODSS is capable of producing valid and reliable Orthostatic and Non-Orthostatic Symptom Scores.

Background: Unplanned hospital readmission is inconvenient for patients, puts them at risk of harm, and is a resource strain. We reviewed available literature on risk factors for readmission following discharge specifically from neurology inpatient services with a focus on factors unique to non-stroke neurology admissions. Methods: We conducted a systematic search using PRISMA methodology of MEDLINE, EMBASE, and CENTRAL databases up to January 1, 2018. Two independent reviewers screened articles for inclusion. English-language articles were included that identified factors related to hospital readmission after discharge from a neurology service. Admissions with stroke as the primary focus were excluded. Results: Of 9508 unique abstracts, 25 met inclusion criteria and were included for review. Multiple factors impacting probability of readmission were identified including age, living alone, history of nonepileptic seizure, length of stay, services consulted during hospital stay, hospital volume, and severity of illness. Conclusions: There are identifiable risk factors that influence likelihood of readmission to hospital following discharge from neurology inpatient services, although the non-stroke literature is sparse. There is a need for future prospective work to investigate modifiable risk factors and opportunities to reduce readmission rates and improve patient safety.

Background: Down Syndrome (DS) has a mosaicism of presentations, but a number of common features. Cerebral evoked potentials (somatosensory, visual and auditory) can be higher in amplitude in DS. The aim of this study is to explore the value of the neurophysiological amplitude of three different modalities in DS individuals undergoing spinal surgery, or epilepsy evaluation. Methods: Standard procedure of EEG evaluation was conducted. We routinely monitor somatosensory (SSEP) and motor evoked potentials (MEP), using peripheral nerves stimulation and transcranial electrical stimulation during surgery. We report findings from 14 DS individuals age-matched to 14 individuals with idiopathic scoliosis Results: The amplitude of the SSEP is significantly higher in DS individuals than in age-matched controls using the same parameters. SSEP;10.2±2.5µV vs 2.4±2.3µV (p<0.05, paired t-test). The threshold for eliciting MEPs was also significantly lower in DS in comparison to controls, 175±20V vs 629±100V, (p<0.05, paired t-test). Interictal EEG showed high amplitude spike and waves, and greater intracortical coherence in DS with epilepsy than non-DS patients Conclusions: Robust neurophysiological findings showed high amplitude sensory evoked potentials, low threshold motor evoked potentials, and high amplitude spikes and wave, all reflect a common process of increased neuronal synchronicity and oscillatory behaviour in Down Syndrome.

Case Description: A 64 year-old male presented with left-sided weakness and altered level of consciousness after a suspected seizure. MR Brain demonstrated right mesial temporal lobe diffusion restriction. Empiric antiviral and antibiotic treatments were initiated despite CSF negative for HSV/VZV and enteroviruses. Lumbar puncture on admission day five was unchanged and empiric treatments were discontinued. On day 13 he deteriorated into status epilepticus necessitating ICU transfer. A third lumbar puncture demonstrated elevated protein and HSV-1 positive PCR. Acyclovir was restarted with guarded prognosis. Discussion: Detection of HSV-1 in CSF is considered the diagnostic gold standard for HSV-1 encephalitis. The validated multiplex assay used in Alberta, Canada has a 95% level of detection significantly better than the recommended threshold for HSV laboratory diagnosis. Previous reports have indicated that CSF PCR may be negative early in the disease course. Others have suggested that initially negative/follow up positive HSV PCR cases may represent secondary reactivation or release from underlying tissue damage. Consideration of the full clinical picture is crucial in patients with HSV negative PCR. Continuation of antiviral therapy may be appropriate in select HSV PCR negative patients.

Background: Phantogeusia associated with Parkinson Disease has not heretofore been reported. Methods: A 59 year old right handed female presented with a four year history of a bitter, sour and sweet taste on her entire tongue and roof of her mouth, 8/10 intensity, constant, persistent, without any external stimuli. Drinking water tasted bitter and sour. The phantogeusia was unresponsive to dietary changes, gabapentine, and allergy medications. Results: Abnormalities in Neurological examination: Decreased blink frequency. Hypokinetic. Hypomimetic face. Mood appears sad. Cranial Nerve (CN) examination: CN lll, lV, Vl: Saccadization of horizontal eye movements. Motor Examination: Pill rolling tremor in right hand. 1+ cogwheel rigidity in left upper extremity. Gait: 2+ retropulsion. Chemosensory testing: Olfactory: Alcohol Sniff Test: 6 (anosmia). SNAP Phenylethyl Alcohol Threshold Testing left -2.5 (hyposmia) & right > -2.0 (anosmia). Gustatory testing: Propylthiouracil Disc Taste test: 10 (normogeusia). Taste Testing Threshold: normogeusia to NaCl, Sucrose, HCl, Urea, and PTC. Other: DOPAPET: positive for Parkinson disease. Upper endoscopy: normal. Conclusions: Investigation for the presence of parkinsonian features in those with phantogeusia is warranted and chemosensory dysfunction including phantogeusia in those who presents with Parkinson’s disease is worthy of exploration.

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.

Background: Post-craniotomy pain can be severe and undermanaged. While opioids are the mainstay treatment, they have the potential to interfere with neurological monitoring. The objectives of this review are: 1) to identify measures to provide opioid-free analgesia 2) to compare the effectiveness of non-opioid to opioid analgesia in post-craniotomy pain. Methods: A comprehensive search of EMBASE, MEDLINE, and the Cochrane Central Registry of Controlled Trials (CENTRAL) databases was conducted for RCTs evaluating the effect of opioid vs non-opioid pain control strategies in patients undergoing supratentorial craniotomy. Results: The literature search yielded 462 citations, 5 RCTs that met the inclusion criteria for a total of 250 patients. Scalp infiltration/block was found to provide equivalent analgesia to morphine1 and fentanyl.2 Morphine was associated with slightly higher postoperative nausea and vomiting. Paracetamol was less likely to induce nausea and vomiting,3,4 but provided inadequate pain relief compared to nalbuphine,3 tramadol,3 morphine4 and sufentanil.4 Dexmedetomidine5 provided similar analgesia to remifentanil but did delay the time to first dose of rescue analgesia with similar side effects. Conclusions: Based on the limited number of RCTs comparing opioid to non-opioid techniques, no definite recommendations can be made with regards to the optimal management of post-craniotomy pain. Considerations should be made for use of multimodal analgesia-including adjuvant analgesics.

Background: The trigemino-cardiac reflex (TCR) is a sudden onset of bradycardia, hypotension, apnea or gastric hypermotility during stimulation of the trigeminal nerve. Methods: We conducted a MEDLINE search for surgical cases of TCR and herein describe a case seen recently at our institution. Results: A 60 year-old female underwent a left orbitozygomatic craniotomy for resection of a skull-base tumor. Pre-operative anesthesia evaluation was unremarkable and negative for a history of cardiovascular disease. Intra-operatively, retraction with moderate force of the temporalis muscle consistently produced asystole. Cessation of retraction resulted in immediate return of sinus rhythm. Otherwise, intra-operative heart rate was 60-90 BPM. Post-operatively, vital signs and clinical course were unremarkable. The patient experienced a similar phenomenon during an operation 6 years earlier, when manipulation of tumor near cranial nerves IX/X resulted in bradycardia. TCR is the result of a polysynaptic brainstem network involving the afferent trigeminal sensory nucleus, the reticular formation, and the efferent vagal motor nucleus. Conclusions: This is a case of exaggerated vagal response following manipulation of the temporalis muscle. Our report emphasizes the importance for neurosurgeons and anesthesiologists alike to be wary of TCR in order to avoid deleterious consequences when operating on structures associated with the trigeminal nerve.