Background: Focal neurological deficits occur in approximately 15% of children with bacterial meningitis. However, cranial nerve involvement such as facial-nerve palsy is uncommon in non-tuberculous bacterial meningitis. Methods: Case Report. Review of the literature was conducted on Pubmed for the search terms: facial nerve palsy and meningitis. Results: We present the case of a 4-year old right-handed girl who presented with a new onset unilateral facial nerve palsy preceded by 5-day history of fever and headaches. The patient had meningeal signs and was identified to have Streptococcal Meningitis. MRI of the brain showed a large previously undiagnosed intranasal encephalocele. The facial palsy resolved within 7 days of antibiotic treatment. Conclusions: Our case represents an unusual combination of facial nerve palsy in context of Streptococcal Meningitis secondary to intranasal encephalocele.

Introduction: Ketamine, an N-methyl D-aspartate (NMDA) receptor antagonist, carries potential benefit in patients with neurological illness. The cerebrovascular/cerebral blood flow (CBF) response to ketamine has been poorly outlined in the literature. Methods: We performed a systematic review of the literature on the cerebrovascular/CBF effects of ketamine in both animal and human subjects. Results: We identified 38 animal studies, and 20 human studies. Within the animal studies, a variety of different models were utilized with the majority focusing changes in global CBF or regional cerebral blood flow (rCBF). Overall, ketamine led to an increase in either global CBF or rCBF, with a vasodilatory effect in medium cerebral vessels. With the human studies a total of 379 patients, 107 of which were control subjects, were studied. Most studies focused on either 131Xe CT or PET imaging with ketamine administration. There was a trend to an increase in global CBF and rCBF with ketamine administration. Conclusions: Animal models indicate an increase in global CBF and rCBF with ketamine administration. Human studies display an Oxford 2b, GRADE C, level of evidence to support an increase in global CBF and rCBF with ketamine administration in both healthy volunteers and elective surgical patients without neurological illness.

Background: In 2014, the Montreal Neurological Hospital reorganized its stroke unit, grouping together all strokes serviced by Neurology/Neurosurgery to ensure continuity of care. This unprecedented change created a new interdisciplinary /interdepartmental team which required a new form of communication to facilitate information dissemination and patient care in a timely manner. Method: Unlike typical rounds, the purpose of the daily huddle is to briefly set the patients’ goals of the day. The huddles are attended by all interdisciplinary team members, lasting approximately 30 minutes. The Assistant Nurse Manager leads the huddle in the morning in the nursing station to discuss the main issues. The huddle outcomes were assessed by: Length of stay (LOS), turnaround time to implement discharge, patient/family and team satisfaction. Results: Length of stay decreased by 4 days, delay to application to disposition was <24 hours. Interdepartmental team stated satisfaction in sharing their expertise in their different domains. Concerns were expressed if the huddle LOS exceeded 30 minutes. Eighty (80%) percent of patients/ families experienced satisfaction that information provided was given in a caring/timely manner. Conclusion: Daily huddles improved LOS and team learning was enhanced. However, huddles need to be more concise.

Background: Most individuals recover from a concussion within 7-10 days. However recovery may be very prolonged. Individuals who do not recover within the usual time are said to have postconcussion syndrome (PCS). The objective of this study was to examine the demography and predictors of PCS. Methods: This was a retrospective cohort study of 284 consecutive concussed patients 221 of whom had PCS on the basis of at least three symptoms persisting at least 1 month. A uniform, internationally accepted definition of concussion was used. Results: The 221 cases showed considerable heterogeneity in clinical features of PCS. They averaged 3.3 concussions with a range of 0 to 12+ concussions, and 62.4% occurred during sports and recreation. The median duration of PCS was 7 months at the time of examination, with 11.8% lasting more than 2 years. Surprisingly, 23.1% with PCS had only 1 concussion. The average age was 27 years (range 10-74). The average number of persistent symptoms was 8.1. 26.2% had a previous psychiatric condition, ADD/ADHD, a learning disability, or previous migraine headaches. The prevalence of arachnoid cysts and Chiari malformation in PCS exceeded the general population. Conclusions: In most of our cases, PCS was disabling, and lasted for months or years.

Background: Acute stroke care pathways are increasingly implemented to improve integration of best-practices, but evidence for impact on functional outcomes is not strong. Elements missing from care-pathways are those directly targeting improvement in function: sit-to-stand and time spent walking. The Stroke Team uses care-pathways to track functional capacity, what the patient can and cannot do, but performance on these key outcomes is difficult to track as the patient is observed by multiple people throughout the day. The purpose of this study is to demonstrate the feasibility and added-value of real-time tracking of patients’ mobility. Methods: A chart review was carried out to identify the extent to which functional capacity and performance is tracked routinely by the Stroke Team. Ethical approval was gained for routine use of accelerometers to be affixed to the unaffected thigh. Results: Swallowing, bladder control, toileting, and feeding were consistently tracked for ~90% of patients. Bed-mobility and capacity to transfer rarely tracked (<12%). Capacity for walking and sit-to-stand was noted but never frequency (performance). Conclusion: Our proof-of concept study will test 30 patients over the next 2 months and link real time performance on transitions and walking to stroke severity and outcome.

Background: Corpus callosotomy is a palliative surgical procedure involving partial or complete disconnection of the corpus callosum. It has been shown to improve outcomes of seizure control with in six months of the procedure. Here, we discuss a challenging case of intractable generalized epilepsy with a delayed response to corpus callosotomy. Methods: This report describes a 23 year old female with onset of seizures since age 16. Patient was followed over 7 year period for evolution of her seizures and treatment. Results: Patient experienced three different types of seizures including atypical absences, drop attacks and grand mal seizures. The most disabling type of seizures were the drop attacks associated with injuries. MRI showed bilateral subependymal heterotopia. Multiple EEG telemetry studies showed generalized spike waves without clear lateralization or focalization. Patient failed seven different antiepileptic medications, ketogenic diet and vagal nerve stimulation. Treatment with anterior corpus callosotomy started to show improvements at 18-24 months after the procedure with less severe drop attacks. Conclusions: Corpus callosotomy usually works few months after surgery. This is a very atypical case in whom callosotomy had a delayed response. This is rarely reported and we do not have a clear explanation. Delayed re-organization of the pathways associated with the seizure initiation may be a potential explanation.

Temporal lobe epilepsy (TLE) is the most common type of epilepsy in adults. The literature in this field supports the notion than many patients become candidates for surgery and little is known about the group of patients who do not require surgical treatment. This is a retrospective cohort study that included all patients with TLE assessed and followed by the Saskatchewan Epilepsy Program since 2007. Mild course was defined as patients not having seizures, using or not AEDs at last follow up. Severe course of TLE was considered in patients with continuous seizures and patients who had epilepsy surgery. Descriptive statistics were used. OR and CI were calculated. One hundred and fifty nine patients were included. Age of patients at last follow up was 46.04 + 14.4 (range 19-88) years. Mean follow up of patients was 43.46+ 22.6 (6 to 84) months. Fourth six patients (29%) were seizure-free with AEDS (mild course TLE) and 113 (61%) had severe course of TLE. Patients with mild course of TLE were older (p 0.002), with a late onset of epilepsy (p< 0.001) and their epilepsy evolution was shorter (p<0.001). Our study shows that not all the patients with TLE require surgery and that a fair percentage of patients can be controlled with medication.

Introduction: The mainstay of diagnosis in Idiopathic Epilepsies (IE) is the electroencephalogram (EEG). The characteristic EEG of each syndrome is an electrographic endophenotype of the larger clinical phenotype of each and more directly associated with potential gene defects than the full phenotype. Endophenotypes represents primary abnormalities elicited by the gene defect, which, in some patients, blossom into full seizures. Revealing the percentage of abnormal EEGs in asymptomatic relative of patients with IE may help to describe the mode of inheritance that would help the ongoing genetics studies to discover the pathologic gene defect. Method: This is a prospective cohort study to identify the percentage of abnormal EEG in asymptomatic first-degree relatives of patients with IE Results: 20 out of 141 EEGs (14%) of first-degree relatives were abnormal. The abnormalities included generalized polyspikes and waves , generalized 3-Hz spike and waves or centro-temporal spikes in 50% of the abnormal EEGs. 50% of the abnormalities were nonspecific. Conclusion: These results may indicate that the EEG endophenotypes in IEs do not follow a Mendelian pattern of inheritance. Nevertheless, the EEG endophenotype is relatively common and thus genetically simpler than the full epilepsy, which will aid in gene identification

Objective: “Epilepsy in the elderly (EE)” is considered a homogeneous, benign syndrome in patients aged > 60 years, with cerebrovascular disease as the most frequent etiology. We challenge this concept by comparing EE and middle-aged adults with epilepsy (MAE). Methods: We compared: 1) seizure dynamics, 2) MRI lesions, 3) EEG findings and 4) treatment course in EE and MAE at the Halifax First Seizure Clinic. Results: 48 EE patients aged > 60.2 years (median 66.9 years). 31 MAE patients aged 50.2 – 59.6 years (median 55.1 years). Seizure dynamics in EE/MAE included first seizure in 50/54.8%, new onset epilepsy (new seizures within 12 months) in 43.8/35.5%, newly diagnosed epilepsy (seizures for >> 12 months) in 9.6/6.3%. First seizure evolved into new onset epilepsy in 12.5/3.2%. MRI in EE/MAE was normal in 22.5/27.6% or showed microangiopathy (25/38.5%), atrophy (10/15.4%), tumors (7.5/11.5%), vascular malformations (7.5/3.8%), hippocampal pathologies (0/3.8%), infarcts (12.5/0%). EEG in EE/MAE was normal in 64.4/65.5% or showed diffuse (6.6/3.5%) or focal slowing (8.8/7%), generalized (4.3/13.7%) or focal (15.4/10.4%) epileptiform activity. At 12 months, 87% of EE and 93.8% of MAE were seizure-free. Conclusions: EE and MAE show similar heterogeneity. We propose an etiology-driven classification of epilepsy syndromes in the aging brain.

Background: Temporal Lobe Epilepsy is associated with bilateral gray (GM) and white matter (WM) loss. After surgical treatment progressive bilateral temporal and extra-temporal WM change occur, however, less is known regarding post-operative GM change. We set out to measure contralateral hippocampal volume (CHV) following TLS. Methods: 1.5T-3D-1mm-isotropic-MPRAGE scans in 26 TLE patients and 3 controls in two groups: longitudinal (n=10)(imaged POD1,2,3,6,60,120 and >360d) and single post-operative scan (n=16). Manual volumetry protocols. Results: We find significant CHV atrophy at delayed scan relative to baseline (mean atrophy 26.8%). In the longitudinal group there is significant and progressive atrophy from baseline to POD4-8 (72.6+/-6.5%), POD60-360 (69.7+/-12.3%) and >360 (58.5+/-10.6%). No significant atrophy in either the control group HV or contralateral CV over time. No significant difference in mean HV at the most delayed exam for surgery type (p=0.13) or side (p=0.24). Conclusions: We find a statistically significant CHV atrophy following surgery which is progressive over time. Our longitudinal within-subject design describes the time course and extent more fully than previous work. Caudate analysis indicates that early CHV atrophy is not due to global atrophy following brain surgery but rather may be due to deafferentation and deefferentation. Finally, we find no significant difference in atrophy when analyzed by surgical approach or surgical side.

Background: In Canada, marijuana is legal for those with seizures. We determined the prevalence of marijuana use in intractable epilepsy patients and assessed the perceived effects. Methods: Information about marijuana use was collected over 12 months from consecutive adult patients admitted to an 8 bed Epilepsy Monitoring Unit using a 27 item self-administered questionnaire. Patients unable to understand and give consent for participation were excluded. Results: 215 of 310 patients median age 36 (interquartile range 27-49) years, 57% female had proven epilepsy. Median duration of seizures was 12 years (interquartile range 5-24) occurring daily or weekly in 47%. 37% of patients used marijuana over the previous year, 85% by smoking and 56% on a daily basis. Mean dose was 1 gm/day. Another 19% had used it previously. Use of cigarettes was 27%, alcohol, 40% and street drugs 2%. Seizure improvement was perceived by 91%, decreased stress by 99%, improved sleep by 98% and reduction in antiepileptic drug side effects by 88%. Minor adverse effects of marijuana occurred in 13% including seizures worse in 3%. Conclusions: Patients investigated for intractable epilepsy use marijuana more than the general population and perceive improved seizure control, lower stress, better sleep and reduced side effects from antiseizure drugs.

Background: Caspr2 is a transmembrane protein facilitating intercellular communication. It is found primarily in the central nervous system, specifically cerebellum and hippocampus. Anti-Caspr2 antibodies, more commonly seen in men (M/F: 4), also bind voltage-gated potassium channels. The antibodies are associated with limbic encephalitis, seizures, Morvan’s syndrome, peripheral nerve hyperexcitability, and cerebellar ataxia. Malignancy exists in 20% of cases. Methods: Case report and review of literature. Results: A 71-year-old man presented with subacute onset refractory seizures failing several anti-convulsants, emotional lability, and rapid decline in memory and executive function. EEG showed an electrographic seizure over the left hemisphere. MRI brain demonstrated mild diffuse cerebral atrophy, chronic ischemic changes, and mild diffusion restriction in the medial frontal lobes. Cerebrospinal fluid was normal. Serum Antithyroid peroxidase and antithyroglobulin antibodies were negative. TSH was slightly elevated and eltroxin didn’t help. Anti-Caspr2 antibodies were highly positive. EMG ruled out neuromyotonia. Body CT and PET scans indicated no malignancy. Treatment with IVIG stopped the seizures and cognition dramatically improved. Conclusions: Recognizing anti-Caspr2 antibody-associated encephalitis in elderly males with new onset refractory epilepsy and rapid cognitive decline is important for timely initiation of immunomodulation to avoid permanent deficits. Rapid executive dysfunction was unique in this case.

Background: The goal of our project is to assess the feasibility of replacing the invasive Wada test considered as the gold standard with non-invasive fMRI test for assessment of language dominance preoperatively. Methods: fMRI test with three language paradigm tasks (verb generation, sentence completion and naming) were conducted on our cohort of patients. fMRI laterality indices (LI) were then defined as a ratio (L-R)/(L+R) between the number of activated voxels in the left and right ROIs for Anterior Language Area (ALA) and Posterior Language Area (PLA). fMRI results were divided into the right (LI < -0.2), left (LI > 0.2) or bilateral (-0.2 < LI <0.2) hemispheric language dominance and compared to the results of the Wada test. Results: 28 patients were studied. The concordance rate between Wada and fMRI tests for the ALA and PLA was 68.2% and 52.2% for sentence completion; 56% and 52% for verb generation and 25% and 35% for naming paradigm, respectively. Conclusions: Sentence completion and verb generation fMRI paradigms showed higher concordance with Wada test than naming paradigm. The higher discordance between the Wada test and fMRI was related to bilateral results suggestive of less stringent thresholds used for either test.

Background: Analysis of infraslow EEG activity (ISA) has shown potential in the evaluation of patients with epilepsy and in differentiating between focal and generalized epilepsies. The purpose of this report is to present a girl with Sturge-Weber Syndrome (SWS) who was identified to have infraslow status epilepticus (ISSE), which successfully resolved after Midazolam administration Methods: The continuous EEG recording of a 5-yr-old girl with known Structural Epilepsy due to Sturge-Weber is presented. The patient presented to the ED with acute confusion, eye deviation and right hemiparesis similar to two previous admissions. ltm-eeg showed diffuse slowing. DWI obtained within 72-hr showed no ischemic changes, analysis of the EEG nfraslow (ISA) activity was undertaken using LFF 0.01 AND HFF of 0.1 Hz respectively. Results: Continuous subclinical unilateral rhythmic ictal ISA was identified. This was only evident in the left hemisphere which correlated with the structural changes due to SWS. A trial of continuous IV midazolam resulted in immediate resolution of the contralateral hemiparesis and encephalopathy. Conclusion: Continuous prolonged rhythmic ictal Infraslow activity (ISA) can cause super-refractory subclinical focal status epilepticus. This has not previously reported, and we propose this should be called Infraslow status epilepticus (ISSE). ISA analysis should be performed in all patients with unexplained subclinical status epilepticus.

Background: Leptomeningeal Carcinomatosis (LMC) is defined as a diffuse or multifocal malignant infiltration of the Pia matter and arachnoid membrane. It is clinically diagnosed in 5-10% of all cancer patients. The most commonly reported cancers associated with LMC are breast, lung and hematological malignancies. Patients with LMC commonly present with multifocal neurological symptoms. Symptoms are related to increase intracranial pressure, hemispheric dysfunction, cranial neuropathies and spinal roots dysfunction. We report a case of LMC secondary to Gastroesophageal junction cancer present initially with Cauda equine syndrome. Methods: A 51 year old male patient with adenocarcinoma of Gastroesophageal junction who underwent surgical resection, chemotherapy and radiation therapy. Nine month after diagnosis he presented with left leg pain, mild weakness and saddle area numbness. Initial radiological examination were unremarkable. Subsequently he had worsening of his leg weakness, fecal incontinence and urine retention. Two days later he developed rapidly progressive cranial neuropathies including facial diplegia, sensorineural hearing loss, dysarthria and dysphagia. Results: MRI with and without contrast showed diffuse enhancement of leptomeninges surrounding the brain, spinal cord and Cauda equine extending to the nerve roots. Cerebrospinal fluid cytology was positive for malignant cells. Conclusion: In cancer patient with Cauda equina syndrome and absence of structural lesion on imaging, LMC should be considered.

Background: IVLBCL is potentially treatable but difficult to diagnose. Methods: A case of progressive leg weakness and sphincter dysfunction diagnosed only at autopsy. Results: A 72 year old female presented with three weeks of increasing leg weakness and three days of urine and stool incontinence. Electrophysiological testing showed reduced tibial and peroneal CMAPs, preserved sural SNAPs, and bilateral gastrocnemius fibrillations, consistent with a pre-ganglionic lesion. Lumbosacral MRI with gadolinium showed no abnormalities. CSF was examined three times (protein 1.18-1.25, glucose 2.6-3.1, normal cell count and cytology). Whole-body FDG PET scanning showed hypermetabolic foci at the tongue base and in the mediastinum, but biopsy of both revealed no abnormality. Leg weakness progressed over three months and spread to the arms despite a course of IVIg. Four months later she died of cardiorespiratory arrest. Autopsy revealed the presence of large atypical B-cells within the lumen of small and medium sized vessels in numerous organs. There was evidence of anterior spinal artery obstruction with lymphocytes and anterior horn infarction in the lumbar cord. Conclusions: Although the literature reports that IVLBCL responds well to chemotherapy, this patient illustrates the difficulty of ante-mortem diagnosis. Nerve root biopsy may be warranted in such patients.

Background: Postural tachycardia syndrome (POTS) is defined as heart rate (HR) increments ≥30 bpm on head-up tilt (HUT) associate with orthostatic symptoms (lightheadedness, dizziness, palpitations, etc.). A large proportion of asymptomatic young adults naturally express excessive HR increments on HUT, which has raised questions regarding whether revision to the diagnostic criteria for POTS in younger patient populations is needed. In addition, poor adaptation to environmental stressors may contribute to the disability experienced by POTS patients. Objectives: 1) determine whether asymptomatic patients were predisposed to developing constitutional symptoms that could result in the full syndrome of POTS, and 2) determine whether these symptoms correlated to postural HR or orthostatic symptoms. Methods: HR response to HUT and orthostatic and constitutional symptoms in 30 asymptomatic POTS patients were evaluated after 1 year. Results: HR increment at follow-up demonstrated no correlation with general fatigue (r = 0.006), body vigilance (r = 0.195), physical (r = -0.087) and mental (r = -0.137) health, or orthostatic scores (r = 0.04). Orthostatic scores significantly correlated with general fatigue (r = 0.374) and body vigilance (r = 0.392) (p<0.05). Conclusion: These data support that the majority of young individuals express benign orthostatic tachycardia and further argue for re-evaluation of the HR criteria for diagnosing POTS in younger populations.

Background: Valsalva maneuver (VM) is a simple and non-invasive technique extensively utilized clinically to detect dysautonomia. VM provides detailed information of baroreflex sensitivity (BRS) which is an important cardiovascular and autonomic marker. However, the current approach for calculating its adrenergic component (BRSa1) is moderately reliable and fails to evaluate atypical VM patterns. Methods: We analyzed typical and atypical VM patterns of 89 young, healthy individuals (30 ±13 years) with the aim of improving BRSa evaluation. Objectives: 1) To determine a new BRSa calculation (BRSa2) applicable to different VM patterns; 2) correlate BRSa2 to BRSa1; 3) compare the internal consistency (ICC) between BRSa1 and BRSa2. Results: The BRSa2 calculation is a complex hemodynamic and time assessment equivalent to the slope in vagal BRS. In contrast to BRSa1, BRSa2 operates with hemodynamic indices easily detectable in any VM pattern. In atypical VM patterns, BRSa2 correlated with BRSa1: “flat-top responses” (r = 0.774, p < 0.01); rapid hemodynamic recovery (r = 0.461, p < 0.05). Most importantly, BRSa2 was more reliable than BRSa1 (ICC= 0.759 versus 0.469). Conclusion: BRSa2 is more reliable and allows atypical responses to VM to be analyzed, which clinically, could help differentiate natural physiological variances and mild adrenergic dysfunction.

We present a 25 year old female veterinarian technician presenting with rapidly progressive quadriplegia in less then 12 hours. Her symptoms occurred at work with initially bilateral hand weakness followed by arm and leg weakness. Before the end of the day she was on a ventilator in the ICU. MRI showed a hyperintense longitudinal T2 signal extending from the cervical medullary junction to T1. Extensive cervical spinal cord edema with cord expansion was noted. CSF showed normal protein and cell count with no oligoclonal banding. A post-infectious inflammatory process causing transverse myelitis was presumed and she was given IVIG, steroids, and plasmapheresis with no improvement. A serum ELISA test for IgG to Toxocara was reactive at titre of 1:800 at 3 weeks after her initial presentation. Her serum IgE levels was elevated at 169 x 10x3 U/L (Normal <87 x 10x3 U/L). At 4 weeks, she was commenced on albendazole at 800 mg per day for two months. A repeat serum ELISA test at 6 weeks and 2 weeks into her treatment with albendazole showed a declining titre of 1:200 consistent with recent Toxocara infection. At 10 weeks, her ELISA test was non-reactive. Unfortunately she did not respond to albendazole treatment and she shows minimal improvement now 1.5 years later.

Idiopathic CD4 lymphopenia is a rare immune disorder that renders patients vulnerable to unusual infections. A 25-year-old female had been diagnosed with idiopathic CD4 lymphopenia (CD4 count of 50/uL) at age 14 after pulmonary infection with atypical mycobacterium, but had since been asymptomatic on Trimethoprim/Sulfamethoxazole prophylaxis. She presented now with 5 weeks of headache, vomiting, diplopia, and vertigo. This had been diagnosed as benign positional vertigo. However, neuro-ophthalmologic exam revealed gaze-evoked nystagmus, impaired smooth pursuit, a left hypertropic skew deviation, left sensorineural hearing loss, gait ataxia and left limb dysmetria. MRI brain showed a 15 mm extra-axial enhancing mass at the left cerebellopontine angle, and chest CT showed pulmonary lesions. CSF Cryptococcal antigen was highly positive (> 1:1024) and CSF culture grew Cryptococcus neoformans variety grubii. She was treated with amphotericin B 175 mg daily and 5-Flucytosine 1000 mg QID for 4 weeks, followed by Fluconazole 400 mg daily, and made an excellent recovery. Cryptococcal infection usually presents as a meningitis, but can occasionally present as a mass lesion. Cryptococcal infection is one of the most common complications in idiopathic CD4 lymphopenia. This case illustrates the importance of neurologic signs in correctly localizing the lesion and the need for high suspicion of serious pathology in patients with rare immune disorders.