bluuebunny/crossref_metadata_2025 huggingface dataset:
Endovascular Therapy of Hypoglossal Canal AVFs Presenting with Orbital Symptoms
Authors: Miropolsky Vladislav , da Costa Leodante B. , Marotta Thomas R. , Spears Julian
Published: December 2014

Objective:

Dural arteriovenous fistulae (DAVF) of the hypoglossal canal region are rare lesions. We describe three cases of DAVF of the hypoglossal canal presenting with ocular symptoms and discuss the endovascular management options.

Methods:

Three consecutive patients with DAVF of the hypoglossal canal region presented with proptosis, chemosis and disturbances of extra-ocular mobility. Each patient was treated using a different endovascular approach, based on variations of the vascular access.

Results:

The cases and treatments are reviewed, with a literature review on the subject. Endovascular treatment, transvenous or trans-arterial was curative in all cases.

Conclusion:

DAVF of the hypoglossal canal region can present with ocular manifestations very similar to DAVF of the cavernous sinus or carotid-cavernous fistulas. Endovascular treatment is usually feasible and effective, but an understanding of the vascular anatomy and pathophysiology of the disease are of utmost importance when planning the approach.
Pediatric Traumatic Dural Arteriovenous Fistula
Authors: Vassilyadi Michael , Mehrotra Naveen , Shamji Mohammed F. , Michaud Jean
Published: December 2014

Background:

Dural arteriovenous fistulas are uncommon lesions in children, with traumatic lesions suspected to arise from incomplete arterial injury in proximity to a vein or draining sinus. Management of symptomatic acquired lesions requires evaluation of patient presentation, neurological status, and pathoanatomic configuration, with special consideration required for surgery secondary to failed endovascular technique.

Case Report:

A 12-year-old male sustained a bicycle fall causing a right temporo-parietal skull fracture associated with non-surgical right epidural hematoma and left contre-coup parietal contusion. Six-weeks later, he complained of a right temporal bruit with subsequent cerebral angiography demonstrating a dural-based fistula between the right middle meningeal artery and a dural vein draining into the sigmoid sinus.

Intervention:

Endovascular treatment of this lesion with glue embolization and coiling was unsuccessful, with angiographic illustration of previously unobserved collateral vessels and coils occupying the sigmoid sinus. A right temporo-parietal craniectomy was required to excise the dural-based fistula, followed by dural defect repair with bovine pericardium and subsequent cranioplasty. Six years later the patient remains neurologically intact with no headaches or bruit.

Conclusions:

Dural arteriovenous fistula can uncommonly occur following traumatic injury in children. Partial injury to the middle mengineal artery may have established arterial communication with the draining vein that became ectatic and tortuous under high pressure. Failure of primary endovascular treatment may complicate secondary surgical intervention.
Skull Base Chondrosarcoma Presenting with Hemorrhage
Authors: Gallia Gary L. , Binder Zev A. , Schwarz Jacob , Moriarity John L. , Weingart Jon D.
Published: December 2014

A 34-year-old white man with a six month history of a feeling of déjà vu presented with an acute onset of headache and blurred vision associated with nausea and vomiting. Neurological examination was significant for a right third cranial nerve palsy. Computerized tomography (CT) demonstrated a 2.0 cm rim calcified right paracavernous mass with intratumoral hemorrhage and an adjacent 2.6 cm by 2.0 cm right temporal lobe hematoma. Intraventricular hemorrhage (IVH) was present in both lateral ventricles, right more than left (Figure 1). Diagnostic cerebral angiography demonstrated no evidence of aneurysm. Brain magnetic resonance (MR) imaging revealed a 2 cm mass in the right paracavernous region extending into the suprasellar region with heterogeneous signal intensity on T1- and T2-weighted images and heterogeneous enhancement after gadolinium administration. Adjacent to the lesion was a right temporal lobe hematoma; intraventricular hemorrhage was also observed in the right lateral ventricle (Figure 2). The patient underwent a right pterional craniotomy for gross total resection of the tumour. Histopathological examination of the tumour demonstrated a low grade hyaline chondrosarcoma. The patient subsequently underwent proton beam radiotherapy and is currently 82 months out from surgery with no evidence of recurrence.
Brain Blood Flow in the Neurological Determination of Death: Canadian Expert Report
Authors: Shemie Sam D. , Lee Donald , Sharpe Michael , Tampieri Donatella , Young Bryan
Published: December 2014

The neurological determination of death (NDD, brain death) is principally a clinical evaluation. However, ancillary testing is required when there are factors confounding the clinical determination or when it is impossible to complete the minimum clinical criteria. At the time of the 2003 Canadian Forum clarifying the criteria for brain death, 4-vessel cerebral angiography or radionuclide angiography were the recommended tests and the electroencephalogram was no longer supported. At the request of practitioners in the field, the Canadian Council for Donation and Transplantation sponsored the assembly of neuroradiology and neurocritical care experts to make further recommendations regarding the use of ancillary testing. At minimum, patients referred for ancillary testing should be in a deep unresponsive coma with an established etiology, in the absence of reversible conditions accounting for the unresponsiveness and the clinical examination should be performed to the fullest extent possible. For newborns, children and adults, demonstration of the absence of brain blood flow by following recommended imaging techniques fulfill the criteria for ancillary testing: 1. radionuclide angiography or CT angiography 2. traditional 4-vessel angiography 3. Magnetic resonance angiography or Xenon CT. In the absence of neuroimaging, an established cardiac arrest, as defined by the permanent loss of circulation, fulfills the ancillary criteria for the absence of brain blood flow. Acknowledging the existing limitations in this field, further research validating current or evolving techniques of brain blood flow imaging are recommended.
CTG Expansion & Haplotype Analysis in DM1 Gene in Healthy Iranian Population
Authors: Shojasaffar Bahareh , Moradin Neda , Kahrizi Kimia , Cobo Ana Maria , Najmabadi Hossein
Published: December 2014

Myotonic dystrophy type 1 (DM1) is due to an unstable expansion of CTG repeat in the DMPK gene (19q13.3). The CTG repeat is highly polymorphic (5 to 37) in healthy individuals. According to the hypothesis that expanded (CTG)n alleles originated from larger normal alleles, there may exist a correlation between the prevalence of DM1 and the frequency of large size normal alleles. Strong linkage disequilibrium between different length alleles and the three biallelic markers, Alu, Hinf1 and Taq1, has been reported.

Objective:

To determine the distribution of normal alleles, the frequency of larger normal alleles and analysis of the three biallelic markers, in healthy Iranian controls.

Material and Methods:

Polymerase chain reaction (PCR) was conducted on two hundred unrelated healthy individuals from different ethnic groups living in Iran to determine the size of the alleles. Markers were analyzed by PCR/RFLP on 174 chromosomes from other control healthy individuals.

Results:

Our data reveals that 23.7% of alleles had 5 CTG repeats and 7.2% of alleles had >18 CTG repeats. The analysis of haplotypes revealed that 75% of CTG5 and 80% of CTG>18 had the (+++) haplotype.

Conclusion:

The frequency of alleles with CTG>18 in Iran is similar to that of Western Europe and Japan.
Deducing the Bioactive Face of Hydantoin Anticonvulsant Drugs Using NMR Spectroscopy
Authors: Tiedje Kathryn E , Weaver Donald F
Published: December 2014

Background:

The general purpose of this study was to deduce the geometry of the bioactive face (pharmacophore) for the hydantoin class of anticonvulsants.

Methods:

Six hydantoin analogs, selected as probes of hydantoin structure, were synthesized. Nuclear magnetic resonance spectroscopy and molecular modelling calculations were used to determine the geometric relationship between the aromatic group and the amide group in the hydantoin pharmacophore.

Results:

In accord with both theoretical and experimental results, the biologically inactive hydantoin analogs containing a benzyl substituent existed in a folded conformation with the benzene flopped over the hydantoin ring. Conversely the biologically active hydantoins had a phenyl ring extended away from the hydantoin ring.

Conclusions:

The bioactive face for hydantoins consists of a N(H)-C(=O)-X-phenyl molecular fragment, where X is a carbon or nitrogen atom and where the distance between the centre of the amide bond and the centroid of the phenyl ring is 4.3 Å.
Sturge-Weber Syndrome. Study of 55 Patients
Authors: Pascual-Castroviejo Ignacio , Pascual-Pascual Samuel-Ignacio , Velazquez-Fragua Ramón , Viaño Juán
Published: December 2014

Purpose:

To review the clinical and neuroimaging features of a large series of patients with Sturge-Weber syndrome (SWS) seen over a 40-year period.

Methods:

Fifty-five patients with SWS (30 males and 25 females), were studied between 1965 and 2004. Results of neurological and ophthalmological examinations, electroencephalographic, and neuroimaging studies were reviewed. All patients were seen by one of the authors (I. P-C).

Results:

Epilepsy, hemiparesis, mental retardation and ocular problems were the most frequent and severe features of patients with Sturge-Weber syndrome in this series. The facial nevus flammeus was unilateral in 35 (63.5%) patients, bilateral in 17 (31%) and absent in 3 (5.5%) of the patients with leptomeningeal angiomas. Seven (41%) of the 17 patients with bilateral nevus flammeus had unilateral leptomeningeal angiomas. Seizures occurred in 47 patients (85.5%). Complete seizure control was obtained in 20 patients (42.5%), but in 2 of these 20 patients seizures were controlled only after lobectomy. All patients with unilateral or bilateral upper eyelid nevus flammeus had ipsilateral, unilateral or bilateral choroid-retinal angiomas. Only 20 (36%) of the 55 patients had low-normal or borderline intelligence (IQs<70). No relationship was observed between the size of the facial nevus flammeus and the severity of the brain lesion.

Conclusions:

Epilepsy, hemiparesis, mental retardation and ocular problems were the most frequent and severe features of patients with Sturge-Weber syndrome in this series. Cerebral lesions followed a progressive course during early childhood, but not later. Early surgical treatment controlled the seizures but other neurological problems such as hemiparesis and intellectual deficits showed a less satisfactory response. Early onset of seizures and poor response to medical treatment, bilateral cerebral involvement and unilateral severe lesions were indicative of a poor prognosis. Limited intelligence and social skills, poor aesthetic appearance and seizures complicated the integration of SWS patients. These features must be addressed in order for the patients improve social interactions, obtain gainful employment and achieve a better quality of life.
Access to Spine Care: A Tale of Two Cities
Authors: Hurlbert R. John , Mobbs Ralph , Teo Charles
Published: December 2014

ABSTRACT:

Introduction:

As governments struggle with increasing demand for accountability within the Canadian Health Care System and set wait-time standards, it is important to objectify data to allow a true understanding of present limitations and to facilitate comparisons to other systems. The purpose of this study was to compare wait list times for a cohort of patients requiring spinal surgery in Calgary, Alberta to a similar cohort in Sydney, Australia.

Methods:

From January 1 until June 30, 2006 all outpatients admitted for spinal surgery to the Foothills Hospital were identified by the surgeons’ office. Two time periods were quantified from their charts: (1) time from referral to surgical consultation; and (2) time from surgical consultation to operative intervention. From July 1 until December 31, 2006 patients were similarly identified through Neurosurgical offices at the Prince of Wales Public and Private Hospitals in Sydney, Australia.

Results:

Four hundred ninety-one surgical patients were captured during the six month period in Calgary and 155 patients during the subsequent six months in Sydney. The majority of patients in Sydney were treated in the Private Health Care system. Public patients in Sydney have access to a surgical consultant twice as fast as public patients in Calgary while private patients have access ten times faster. Access to operating room time within the public system is a rate limiting step in both countries. However, Sydney private patients receive their surgery four times faster than Calgary patients.

Conclusions:

Compared to Calgary, access to specialized spine care in Sydney appears more efficient not only in the Private but also the Public Health Care System. Part of this efficiency may arise from offloading from the public into the private system. Solutions proposed to reduce wait list times should consider benefits of a Private Health Care System.
A Novel Mutation in a Large French-Canadian Family with LGMD1B
Authors: Chrestian Nicolas , Valdmanis Paul N. , Echahidi Najmeddine , Brunet Denis , Bouchard Jean-Pierre , Gould Peter , Rouleau Guy A. , Champagne Jean , Dupré Nicolas
Published: December 2014

ABSTRACT

Background:

Limb girdle muscular dystrophy type 1B is an autosomal dominant disease characterized by late onset proximal muscle involvement associated with cardiac complications such as atrioventricular conduction blocks, dilated cardiomyopathy, and sudden death.

Objective:

Define the full phenotypic spectrum of a new mutation in the LMNA gene causing limb girdle muscular dystrophy type 1B.

Methods:

We identified a large French Canadian family with the LGMD 1B phenotype and a cardiac conduction disease phenotype that carried a new mutation in the LMNA gene and sought to define its full phenotypic spectrum by performing complete neurological and cardiac evaluations, muscle biopsy, RNA and DNA studies.

Results:

The proband and 12 living at risk relatives were tested. In total, we identified seven carriers of a new (IVS9-3C>G) LMNA gene mutation. Of the three symptomatic patients, all had cardiac involvement, but only two presented proximal limb weakness. The one available muscle biopsy demonstrated a normally expressed lamin A/C protein, localized at the nuclear envelope. RNA study revealed a loss of exon 10 transcription caused by the IVS9-3C to G splicing mutation.

Conclusions:

We have identified a new mutations in the LMNA gene in a French-Canadian family. This diagnosis has important implications for affected patients and their siblings since they may eventually require pacemaker implantation.
Restless Legs Syndrome in Patients with Chronic Obstructive Pulmonary Disease
Authors: Kaplan Yuksel , Inonu Handan , Yilmaz Ayse , Ocal Serpil
Published: December 2014

Objective:

To evaluate the prevalence of restless legs syndrome (RLS) in patients with chronic obstructive pulmonary disease (COPD) and the relationship between RLS and clinical/laboratory findings of COPD.

Methods:

One hundred and thirty-four COPD patients without secondary causes of RLS were included. Thirty-nine (29.1%) patients were diagnosed with RLS and classified as Group 1. The control group consisted of 65 age-matched COPD patients without RLS. Group 1 was divided into subgroups according to the Johns Hopkins Severity (JHS) scale. Patients with a score of 0, 1, or 2 were classified as JHS 0-2 and those with a score of 3 as JHS 3. Group 1 and the control group and subgroups were compared for clinical and laboratory characteristics.

Results:

We found that the duration of COPD was longer and that airway obstruction, hypercapnia, and hypoxia were more evident in patients with RLS than those without. Similar differences were also detected between JHS subgroups 3 (more severe) and 0-2. Polyneuropathy frequency was significantly higher in Group 1 compared to controls. However, Group 1 subgroups showed a similar frequency of polyneuropathy. In a multivariate analysis, hypercapnia made a significant independent contribution to both JHS 0-2 and JHS 3 patients when RLS severity was set as the dependent variable. Polyneuropathy and the duration of COPD were significant independent variables for patients in the JHS 3 subgroup. Polyneuropathy was the strongest predictor for the JHS 3 patients.

Conclusions:

We conclude that RLS is frequent in COPD, particularly in patients with severe hypoxemia/hypercapnia and in late stages of the disease.
Intravascular Lymphoma with Conus Medullaris Syndrome Followed by Encephalopathy
Authors: Savard Martin , Verreault Steve , Gould Peter V. , Bernier Vincent , Bouchard Jean-Pierre
Published: December 2014

Background:

Intravascular large cell lymphoma (ILCL) is a diagnostic challenge, with neurological, cutaneous and constitutional symptoms. The natural history is usually an evolution to a comatose state. As invasive procedures are usually required for diagnosis, recognizing the typical clinical pattern is critical since an effective treatment is available.

Method:

After an extensive literature review of the subject, we report a case of ILCL, analyzing clinical, laboratory, radiological and pathological data. We will also give a special attention to the clinical picture of a conus medullaris (CM) lesion with subsequent encephalopathy in the same patient.

Results:

We report here a 61-year-old woman with a paraplegia caused by a CM lesion, evolving about one year latter to encephalopathy and eventual coma, with the diagnosis of ILCL confirmed by autopsy. The present case is similar to eight other cases in literature who had CM lesion associated with ILCL, knowing that 80-90% of these patients will eventually evolve to encephalopathy without treatment. Conclusions: ILCL is a recognized but rare cause of coma. Diagnosing it is tremendously important since it is fatal if left untreated. We propose that this specific picture (conus medullaris lesion, eventually evolving to encephalopathy) is quite characteristic and will directly result in better outcome if recognized.
Sphenoid Sinus Mucocele - Rupture Causing Brainstem Inflammation and Stroke
Authors: Busche Kevin , Morrish Wiliam F.
Published: December 2014

Background:

Posterior rupture of a sphenoid sinus mucocele is a rare cause of brainstem injury.

Methods:

Case report.

Results:

A healthy young woman with a history of prior surgical excision of nasal polyps presented with a headache and evolving neurologic symptoms. The clinical presentation and imaging studies were consistent with a posterior rupture of a large sphenoid mucocele with subsequent inflammatory changes in the brainstem and evidence of brainstem stroke. Relatively rapid recovery coincided temporally with the resolution of local inflammation. Longer-term recovery continued over months, as expected following stroke.

Conclusions:

Posterior rupture of a sphenoid sinus mucocele may cause brainstem injury by multiple mechanisms.
Stigma of Epilepsy
Authors: Bandstra Nancy F. , Camfield Carol S. , Camfield Peter R.
Published: December 2014

Epilepsy directly affects 50 million people worldwide.Most can achieve excellent seizure control; however, people living with epilepsy continue to suffer from enacted or perceived stigma that is based on myths, misconceptions and misunderstandings that have persisted for thousands of years. This paper reviews the frequency and nature of stigma toward epilepsy. Significant negative attitudes prevail in the adolescent and adult public worldwide leading to loneliness and social avoidance both in school and in the workplace. People with epilepsy are often wrongly viewed as having mental health and antisocial issues and as being potentially violent toward others. Twenty-five percent of adults having epilepsy describe social stigma as a result of their epilepsy. They fear rejection and often feel shame or loneliness from this diagnosis. The psychosocial and social impact of epilepsy is significant. Yet few specific interventions have been demonstrated to alter this perception. The effect on public education is primarily short-term, while change over the long-term in attitudes and inaccurate beliefs have not presently been proven effective. School education programming demonstrates improved knowledge and attitude a month after a classroom intervention, but persisting change over a longer period of time has not been evaluated. In-depth adult psycho-educational programs for adults with epilepsy improves knowledge, coping skills and level of felt stigma. However these gains have not demonstrated persistence over time. Myths, misconceptions and misunderstandings about epilepsy continue and programs aimed at increasing knowledge and reducing negative public attitudes should be enhanced.
Severe Head Injury: Clinicians’ Awareness of the Literature
Authors: Jacka Michael J , Des Ordons Amanda Roze , Zygun David
Published: December 2014

Objectives:

1. To determine the awareness of the literature concerning therapeutic manoeuvres in severe closed head injury (CHI) among Canadian critical care clinicians and neurosurgeons, 2. To identify factors that affect utilization of these manoeuvres, and 3. To compare reported appropriateness and frequency of use with #1 and #2.

Methods:

The study design was a systematic scenario-based survey of all neurosurgeons and critical care physicians treating patients with severe CHI in Canada.

Results:

Fifty-nine of 99 neurosurgeons and 82 of 148 critical care physicians responded (57%). The majority of respondents were not able to identify the highest level of published evidence for most manoeuvres, except for the avoidance of corticosteroids (51%). The factor identified by most respondents as being most important in motivating use of any given manoeuvres was the level of published evidence (25%). Although reported appropriateness and frequency of use of most manoeuvres correlated well with each other, they did not correlate with awareness of evidence. In the case of corticosteroids, there was a strong correlation between non-use of steroids and awareness of evidence (R = - 0.30, p = 0.0003).

Conclusions:

Respondents to this survey of Canadian physicians treating patients with severe head injury reported published evidence as being the most significant factor affecting use of a therapy. However, most respondents did not correctly identify the highest published level of evidence for most therapies. This study has identified difficulty with research translation that may have clinical implications.
NRAMP1Polymorphism and Viral Factors in Sardinian Multiple Sclerosis Patients
Authors: Gazouli Maria , Sechi Leonardo , Paccagnini Daniela , Sotgiu Stefano , Arru Giannina , Nasioulas George , Vassilopoulos Dimitrios
Published: December 2014

Background:

Multiple sclerosis (MS) is believed to be an autoimmune disease occurring in genetically predisposed individuals after an appropriate environmental exposure such as viral infections. Recent studies suggest a significant association between MS and the functional 5’-(GT)n polymorphism in the promoter region of theNRAMP1gene. In the present study we aimed to evaluate the contribution of the allelic variation in theNRAMP1promoter to MS susceptibility and to study the role of viral infection in relation to specificNRAMP1genotypes, in a Sardinian cohort.

Methods:

Sixty MS patients and 66 healthy individuals were genotyped, and screened for the presence of Epstein-bar virus (EBV) and JC virus (JCV) sequences.

Results:

Consistent with previous autoimmune disease studies, allele 3 at the functional 5’(GT)n promoter region repeat polymorphism, was significantly overrepresented among MS patients when compared to controls (p=0.02). The EBV and JCV sequences were detected in 8/60 (13.33%) and in 4/60 (6.66%) of MS patients respectively and in 5/66 (7.57%) and in 0/66 of controls.

Conclusion:

The allelic variation in the NRAMP1 promoter may contribute to MS susceptibility in the Sardinian population. The viral sequences were not confined to a specific NRAMP1 genotype.
Hemicraniectomy for Massive Middle Cerebral Artery Infarction: A Review
Authors: Manawadu Dulka , Quateen Ahmed , Findlay J. Max
Published: December 2014

Hemicraniectomy and opening underlying dura mater permits the expansion of infarcted, swollen brain outwards, reversing dangerous intracranial pressure elevations and the risk of fatal transtentorial temporal lobe or diencephalic herniation. Recently published randomized controlled trials have proven this procedure a powerful life-saving measure in the setting of malignant middle cerebral artery infarction and allayed concerns that a reduction in mortality is accompanied by an unacceptable increase in patients suffering severe neurological impairments. Appropriate patients are relatively young, in the first five decades of life, suffering infarction of a majority of the middle cerebral artery (MCA) territory in either hemisphere, and decompression should be performed prior to progression to coma or two dilated, fixed pupils. Lethargy combined with midline shift and uncal herniation on neuroimaging is an appropriate trigger to consider and discuss surgical intervention. Families and, when possible, patients themselves, should be informed of the certainty of at least moderate to mild permanent deficits, and the possibility of worse. To be successful decompression must be extensive, targeting a bone flap measuring 14 cm from front to back, and extending 1 to 2 cms lateral to the midline sagittal suture to the floor of the middle cranial fossa at the level of the coronal suture. An augmentation duraplasty is mandatory.
Acute Stroke with Hyperdense Middle Cerebral Artery Sign Benefits from IV rtPA
Authors: Tartaglia M C , Di Legge S , Saposnik G , Jain V , Chan R , Bussière M , Hachinski V , Frank C , Hesser K , Pelz D
Published: December 2014

Objectives:

The current management of acute ischemic stroke is intravenous (IV) recombinant tissue plasminogen activator (rtPA). The presence of a hyperdense middle cerebral artery sign (HMCAS) on pre-treatment head computed tomogram (CT) is considered a poor prognostic sign. We compared the clinical outcome in IV rtPA-treated patients with and without a HMCAS.

Design:

Retrospective analysis of prospectively collected cases treated with IV rtPA within three hours. Inclusion criteria were the presence of: i) an anterior circulation stroke; ii) a pre-treatment CT available; iii) a pre-treatment National Institutes of Health (NIH) stroke scale (NIHSS) score; and iv) a modified Rankin Score (mRS) at three months.

Results:

One hundred and thirty patients were eligible for the analysis, 64 (49%) had a HMCAS. The HMCAS group had a trend toward a higher mean (±SD) pre-treatment NIHSS score compared to the non-HMCAS group (13.9±6 vs. 12.2±6; p=0.12). Accordingly, there were more patients with severe strokes (NIHSS>10) in the HMCAS group compared to the non-HMCAS one (48/64=75% vs. 35/66=53%; p=0.009). The mean (±SD) NIHSS score 24 hours after treatment was 10.6 (±8) in the HMCAS group and 8.3 (±7) in the non-HMCAS group (p=0.08). In a multiple logistic regression analysis, the only independent predictor of poor outcome (mRS 3-6) was pre-treatment NIHSS score (p<0.001).

Conclusion:

Patients with a HMCAS receiving IV rtPA did not fare worse at three months despite a greater proportion of patients with more severe strokes. Based on the current knowledge, IV rtPA remains a good treatment for patients with a HMCAS within three hours of symptom onset.
Prevalence of Multiple Sclerosis in Canada: A Systematic Review
Authors: Poppe Alexandre Y. , Wolfson Christina , Zhu Bin
Published: December 2014

Background:

Studies of the prevalence of multiple sclerosis (MS) in Canada have generally been isolated to specific regions. Given the importance of multiple sclerosis as a cause of disability in adults, a comprehensive review of Canadian MS prevalence examining current data, interregional variation, deficiencies in knowledge and frontiers for research is timely.

Methods:

A systematic review of all studies addressing the prevalence of MS in Canada or regions within Canada, published in English or French since 1985, was conducted. Studies were identified using MEDLINE, EMBASE and bibliographic review. Ten studies were evaluated for methodological rigour and a test of heterogeneity across studies was performed and a measure of consistency (I2) estimated.

Results:

Studies were generally of high quality. Nine were restricted to regions within Canada and one provided an estimated national prevalence based on self-reported cases. All reported a high prevalence (>50 per 100 000). Latitude and longitude gradients were not striking while assessment of heterogeneity confirmed that regional differences were unlikely to be the result of sampling variability.

Conclusions:

This review confirms Canada as a country of very high MS prevalence and it is the first study to demonstrate that variation in regional estimates represents true differences in prevalence within Canada. Avenues for future MS prevalence research, including adoption of a national MS registry, are proposed.
Progressive Supranuclear Palsy: A Review of Co-existing Neurodegeneration
Authors: Keith-Rokosh J , Ang L C
Published: December 2014

Objectives:

The neuropathological findings of 32 progressive supranuclear palsy (PSP) cases over a period of 17 years were reviewed.

Results:

Of the 26 cases with adequate clinical data, 20 patients either presented with cognitive dysfunction or developed a cognitive impairment subsequently during the course of the disease. Co-existing changes of argyrophilic grains and corticobasal degeneration (CBD) were found in 28% and 32% of the cases respectively. Alzheimer-related pathology was found in 69% of cases but only 18.75% of cases fulfilled the consortium to establish a registry for Alzheimer's disease (CERAD) criteria for either definite or probable Alzheimer's disease. Lewy bodies were noted in four cases (12.5%), all in the subcortical regions. Only seven cases of PSP showed no pathological evidence of other co-existing neurodegenerative diseases. The severity of the cerebrovascular pathology in this cohort was insufficient to explain any clinical symptomatology.

Conclusions:

As in previous studies, this study has demonstrated the frequent co-existence of pathological changes usually noted in other neurodegenerative diseases in PSP. Whether these coexisting pathological changes contribute to the cognitive impairment in PSP remains uncertain.
Reliability of the MMSE Administered In-Person and by Telehealth
Authors: McEachern Wendaline , Kirk Andrew , Morgan Debra G. , Crossley Margaret , Henry Carol
Published: December 2014

Background:

Recent advances in telehealth have improved access to health care for those in rural areas. It is important that examinations conducted via telehealth are comparable to in-person testing. A rural and remote memory clinic in Saskatoon provided an opportunity to compare scores on the Mini-Mental State Examination (MMSE) administered in-person and via telehealth.

Methods:

After an initial one day assessment in Saskatoon, patients were seen in follow-up at 6 and 12 weeks. Individual patients were randomly assigned to either in-person follow-up assessment in Saskatoon or telehealth assessment in their home community. Patients who initially received in-person assessments were seen by telehealth for their next follow-up visit and vice-versa. The same neurologist administered MMSEs at all visits. The first 71 patients with both 6 and 12 week follow-up assessments were included in this study. The scores of in-person and telehealth MMSE administrations were compared using the methods of Bland and Altman as well as a paired t-test.

Results:

MMSE scores did not differ significantly between telehealth (22.34 +/- 6.35) and in-person (22.70 +/- 6.51) assessments.

Conclusion:

Telehealth provides an acceptable means of assessing mental status of patients in remote areas.
In Situ Cranioplasty for Hyperostosing Meningiomas of the Cranial Vault
Authors: Bloch Orin , McDermott Michael W.
Published: December 2014

Objective:

Hyperostosis of the bone overlying meningiomas has been reported in up to 50% of cases. The skull becomes infiltrated by meningothelial tumor cells, necessitating removal of the hypertrophied bone to achieve a complete tumor resection. Unfortunately, aesthetic reconstruction of large bony defects can pose a significant challenge intra-operatively. Custom cranioplasty implants are very expensive and can only be fabricated after the bony defect exists, requiring a second surgery for implantation. Although numerous composite materials exist to repair the defects at the time of tumor resection, the challenge is to create an implant that fits appropriately without shifting and approximates the natural curvature of the skull. We have developed a technique for an “in situ cranioplasty” using a composite construct with strength in compression and tension.

Technique:

After the skull is reshaped by shaving down part of the hyperostotic bone, titanium mesh is molded to the surface of the skull and screwed into the surrounding normal bone. The bone flap is then removed by drilling a trough at the outer margin of the tumor-involved skull and removing a ring of normal surrounding bone. The central portion of tumor involved skull is then craniectomized. The mesh can be reapplied and the full thickness of the central bone can be reconstructed with polymethylmethacrylate, yielding a solid construct perfectly matched to the patient's natural head shape.

Conclusion:

This novel technique yields a sturdy, aesthetic, and cost-effective result which can be used to address any cranial vault defect at the time of tumor resection.
Gender, Patient Comfort and the Neurosurgical Operating Room
Authors: Zener Rebecca , Bernstein Mark
Published: December 2014

Background:

Neurosurgical patients may be unaware of components of their intra-operative care. The relationship between patient gender and comfort level in the neurosurgical operating room (OR) has not been previously studied. Our objective was to gain insight into patients' perspective of the OR environment, including staffing and observers, the role of medical students, catheterization, exposure, and verbiage, using a qualitative needs assessment.

Methods:

Face-to-face semi-structured interviews were conducted with 20 patients (14 female, six male) who had a neurosurgical operation under general anesthetic within the previous two years. The majority underwent craniotomy for benign tumours. Interviews were transcribed and subjected to modified thematic analysis.

Results:

Nine themes emerged: 1) perception of the intra-operative environment varies between men and women; 2) lacking awareness about observers is anxiety-provoking for women; 3) being unaware of the hands-on involvement of students is a concern for all patients; 4) disclosure of implantation of foreign and permanent materials into patients is important; 5) catheterization is anxiety provoking for women; 6) pre-operative menstruation screening may minimize embarrassment for women; 7) patients perceive extraneous conversation as a distraction for surgeons; 8) patients trust their surgeon; 9) a relationship exists between interviewer gender and patient comfort in the interview.

Conclusion:

Although most male and female patients are unaware of OR activities, they are generally not fearful since they trust their surgeon. Women appear to have greater information needs. Patients' information needs must be met without provoking anxiety and yet preserving their personal sense of modesty in the intra-operative environment.
Head Pre-Cooling Improves Symptoms of Heat-Sensitive Multiple Sclerosis Patients
Authors: Reynolds Luke F. , Short Christine A. , Westwood David A. , Cheung Stephen S.
Published: December 2014

Background:

Damage to the central nervous system by Multiple Sclerosis (MS) leads to multiple symptoms, including weakness, ambulatory dysfunction, visual disturbances and fatigue. Heat can exacerbate the symptoms of MS whereas cooling can provide symptomatic relief. Since the head and neck areas are particularly sensitive to cold and cooling interventions, we investigated the effects of cooling the head and neck for 60 minutes on the symptoms of MS.

Methods:

We used a double blinded, placebo controlled, cross-over study design to evaluate the effects of head and neck cooling on six heat-sensitive, stable, ambulatory females with MS (Extended Disability Status Scale 2.5-6.5). To isolate the effects of perceived versus physiological cooling, a sham cooling condition was incorporated, where subjects perceived the sensation of being cooled without any actual physiological cooling. Participants visited the clinic three times for 60 minutes of true, sham, or no cooling using a custom head and neck cooling hood, followed by evaluation of ambulation, visual acuity, and muscle strength. Rectal and skin temperature, heart rate, and thermal sensation were measured throughout cooling and testing.

Results:

Both the true and sham cooling elicited significant sensations of thermal cooling, but only the true cooling condition decreased core temperature by 0.37°C (36.97±0.21 to 36.60±0.23°C). True cooling improved performance in the six minute walk test and the timed up-and-go test but not visual acuity or hand grip strength.

Conclusions:

Head and neck cooling may be an effective tool in increasing ambulatory capacity in individuals with MS and heat sensitivity.
MRI of Tibialis Anterior Skeletal Muscle in Myotonic Dystrophy Type 1
Authors: Coté Chantal , Hiba Bassem , Hebert Luc J. , Vial Christophe , Remec Jean François , Janier Marc , Puymirat Jack
Published: December 2014

Objective:

The aim of this study was to evaluate whether magnetic resonance imaging (MRI) can be used as a noninvasive approach to assessment of disease severity and muscle damage in Myotonic Dystrophy type 1 (DM1).

Methods:

The MRI findings in legs of 41 patients with DM1 were evaluated with respect to the tibialis anterior (TA) skeletal muscle impairment. Magnetic resonance imaging findings were compared with TA strength measurements obtained by quantitative manual testing, duration of the disease and with the length of the CTG repeats.

Results:

Muscle MRI abnormalities were observed in 80% of DM1 patients, ranging from edema-like abnormalities alone to severe atrophy / fatty replacement. Edema-like abnormalities seem to be an earlier MRI marker of the disease. Fatty infiltration/atrophy correlated with the TA muscle force (r = 0.95), the severity (P = 0.00001) of the disease but not with the duration of the disease (P = 0.3) or the length of the CTG repeats (P > 0.10), measured in peripheral leukocytes. Evaluation of other muscles of the legs revealed that the medial gastrocnemius and soleus muscles were the most frequently and severely affected muscles, while tibialis posterior muscles were relatively spared. Edema-like abnormalities are most frequently observed in the skeletal muscles of the anterior compartment.

Conclusion:

Muscle MRI is helpful to depict muscle abnormalities but does not seem to be a reliable indicator of skeletal muscle involvement in DM1 since the decrease in TAmuscle force is not correlated with MRI abnormalities in some patients.
The Design of the Canadian UnRuptured Endovascular versus Surgery (CURES) Trial
Authors: Darsaut Tim E. , Findlay J. Max , Raymond Jean
Published: December 2014

Background:

Once a decision has been made to treat an intact aneurysm, the best treatment remains uncertain. Both surgical and endovascular management strategies are commonly performed for these lesions. Surgical clipping, for years the standard treatment, is gradually becoming supplanted by endovascular treatment. However, there is no randomized data available to compare the results of surgery versus endovascular treatment of unruptured aneurysms (UIAs).

Methods:

We report the design of the Canadian UnRuptured Endovascular versus Surgery (CURES) trial to compare angiographic and clinical outcomes following treatment of UIAs.

Results:

The Canadian pilot phase will serve two purposes: i) to determine feasibility of the pivotal international study, and ii) to determine the incidence of treatment failure, a composite primary end-point comprising the occurrence of either: failure to accomplish aneurysm obliteration with the initial treatment modality, a major saccular aneurysm remnant or recurrence, or intracranial hemorrhage following treatment at one year. The pivotal international study will address which strategy leads to the best overall clinical outcomes in terms of mortality, morbidity, and clinical efficacy. CURES is designed to be a pragmatic management trial with loose inclusion criteria. The pilot study plans to enroll 260 patients, a size sufficient (at 80% power and 0.05 significance) to detect a decrease in the incidence of treatment failure from 13% to 4%. The formulation of specific hypotheses for the pivotal phase awaits the preliminary CURES morbidity and mortality results.

Conclusions:

The CURES trial intends to test surgical versus endovascular management strategies for the treatment of unruptured intracranial aneurysms.
Web-Based Software to Assist in the Localization of Neuroanatomical Lesions
Authors: Lewis Evan Cole , Strike Melanie , Doja Asif , Ni Andy , Weber Jonathan , Wiper-Bergeron Nadine , Sell Erick
Published: December 2014

Objective:

To evaluate the educational effectiveness of a novel, web-based neuroanatomical localization application.

Methods:

A prototype version of a neuroanatomical localization application was developed, limited to lesions involving Cranial Nerve (CN) VII. Second year medical students at the University of Ottawa were recruited to participate in the study. Participants were exposed to a didactic teaching session on CN VII anatomy. They were subsequently randomized to two groups - one group was granted access to the localization application (the “intervention group”), while the other group was given a booklet of standard textbook resources (the “control group”). Participants then completed a case-based multiple choice test on localization of neurologic lesions associated with CN VII, followed by a questionnaire regarding the experience.

Results:

Thirty-nine students volunteered to participate. Twenty were randomized to the intervention group and 19 to the control group. There was a mean test score difference of 1.3 (CI.95 = 0.2, 2.3) that was significantly higher in the intervention group when compared to the control group. Significance was determined by aWilcoxon rank test (p = 0.028). Questionnaire results were similar for both groups, showing an overall favourable evaluation of the localization application.

Conclusions:

The results support our hypotheses that students using the application would perform better on the multiple choice question (MCQ) test and there would be an overall preference for its use. The demonstrated educational benefit of the application, in addition to the demand for such a resource expressed by the participants, warrant further investigation into the development of a neurological localization application.
Normal-Pressure Hydrocephalus: Is There a Genetic Predisposition?
Authors: Cusimano M. D. , Rewilak D. , Stuss D. T. , Barrera-Martinez J. C. , Salehi F. , Freedman M.
Published: December 2014

Background:

Normal-pressure hydrocephalus (NPH) is characterized by gait disturbance, cognitive impairment, with or without urinary incontinence, enlarged ventricles with or without cerebral atrophy and normal cerebrospinal fluid pressure.

Methods:

We report two sisters with NPH who lived together their entire lives and whose natural history might provide insights into genetic and environmental mechanisms underlying this disorder. Both patients were in their early seventies, single, had similar daily habits and hypertension. No other family members had NPH.

Results:

They both underwent shunt placement and showed improvement documented by history and neuropsychological assessment. Both showed a delayed deterioration due to vasculopathy. Both patients were homozygous for the apolipoprotein E (ApoE) e3 allele on chromosome 19. No environmental factors that might have influenced the development of NPH were identified.

Conclusion:

Our report of two sisters with NPH may indicate the presence of genetic predisposition and further studies involving genetics and environmental factors are necessary to elucidate their role in the pathogenesis of NPH.
The Neuropathies of Waldenström's Macroglobulinemia (WM) and IgM-MGUS
Authors: Klein Christopher J. , Moon Joon-Shik , Mauermann Michelle L. , Zeldenrust Steven R. , Wu Yanhong , Dispenzieri Angela , Dyck Peter J.
Published: December 2014

Background:

Neuropathy is common in Waldenström's macroglobulinemia (WM, an IgM-associated lymphoplasmacytic lymphoma) and in IgM-monoclonal gammopathy of undetermined significance (IgM-MGUS). Paraneoplastic or paraimmune mechanisms are thought to be involved in the pathogenesis of these neuropathies. Attempts at distinguishing WM and IgM-MGUS neuropathies are lacking especially among bone marrow (BM) confirmed patients.

Methods:

Retrospective analyses were performed on BM confirmed WM (N=30) and IgM-MGUS (N=73) neuropathy patients with neurologic assessments and hematologic features.

Results:

The presence of anemia and quantity of IgM monoclonal protein were significantly greater in WM. Based on multiple neurologic assessments differences were not found for: 1) length of time from neurologic symptom onset to evaluation; 2) chief complaint of painless loss of feeling in the feet, Romberg's sign and tremor; and 3) clinical motor, sensory and reflex abnormalities. Autonomic testing was normal in both diseases. Using nerve conduction (NCS) criteria for demyelination, 62% of IgM-MGUS and 27% of WM met this criteria (p=0.013). IgM MGUS patients had greater terminal conduction slowing by ulnar residual latency calculation (<0.01). The degree of axonal loss as measured by summated compound muscle action potentials and available nerve biopsy was not significantly different between diseases.

Conclusion:

Although WM and IgM-MGUS must be distinguished for hematologic prognosis and treatment, clinical neuropathy presentations of WM and IgM-MGUS are similar and likely related to comparable axonal loss in both conditions. Despite these similarities, evidence of demyelination was found by electrophysiologic studies much more commonly in IgM-MGUS. This difference may reflect varied immune mechanism(s) in the two disorders.
Variations in5-HT2AInfluence Spatial Cognitive Abilities and Working Memory
Authors: Gong Pingyuan , Li Jing , Wang Jian , Lei Xu , Chen Dongmei , Zhang Kejin , Zhang Wenjiang , Zhen Anyuan , Gao Xiaocai , Zhang Fuchang
Published: December 2014

Background:

5-hydroxytryptamine receptor 2A (5-HT2A) participates in diverse psychiatric disorders by regulating the activity of serotonin. Some previous studies have also suggested that the receptor is involved in cognitive abilities of disease groups. We hypothesize that some functional genetic variants in5-HT2Ahave certain specific influences on cognitive abilities in a normal population.

Method:

To confirm this hypothesis, two polymorphisms (rs6313 and rs4941573) in5-HT2Awere selected, and a population-based study was performed in a young healthy Chinese Han cohort.

Results:

The results indicated that the rs6313 and rs4941573 were associated with touching blocks and mental rotation-3D error ratio in males, and the rs4941573 was associated with visuo-spatial working memory in the whole cohort.

Conclusion:

All the findings suggest that5-HT2Aparticipates in human spatial cognitive abilities and spatial working memory.