bluuebunny/crossref_metadata_2025 huggingface dataset:
Population-Based Study of Pseudoprogression after Chemoradiotherapy in GBM
Authors: Roldán Gloria B. , Scott James N. , McIntyre John B. , Dharmawardene Marisa , de Robles Paula A. , Magliocco Anthony M. , Yan Elizabeth S. Y. , Parney Ian F. , Forsyth Peter A. , Cairncross J. Gregory , Hamilton Mark G. , Easaw Jacob C.
Published: December 2014

Introduction:

Chemoradiotherapy followed by monthly temozolomide (TMZ) is the standard of care for patients with glioblastoma multiforme (GBM). Case reports have identified GBM patients who experienced transient radiological deterioration after concurrent chemoradiotherapy which stabilized or resolved after additional cycles of adjuvant TMZ, a phenomenon known as radiographic pseudoprogression. Little is known about the natural history of radiographic pseudoprogression.

Methods:

We retrospectively evaluated the incidence of radiographic pseudoprogression in a population-based cohort of GBM patients and determined its relationship with outcome and MGMT promoter methylation status.

Results:

Out of 43 evaluable patients, 25 (58%) exhibited radiographic progression on the first MRI after concurrent treatment. Twenty of these went on to receive adjuvant TMZ, and subsequent investigation demonstrated radiographic pseudoprogression in 10 cases (50%). Median survival (MS) was better in patients with pseudoprogression (MS 14.5 months) compared to those with true radiologic progression (MS 9.1 months, p=0.025). The MS of patients with pseudoprogression was similar to those who stabilized/responded during concurrent treatment (p=0.31). Neither the extent of the initial resection nor dexamethasone dosing was associated with pseudoprogression.

Conclusions:

These data suggest that physicians should continue adjuvant TMZ in GBM patients when early MRI scans show evidence of progression following concurrent chemoradiotherapy, as up to 50% of these patients will experience radiologic stability or improvement in subsequent treatment cycles.
Febrile Seizures: Current Views and Investigations
Authors: Reid Aylin Y. , Galic Michael A. , Teskey G. Campbell , Pittman Quentin J.
Published: December 2014

Febrile seizures (FSs) are seizures that occur during fever, usually at the time of a cold or flu, and represent the most common cause of seizures in the pediatric population. Up to 5% of children between the ages of six months and five years-of-age will experience a FS. Clinically these seizures are categorized as benign events with little impact on the growth and development of the child. However, studies have linked the occurrence of FSs to an increased risk of developing adult epileptic disorders. There are many unanswered questions about FSs, such as the mechanism of their generation, the long-term effects of these seizures, and their role in epileptogenesis. Answers are beginning to emerge based on results from animal studies. This review summarizes the current literature on animal models of FSs, mechanisms underlying the seizures, and functional, structural, and molecular changes that may result from them.
EEG in Suspected Syncope: Do EEGs Ordered by Neurologists Give a Higher Yield?
Authors: Poliquin-Lasnier Laurence , Moore Fraser G. A.
Published: December 2014

Background:

Prior studies have shown that the electroencephalogram (EEG) is of low diagnostic yield in the evaluation of syncope but have not looked at the yield according to referring physician specialty. The goals of this study were to determine if the yield of the EEG is higher when ordered by neurologists and whether EEGs with abnormal findings resulted in any significant change in patient management.

Methods:

We retrospectively reviewed the records of the EEGs requested for a clinical diagnosis of syncope, convulsive syncope, loss of consciousness, or falls from 2003 to 2007 at our institution. We obtained further information from the medical record of patients with an abnormal EEG.

Results:

Of 517 EEGs meeting our inclusion criteria, only 57 (11.0%) were read as abnormal. No EEG was positive for epileptiform activity and only 9 (1.6%) showed potentially epileptic activity. EEGs ordered by neurologists did not have a higher yield compared to non-neurologists. Five abnormal EEGs resulted in further investigations being ordered. One patient was ultimately started on phenytoin.

Conclusions:

EEGs requested for the evaluation of patients with suspected syncope have an extremely low diagnostic yield and do not significantly alter the management of the patients, regardless of the specialty of the referring physician.
Population-based Study of Medulloblastoma: Outcomes in Alberta from 1975 to 1996
Authors: Roldán Gloria , Brasher Penny , Vecil Giacomo , Senger Donna , Rewcastle Barry , Cairncross Gregory , Forsyth Peter , Hamilton Mark
Published: December 2014

Background:

The purpose of this study was to determine incidence, survival rate, and prognostic factors as well as the frequency of Collins’ Law Violators (CLVs) in an unselected population of medulloblastoma patients. Collins’ Law dictates that ’cure’ of a child with a tumor occurs after a period that includes the child’s age at diagnosis plus 9 months.

Methods:

Using the Alberta Cancer Registry a population-based review identified 49 patients with medulloblastoma (19 adults, 30 children) diagnosed from 1975-96. Pathology was reviewed in all cases. All patients had surgical resection, followed by radiotherapy in 47 patients and chemotherapy in 17.

Results:

The overall 5-year survival was 50%. There was a trend for the extent of resection to be associated with a longer survival (Long rank test, p< 0.06) but this was not significant. Tumor recurrence occurred a median of 22.4 months (range, 6.4-192.3) after diagnosis and median survival after recurrence was 9.3 months (range, 0.4-64.9). The survival curve did not appear to plateau but was affected by tumor-related deaths in 3 (21.4%) of the 21 long-term survivors diagnosed in childhood. These three patients had recurrences a mean of 11.7 years after diagnosis and are designated as CLVs.

Conclusions:

The survival rate in an unselected population of patients with medulloblastoma is poor. Aggressive resection of the tumors prolongs survival. The Collins’ Law Violators were relatively common and we suggest this concept be abandoned in medulloblastoma.
Lipomatous Lumbar Mass with an Attached Digit and Associated Split Cord Malformation
Authors: Snelling CMH , Ellis PM , Smith RML , Rossiter JP
Published: December 2014

Background:

A male infant was born with a digit attached to a skin-covered lumbar lipomatous mass and an underlying split cord malformation.

Methods:

Surgical removal of the mass was performed at four months-of-age. By this time the digit had grown a nail and imaging and histology showed ongoing development of articulated phalanges.

Results:

The lipomatous mass contained a long bone, a clavicle-and scapula-like structure and a variety of other mature germ layer derivatives. These features raised a number of diagnostic considerations, including: mature teratoma, hamartoma, rudimentary parasitic twin, lipomyelomeningocele and dorsal accessory limb.

Conclusions:

Based on review of the literature, the authors hypothesize that there is a pathogenetically related spectrum of skin-covered dorsal mass lesions, often associated with spinal dysraphism. These consist of a major lipomatous component and a variety of mature germ layer derivatives that can vary widely in their degree of anatomical organization from case to case.
Reliability of Point-of-Care Testing of INR in Acute Stroke
Authors: Green Theresa L. , Mansoor Adnan , Newcommon Nancy , Stephenson Caroline , Stewart Eileen , Hill Michael D.
Published: December 2014

Background:

In the emergency department, portable point-of-care testing (POCT) coagulation devices may facilitate stroke patient care by providing rapid International Normalized Ratio (INR) measurement. The objective of this study was to evaluate the reliability, validity, and impact on clinical decision-making of a POCT device for INR testing in the setting of acute ischemic stroke (AIS).

Methods:

A total of 150 patients (50 healthy volunteers, 51 anticoagulated patients, 49 AIS patients) were assessed in a tertiary care facility. The INR’s were measured using the Roche Coaguchek S and the standard laboratory technique.

Results:

The interclass correlation coefficient and 95% confidence interval between overall POCT device and standard laboratory value INRs was high (0.932 (0.69 - 0.78). In the AIS group alone, the correlation coefficient and 95% CI was also high 0.937 (0.59 - 0.74) and diagnostic accuracy of the POCT device was 94%.

Conclusions:

When used by a trained health professional in the emergency department to assess INR in acute ischemic stroke patients, the CoaguChek S is reliable and provides rapid results. However, as concordance with laboratory INR values decreases with higher INR values, it is recommended that with CoaguChek S INRs in the > 1.5 range, a standard laboratory measurement be used to confirm the results.
Simultaneous EEG-fMRI in Human Epilepsy
Authors: Cunningham Cameron JB , Zaamout Mohammed El-Fateh , Goodyear Bradley , Federico Paolo
Published: December 2014

Electroencephalography (EEG) has been used to study and characterize epilepsy for decades, but has a limited ability to localize epileptiform activity to a specific brain region. With recent technological advances, high-quality EEG can now be recorded during functional magnetic resonance imaging (fMRI), which characterizes brain activity through local changes in blood oxygenation. By combining these techniques, the specific timing of interictal events can be identified on the EEG at millisecond resolution and spatially localized with fMRI at millimeter resolution. As a result, simultaneous EEG-fMRI provides the opportunity to better investigate the spatiotemporal mechanisms of the generation of epileptiform activity in the brain. This article discusses the technical considerations and their solutions for recording simultaneous EEG-fMRI and the results of studies to date. It also addresses the application of EEG-fMRI to epilepsy in humans, including clinical applications and ongoing challenges.
Symptom Predictors of Cerebrospinal Fluid Leaks
Authors: Ohwaki Kazuhiro , Yano Eiji , Ishii Teruyuki , Takanashi Shigehiko , Nakagomi Tadayoshi
Published: December 2014

Background:

Spinal cerebrospinal fluid (CSF) leaks, which are considered a cause of intracranial hypotension, generally do not cause any local symptoms. Although symptoms are key elements for further evaluation, few studies have examined symptom predictors of intracranial hypotension. The aim of this study was to determine what symptoms are predictors of CSF leaks in patients suspected of intracranial hypotension.

Methods:

We performed radionuclide cisternography in 207 consecutive patients suspected of intracranial hypotension. Intracranial hypotension was suspected when a patient had a history of minor trauma and complained about uncontrolled headache, cranial nerve dysfunction, autonomic dysfunction, or higher brain dysfunction. The leakage of CSF was defined as direct signs of tracer leak into the spinal epidural space or early accumulation of the tracer in the urinary bladder. We obtained information on 16 symptoms commonly reported in previous studies.

Results:

CSF leaks were observed in 154 cases (74%). Back pain, limb pain, and limb numbness were inversely associated with CSF leaks (p = 0.042, p = 0.045, and p = 0.006, respectively). In logistic regression analysis, diplopia was a positive predictor of CSF leaks (odds ratio [OR], 6.53; 95% confidence interval [CI], 1.49 to 28.51), whereas limb numbness was a negative predictor (OR, 0.38; 95% CI, 0.17 to 0.84). Of the 21 patients in whom diplopia was present and limb numbness was absent, 20 had CSF leaks (specificity, 98%; positive predictive value, 95%).

Conclusion:

Some symptoms may be helpful in the diagnosis of CSF leaks in patients suspected of intracranial hypotension.
Epidemiology of Guillain-Barré Syndrome in the Province of Quebec
Authors: Deceuninck Geneviève , Boucher Renée-Myriam , De Wals Philippe , Ouakki Manale
Published: December 2014

Background:

In the province of Quebec, a population-based study of Guillain-Barré syndrome (GBS) was conducted at the time of a mass immunization campaign against meningococcal disease, in 2001.

Methods:

The study population included residents aged 2 months to 20 years observed from November 1st, 2000 to December 31, 2002, representing 4 075 465 person-years of observation. GBS cases were identified in the provincial hospital database Med-Echo and medical records were reviewed.

Results:

Thirty-three incident GBS cases were identified, including 27 cases of acute inflammatory demyelinating polyradiculopathy. The overall GBS incidence rate was 0.8/100 000 person-years, higher in persons aged 1 to 4 years (2.1/100 000) than in those 5 years or more (0.6/100 000). There was a female preponderance and no significant seasonal variation. All patients survived.

Conclusion:

Results could be used to interpret reports of adverse events associated with the introduction of new vaccines in this age-group in Canada.
Variability in Physician Care Practices for Glucose Treatment in Stroke Patients
Authors: Casaubon Leanne K. , Saltman Alexandra , Peeva Valentina , Ennis Marguerite , Lam Ngan , Silver Frank L. , Kapral Moira K.
Published: December 2014

Background:

Hyperglycemia is noted in up to 60% of stroke patients. Practice guidelines recommend glucose monitoring following stroke but provide few management recommendations. We examined physician care practices for glucose management in stroke patients.

Methods:

Emergency physicians, family physicians, general internists, intensive care specialists and neurologists in Ontario comprised the study population. A mailed, self-administered survey inquired about glucose management practices. Proportions of responses for survey questions were determined. Chi-square analysis was used for comparing physician groups.

Results:

Surveys were mailed to 2,280 physicians; 26.8% returned surveys. There were 278 respondents who reported providing stroke patient care. For physicians treating glucose in stroke patients, 16.6% targeted glucose 4.0-6.0 mmol/l, 52% targeted 6.1-8.0 mmol/l, 13.6% targeted 8.1-12.0 mmol/l, 0.8% targeted 12.1-15.0 mmol/l, and 7.5% were unsure. Comparing specialties, 32% of intensivists, 17.5% of neurologists, 13% of general internists, 14% of emergency physicians, and 0% of family physicians reported targeting 4.0-6.0 mmol/l (p=0.026). Overall, 44% reported aiming for target glucose within 12 hours and 77% within 24 hours from hospital presentation. Intensive care specialists treated glucose most aggressively, including 20% treating, with insulin infusion, patients with no diabetes and initial glucose 6.0-8.0 mmol/l. Emergency physicians were most conservative when treating glucose in stroke patients.

Conclusion:

There is variability in the aggressiveness of glucose management in stroke patients by different physician specialty groups, reflecting the lack of evidence available to guide hyperglycemia management in this setting. These results highlight an important gap in knowledge and recommendations for stroke patient care that must be addressed to ensure optimal patient outcomes.
Alexia With and Without Agraphia: An Assessment of Two Classical Syndromes
Authors: Sheldon Claire A. , Malcolm George L. , Barton Jason J.S.
Published: December 2014

Background:

Current cognitive models propose that multiple processes are involved in reading and writing.

Objective:

Our goal was to use linguistic analyses to clarify the cognitive dysfunction behind two classic alexic syndromes.

Methods:

We report four experiments on two patients, one with alexia without agraphia following occipitotemporal lesions, and one with alexia with agraphia from a left angular gyral lesion.

Results:

The patient with occipital lesions had trouble discriminating real letters from foils and his reading varied with word-length but not with linguistic variables such as part of speech, word frequency or imageability. He read pseudo-words and words with regular spelling better, indicating preserved use of grapheme-to-phoneme pronunciation rules. His writing showed errors that reflected reliance on ‘phoneme-to-grapheme’ spelling rules. In contrast, the patient with a left angular gyral lesion showed better recognition of letters, words and their meanings. His reading was better for words with high imageability but displayed semantic errors and an inability to use ‘grapheme-to-phoneme’ rules, features consistent with deep dyslexia. His agraphia showed impaired access to both an internal lexicon and ‘phoneme-to-grapheme’ rules.

Conclusion:

Some cases of pure alexia may be a perceptual word-form agnosia, with loss of internal representations of letters and words, while the angular gyral syndrome of alexia with agraphia is a linguistic deep dyslexia. The presence or absence of agraphia does not always distinguish between the two; rather, writing can mirror the reading deficits, being more obvious and profound in the case of an angular gyral syndrome.
Concussion Incidence and Time Lost from Play in the NHL During the Past Ten Years
Authors: Wennberg Richard A. , Tator Charles H.
Published: December 2014

Background:

The problem of concussions in professional hockey has attracted much recent attention. To evaluate the current state of this injury in the National Hockey League (NHL), we analyzed the concussion incidence and time lost from play due to concussions during the past ten NHL seasons.

Methods:

Data were obtained from a complete review of injury reports in two different sports media sources covering the NHL seasons 1997-98 through 2007-08. Time lost from play was measured in missed games per concussion.

Results:

The incidence of concussions reported in the regular season ranged from a high of 1.81/1000 athlete exposures in 1998-99 to a low of 1.04/1000 athlete exposures in 2005-06. There was a downward trend in the number of concussions reported per season during the past ten years (p=0.01). However, average time lost from play per concussion increased over the same period (p<0.0005). Forwards suffered a disproportionately high percentage of concussions (p<0.0001).

Conclusions:

Possibly related to injury reduction efforts, the number of concussions reported per season in the NHL has trended downward in recent years. However, the incidence of concussion remains high and the average time lost from play per concussion has increased. This may reflect increased injury severity in recent years or, alternatively, increased adherence to modern management guidelines preventing premature return to play.
Proximity to the Treating Centre and Outcomes Following Subarachnoid Hemorrhage
Authors: O'Kelly Cian J. , Spears Julian , Urbach David , Wallace M. Christopher
Published: December 2014

Background:

In the management of subarachnoid hemorrhage (SAH), the potential for early complications and the centralization of limited resources often challenge the delivery of timely neurosurgical care. We sought to determine the impact of proximity to the accepting neurosurgical centre on outcomes following aneurysmal SAH.

Methods:

Using administrative data, we analyzed patients undergoing treatment for aneurysmal subarachnoid hemorrhage at neurosurgical centres in Ontario between 1995 and 2004. We compared mortality for patients receiving treatment at a centre in their county (in-county) versus those treated from outside counties (out-of-county). We also examined the impact of distance from the patient's residence to the treating centre.

Results:

The mortality rates were significantly lower for in-county versus out-of-county patients (23.5% vs. 27.6%, p=0.009). This advantage remained significant after adjusting for potential confounders (HR=0.84, p=0.01). The relationship between distance from the treating centre and mortality was biphasic. Under 300km, mortality increased with increasing distance. Over 300km, a survival benefit was observed.

Conclusions:

Proximity to the treating neurosurgical centre impacts survival after aneurysmal SAH. These results have significant implications for the triage of these critically ill patients.
Spontaneous Intracranial Hypotension: Case Series of Rare Clinical Presentations
Authors: Chaudhary N. , Cooper P. , Lownie S.P. , Ng W. , Duggal N.
Published: December 2014

Background:

Spontaneous intracranial hypotension (SIH) is an increasingly described entity, with over 70 cases reported in the literature. The classic triad includes orthostatic headache, diffuse pachymeningeal enhancement on magnetic resonance imaging (MRI) with gadolinium, and low cerebrospinal fluid pressure (CSF) in the lateral decubitus (<60 mm H2O) and sitting positions.

Method:

We present four rare clinical presentations of SIH, two of which have not been previously described in the literature, to the best of our knowledge.

Results:

Patient 1 presented with dyspnea, dysphagia, bilateral ptosis, diplopia and seizures. Patient 2 presented with a paradoxical positional pattern of orthostatic hypotension. In Patient 3, bilateral subdural hematomas (SDH) were encountered; while in Patient 4, a recurrent unilateral SDH requiring multiple surgical interventions was demonstrated.

Conclusion:

Although uncommon clinical presentations, all four cases of intracranial hypotension were spontaneous, demonstrated diagnostic MRI findings, and responded favorably to blood patches or saline injections.
Intracranial Caseating Granulomas with No Infectious Organism Detected
Authors: Ghavanini Amer A. , Munoz David G.
Published: December 2014

Background:

Caseating granuloma is a classic histopathological feature of mycobacterial infections. Occasionally, no infectious organism is demonstrated despite extensive examination of intracranial caseating granulomas. The pathogenesis and optimal management strategy for patients with such intracranial caseating granulomas with no detectable infectious organism (ICGNs) remain unclear.

Methods:

The study was a retrospective case-series design in a referral hospital setting. Patients with intracranial caseating granulomas in whom no infectious etiology was identified after appropriate investigations were reviewed.

Results:

Eight patients with ICGN (four females and four males) were identified in an eight-year-period. Average age on presentation was 46 years (range 21-69 years). Cerebrospinal fluid showed lymphocytic pleocytosis, elevated protein and decreased glucose. Neuroimaging showed multiple or single intraparenchymal and meningeal enhancements. Intracranial ICGN were demonstrated on biopsy. Immunomodulation was tried and resulted in improvement in five out of eight patients. In four patients, anti-mycobacterial treatment resulted in no improvement or worsening of clinical or radiological features.

Conclusions:

The response to therapy of intracranial caseating granulomas where no organism is identified after thorough investigations hints to non-infectious causes, and suggests current dogma regarding the significance of necrosis in granulomatous diseases should be re-evaluated. Our retrospective series suggests that patients may benefit from an early trial of immunomodulation therapy, a hypothesis to be tested in a randomized trial.
A Quantitative Analysis of Suspected Environmental Causes of MS
Authors: Sloka Scott , Silva Claudia , Pryse-Phillips William , Patten Scott , Metz Luanne , Yong V. Wee
Published: December 2014

Background:

Multiple sclerosis (MS) is a disease with purported environmental causes. Consistent correlations have been found in various settings for latitude, smoking exposure, sunlight, and vitamin D deficiency. We analysed the contribution of various environmental factors to the risk of developing MS from a population perspective.

Methods:

We collated global data of MS prevalence from 54 studies over the previous ten years and calculated the degree of risk contributed by latitude, longitude, ultraviolet radiation (from NASA satellite data and formulae for available sunlight hours), population smoking rates (from WHO data), gender, study date, study demographics, and several socioeconomic factors. We report a very significant negative correlation between MS prevalence and available ultraviolet (UV) radiation.

Results:

The lack of available UV radiation outweighs other factors by at least 20 fold (p<10∧-8) from single variate regression analysis. Multiple regression analysis revealed that latitude and longitude are also significant factors; smoking may also provide a very minimal role. The eight prevalence studies from Scandinavia produced prevalences that were lower than expected, given their global geospatial positioning.

Conclusions:

The available ultraviolet radiation is a significant environmental factor, moreso than all the other factors examined.
Procalcitonin Levels in Migraine Patients
Authors: Turan Hale , Horasanli Bahriye , Ugur Murat , Arslan Hande
Published: December 2014

Objectives:

Migraine is a risk factor for ischemic stroke. Sterile vascular inflammation may develop during migraine attacks. This study aims to investigate procalcitonin (PCT) levels amongst migraine patients as they are important markers for infection and sepsis, but can also be found at elevated levels in various cases of inflammation.

Methods:

Eighty adult migraine patients participated in our study. Patients were initially separated into two main groups; Group-1 consisted of 34 patients who had migraines during the attack period. Group-2 consisted of 46 patients during the period in-between attacks. Afterwards, patients were further divided into four subgroups based on their aura status; Group-1a Migraine without aura, 27 patients during attack period, Group-1b Migraine with aura, 7 patients during attack period, Group-2a Migraine without aura, 40 patients during the period in-between attacks, Group-2b Migraine with aura, 6 patients during the period in-between attacks.

Results:

Average PCT levels in patients during attack periods were found to be higher than the average PCT levels of patients during the period in-between attacks. These elevated levels were determined to be statistically significant(p<0.01). Serum PCT levels of the patients with migraine without aura during the attack period were significantly higher than those of patients during the period in-between attacks(p<0.01).

Conclusions:

Based on significantly high levels of PCT, our results support the idea that sterile inflammation plays a role in migraine pathogenesis. Further studies are necessary to understand whether PCT is a marker for ischemic stroke risk in patients who go through frequent migraine attacks.
Reversible Wall Enhancement in Pediatric Cerebral Arteriopathy
Authors: Payne Eric T. , Wei Xing-Chang , Kirton Adam
Published: December 2014

A previously well and developmentally normal five-year-old right-handed girl, presented with acute left-sided weakness and dysarthria. Investigations confirmed an acute right lenticulostriate stroke secondary to probable childhood primary angiitis of the CNS(cPACNS), including evidence of vessel wall enhancement (Figure). Despite extensive investigations, no alternative etiology for her stroke was detected. After three weeks of steroids, the child improved and the vessel wall enhancement resolved. Moderate hemiparesis, hemidystonia, and behavioural sequelae are present at 18-month follow-up.
Low Grade Glioma: A Qualitative Study of the Wait and See Approach
Authors: Hayhurst Caroline , Mendelsohn Daniel , Bernstein Mark
Published: December 2014

Background:

There is no consensus on the best management of adults with presumed low grade glioma (LGG). Studies have suggested uncertainty and anxiety associated with a wait and see approach contribute to reduced quality of life. This study aims to explore the impact of a diagnosis of LGG, to address concerns regarding the uncertainty of the diagnosis and the role of wait and see from the patient's perspective.

Methods:

Qualitative research methodology was used. A semi-structured interview was conducted with 24 patients with imaging evidence of LGG but no prior intervention. All patients had been followed for at least one year prior to interview. Verbatim transcripts were subjected to thematic analysis.

Results:

The median age of participants was 47 (range 21 - 82) and the median duration of follow-up 37 months (range 12 - 156 months). Fifty percent presented with seizures. Five overarching themes emerged from the data; 1) patients experience initial devastation followed by acceptance and low anxiety; 2) absence of symptoms mitigates anxiety concerning the possibility of progression; 3) patients would prefer to defer surgery until there is progression or a change in their quality of life; 4) anxiety is reduced by trust in the physician; 5) quality of life is not affected by the diagnosis, as fear of morbidity from intervention is greater than the fear of uncertainty.

Conclusions:

The wait and see approach does not contribute to anxiety or reduction in quality of life in patients with LGG.
Minimally Invasive versus Open Approach for Cervical Laminoforaminotomy
Authors: Winder Mark J. , Thomas Kenneth C.
Published: December 2014

Background:

Minimally invasive posterior cervical foraminotomy for radicular symptoms has become more prevalent. The reported experience with microscopic tubular assisted posterior cervical laminoforaminotomy (MTPF) for the treatment of radicular pain is lacking. Tubular assisted techniques have been considered to offer significant benefit, over open procedures, in terms of minimizing tissue damage, operative time, blood loss, analgesic requirements and length of hospital stay. We hypothesized that MTPF reduces post-operative analgesic requirements and length of hospital stay over the traditional open laminoforaminotomy, with no difference in complication rates and, secondly, that MTPF is comparable to endoscopic posterior foraminotomy (EPF).

Methods:

We conducted a retrospective review of 107 patients who underwent posterior cervical laminoforaminotomy for radicular pain between 1999 and 2009. Patient demographics, intra-operative parameters, length of hospitalization, post-operative analgesic use, complications and short-term neurological outcome were compared between groups.

Results:

Between 1999 and 2009, a total of 107 patients were identified to have undergone a cervical foraminotomy. An open approach was used in 65 patients, while 42 underwent MTPF. Operative time and complications were comparable between groups. Significant differences favoring MTPF were observed in operative blood loss, post-operative analgesic use and length of hospital stay (p<0.001). All results were comparable to previous reports utilizing EPF.

Conclusions:

MTPF for the treatment of cervical radiculopathy significantly reduces blood loss, post-operative analgesic use and length of hospital stay compared to the standard open approach. Operative time and complication rates were comparable between both techniques, whilst MTPF offered similar results compared to EPF.
Meaningful Change in Cognition in Multiple Sclerosis: Method Matters
Authors: Walker L. A. S. , Mendella P. D. , Stewart A. , Freedman M. S. , Smith A. M.
Published: December 2014

Objective:

To determine if different methods of evaluating cognitive change over time yield measurably different outcomes.

Methods:

Twelve cognitively impaired patients with clinically definite Multiple sclerosis (10 relapsing-remitting, 2 secondary progressive) underwent neuropsychological testing (baseline, 6, 12 months). Data was analysed using: t-tests evaluating group differences on individual tests, group differences in composite scores, reliable change analyses at the level of the individual, and comparisons regarding number of tests failed at each time point.

Results:

Group t-tests on individual tests yielded no change. When tests were grouped according to theoretical constructs, analyses revealed change in processing speed. Reliable change estimates revealed that 16% of the sample deteriorated. When change was measured with respect to the number of domains affected at each time point, 58% of the sample deteriorated on at least one subtest.

Conclusions:

Methodology has a significant impact on interpretation of longitudinal data. In the same group of subjects, traditional group analyses documented no change in individual test scores or change on a single composite score. Analyses of individual results documented change from 16 to 58% of the sample. Advantages and disadvantages of each method were discussed. Findings have implications for interpretation of longitudinal studies.
Hyperekplexia: Treatment of a Severe Phenotype and Review of the Literature
Authors: Mineyko Aleksandra , Whiting Sharon , Graham Gail E.
Published: December 2014

Hyperekplexia is a rare disorder caused by autosomal dominant or recessive modes of inheritance and characterized by episodes of exaggerated startle. Five causative genes have been identified to date. The syndrome has been recognized for decades and due to its rarity, the literature contains mostly descriptive reports, many early studies lacking molecular genetic diagnoses. A spectrum of clinical severity exists. Severe cases can lead to neonatal cardiac arrest and death during an episode, an outcome prevented by early diagnosis and clinical vigilance. Large treatment studies are not feasible, so therapeutic measures continue to be empiric. A marked response to clonazepam is often reported but refractory cases exist. Herein we report the clinical course and treatment response of a severely affected infant homozygous for an SLC6A5 nonsense mutation and review the literature summarizing the history and genetic understanding of the disease as well as the described comorbidities and treatment options.
Outcomes after Carotid Angioplasty and Stenting in Symptomatic Octogenarians
Authors: Almekhlafi M. A. , Couillard P. L. , Pandya A. , Shobha N. , Morrish W. F. , Wong J. H. , Hill M. D.
Published: December 2014

Objective:

Octogenarians were excluded from participation in many carotid endarterectomy trials due to the high complication rates observed in past studies. However, stroke resulting from carotid stenosis is expected to increase with the aging population. Moreover, advances in Carotid Angioplasty and Stenting (CAS) techniques have resulted in perceived improved safety of this procedure. We sought to review our experience with carotid stenting in symptomatic octogenarians with an emphasis on short-term outcomes and complications.

Methods:

This is a retrospective longitudinal cohort study of all symptomatic patients who underwent CAS in our center between 1997 and 2007. Thirty-day stroke and death rates, and length of hospitalization were compared between the symptomatic octogenarians and non-octogenarians.

Results:

A total of 214 procedures were performed on 211 symptomatic patients (56 females). Fifty-nine patients (14 females) were octogenarians. The median (interquartile range) age on procedure date for the octogenarian cohort was 83 (4) years. Periprocedural death occurred in two (3.4%) octogenarians and five (3.3%) non-octogenarians (p = 0.97). At 30 days from the procedure, stroke occurred in four (6.8%) octogenarians and seven (4.6%) non-octogenarians (p= 0.52). The mean hospital stay (4.8 days) was not different between the two cohorts. Age was not a predictor of the 30-day risk of composite stroke or death.

Conclusion:

The complications rate observed in octogenarians was not significantly higher than non-octogenarians. Our findings suggest that octogenarians should be included in randomized trials examining CAS to better define the risk-benefit profile of this procedure in the elderly.
Posterior Fossa Measurements in Patients With and Without Chiari I Malformation
Authors: Dufton John A. , Habeeb Syed Yaser , Heran Manraj K. S. , Mikulis David J. , Islam Omar
Published: December 2014

Purpose:

To determine if there is a correlation between cerebellar tonsillar descent in patients with and without Chiari I malformation and three skull morphometric measurements: clivus length, anteroposterior diameter of the foramen magnum, and Boogard's angle.

Methods:

Cerebellar tonsillar descent, clivus length, anteroposterior diameter of the foramen magnum, and Boogard's angle were measured in mid-sagittal T1-weighted magnetic resonance images of 188 patients. The study included 81 patients with Chiari I malformations (CMI). Without identifiable pathology, 107 patients served as a comparison group. Two-sample t-tests were used to assess for significance. A Pearson correlation matrix was constructed to assess the strength of linear dependence between measured parameters for the study population.

Results:

A negative correlation was found between tonsillar herniation and clivus length (r = -0.30, P < 0.001), while a positive correlation was found between tonsillar herniation and foramen magnum size (r = 0.15, P = 0.0431), and Boogard's angle (r = 0.23, P = 0.0014). Clivus length was shorter (P = 0.0009) in CMI patients (4.02 cm ± 0.45) than comparison patients (4.23 cm ± 0.42). In addition, the anteroposterior diameter of the foramen magnum was wider (P = 0.0412) (3.74 cm ± 0.40 compared to 3.63 ± 0.30) and Boogard's angle was larger (P = 0.0079) (123.58 degrees ± 8.27 compared to 120.62 degrees ± 6.79) with CMI.

Conclusion:

A greater degree of cerebellar tonsillar herniation is associated with a shorter clivus length, a wider anteroposterior diameter of foramen magnum, and a wider Boogard's angle.
A Canadian Perspective on Ethics Review and Neuroimaging: Tensions and Solutions
Authors: Racine Eric , Northoff Georg , Menon Ravi S. , Kimmelman Jonathan , Illes Judy
Published: December 2014

Neuroimaging research has raised ethical concerns such as the management of unexpected findings and the classification and assessment of risks. Research ethics boards (REBs) bear responsibility for the oversight of these challenges but neuroimagers struggle with the practical aspects of ethics review and report that administrative load and inconsistency contribute to eroding confidence and trust in ethics review. Our goal was to discuss and propose strategies for institutional and educational change to improve ethics review. We used an iterative and deliberative workshop-based writing process involving multiple disciplines. We propose recommendations in three tension areas: (1) communication between researchers and REBs; (2) collaboration and sharing of expertise between REBs; and (3) practical considerations and the needs of neuroimagers engaged in the ethics review process. Our recommendations are intended as openings rather than endpoints. Researchers and research ethics governance communities should decide on the future uptake of these recommendations.
Risk Profiles of Alzheimer Disease
Authors: Bilbul Melanie , Schipper Hyman M.
Published: December 2014

Alzheimer disease (AD) is a dementing, neurodegenerative disorder that affects approximately 500,000 Canadians and its prevalence is expected to double over the next 30 years. Although several medications may temporarily augment cognitive abilities in AD, there presently exists no proven method to avoid the inevitable clinical deterioration in this devastating condition. The delineation of risk factors for the development of AD offers hope for the advent of effective prevention or interventions that might retard the onset of symptoms. In this article, we provide a comprehensive review of midlife risk factors implicated in the etiopathogenesis of sporadic AD. Although some risk factors are heritable and largely beyond our control, others are determined by lifestyle or environment and are potentially modifiable. In a companion paper, we introduce the concept of an Alzheimer Risk Assessment Clinic for ascertainment and mitigation of these and other putative dementia risk factors in middle-aged adults.
Angioplasty as an Adjuvant Therapy for the Treatment of Acute Ischemic Stroke
Authors: Alhazzaa Mohammed , Murphy Amanda , Lum Cheemun , dos Santos Marlise P. , Lesiuk Howard , Bussière Miguel
Published: December 2014

Background:

Different endovascular techniques can be employed to achieve vessel recanalization in acute stroke. We assessed whether an endovascular strategy that included angioplasty was safe and effectively recanalized acutely occluded intracranial vessels.

Methods:

We retrospectively reviewed 70 patients that received intra-arterial therapy for acute stroke. Patients were divided into two groups depending on whether they had received angioplasty as part of their endovascular treatment.

Results:

Angioplasty was used in the treatment of 35/70 patients (50%). Median baseline NIHSS was 15. The site of occlusion was at the M1 in 11 patients, M1/M2 in 3, ICA/M1 in 13 and vertebrobasilar in 8 patients. Intravenous thrombolysis was administered to 16/35 patients (46%). Angioplasty was used alone in 4 patients, in combination with intra-arterial thrombolysis in 27 and with a mechanical retrieval device or stent in 13 patients. Recanalization (TICI 2-3) was achieved in 23/35 patients (66%). Median time from symptom onset to recanalization was six hours. In patients where angioplasty was employed, symptomatic intracranial hemorrhage occurred in 2/35 (6%), which was similar to patients that were not treated with angioplasty. A favorable functional outcome (mRS=2) was achieved in 20% (7/35) at 24 hour and 34% (12/35) at one month. All patients that had a favorable outcome had recanalized.

Conclusion:

In this small cohort, an endovascular treatment strategy that employed angioplasty was safe and effectively recanalized acutely occluded intracranial vessels. Angioplasty should be considered as a potential treatment option in interventional acute stroke trials.
Delirium: A Review
Authors: Gofton Teneille Emma
Published: December 2014

Delirium affects a diverse patient population, may present with highly variable clinical features, is a source of distress for patients and their caregivers, prolongs hospital stays and may herald a poor prognosis. Many cases of delirium are reversible and therefore a full history, physical examination and investigations should be performed. A high degree of suspicion is required for detecting delirium and thorough investigations are necessary in order to determine the underlying etiology and to maximize the potential for reversibility. The following review outlines important aspects of a clinical approach to delirium, the differential diagnosis of delirium, investigation of a patient presenting with delirium, management of delirium, the pathophysiology of delirium and the prognosis accompanying delirium.
A Critical Appraisal of Sedation, Analgesia and Delirium in Neurocritical Care
Authors: Teitelbaum Jeanne S. , Ayoub Omar , Skrobik Yoanna
Published: December 2014

Administering analgesics, sedatives and antipsychotics is challenging in the Neurological Intensive Care Unit (NICU). We reviewed this literature and our current practice to better inform the critical care practitioner and to identify gaps for future research. We electronically searched observational, intervention and outcome studies addressing sedation, analgesia and delirium in the NICU, and their bibliographies. Practice patterns were assessed in three critical care units with specialized neurological care in Montreal. Bedside pain assessment tools are psychometrically validated in the neuro-critically ill but sedation and delirium tools are not. Rigorous pain and sedation assessments appear feasible; delirium screening has not been tested. Publications addressing outcomes and responses to pharmacologic treatment lack consistency, rigor or both. In daily practice, pharmacologic management varies greatly. Clearly, little information exists on analgesia, sedation and delirium in the NICU. Systematic evaluation of pain improves outcome. No evidence-based therapeutic recommendations can be proffered.
Neurolathyrism in Vapniarka: Medical Heroism in a Concentration Camp
Authors: Garfinkle Jarred , Andermann Frederick , Shevell Michael I.
Published: December 2014

Stories abound about the medical abuses that have come to define medicine and the “pseudo”-neurosciences in the Third Reich. Well known are the Nazi program of euthanasia and the neuroscientific publications that arose from it. Nevertheless, during this widespread perversion of medical practice and science, true medical heroics persisted, even in the concentration camps. In December 1942, inmates of Camp Vapniarka began experiencing painful lower extremity muscle cramps, spastic paraparesis, and urinary incontinence. In order to reduce the cost of feeding the 1200, mostly Jewish, inmates of Camp Vapniarka and surreptitiously hasten their deaths, the Nazi-affiliated Romanian officers of the camp had begun feeding them a diet high inLathyrus sativus. L. sativusis the neurotoxin implicated in neurolathyrism, a degenerative disease of the upper motor neurons. Dr. Arthur Kessler, one of the camp's prisoners, eventually identified the source of the epidemic. Armed with this knowledge, the inmates collectively organized to halt its spread.
Vagus Nerve Stimulation for Epilepsy: The Notre-Dame Hospital Experience
Authors: Qiabi Mehdi , Bouthillier Alain , Carmant Lionel , Nguyen Dang Khoa
Published: December 2014

Purpose:

Retrospective study assessing the efficacy and tolerability of vagus nerve stimulation (VNS) for the treatment of refractory epilepsy at Notre-Dame Hospital.

Methodology:

Chart review of all adult epileptic patients treated by VNS with ≥ 1 year follow-up. Responders were defined as patients with ≥ 50% reduction of baseline seizure frequency.

Results:

Thirty-four patients (14M; mean age = 29.9 yrs) received a VNS. Sub-pectoralis implantation (n = 25) was more frequent than subcutaneous (n = 9). Most patients suffered from intractable partial epilepsy. After 6 months, 12 months, 24 months, and 36 months, 14/34 patients (41%), 16/34 patients (47%), 17/30 patients (57%) and 12/20 patients (60%) respectively were responders. Two patients (6%) became seizure-free. Complications related to implantation were minor: eight cases of limited cervical hypoesthesia, two minor scar infections and one Horner syndrome. Adverse events (voice hoarseness, throat paresthesia, coughing) related to stimulation were generally mild and tended to wane over time. However, a reduction in seizure frequency did not translate into a reduction in medication, as only 9% of responders had less antiepileptic medication at last follow-up compared to baseline.

Conclusion:

VNS as practiced at Notre-Dame hospital is an efficacious and safe treatment for refractory epilepsy. Quotas allotted to epilepsy centers in the province of Quebec should be lifted or increased to allow more patients to benefit from this therapeutic device.