bluuebunny/crossref_metadata_2025 huggingface dataset:
Survey of Management of Severe Head Injury in Canada
Authors: Jacka Michael J. , Zygun David
Published: December 2014

Objective:

To determine: 1. the degrees of consensus and disagreement among Canadian critical care clinicians regarding the appropriateness (benefit exceeding risk) of common therapeutic manoeuvres in patients with severe closed head injury (CHI), and 2. the frequency with which clinicians employed these manoeuvres.

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:

In the scenario of acute epidural hematoma with mass effect, respondents agreed very strongly that surgery was appropriate. Clinicians reported mannitol and hypertonic saline as appropriate. Beyond these two interventions, agreement was less strong, and the use of the extraventricular drain (EVD), phenytoin, cooling, hyperventilation, nimodipine, and jugular venous oximetry (JVO) were of uncertain appropriateness. Steroids were considered inappropriate. In a scenario of diffuse axonal injury (DAI), clinicians agreed strongly that fever reduction, early enteral feeding, intensive glucose control, and cerebral perfusion pressure (CPP)-directed management were appropriate. The use of mannitol, hypertonic saline, EVD, JVO, narcotics and propofol were also appropriate. Neuromuscular blockade, surgery, and hyperventilation were of uncertain appropriateness. The appropriateness ratings of the interventions considered in the scenario of an intracranial contusion mirrored the DAI scenario. In general, correlations between the reported appropriateness and frequency of use of each intervention were very high. An exception noted was the use of the JVO. The correlation between CPP-guided therapy and the use of the EVD was weak.

Conclusions:

This survey has described current practice with regard to treatment of patients with severe CHI. Areas of variation in perceived appropriateness were identified that may benefit from further evaluation. Suggested priorities for evaluation include the use of osmotic diuretics, anticonvulsants, and intracranial manometry.
Metastatic Melanoma to the Pituitary Gland
Authors: McCutcheon Ian E. , Waguespack Steven G. , Fuller Gregory N. , Couldwell William T.
Published: December 2014

Background:

Metastasis to the pituitary gland is unusual, and occurs most often in patients with carcinomas of the breast or lung. Despite its propensity for spread to the brain, metastatic melanoma has rarely been described within the sella.

Methods:

We report two cases of malignant melanoma pathologically confirmed within the pituitary, both metastatic from a primary site on the chest wall. In each patient, transsphenoidal resection of the tumor was incomplete and each received local radiotherapy after surgery.

Results:

One patient recurred quickly and developed brain metastasis as well. He died four months after resection of the pituitary metastasis, but the second patient survived six months without recurrence. As intrasellar metastasis portends widespread systemic disease and may be synchronous with parenchymal brain metastasis, survival in such patients is limited regardless of adjunctive therapy.

Conclusions:

Such cases are likely to arise more commonly in future due to the increasing incidence of melanoma. Identifying them by imaging alone is difficult due to inconsistent signal characteristics on MRI (as shown by these cases) and the confusion introduced by any associated intratumoral hemorrhage.
Ophthalmoplegic Migraine: Inflammatory Neuropathy with Secondary Migraine?
Authors: McMillan H J , Keene D L , Jacob P , Humphreys P
Published: December 2014

Background:

This critical review provides a summary of the clinical presentation, neuroimaging, treatment and prognosis in pediatric ophthalmoplegic migraine (OM). The features of OM are not in keeping with its classification as a migraine-variant.

Method:

We review 3 new and 37 reported pediatric OM cases.

Results:

Headache was an inconsistent feature, with 25% patients showing no evidence of pain at the initial OM episode.

Patients demonstrated:

1) prolonged time for symptom resolution to occur (median time 3 weeks); 2) tendency for recurrent episodes to have more severe and persistent nerve involvement; 3) evidence of permanent neurological sequelae with recurrent episodes (30% of patients); 4) rapid improvement and shortened duration with corticosteroid therapy and; 5) transient, reversible MRI contrast enhancement of the affected cranial nerve (86% of patients). These features would not be expected in primary migraine headache.

Conclusion:

A detailed understanding of the natural history of OM is essential for the clinical. This review provides support that OM may result from cranial nerve inflammation with headache a secondary and later feature of this condition.
A Palliative Approach to Neurological Care: A Literature Review
Authors: Gofton Teneille E. , Jog Mandar S. , Schulz Valerie
Published: December 2014

This review assesses the current opinion towards early palliative care in neurology and discusses the existing evidence base. A comprehensive literature search resulted in 714 publications with 53 being directly relevant to the scope of this review. The current literature reflects primarily expert opinion and describes a growing interest in the early introduction of palliative principles into neurological care. Early initiation of palliative interventions has the potential to improve quality of life, enhance symptom management and assist in advance care planning. Further data is required to determine whether this shift in philosophy has a positive impact on patient care.
The Script Concordance Test: A New Tool Assessing Clinical Judgement in Neurology
Authors: Lubarsky Stuart , Chalk Colin , Kazitani Driss , Gagnon Robert , Charlin Bernard
Published: December 2014

Background:

Clinical judgment, the ability to make appropriate decisions in uncertain situations, is central to neurological practice, but objective measures of clinical judgment in neurology trainees are lacking. The Script Concordance Test (SCT), based on script theory from cognitive psychology, uses authentic clinical scenarios to compare a trainee’s judgment skills with those of experts. The SCT has been validated in several medical disciplines, but has not been investigated in neurology.

Methods:

We developed an Internet-based neurology SCT (NSCT) comprising 24 clinical scenarios with three to four questions each. The scenarios were designed to reflect the uncertainty of real-life clinical encounters in adult neurology. The questions explored aspects of the scenario in which several responses might be acceptable; trainees were asked to judge which response they considered to be best. Forty-one PGY1-PGY5 neurology residents and eight medical students from three North American neurology programs (McGill, Calgary, and Mayo Clinic) completed the NSCT. The responses of trainees to each question were compared with the aggregate responses of an expert panel of 16 attending neurologists.

Results:

The NSCT demonstrated good reliability (Cronbach alpha = 0.79). Neurology residents scored higher than medical students and lower than attending neurologists, supporting the test’s construct validity. Furthermore, NSCT scores discriminated between senior (PGY3-5) and junior residents (PGY1-2).

Conclusions:

Our NSCT is a practical and reliable instrument, and our findings support its construct validity for assessing judgment in neurology trainees. The NSCT has potentially widespread applications as an evaluation tool, both in neurology training and for licensing examinations.
Myocardial Infarction Following t-PA for Acute Stroke
Authors: Mehdiratta M. , Murphy C. , Al-Harthi A. , Teal P. A.
Published: December 2014

Background and Purpose:

Complications of intravenous (IV) thrombolysis with tissue plasminogen activator (t-PA) for acute stroke are commonly related to hemorrhage, anaphylaxis, or arterial re-occlusion. Embolic complications of t-PA are beginning to be recognized with increased use of t-PA for acute ischemic stroke. We hypothesize that disruption of intra-cardiac thrombus may result in myocardial infarction (MI) after use of t-PA for acute ischemic stroke.

Summary of Cases:

We describe three cases of acute MI immediately following IV t-PA infusion for acute stroke. In patient #1 apical thrombus was visualized on cardiac echocardiogram accounting for the MI after t-PA for acute stroke. Patient #2 had fresh thrombus seen on cardiac catherization after use of t-PA for acute stroke. Patient #3 developed a significant troponin rise 15 hours after the t-PA for stroke infusion with an echocardiogram revealing new wall motion abnormalities. Patient # 1 and #2 died secondary to multi-organ failure.

Discussion:

Acute MI immediately following t-PA treatment for stroke is a rare but serious complication. The disruption of intra-cardiac thrombus and subsequent embolization to the coronary arteries may be an important mechanism in the development of MI after t-PA treatment for acute ischemic stroke.
A Novel GDAP1 Mutation P78L Responsible for CMT4A Disease in Three Moroccan Families
Authors: Bouhouche Ahmed , Birouk Nazha , Benomar Ali , Ouazzani Reda , Chkili Taïeb , Yahyaoui Mohamed
Published: December 2014

Background:

The gene encoding the ganglioside-induced-differentiation-associated protein 1 (GDAP1) has been associated with both axonal and demyelinating neuropathy. Up to date, 25 mutations in the GDAP1 gene have been reported in patients from different origins.

Methods:

Three Moroccan families with early onset ARCMT1 and autosomal recessive inheritance were genotyped to test linkage to 8q21.3 and their GDAP1 gene coding exons screened for mutations.

Results:

A novel C233T transversion at codon 78 (P78L) was detected in 6 patients from 3 unrelated families. The mutation was found to be homozygous in two families and compound heterozygous in association with the already reported S194X mutation in one family. The P78L mutation was associated with a common haplotype suggesting a Moroccan founder mutation. The patients had symptoms within the two first years of life and developed common phenotype of CMT4A with evident hoarse-voice in two cases with the longer disease duration.

Conclusion:

P78L mutation was associated with a common haplotype suggesting a common ancestor.
Skeletal Muscle Metabolic Dysfunction in Obesity and Metabolic Syndrome
Authors: Wells Greg D. , Noseworthy Michael D. , Hamilton Jill , Tarnopolski Mark , Tein Ingrid
Published: December 2014

Obesity and the related metabolic syndrome have become a worldwide epidemic. Inactivity appears to be a primary causative factor in the pathogenesis of this obesity and metabolic syndrome. There are two possible, perhaps not mutually exclusive, events that may lead to intramyocellular lipid accumulation and mitochondrial dysfunction in patients with obesity. First, obesity, with high intake-associated lipid accumulation in muscle may interfere with cellular mitochondrial function through generation of reactive oxygen species leading to lipid membrane peroxidative injury and disruption of mitochondrial membrane-dependent enzymes. This in turn leads to impaired oxidative metabolism. Secondly, a primary defect in mitochondrial oxidative metabolism may be responsible for a reduction in fatty acid oxidation leading to intramyocellular lipid accumulation as a secondary event. Non-invasive techniques such as proton (1H) and phosphorus (31P) magnetic resonance spectroscopy, coupled with specific magnetic resonance imaging techniques, may facilitate the investigation of the effects of various ergometric interventions on the pathophysiology of obesity and the metabolic syndrome. Exercise has positive effects on glucose metabolism, aerobic metabolism, mitochondrial density, and respiratory chain proteins in patients with metabolic syndrome, and we propose that this may be due to the exercise effects on AMP kinase, and a prospective physiological mechanism for this benefit is presented. A physiological model of the effect of intramyocellular lipid accumulation on oxidative metabolism and insulin mediated glucose uptake is proposed.
Methylprednisolone for Acute Spinal Cord Injury: 5-Year Practice Reversal
Authors: Hurlbert R. John , Hamilton Mark G.
Published: December 2014

Objective:

To re-evaluate practice patterns for methylprednisolone (MP) administration in patients with acute spinal cord injury (SCI) within the spinal surgery community across Canada five years after the publication of practice recommendations.

Methods:

Canadian orthopedic and neurological spine surgeons were surveyed at their respective annual meetings about their practice of steroid administration for acute SCI by means of a questionnaire comprised of the same seven questions posed five years ago plus an additional question related to change of view.

Results:

Forty-two surgeons and twenty-one residents directly involved in the acute management of SCI completed the questionnaire. Seventy-six percent of spinal surgeons do not prescribe MP for SCI in sharp contrast to 76% who prescribed it five years ago. Of the 24% who use steroids, the NASCIS II dosing regimen is most commonly followed. One third of physicians continue to administer MP because of fear of litigation.

Conclusions:

Over a five year period there has been a complete reversal in practice patterns of MP administration for SCI, along with an increased familiarity of the published literature. Attendance at meetings, participation in local group discussions, and peer-reviewed publications appear effective in altering practice preferences arising from peer pressure and even fear of litigation.
The Relation of Ultraviolet Radiation and Multiple Sclerosis in Newfoundland
Authors: Sloka JS , Pryse-Phillips WEM , Stefanelli M
Published: December 2014

Background:

It has been thought that the occurrence of multiple sclerosis (MS) could be associated with daily ultraviolet exposure. In this study we investigated the geospatial association between average daily ultraviolet B (UVB) irradiance and MS prevalence in Newfoundland and Labrador (NL), Canada.

Methods:

A complete list of patients diagnosed with MS in the province of NL was constructed. Places of habitation from birth to diagnosis were ascertained by mailout survey.

Results:

A 74% rate of return on the survey results was obtained. A plot of the average daily erythemal UV over the available five years (1998-2002) shows that the distribution of MS follow a north-south gradient. Average daily UVB measurements are lower in the higher latitudes. A statistically significant negative correlation of MS incidence with erythemal UVB was found that is stronger than the correlation using latitude. This correlation appears to be strongest in the first year of life and declines when subsequent years are examined up to age ten. No significant correlation was found for the subjects' locale of habitation at the time of their first MS attack.

Conclusions:

This study suggests that UVB radiation may contribute to the pathogenesis of MS.
Complications in 622 Cases of Frame-Based Stereotactic Biopsy, a Decreasing Procedure
Authors: Kongkham Paul N. , Knifed Eva , Tamber Mandeep S. , Bernstein Mark
Published: December 2014

Background:

Frame-based stereotactic brain biopsy has played an important role in the management of patients with suspected neoplastic intracranial lesions over the last three decades. We reviewed the surgical experience of one surgeon to determine the nature and frequency of complications associated with this procedure.

Methods:

Records were reviewed for 858 patients undergoing frame-based stereotactic procedures from January 1986 to May 2006. Data on each case were prospectively collected by the senior author. Procedures for Ommaya reservoir placement, brachytherapy, stereotactic craniotomy flap localization, shunt placement, or treatment of previously-diagnosed intracranial cystic lesions were excluded, leaving 614 patients in whom a total of 622 procedures were performed for purely diagnostic purposes. Complication rates and their association with clinical variables were sought.

Results:

Morbidity and mortality rates were 6.9% (43/622) and 1.3% (8/622), respectively. The risk of symptomatic hemorrhage (intracerebral hemorrhage [ICH], subarachnoid hemorrhage [SAH], intraventricular hemorrhage [IVH]) was 4.8%. The risks of transient or permanent neurological deficits were 2.9% (18/622) and 1.5% (9/622), respectively. Biopsy of deep-seated lesions was associated with increased overall complication rate, while biopsy of Glioblastoma Multiforme (GBM) was associated with perioperative mortality.

Conclusions:

Overall, complication rates were comparable with those in previous reports. The subgroup of patients with deep-seated lesions or a histologic diagnosis of GBM may possess an elevated risk of overall complications or mortality, respectively, compared to other patients undergoing frame-based stereotactic brain biopsy.
Left Eye Proptosis in an 11-Year-Old Child
Authors: Alkhani Ahmed
Published: December 2014

Aneurysmal bone cysts (ABC) are uncommon non-neoplastic expansile lesions of the bone. They usually affect long bones and vertebrae in childhood and early adulthood. Skull involvement is quite rare. Only a few cases of orbital involvement were described in the literatures. We are presenting a case of ABC that affects the orbit causing proptosis and external ophthalmoplegia.

An 11-year-old girl known to have a chronic seizure disorder and school performance difficulties presented with a four month history of rapidly progressing left orbital swelling and redness. This was associated with double vision, occasional throbbing headache and feelings of nausea. The patient and her family denied any history of trauma. Her past medical and family histories were otherwise non-contributory. Physical examination revealed a left eye proptosis with mild conjunctivitis (Figure 1). She had complete left external ophthalmoplegia with pupil sparing. The left orbit was non-tender with no detectable bruit. Visual acuity in both eyes was 20/20 (right) and 20/50 (left). Fundoscopic examination revealed mild papilledema in the left eye. The rest of the physical examination was essentially normal. Computerized tomography scan of the brain revealed a left fronto-orbital expansile bony lesion with multiple cystic compartments, distorting the left orbital roof (Figure 2). The lesion pushed the eye globe laterally and externally. Magnetic resonance imaging of the left orbit confirmed that the lesion was extradural, displacing rather than infiltrating the left frontal dura, causing mass effect on the left frontal lobe.Within the lesion, the cystic components exhibited fluid-fluid levels (Figure 3 a,b,c).
Spontaneous C1-2 CSF Leak Treated with High Cervical Epidural Blood Patch
Authors: Peng Philip WH , Farb Richard
Published: December 2014

Spontaneous intracranial hypotension (SIH) is a condition caused by spontaneous spinal cerebrospinal fluid (CSF) leaks. Clinically, it is characterized by orthostatic headache and may respond well to epidural blood patch. Other neurological presentations of SIH include diplopia, memory loss, hearing deficits, Parkinsonism, ataxia, obtundation and even coma.

Magnetic resonance imaging (MRI) is crucial in the diagnosis of this condition. Typical radiological features include diffuse pachymeningeal enhancement, descent of the cerebellar tonsil, a decrease in the size of prepontine and perichiasmatic cisterns, and subdural fluid collections. However, radiological imaging such as radionuclide cisternography or spinal MRI are not reliable in detecting the site of leakage. Myelography with iodinated contrast followed by thin-cut computed tomography (CT) or MRI of the entire spine has been shown as the technique of choice in defining the location of the CSF leak.
Early Identification and Incidence of Mild TBI in Ontario
Authors: Ryu Won Hyung A. , Feinstein Anthony , Colantonio Angela , Streiner David L. , Dawson Deirdre R.
Published: December 2014

Objectives:

(1) To examine the variability in diagnosis of mild traumatic brain injury (mTBI) in primary care relative to that of an expert reviewer; and (2) to determine the incidence rate of mTBI in Ontario, Canada.

Method:

Potential mTBI cases were identified through reviewing three months of Emergency Department (ED) and Family Physician (FP) health records. Potential cases were selected from ED records using the International Classification of Disease, 9th revision, Clinical Modification and External Cause codes and from all FPs records for the time period. Documented diagnoses of mTBI were compared to expert reviewer diagnosis. Incidence of mTBI was determined using the documented diagnoses and data from hospital catchment areas and population census.

Results:

876 potential mTBI cases were identified, 25 from FP records. Key indicators of mTBI were missing on many records (e.g., 308/876 records had Glasgow Coma Scale (GCS) scores). The expert reviewer disagreed with the documented diagnosis in 380/876 cases (kappa=0.19). The expert reviewer was more likely to give a diagnosis if the GCS was 13-14, if there was documented loss of consciousness and/or post-traumatic amnesia, and/or if there was pathology found on an acute brain scan. Calculated incidence rates of hospital-treated mTBI were 426 or 535/100,000 (expert review - hospital diagnosis). Including family physician cases increased the rate to 493 or 653/100,000.

Conclusion:

Health record documentation of key indicators for mTBI is often lacking. Notwithstanding, some patients with mTBI appear to be missed or misdiagnosed by primary care physicians. A more comprehensive case definition resulted in estimated incidence rates higher than previous reports.
Blood Glucose Control Among Critically Ill Patients with Brain Injury
Authors: Jacka Michael J. , Torok-Both Clinton J. , Bagshaw Sean M.
Published: December 2014

Objective:

To evaluate the incidence of hypoglycemia, hyperglycemia and blood glucose (BG) variability in brain-injured patients and their association with clinical outcomes.

Methods:

Retrospective cohort study of brain-injured patients admitted to an 11- bed neurosciences intensive care unit (ICU) from January 1 to December 31, 2003.

Results:

We included 606 patients. Mean age was 52.3 years, 60.6% were male, 11.9% had diabetes mellitus, and 64% were post-operative. Seventy-five (12.4%) received intensive insulin therapy (IIT) for a median (IQR) 72 (24-154) hours. Hypoglycemia and hyperglycemia occurred in 4.6% (96.4% receiving IIT) and 9.6% (77.6% receiving IIT). Median number of episodes per patient was 3 (75% with ≥2) and 4 (81% with ≥2) for hypoglycemia and hyperglycemia. Variable glycemic control occurred in 3.8% (100% receiving IIT) with median number of 13 episodes per patient. In-hospital mortality was 16.7%, median (IQR) ICU and hospital lengths of stay were 2 (1-5) and 8 (3-19) days. Hypoglycemia, hyperglycemia and BG variability showed non-significant but consistent associations with hospital mortality and prolonged lengths of ICU and hospital stay. The rate of recurrence of episodes showed stronger and significant associations with outcome, in particular for BG variability and hyperglycemia.

Conclusions:

Hypoglycemia, hyperglycemia and BG variability are relatively common in brain-injured patients and are associated with IIT. An increased frequency of episodes, in particular for BG variability and hyperglycemia, was associated with greater risk of both hospital death and prolonged duration of stay.
Mild to Moderate Early Exercise Promotes Recovery from Cerebral Ischemia in Rats
Authors: Lee Shi-Uk , Kim Dae-Yul , Park Sung-Hye , Choi Deok-Hyung , Park Hee-Won , Han Tai Ryoon
Published: December 2014

Objective:

We examined the effects of various exercise intensities on recovery from middle cerebral artery occlusion (MCAO) in rats.

Methods:

First, we administered a 120-minute left MCAO to male Sprague-Dawley rats and randomly assigned them to one of four groups: no exercise (Group 1), mild exercise (Group 2), moderate exercise (Group 3), and severe exercise (Group 4). Then, we trained the rats for 30 min per day for one week or two weeks. We used a five-point neurological evaluation scale to measure neurological deficits 1-day, 4-days, 7-days, 10-days and 14-days after MCAO and measured infarct volume by use of 2% 2,3,4-triphenyltetrazolium chloride in exercised brains. We also performed immunohistochemistry analysis of the brain to observe reactive astrocytosis at the peri-infarct region.

Results:

Neurological examination indicated that Group 2 and 3 recovered better than Group 1 after one week and two weeks (p<0.05). Moreover, Group 2 and 3 had reduced brain infarct volume compared with Group 1 after one week (p<0.05). There were no significant differences between Group 4 and Group 1. The thickness of the peri-infarct astrocytosis was significantly reduced in Group 4 relative to Group 1 after one week. There was a significant negative correlation between the extent of reactive astrocytosis and neurological recovery (r= -0.648, p<0.01).

Conclusion:

This study demonstrates that mild to moderate exercise that begins soon after induced cerebral ischemia promotes recovery and that astrocytes may have an important role in the recovery process.
Defining the CT Angiography ‘Spot Sign’ in Primary Intracerebral Hemorrhage
Authors: Thompson Andrew L. , Kosior Jayme C. , Gladstone David J. , Hopyan Julia J. , Symons Sean P. , Romero Francisco , Dzialowski Imanuel , Roy Jayanta , Demchuk Andrew M. , Aviv Richard I. ,   for the PREDICT/Sunnybrook ICH CTA Study Group
Published: December 2014

Purpose:

The computed tomogram angiography (CTA) ‘spot sign’ describes foci of intralesional enhancement associated with hematoma expansion in primary intracerebral hemorrhage patients. A consistent radiological definition is required for two proposed recombinant Factor VIIa trials planning patient dichotomization according to ‘spot sign’ presence or absence. We propose radiological criteria for diagnosis of the CTA ‘spot sign’ and describe different morphological patterns.

Material and Methods:

A prospective cohort of 36 consecutive patients presenting with primary intracerebral hemorrhage (ICH) were enrolled in a multicenter collaborative study, and have been included for the present analysis. Three reviewers analyzed the CTA studies in a blinded protocol. Analysis of specific ICH and ‘spot sign’ features was performed including prevalence, number, size, location, morphology and Hounsfield unit density.

Results:

Twelve of thirty-six patients (33%) demonstrated a total of 19 enhancing foci consistent with the CTA ‘spot sign’. Mean maximal axial ‘spot sign’ dimension was 3.7±2.2 mm and mean density was 216±57.7 HU. No significant differences in age or blood pressure (p=0.7), glucose (p=0.9), INR/PTT (p=0.3 and 0.4) or hematoma location (p=0.3) were demonstrated between patients with or without the ‘spot sign’. Consensus definition and classification criteria for the CTA ‘spot sign’ are proposed.

Conclusion:

The ‘spot sign’ is defined as spot-like and/or serpiginous foci of enhancement, within the margin of a parenchymal hematoma without connection to outside vessels. The ‘spot sign’ is greater than 1.5 mm in maximal dimension and has a Hounsfield unit density at least double that of background hematoma density.
Ataxia-Telangiectasia: Atypical Presentation and Toxicity of Cancer Treatment
Authors: Yanofsky Rochelle A. , Seshia Sashi S. , Dawson Angelika J. , Stobart Kent , Greenberg Cheryl R. , Booth Frances A. , Prasad Chitra , Del Bigio Marc R. , Wrogemann Jens J. , Fike Francesca , Gatti Richard A.
Published: December 2014

Background:

The onset of progressive cerebellar ataxia in early childhood is considered a key feature of ataxiatelangiectasia (A-T), accompanied by ocular apraxia, telangiectasias, immunodeficiency, cancer susceptibility and hypersensitivity to ionizing radiation.

Methods:

We describe the clinical features and course of three Mennonite children who were diagnosed with A-T following the completion of therapy for lymphoid malignancies.

Results:

Prior to cancer therapy, all had non-progressive atypical neurological abnormalities, with onset by age 30 months, including dysarthria, dyskinesia, hypotonia and/or dystonia, without telangiectasias. Cerebellar ataxia was noted in only one of the children and was mild until his death at age eight years. None had severe infections. All three children were “cured” of their lymphoid malignancies, but experienced severe adverse effects from the treatments administered. The two children who received cranial irradiation developed supratentorial primitive neuroectodermal tumors of the brain, an association not previously described, with fatal outcomes.

Conclusions:

The range of neurological presentations of A-T is broad. Ataxia and telangiectasias may be minimal or absent and the course seemingly non-progressive. The diagnosis of A-T should be considered in all children with neuromotor dysfunction or peripheral neuropathy, particularly those who develop lymphoid malignancies. The consequences of missing the diagnosis may be dire. Radiation therapy and radiomimetic drugs should be avoided in individuals with A-T.
Multiple Sclerosis: Change in Health-Related Quality of Life Over Two Years
Authors: Hopman Wilma M. , Coo Helen , Pavlov Andrey , Day Andrew G. , Edgar Catherine M. , McBride Evelyn V. , Brunet Donald G.
Published: December 2014

Background:

Cross-sectional research has demonstrated poorer function and health-related quality of life (HRQOL) in those with multiple sclerosis (MS) but less is known about change over time. The goals of this study were to measure change in HRQOL and identify factors associated with change.

Methods:

HRQOL was assessed at baseline and annually over two subsequent years using the Multiple Sclerosis Quality of Life Inventory. Function was assessed using the Expanded Disability Status Scale (EDSS) and the Multiple Sclerosis Functional Composite. Annualized rate of change was calculated for all twenty outcomes. Mixed effects modeling (univariate followed by multivariate) was used to examine the associations among patient characteristics and the age- and sex-adjusted Physical Component Summary (PCS) and Mental Component Summary (MCS) at study initiation and over the two years of follow-up.

Results:

Of 300 participants, 288 (96%) provided at least one assessment and are included in this analysis. Although 14 of the 20 outcomes showed a mean decline, only two (SF-36 physical function, p=0.018 and the EDSS, p<0.001) were statistically significant. The SF-36 social function showed a significant improvement (p=0.031). Only two variables were significantly associated with a decreased rate of decline or improvement over two years, including being female (PCS, p=0.001) and use of visiting nurse services (MCS, p<0.001).

Conclusions:

HRQOL is relatively stable over two years of follow-up, particularly for mentally-oriented outcomes. Further research with a longer period of follow-up is needed to provide additional insight into factors associated with change in HRQOL in patients with MS.
Experiences from an International Tele-Epilepsy Collaboration
Authors: Ahmed S. Nizam , Mann Carly , Siddiqui Fowzia , Sheerani Mughis , Syed Nadir Ali , Snyder Thomas , Enam S. Ather , Boling Warren
Published: December 2014

Objective:

Our main objective was to use videoconferencing as a primary means to: a) assist in launching an epilepsy surgery program in Pakistan; 2) participate in case conferences on complex epilepsy patients in each country.

Methods:

Extensive testing using both point to point and bridged integrated service digital network (ISDN) and internet protocol (IP) connections was carried out using bandwidths of 384-768 kilobits per second (kbps). Videoconferences between sites were arranged two to three weeks in advance and connections were tested a day prior to the scheduled conference. Sharing of PowerPoint presentations, neuroimaging and video-EEG was available to all sites. Discussions centered on patients with medically refractory epilepsy.

Results:

Between July 2006 and June 2008, 17 sessions were booked. Five of these conferences bridged in specialists from West Virginia University. Most successful connections occurred using IP point to point calls or a bridge connecting end points through IP at 512 kbps. We conducted three surgeries for medically refractory temporal lobe epilepsy in Pakistan. At follow-up in January 2009, two patients have been seizure free and one had two breakthrough seizures after sudden unsupervised discontinuation of Levetiracetam.

Conclusion:

Our international tele-epilepsy collaboration has proven feasible and valuable to all participants. Our experience suggests considerable thought and preparation are needed before a teleconference to ensure its success. We provide a recipe to set-up similar telemedicine collaborations. Considerations include time zone differences, equipment type, interoperability between endpoints, connection capabilities, bandwidth availability, and backup plans for unsuccessful connections. Telemedicine can facilitate epilepsy care around the world, identifying with the concept of a “Global Health Village”.
Pre-Operative Factors Affecting Resectability of Giant Intracranial Meningiomas
Authors: Quiñones-Hinojosa Alfredo , Kaprealian Tania , Chaichana Kaisorn L. , Sanai Nader , Parsa Andrew T. , Berger Mitchel S. , McDermott Michael W.
Published: December 2014

Objective:

Larger intracranial tumors require extended operating times and may be associated with increased perioperative complications. There are few reports describing the experience of resecting large meningiomas ≥5cm in a variety of locations. As a group, it remains largely unknown whether these relatively rare lesions are amenable to radical resection, and what factors influence their resectability.

Methods:

Sixty-seven patients undergoing surgery for a large intracranial meningioma (≥5cm in the longest dimension) between 1998 and 2004 were retrospectively reviewed. The surgeries were performed at a single institution University of California at San Francisco. Predictors of resectability were assessed via multivariate logistical regression analysis.

Results:

Thirty-nine (58%) patients underwent gross total resection (GTR) (Simpson grades I/II). There were no cases of perioperative mortality. At last follow-up, symptoms improved in 39 (58%) patients, remained unchanged in 20 (30%), and were aggravated in 8 (12%). In the multivariate model for all large meningiomas, age>45 years [OR(95%CI);0.127 (0.026-0.616),p=0.01] and superior sagittal sinus involvement [OR(95%CI);0.160 (0.026-0.976),p=0.05] were negative predictors of GTR, while preoperative embolization [OR(95%CI);8.087(1.719-38.044),p=0.008] was positively associated with GTR. For only supratentorial meningiomas, superior sagittal sinus involvement [OR (95%CI);0.077 (0.010-0.571),p=0.01] and preoperative embolization [OR(95%CI);10.492(1.961-56.135),p=0.006] were independently associated with GTR.

Conclusions:

This study evaluated a subset of large intracranial meningiomas ≥5cm. The results indicate that GTR can be achieved in the majority of cases with limited morbidity and mortality, where symptoms will likely improve and recurrences seldom occur. This study may provide useful insights for patients undergoing surgery for large intracranial meningiomas.
Cine Phase-Contrast MR Images Failed to Predict Clinical Outcome Following ETV
Authors: Di Xiao , Ragab M. , Luciano Mark G.
Published: December 2014

Background:

To predict success of endoscopic third ventriculostomy (ETV) in patients with obstructive hydrocephalus, we evaluated pre- and post- operative phase-contrast cine magnetic resonance images (PC MRI) on cerebrospinal fluid (CSF), cine flow in basal cisterns around the brain stem, and cervical medullar junction (CMJ) retrospectively.

Methods:

The study involved 102 patients with mean age of 36.74±23.25, and F/M = 1.55. All patients had PC MRI taken both pre- and post-operatively. A dynamic MRI video of PC MRI was reviewed at sagittal, axial, and coronal sections to determine cistern flows around the brain stem and CMJ. For quantitative analysis, quadrants were divided around midbrain axially to evaluate interpeduncular, quadrageminal, and ambient cisterns of both sides using scores of 0 to 60. Pre- and post- pontine and CMJ flows were shown in sagittal view and scored 0 to 20, and lateral cerebellopontine cisterns of both sides were measured on coronal image and scored 0 to 20.

Results:

No significant difference in CSF flow was seen from three individual views and total cine score, or between ETV success and failure groups by multivariate analysis of variance. Kaplan-Meier Analysis and Spearman's Correlation Test produced no relationship between MRI cine flow scores and interval period after surgery to ETV failure.

Conclusion:

PC MRI cine flow failed to demonstrate significant differences between successful and failed ETV groups. This indicates in addition to achieving an adequate fenestration, CSF pathways beyond the basal cisterns around the brain stem and CMJ may play an essential role in achieving ETV success.
Tension-Type Headache in Children: The Cinderella of Headache Disorders!
Authors: Seshia Shashi S. , Abu-Arafeh Ishaq , Hershey Andrew D.
Published: December 2014

Tension-type headache (TTH) may be as common a headache disorder as migraine in children and adolescents. TTH has a neurobiological basis with genetic and environmental factors making variable contributions to the different sub-types. The diagnostic criteria for TTH in the second edition of the “International Classification of Headache Disorders” appear to be applicable to children. Anxiety and mood disorders may be co-morbid with frequent episodic and chronic TTH. Psychosocial stressors play an important role in precipitating and maintaining TTH. Hence, a biopsychosocial approach should be adopted for care. Standardized histories and examinations together with prospective headache diaries are the foundations for good management; attention to ‘red flags’ will help identify secondary causes that present with headache similar to TT. There are no randomized controlled drug trials for the treatment of TTH. Relaxation and cognitive behavioral therapies are effective. TTH in children and adolescents warrants greater recognition from the clinician and scientist. Studies focusing on TTH are overdue.
Postoperative Surveillance Magnetic Resonance Imaging for Cerebellar Astrocytoma
Authors: Vassilyadi Michael , Shamji Mohammed F. , Tataryn Zachary , Keene Daniel , Ventureyra Enrique
Published: December 2014

Introduction:

Patients with low grade astrocytomas generally have good prognosis when total resection can be achieved, but surveillance neuroimaging is commonly performed to detect recurrence or progression. This study evaluated the utility and yield of such strategy for pilocytic and non-pilocytic cerebellar astrocytomas.

Methods:

A 20-year retrospective review was performed of patients undergoing resection of cerebellar astrocytoma at a single institution. A negative MRI string (NMS) ratio was computed as the fraction of total follow-up period over which surveillance neuroimaging was negative for recurrence or progression. Chi-squared analysis differentiated NMS ratio by resection extent and lesion histopathology.

Results:

Twenty-eight patients with pilocytic (n=15) and non-pilocytic (n=13) astrocytoma underwent 34 craniotomies, with total resection in 19 cases. Surveillance MRIs (n=167) among total resection patients were uniformly negative for recurrent disease at average seven years follow-up (NMS ratio = 1.0). The 43 surveillance MRIs among subtotal resection patients revealed disease progression in two patients within six months of operation (NMS ratio = 0.78, p<0.05). No differences in NMS ratio were observed between pilocytic and non-pilocytic astrocytoma subtypes.

Discussion:

This study illustrates pediatric patients with low-grade cerebellar astrocytomas undergoing total resection may not benefit from routine surveillance neuroimaging, primarily because of low recurrence likelihood. Patients with subtotal resection may benefit from surveillance of residual disease, with further work aimed at exploring the schedule of such follow-up.
The Cerebellar Dysplasia of Chiari II Malformation as Revealed by Eye Movements
Authors: Salman Michael S. , Dennis Maureen , Sharpe James A.
Published: December 2014

Introduction:

Chiari type II malformation (CII) is a developmental deformity of the hindbrain. We have previously reported that many patients with CII have impaired smooth pursuit, while few make inaccurate saccades or have an abnormal vestibuloocular reflex. In contrast, saccadic adaptation and visual fixation are normal. In this report, we correlate results from several eye movement studies with neuroimaging in CII. We present a model for structural changes within the cerebellum in CII.

Methods:

Saccades, smooth pursuit, the vestibulo-ocular reflex, and visual fixation were recorded in 21 patients with CII, aged 8-19 years and 39 age-matched controls, using an infrared eye tracker. Qualitative and quantitative MRI data were correlated with eye movements in 19 CII patients and 28 controls.

Results:

Nine patients with CII had abnormal eye movements. Smooth pursuit gain was subnormal in eight, saccadic accuracy abnormal in four, and vestibulo-ocular reflex gain abnormal in three. None had fixation instability. Patients with CII had a significantly smaller cerebellar volume than controls, and those with normal eye motion had an expanded midsagittal vermis compared to controls. However, patients with abnormal eye movements had a smaller (non-expanded) midsagittal vermis area, posterior fossa area and medial cerebellar volumes than CII patients with normal eye movements.

Conclusions:

The deformity of CII affects the structure and function of the cerebellum selectively and differently in those with abnormal eye movements. We propose that the vermis can expand when compressed within a small posterior fossa in some CII patients, thus sparing its ocular motor functions.
Seroprevalence ofChlamydia PneumoniaeAntibodies in Stroke in Young
Authors: Bandaru V. C. S. Srinivasarao , Boddu D. Babu , Laxmi V. , Neeraja M. , Kaul S.
Published: December 2014

Background:

Younger patients, aged below 45 years, usually lack the conventional risk factors of stroke whereas infections, especially in developing countries, may play a role. There have been many reports in the last decade about the association ofChlamydia pneumoniae(C.pneumoniae) and atherosclerosis involving cerebral vessels.

Objective:

To investigate the seroprevalence ofC.pneumoniaeIgG and IgA antibodies in patients aged below 45 years with acute ischemic stroke.

Methods:

This study was done at a tertiary care hospital in South India between January 2004 and December 2006 where we recruited consecutive patients aged less than 45years with acute ischemic stroke. Age and sex matched controls were recruited from the outpatient department with non stroke diagnosis. All stroke patients underwent CT (Computerized Tomography), MRI (Magnetic Resonance Image), MRA (Magnetic Resonance Angiography), Transthoracic Echocardiography and Carotid Doppler for stroke sub group diagnosis. We measuredC.pneumoniaeantibodies IgG and IgA by microimmunofluorescence technique in all patients and controls.

Results:

A total of 120 patients and 120 controls were studied over a period of two years. We foundC.pneumoniaeantibodies in 29.1% (35/120) stroke patients and in 12.5% (15/120) control subjects (p=0.002).C.pneumoniaeIgG antibodies were found in 27.5 % (33/120) of stroke patients and 12.5% (15/120) of controls (p=0.006). IgAantibodies were observed in 5 % (6/120) of strokes and none in control group (p=0.03). After adjustment of all risk factorsC.pneumoniaeIgG seropositivity showed odds ratio of 2.6; 95% Confidence Interval 1.2-5.6.

Conclusions:

C.pneumoniaeIgG antibodies were found to be associated with ischemic stroke in young.
Evaluating an Organized Palliative Care Approach in Patients with Severe Stroke
Authors: Blacquiere Dylan P. V. , Gubitz Gord J. , Dupere David , McLeod Deborah , Phillips Stephen
Published: December 2014

Background:

A recent survey found few guidelines on the provision of palliative care following stroke; none examined the efficacy or results of any such process. The role of the patient's family in decision making and in conflicts with staff has not been evaluated. We sought to formally evaluate the use of locally-developed palliative care guidelines on our Acute Stroke Unit (ASU).

Methods:

We retrospectively examined records of 104 patients who died on our ASU over a two-year period to determine if our existing palliative guidelines were reflected in clinical practice, and to identify conflicts that arose. Data on medical and nursing care, palliative decisions, and medication use were compared to the ASU's existing palliative care guidelines. Family concerns about the palliative process were also reviewed.

Results:

Of patients admitted to the stroke unit, 104 (16% of total admissions) died. Ninety-four (90.4%) of these were palliated; all received routine nursing and comfort care prior to death. Median time from admission to palliation was 3.6 days; median time from admission to death was 8.5 days. Most had vital signs (98.9%), investigations (100%) and non-palliative medications (95.7%) stopped, and had nasogastric feeding (96.8%) and intravenous fluids (87.2%) withdrawn or never begun. Most were treated with morphine (93.6%) and scopolamine (81.9%). Concerns raised by family members centered around hydration and feeding (45.7%), doubts about palliative care (27.8%) and patient comfort (18.2%).

Conclusions:

A formal approach to palliation results in timely decisions regarding end of life care with relatively few conflicts. Further work to address the specific concerns of families is needed.
Evaluation of ThinkFirst for Kids Injury Prevention Curriculum for Grades 7/8
Authors: Vassilyadi Michael , Duquette Cheryll , Shamji Mohammed F. , Orders Shari , Dagenais Simon
Published: December 2014

Background:

Head injury causes substantial morbidity and mortality in children. The ThinkFirst For Kids (TFFK) program improves knowledge of safe behaviours in kindergarten to Grade 6 students.

Methods:

This study evaluated the TFFK curriculum for grade 7/8 students. Knowledge acquisition was assessed quantitatively by an injury prevention test at baseline, at curriculum completion, and six weeks later. Participant experiences and behaviours were explored qualitatively by interviews and focus groups.

Results:

Students (n=204) and teachers (n=6) from four schools participated in this study. Test scores improved from baseline (26.48±0.17, n=204), to completion (27.75±0.16, n=176), to six weeks post-completion (28.65±0.13, n=111) (p<0.05). Most students reported their decision-making and participation in risky behaviors was altered by the curriculum.

Interpretation:

The TFFK curriculum may promote education about head injury prevention among Grade 7/8 students, with a suggestion of long-term knowledge retention. The curriculum was wellreceived and may be suitable to reduce risky behavior and injuries in children.