bluuebunny/crossref_metadata_2025_split huggingface dataset:
Syndrome de vasoconstriction cérébrale segmentale réversible ou angéite primitive du Systeme nerveux central?
Authors: Gerretsen Philip , Kern Ralph Z. 
Published: December 2014

Background:Reversible Cerebral Vasoconstriction Syndrome (RCVS) may present as thunderclap headache (TCH), accompanied by reversible cerebral vasospasm and focal neurological deficits, often without a clear precipitant. RCVS may be mistaken for Primary Angiitis of the Central Nervous System (PACNS) due to the presence of similar angiographic features of segmental narrowing of cerebral arteries. We discuss the clinical features of a young female migraine patient who developed TCH and was found to have RCVS following initial treatment with corticosteroids for PACNS, in the context of a systematic review of the available medical literature.Methods:A Medline™ search was performed to identify all case reports since 1966 describing RCVS and PACNS that provide sufficient clinical detail to permit diagnostic classification according to published criteria. RCVS included case studies in which there was angiographic or transcranial Doppler ultrasound evidence of near-to-complete resolution of cerebral vasoconstriction in the absence of a well-recognized secondary cause. PACNS included reports of histologically confirmed PACNS either through biopsy or necropsy.Results:Reversible Cerebral Vasoconstriction Syndrome occurs primarily in females and is characterized by sudden, severe headache at onset, normal CSF analysis, vasoconstriction involving the Circle of Willis and its immediate branches, and angiographic or TCD ultrasound evidence of near-to-complete vasospastic resolution within 1-4 weeks. It occurs typically in the context of vasoconstrictive drug use, the peripartum period, bathing, and physical exertion.Conclusion:Initial and follow-up (within 4 weeks) non-invasive angiographic studies are indicated in patients who present with TCH or who have clinical presentations that could be consistent with RCVS or PACNS in the absence of a well-recognized secondary cause, such as subarachnoid haemorrhage. Early reversibility of cerebral vasospasm is the key neuroradiological feature that supports the clinical diagnosis of RCVS.
Metabolic Myopathies Discovered During Investigations of Statin Myopathy
Authors: Baker Steven K. , Vladutiu Georgirene D. , Peltier Wendy L. , Isackson Paul J. , Tarnopolsky Mark A. 
Published: December 2014

The statins have emerged as the dominant class of drug for the treatment of hypercholesterolemia. These medications are generally well tolerated. However, myalgias, the most frequent side-effect, occur in up to 7% of patients. Transaminitis and skeletal myotoxicity, with elevated serum creatine kinase (CK) levels (i.e., >10 times the upper limit of normal), occur with reported frequencies of 1% and 0.1%, respectively. Various hypotheses have been proposed to explain the relationship between statin therapy and the spectrum of muscle dysfunction manifested by myalgia, myopathy, and rhabdomyolysis.Statin-mediatd inhibition of mevalonate metabolism impairs the synthesis of isoprenylated products–the most notable of which is ubiquinone. However, isoprenylation is responsible for the post-translational modification of up to 2% of cellular proteins. Therefore, numerous metabolic pathways are potentially modified by statin-mediated hypoprenylation. Subclinical defects in one or more energy-deriving pathways may be unmasked upon exposure to the pleotropic effects of statins. Such pharmacogenomic synergism may underlie the development of “statin myopathy” in a subset of patients. In this regard, we describe four patients with mutations in the myophosphorylase (PYGM; MIM 232600), myoadenylate deaminase (AMPD1; MIM 102770), and carnitine palmitoyltransferase (CPT2; MIM 600650) genes whose diagnoses became apparent during the course of investigations for statin-induced myalgias and hyperCKemia.
Anterior Corpectomy Approach for Removal of a Cervical Intradural Schwannoma
Authors: Casha Steven , Xie Jing Cheng , Hurlbert R. John 
Published: December 2014

Spinal schwannomas are typically intradural-extramedullary neoplasms thought to arise from Schwann cells or their progenitors which occur proportionally throughout the spinal canal. They most typically arise from dorsal sensory rootlets and occupy a posterior-lateral location in the spinal canal. Thus, posterior surgical procedures have become the conventional method to remove these tumors providing adequate exposure in most cases. More anteriorly located tumors may be approached through a posterolateral direction with section of the dentate ligament and gentle rotation of the spinal cord. However, posterior and posterolateral approaches may be problematic for removing tumors located in the midline and ventral to the spinal cord.Although the anterior approach has been applied widely to treat cervical spondylosis, it has rarely been used to remove intradural tumors. Here, we present a case of a ventral cervical spinal schwannoma removed through an anterior approach followed by spinal reconstruction.
α-B-Crystallin as a Tissue Marker of Epileptic Foci in Paediatric Resections
Authors: Sarnat Harvey B. , Flores-Sarnat Laura 
Published: December 2014

Background:We studied α-B-crystallin, a small heat shock chaperone protein upregulated by various “stresses”, as an immunocytochemical tissue marker of epileptic foci.Methods:We examined 45 resected brain tissues of epileptic patients, 16 months to 23 years. Postmortem brains of 2 epileptic children and 20 normal fetuses and neonates of 10-41 weeks gestation similarly were studied. Immunocytochemical demonstration of α-B-crystallin was supplemented by neuronal, glial and inflammatory cell markers and electron microscopy (EM) in surgical cases. Autopsy brain tissue of children without epilepsy or neurological disease served as controls.Results:In all resections, α-B-crystallin was overexpressed in astrocytes and oligodendrocytes, including satellite cells adherent to neurons, and occasionally in neurons of neocortex, hippocampus and amygdala. In six cases, reactivity was most intense at or near the epileptic focus, with a diminishing gradient of intensity for 2-3 cm; similar focal expression was seen in autopsy cases. Presence or absence of histological structural lesions was independent of α-B-crystallin expression. Balloon cells and giant atypical cells in tuberous sclerosis were intensely reactive. Reactivity was present in DNETs. No correlation occurred with microglial activation, inflammation or gliosis; no ultrastructural alterations were seen. No expression was seen in fetal brains at any age.Conclusions:Immunoreactive α-B-crystallin is a reliable tissue marker of epileptic foci, regardless of presence or absence of structural lesions; at times it maps the extent of a focus.
Temporolimbic Activation by Intracranial Electrical Stimulation
Authors: Ure Jorge A. , Olivier André , Quesney Luis Felipe , Bravo Mauricio , Perassolo Mónica 
Published: December 2014

Background:To evaluate the results of intracranial electrical stimulation (ICES) as a pre-surgical tool in order to select the side of the operation in bitemporal lobe epilepsy (BTLE) patients who underwent depth electrode (DE) implantation.Methods:We reviewed the files of 77 medically intractable BTLE patients who underwent ICES with positive results through implanted DEs and then were under surgical treatment. One year or more after surgery, we evaluated the outcome. ICES was performed through: 1) Square-wave bipolar stimulation with symmetrical pulses of 60 Hz for 0.5 ms was delivered by a constant current Nuclear Chicago stimulator; 2) An initial intensity of 0.5 mA, and subsequently progressively stronger currents at 1-2 and occasionally 3 or 4 mA; 3) The duration of a single stimulation was usually 5 seconds; 4) The volume of tissue effectively stimulated did not exceed 5 mm.Results:We obtained habitual auras or seizures (clinical responses, CRs) in 74 patients and after-discharges, ADs in 61 of them, according to Engel's classification for post surgery outcomes. If CRs or ADs were obtained by stimulation of only one temporal lobe the result of epilepsy surgery tended to be better (Engel classes I or II) when the operation was done on the same side of positive CRs (15 cases) or ADs (14 cases), and tended to be worse (Engel classes III or IV) when the ICES had provoked bilateral responses or when the side operated on was contra-lateral to positive CRs (33 cases) or ADs (28 cases). Statistical analyses were performed in order to test these results and we found better post-operative results when the resection took place in the same side of positive responses to ICES (CRs: χ2 4.74 and p=0.0295; ADs: χ2 7.57 and p=0.0059).Conclusion:In addition to other methods (PET, MRI and neuropsychology) presurgical ICES can provide useful data in the process of identifying the temporal lobe to be targeted for resection in BTLE patients.
Detection of Cognitive Impairment and Dementia Using the Animal Fluency Test: The DECIDE Study
Authors: Sebaldt Rolf , Dalziel William , Massoud Fadi , Tanguay André , Ward Rick , Thabane Lehana , Melnyk Peter , Landry Pierre-Alexandre , Lescrauwaet Benedicte 
Published: December 2014

Objectives:To evaluate the performance of a one-minute screening test measured against a validated 10-minute screening test for mild cognitive impairment (MCI) in detecting CI in patients aged ≥ 65 years with two or more vascular risk factors (VRF).Methods:Patients (n=1523) aged 65 years or older without documented CI symptoms or dementia with two or more VRF participated in this study set in Canadian primary care practice. Baseline data was collected, followed by the 1-minute animal fluency (AF) test and the 10-minute Montreal Cognitive Assessment (MoCA). Physicians (n=122) completed case reports during patient interviews and reported their diagnostic impression. AF test sensitivity, specificity, and accuracy in predicting a positive MoCA was assessed.Results:Study sample mean age was 79.7 years, 55% were female, 97.6% were Caucasian and 75% had ≤ 12 years of education. The AF test and MoCA detected CI in 52 and 56 percent of the study population, respectively. The AF test demonstrated sensitivity, specificity, and accuracy in predicting a positive MoCA of 67 percent each. Physicians diagnostic impression of MCI was reported for 37% of patients, and of dementia for 6%.Conclusion:In an elderly population with at least two VRF, using AF can be useful in detecting previously unknown symptoms of CI or dementia. Screening for CI in this high risk population is warranted to assist physician recognition of early CI. The short AF administration time favours its incorporation into clinical practice.
Can Admission S-100β Predict the Extent of Brain Damage in Head Trauma Patients?
Authors: Schültke E. , Sadanand V. , Kelly M.E. , Griebel R.W. , Juurlink B.H.J. 
Published: December 2014

Background:As has been shown previously, S-100β levels in serum can be a useful predictor of brain damage after head trauma. This pilot study was designed to investigate whether urine samples, which are much easier to obtain, could be used for the same purpose instead of serum samples.Methods:Ninety-six consecutive patients admitted with head trauma were recruited in the study. After exclusion of 54 patients, mostly because of significant additional trauma, S-100β levels were analyzed in serum and urine of 42 patients using a luminometric assay. A range for normal values was established based on samples from ten healthy volunteers.Results:S-100β serum levels increased proportional to the severity of the head trauma, as had been previously shown by several other groups. In many patients, initial increases in urine S-100β levels were seen later than in serum, after which the kinetics of S-100β levels in urine seemed to follow that established for serum levels. S-100β values in urine were on average about 54% lower in urine than in serum.Conclusions:S-100β levels in urine obtained on admission to the hospital are not a good indicator for the extent of brain damage. However, urine S-100β levels obtained at later time points might be a useful indicator for the development of secondary brain injury.
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.
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.
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.