bluuebunny/crossref_metadata_2025_split huggingface dataset:
Occipital Condyle Syndrome as the First Sign of Metastatic Cancer
Authors: Moeller Jeremy J , Shivakumar Sudeep , Davis Mary , Maxner Charles E 
Published: December 2014

Background:Occipital condyle syndrome is characterized by severe, unilateral, occipital headache and ipsilateral twelfth-nerve palsy. It is associated with skull-base metastasis.Cases:We identified two patients with sub-acute onset of severe, unilateral, occipital headache and ipsilateral tongue paralysis. The first patient was a 58-year-old woman with a history of limited stage small-cell lung cancer in clinical remission. The second patient was an otherwise healthy 36-year-old man. Neither patient had any other findings on general medical or neurological examination. One patient had only equivocal findings on initial magnetic resonance imaging (MRI), and the other patient's MRI was normal. Although initial work-up for metastatic disease was normal, the first patient developed severe bone pain over the next few months, and follow-up investigations demonstrated metastases to her spine, tibia, skull base and brain. The second patient improved initially, but was admitted to hospital three months later with constitutional symptoms and pancytopenia. Bone marrow and lymph node biopsies were consistent with Stage IVB Hodgkin's lymphoma.Conclusion:Occipital condyle syndrome can be the first presentation of disseminated malignancy. Initial imaging of the brain and skull base may be normal, and recognition of this syndrome warrants thorough investigation and close follow-up.
Endovascular Management of an Embedded Intracranial Knife
Authors: Darsaut T E , Ashforth R A , Chow M M , Findlay J M 
Published: December 2014

Background:Endovascular therapy (ET) is infrequently used in the setting of acute penetrating intracranial trauma.Methods:We report a case where ET was used immediately prior to and following removal of an embedded intracranial knife, which was found to be disrupting the anterior cerebral artery.Results:The proximal vessel was coiled and angiographically occluded and then the knife was removed with the microcatheter in place. Immediate angiography allowed us to see and quickly treat the contrast extravasation with further coiling. Despite full medical management, the patient died of elevated intracranial pressure.Conclusion:In this severely injured patient, endovascular therapy represented the most suitable means to attempt safe removal of the knife.
Seizure Semiology: Value in Identifying Seizure Origin
Authors: Jan Mohammed M.S. , Girvin John P. 
Published: December 2014

ABSTRACT:The diagnosis of epilepsy depends upon a number of factors, particularly detailed and accurate seizure history, or semiology. Other diagnostic data, consisting of electroencephalography, video-monitoring of the seizures, and magnetic resonance imaging, are important in any comprehensive epilepsy program, particularly with respect to lateralizing and localizing the seizure focus, if such a focus exists, and with respect to determining the type of seizure or seizure syndrome. The aim of this review is to present a survey of important semiologic characteristics of various seizures that provide the historian with observations, which help to lateralize and localize epileptic zones. Clinical semiology is the starting point of understanding a seizure disorder and making the diagnosis of epilepsy. While it may not provide unequivocal evidence of localization of the epileptic focus, nevertheless it usually directs subsequent investigations, whose concordance is necessary for the ultimate localization.
Multidisciplinary Treatment for Headache in the Canadian Healthcare Setting
Authors: Sauro Khara M. , Becker Werner J. 
Published: December 2014

Background/Objectives:Multidisciplinary treatment programs are seen as an effective way to treat patients with chronic illness. The purpose of this study was to describe a multidisciplinary headache program which was developed in the Canadian public healthcare setting, and to report on patient perceptions of the program and patient treatment outcomes.Methods:The Calgary Headache Assessment and Management Program (CHAMP) was developed with initial funding from Alberta Health, and continued with function from the Calgary Health Region. Patient perceptions of the program were obtained with questionnaires. Outcome measures for a cohort of patients who completed the Self-Management Workshop were obtained using standard headache related disability measures.Results:Patient perceptions of the education session, the Lifestyle Assessment, and the Self-Management Workshop were very positive. Headache Disability Inventory scores fell from 56.2 to 46.3 from baseline to three months post Self-Management Workshop (p<.001). Corresponding scores for the HIT-6 were 63.6 and 58.2 (p <.001).Conclusions:Multidisciplinary headache treatment programs can be developed in the Canadian public healthcare system. The program described here was well accepted by many patients and perceived to be useful by them. Headache related disability as measured by standard measures was significantly reduced after participation in the program.
3D Conformal Radiotherapy and Cisplatin for Recurrent Malignant Glioma
Authors: VanderSpek Lauren , Fisher Barbara , Bauman Glenn , Macdonald David 
Published: December 2014

Purpose:To determine the maximum tolerated dose of 3D conformal radiotherapy in combination with Cisplatin for patients with recurrent malignant gliomas.Methods:From 1999-2003, nine patients with recurrent malignant glioma received fractionated radiotherapy and Cisplatin (20 mg/m2/d IV on days 1-5) in a Phase I radiation dose escalation trial. Three sequential dose levels were evaluated: 25 Gy, 30 Gy, and 35 Gy, using 5 Gy fractions. All patients received prior external beam radiation (median dose 59.4 (20-60) Gy) and five patients received prior chemotherapy.Results:Six male and three female patients were enrolled with a median age of 52 years, and a median Karnofsky performance status score of 70. The median re-irradiated tumor volume was 18.9 (0.1-78.5) cm3 and the median follow-up was 8.8 (3.2-31.2) months. One patient (30 Gy/ 6 fractions) experienced medically reversible acute grade 3 toxicity. A second patient (35 Gy/ 7 fractions) experienced acute grade 2 toxicity and histology showed tumor and radiation effect. A third patient (25 Gy/ 5 fractions) experienced late grade 3 toxicity from radiation necrosis. The radiological responses consisted of complete response (1 patient), partial response (1 patient), and stable disease (2 patients). The median overall survival was 8.8 months (95% CI 8.0-9.9), and the median disease free interval was 2.0 months (95% CI 1.4-4.4). Seven patients received chemotherapy following re-irradiation and Cisplatin.Conclusion:The maximum tolerated dose of 3D conformal fractionated radiotherapy was 30 Gy in 6 fractions with low dose Cisplatin, which was well tolerated in terms of acute toxicity for our patient population. This regimen demonstrated only modest efficacy in the treatment of recurrent malignant glioma. Combinations of conformal re-irradiation and other systemic agents may merit investigation. Currently our recommended dose is 30 Gy in 6 fractions for selected patients.
Outcome of Stereotactic Radiotherapy for Patients with Uncontrolled Acromegaly
Authors: Imran Syed Ali , Fleetwood Ian G. , O'Connell Colleen M. , Ransom Thomas P. , Mulroy Liam A. , Ur Ehud , Clarke David B. 
Published: December 2014

Objective:Linear accelerator based stereotactic radiation therapy (SRT) has been used for the treatment of pituitary tumours; however, little is known concerning the use of this modality for the treatment of patients with acromegaly. We have prospectively studied the short-term outcome of SRT in 12 acromegaly patients who failed to achieve biochemical remission despite surgery and/or pharmacologic therapy.Methods:We identified all patients who had biochemically uncontrolled acromegaly and were treated with SRT between April 2003 and December 2006. All patients were followed prospectively based on a pre-defined protocol that included Goldman visual field examination, MRI of the sella, and pituitary hormone testing at 3, 6, 12 months, and then yearly.Results:A total of 12 patients with acromegaly were treated with SRT. There were 9 females and the median age of the group was 50 years. The median follow-up was 28.5 months during which time the mean tumor volume decreased by 40%, the median GH fell from 4.1 μg/L to 1.3 μg/L (p=0.003) and the median IGF-1 dropped more than half from 545.5 μg/L to 260.5 μg/L (p=0.002). Four patients achieved normal, while an additional 2 achieved near-normal, IGF-1 levels. One patient was able to discontinue and two were able to reduce their acromegaly medications while maintaining a normal IGF-1. A new pituitary hormonal deficit was found at 24 months in one patient who developed hypoadrenalism requiring corticosteroid replacement.Conclusion:Based on our early experience, we believe that SRT should be considered in treating patients with uncontrolled acromegaly.
Gait Apraxia in Multiple Sclerosis
Authors: Abou Zeid Nuhad E. , Weinshenker Brian G. , Keegan B. Mark 
Published: December 2014

Background:Gait apraxia is a gait disorder not attributable to motor, cerebellar, or sensory dysfunction. Gait impairment is common in Multiple Sclerosis (MS), but is mostly attributed to spasticity and ataxia. Impairment ratings scales are designed accordingly and do not separately evaluate apraxia.Objective:To describe 15 patients with gait apraxia resulting from MS as their major functional impairment.Methods:The Mayo Clinic database (1994-2007) was searched for the terms MS and gait apraxia. Inclusion criteria: Definite MS, significant gait apraxia. Exclusion criteria: alternative disorder causing apraxia, predominantly spastic/ataxic gait disorder.Results:9 (60%) of the patients were women, and 12 (80%) had a progressive MS course. Gait apraxia was evident at a median of 8 years (range 0-34) following MS onset. Median EDSS at recognition of gait apraxia was 6.5 (range 5-8). Cognitive dysfunction was present in 11 (73%) and neurogenic bladder dysfunction in 14 (93%). The commonest MRI findings were confluent periventricular T2 lesions, T1 hypointensity and generalized cerebral atrophy with symmetrical ex vacuo ventricular enlargement.Conclusion:Gait apraxia can cause significant functional impairment in MS patients, and may be underrecognized. The natural course of the neurological deficit in such patients is unknown, and may differ from that of MS patients with other ambulatory disabilities.
The Epidemiology of Hospitalized Head Injury in British Columbia, Canada
Authors: Phillips Leah A. , Voaklander Don C. , Drul Colleen , Kelly Karen D. 
Published: December 2014

Objective:This descriptive study seeks to identify the incidence rates of head injuries in a large Canadian province, given incident cases for a ten year period. It describes cases in terms of age standardized rates, demographics, and health care utilization.Methods:The analyses were done using descriptive statistics. Incidence rates were calculated using the direct method. The indicators of hospital resource utilization were: mean length of hospital stay, number of intensive care unit (ICU) stays, and mean length of stay in an ICU.Results:In the ten year period, British Columbia saw 48,753 admissions due to an incident head injury. The most common head injury diagnosis was an “Intracranial” injury. The year with the highest total age standardized rate was 1991/92 (174.18/100 000). The mean length of hospital stay was 7.4 days. Ten percent had an ICU stay and the mean length of stay was 4.4 days (± 4.8). The diagnosis with the longest mean length of stay was a “Fractured Skull” while of the top five E-code categories; “Motor Vehicle Traffic” had the highest mean length of stay with 12.2 days.Conclusions:Our study provides a much needed analysis of the incidence of head injuries in British Columbia. These rates can be compared to other provinces using the 2001 Canadian population as the standardized population. Our results indicate that there are certain “at risk” groups that warrant attention, in particular, younger men with lower socioeconomic standing. Indicators of health care utilization presented in the study should generate policy discussions.
Effects of Surgical Excision and Radiation on Medulloblastoma Cell Invasiveness
Authors: Ranger Adrianna , McDonald Warren , Bauman Glenn S. , Del Maestro Rolando 
Published: December 2014

Objectives:Surgical resection and adjuvant radiation are mainstays of medulloblastoma (MB) patient management. We utilized a novel 3-dimensional assay to identify how (a) radiation, (b) excision of the primary tumour aggregate, and (c) both treatments combined influence MB cell invasiveness.Methods:Five MB cell lines (UW228-1, 2 and 3; Daoy, and Madsen) were implanted onto a 3-dimensional, type I collagen gel assay to assess tumour invasion distance over five days, in response to (1) needle-assisted excision of the central cell aggregate; (2) pre-exposure to single-dose and fractionated dose irradiation in doses from 6-25 and 8-24 Gy, respectively; and (3) excision plus either single-dose or fractionated radiation.Results:Within hours, individual MB cells detached from the surface of the cell aggregates and invaded the collagen matrix, to distances up to 1200 μm and at rates up to 300 μm daily. The UW228-1 cell line was less invasive than the other cell lines and was dropped from further analysis. In the four remaining lines, a dose-dependent decline in tumour invasiveness was identified, both for single-dose and fractionated radiation, which achieved statistically decreased invasion distances at higher doses, especially of fractionated irradiation. Excision of the central tumour aggregate tended towards exerting a late effect on cell invasion, but exerted no significant influence on the radio-sensitivity of residual cells.Conclusions:Both single-dose and fractionated dose irradiation appear to inhibit MB cell invasiveness in a dose-dependent manner, whereas excision of the central cell aggregate exerts no effect on residual invading cells.
Contrast Echocardiography and Migraine in Divers with Patent Foramen Ovale
Authors: Di Fabio Roberto , Giugni Elisabetta , Angeloni Imerio , Vanacore Nicola , Casali Carlo , Pierallini Alberto , Vadalà Rita , Pierelli Francesco 
Published: December 2014

Background:It has been proposed that the patent foramen ovale (PFO) may be associated with migraine, in particular migraine with aura. However, it is not clear whether paradoxical embolism triggers crises of headache. Cerebral embolization is provoked in subjects with PFO through contrast echocardiography, a safe method to diagnose the presence of foramen ovale pervium.Methods:Twenty-four men practicing diving, an activity characterized by increased prevalence of PFO and migraine, underwent trans-thoracic echocardiography with contrast solution, composed of saline and air mixture and checked for the occurrence of migraine in the following 24 hours.Results:A PFO (five of minimal size, i.e. visible only during Valsalva, one of small and two of medium size) was detected in 8/24 divers (33%). No one reported headache over the 24 hours after the procedure.Discussion:Our preliminary data suggest that cerebral micro-embolism, provoked by contrast echocardiography, does not systematically trigger migraine crises when a minimal-to-medium sized patent foramen ovale is present.
Endovascular Therapy of Hypoglossal Canal AVFs Presenting with Orbital Symptoms
Authors: Miropolsky Vladislav , da Costa Leodante B. , Marotta Thomas R. , Spears Julian 
Published: December 2014

Objective:Dural arteriovenous fistulae (DAVF) of the hypoglossal canal region are rare lesions. We describe three cases of DAVF of the hypoglossal canal presenting with ocular symptoms and discuss the endovascular management options.Methods:Three consecutive patients with DAVF of the hypoglossal canal region presented with proptosis, chemosis and disturbances of extra-ocular mobility. Each patient was treated using a different endovascular approach, based on variations of the vascular access.Results:The cases and treatments are reviewed, with a literature review on the subject. Endovascular treatment, transvenous or trans-arterial was curative in all cases.Conclusion:DAVF of the hypoglossal canal region can present with ocular manifestations very similar to DAVF of the cavernous sinus or carotid-cavernous fistulas. Endovascular treatment is usually feasible and effective, but an understanding of the vascular anatomy and pathophysiology of the disease are of utmost importance when planning the approach.
Pediatric Traumatic Dural Arteriovenous Fistula
Authors: Vassilyadi Michael , Mehrotra Naveen , Shamji Mohammed F. , Michaud Jean 
Published: December 2014

Background:Dural arteriovenous fistulas are uncommon lesions in children, with traumatic lesions suspected to arise from incomplete arterial injury in proximity to a vein or draining sinus. Management of symptomatic acquired lesions requires evaluation of patient presentation, neurological status, and pathoanatomic configuration, with special consideration required for surgery secondary to failed endovascular technique.Case Report:A 12-year-old male sustained a bicycle fall causing a right temporo-parietal skull fracture associated with non-surgical right epidural hematoma and left contre-coup parietal contusion. Six-weeks later, he complained of a right temporal bruit with subsequent cerebral angiography demonstrating a dural-based fistula between the right middle meningeal artery and a dural vein draining into the sigmoid sinus.Intervention:Endovascular treatment of this lesion with glue embolization and coiling was unsuccessful, with angiographic illustration of previously unobserved collateral vessels and coils occupying the sigmoid sinus. A right temporo-parietal craniectomy was required to excise the dural-based fistula, followed by dural defect repair with bovine pericardium and subsequent cranioplasty. Six years later the patient remains neurologically intact with no headaches or bruit.Conclusions:Dural arteriovenous fistula can uncommonly occur following traumatic injury in children. Partial injury to the middle mengineal artery may have established arterial communication with the draining vein that became ectatic and tortuous under high pressure. Failure of primary endovascular treatment may complicate secondary surgical intervention.
CTG Expansion & Haplotype Analysis in DM1 Gene in Healthy Iranian Population
Authors: Shojasaffar Bahareh , Moradin Neda , Kahrizi Kimia , Cobo Ana Maria , Najmabadi Hossein 
Published: December 2014

Myotonic dystrophy type 1 (DM1) is due to an unstable expansion of CTG repeat in the DMPK gene (19q13.3). The CTG repeat is highly polymorphic (5 to 37) in healthy individuals. According to the hypothesis that expanded (CTG)n alleles originated from larger normal alleles, there may exist a correlation between the prevalence of DM1 and the frequency of large size normal alleles. Strong linkage disequilibrium between different length alleles and the three biallelic markers, Alu, Hinf1 and Taq1, has been reported.Objective:To determine the distribution of normal alleles, the frequency of larger normal alleles and analysis of the three biallelic markers, in healthy Iranian controls.Material and Methods:Polymerase chain reaction (PCR) was conducted on two hundred unrelated healthy individuals from different ethnic groups living in Iran to determine the size of the alleles. Markers were analyzed by PCR/RFLP on 174 chromosomes from other control healthy individuals.Results:Our data reveals that 23.7% of alleles had 5 CTG repeats and 7.2% of alleles had >18 CTG repeats. The analysis of haplotypes revealed that 75% of CTG5 and 80% of CTG>18 had the (+++) haplotype.Conclusion:The frequency of alleles with CTG>18 in Iran is similar to that of Western Europe and Japan.
Deducing the Bioactive Face of Hydantoin Anticonvulsant Drugs Using NMR Spectroscopy
Authors: Tiedje Kathryn E , Weaver Donald F 
Published: December 2014

Background:The general purpose of this study was to deduce the geometry of the bioactive face (pharmacophore) for the hydantoin class of anticonvulsants.Methods:Six hydantoin analogs, selected as probes of hydantoin structure, were synthesized. Nuclear magnetic resonance spectroscopy and molecular modelling calculations were used to determine the geometric relationship between the aromatic group and the amide group in the hydantoin pharmacophore.Results:In accord with both theoretical and experimental results, the biologically inactive hydantoin analogs containing a benzyl substituent existed in a folded conformation with the benzene flopped over the hydantoin ring. Conversely the biologically active hydantoins had a phenyl ring extended away from the hydantoin ring.Conclusions:The bioactive face for hydantoins consists of a N(H)-C(=O)-X-phenyl molecular fragment, where X is a carbon or nitrogen atom and where the distance between the centre of the amide bond and the centroid of the phenyl ring is 4.3 Å.
Sturge-Weber Syndrome. Study of 55 Patients
Authors: Pascual-Castroviejo Ignacio , Pascual-Pascual Samuel-Ignacio , Velazquez-Fragua Ramón , Viaño Juán 
Published: December 2014

Purpose:To review the clinical and neuroimaging features of a large series of patients with Sturge-Weber syndrome (SWS) seen over a 40-year period.Methods:Fifty-five patients with SWS (30 males and 25 females), were studied between 1965 and 2004. Results of neurological and ophthalmological examinations, electroencephalographic, and neuroimaging studies were reviewed. All patients were seen by one of the authors (I. P-C).Results:Epilepsy, hemiparesis, mental retardation and ocular problems were the most frequent and severe features of patients with Sturge-Weber syndrome in this series. The facial nevus flammeus was unilateral in 35 (63.5%) patients, bilateral in 17 (31%) and absent in 3 (5.5%) of the patients with leptomeningeal angiomas. Seven (41%) of the 17 patients with bilateral nevus flammeus had unilateral leptomeningeal angiomas. Seizures occurred in 47 patients (85.5%). Complete seizure control was obtained in 20 patients (42.5%), but in 2 of these 20 patients seizures were controlled only after lobectomy. All patients with unilateral or bilateral upper eyelid nevus flammeus had ipsilateral, unilateral or bilateral choroid-retinal angiomas. Only 20 (36%) of the 55 patients had low-normal or borderline intelligence (IQs<70). No relationship was observed between the size of the facial nevus flammeus and the severity of the brain lesion.Conclusions:Epilepsy, hemiparesis, mental retardation and ocular problems were the most frequent and severe features of patients with Sturge-Weber syndrome in this series. Cerebral lesions followed a progressive course during early childhood, but not later. Early surgical treatment controlled the seizures but other neurological problems such as hemiparesis and intellectual deficits showed a less satisfactory response. Early onset of seizures and poor response to medical treatment, bilateral cerebral involvement and unilateral severe lesions were indicative of a poor prognosis. Limited intelligence and social skills, poor aesthetic appearance and seizures complicated the integration of SWS patients. These features must be addressed in order for the patients improve social interactions, obtain gainful employment and achieve a better quality of life.