bluuebunny/crossref_metadata_2025_split huggingface dataset:
Endovascular Treatment of a “Blister-like” Aneurysm of the Internal Carotid Artery
Authors: McNeely P.D. , Clarke D.B. , Baxter B. , Vandorpe R.A. , Mendez I. 
Published: December 2014

Backgound:“Blister-like” aneurysms of the supraclinoid internal carotid artery have recently been recognized as having unique pathological and clinical features. Little is known regarding their optimal treatment modality.Methods:We report a case of a “blister-like” aneurysm of the internal carotid artery treated with Guglielmi detachable coil (GDC) embolization. Case Report: A 55-year-old man presented with a Hunt & Hess grade II subarachnoid hemorrhage. Computed tomography revealed diffuse subarachnoid blood. Cerebral angiography demonstrated a broad-based bulge on the medial wall of the right distal internal carotid artery. The patient was taken to the operating room and underwent a right pterional craniotomy and wrapping of this unclippable aneurysm. On postoperative day 11, he developed signs of vasospasm, and repeat angiography showed remarkable growth of the aneurysm. The aneurysm was believed to be amenable to endovascular therapy and was treated by GDC embolization. The patient recovered well and remained neurologically intact on follow-up examinations. Repeat cerebral angiography was performed three and nine months following his initial presentation and revealed a significant aneurysm neck remnant. This neck remnant was treated by repeat GDC embolization 13 months following his subarachnoid hemorrhage.Conclusion:“Blister-like” aneurysms of the internal carotid artery are important to recognize and are difficult to manage using traditional surgical approaches. Early repeated cerebral angiography is indicated and, where appropriate, endovascular therapy should be considered in the management of these patients.
Minimum Standards for Electromyography in Canada: A Statement of the Canadian Society of Clinical Neurophysiologists
Authors: Bolton C.F. , Benstead T.J. , Grand'Maison F. , Tardif G.S. , Weston L.E. 
Published: December 2014

Background:Electromyography (EMG) is a widely used diagnostic technique for disorders of the nervous system. The Canadian Society of Clinical Neurophysiologists (CSCN) promotes the education, evaluation and standards of EMG in Canada. A statement of practice standards was needed to clarify the position of the CSCN on several issues relevant to the practice of EMG.Methods and Results:A subcommittee of the CSCN reviewed current patterns of practice and established guidelines for review by the CSCN. The guidelines developed by the subcommittee were reviewed by the CSCN and adopted as recommendations for EMG practice. The subcommittee was charged with formulation of a document for publication.Conclusion:This document deals with minimum standards for electromyographer education, laboratory operation, equipment and a variety of special circumstances relevant to the practice of EMG. The standards can be adopted by EMG laboratories to guide quality assurance.
Vertebral Artery Dissection: Warning Symptoms, Clinical Features and Prognosis in 26 Patients
Authors: Saeed Abdullah Bin , Shuaib Ashfaq , Emery Derek , Al-Sulaiti Ghanem 
Published: December 2014

Background and objectives:Internal carotid artery dissection has been extensively studied and well-described. Although there has been a recent increase in the number of reported cases of vertebral artery (VA) dissection, the clinical variety of presentation and the early warning symptoms have not been well-described before. Our objectives in this study include: (1) To determine the early symptoms and warning signs which may help the clinician in the early identification and treatment of patients with VAdissection. (2) To explore the variety of clinical presentation of VA dissection and its relation to prognosis.Design and setting:Retrospective analysis of hospital records in a tertiary academic centre for the period 1989-1999.Results:Twenty-six patients were identified (13 men and 13 women). The mean age was 48. Possible precipitating factors were identified in 14 patients (53%). Sporting activity and chiropractic manipulations were the most common (15% and 11% respectively). Headache and/or neck pain was the prominent feature in 88% of patients and was a warning sign in 53%, preceding onset of stroke by up to 14 days. The most common clinical features included vertigo (57%), unilateral facial paresthesia (46%), cerebellar signs (33%), lateral medullary signs (26%) and visual field defects (15%). Bilateral VA dissection presented in six patients (24%). The most common region of dissection was the C1-C2 level (16 arteries, 51%). Intracranial VAdissection was found in eight arteries (25%). The majority of patients (83%) had favorable outcome. Poor prognosis was associated with (1) bilateral dissection; (2) intracranial VA dissection accompanied by subarachnoid hemorrhage. Only two patients reported stroke recurrence.Conclusion:Our findings show that VA dissection affects mainly middle age persons and involves both sexes equally. Headache and/or neck pain followed by vertigo or unilateral facial paresthesia is an important warning sign that may precede onset of stroke by several days. Although the majority of patients will have excellent prognosis, this was less likely in patients presenting with subarachnoid hemorrhage or bilateral VA dissection. Recurrence rate was low.
Familial Adenomatous Polyposis and Benign Intracranial Tumors: A New Variant of Gardner's Syndrome
Authors: Leblanc Richard 
Published: December 2014

Introduction:Familial adenomatous polyposis (FAP) is associated with malignant tumors of the central nervous system, predominantly medulloblastomas and glioblastoma multiforme (Turcot's syndrome) and with craniofacial osteomas (Gardner's syndrome). This report details the occurrence of benign, intracranial tumors in two related individuals with Gardner's syndrome, an asso ciation not previously described.Patients and Methods:A 57-year-old woman (the propositus), her sister, two of her nieces and one of her grandnephews were previously diagnosed with Gardner's syndrome. The propositus came to neurosurgical attention because of vertigo associated with what proved to be an epidermoid cyst of the cerebellopontine angle. Her unaffected children and her relatives with Gardner's syndrome were examined and underwent computed tomography or magnetic resonance imaging.Results:A 39-year-old woman with Gardner's syndrome, the niece of the propositus, was found to harbor an asymptomatic left frontal meningioma.Discussion:Familial adenomatous polyposis, Gardner's syndrome, and that variant of Turcot's syndrome in which medulloblastoma predominate, are associated with a mutation of the adenomatous polyposis coli gene. The demonstration that patients with Gardner's syndrome can also have benign, nonneuroglial, intracranial tumors adds to the previously known extracolonic lesions associated with FAP. The molecular characterization of our patients should reveal if benign intracranial tumors represent a pleiotropic manifestation of the adenomatous polyposis coli gene mutation or if other genes are implicated.
PCV for Oligodendroglial Tumors: In Search of Prognostic Factors for Response and Survival
Authors: Fortin David , Macdonald David. R. , Cairncross J. Gregory , Stitt Larry 
Published: December 2014

Background:We report survival and pretreatment prognostic factors for survival and chemosensitivity in 53 oligodendrogliomas treated with PCV (procarbazine, lomustine and vincristine) chemotherapy.Methods:A total of 53 patients with histologically proven oligodendroglioma, anaplastic oligodendroglioma or oligo-astrocytoma and treated with PCVwere extracted from the London Regional Cancer Center database. A retrospective review was conducted to evaluate overall survival and pretreatment prognostic factors for survival and chemosensitivity.Results:The median survival time from diagnosis was 123.6 months. The overall five- and ten-year survival rates were 72.7% and 52.7% respectively. Age <40, seizure as an initial symptom, absence of cognitive deficit and presence of a homogeneous hypodense lesion without contrast enhancement on the initial pretreatment CT scan were all factors independently associated with favorable outcome. The presence of increased cellularity, pleomorphism, mitosis, vascular proliferation and grading as an anaplastic lesion using these surrogates on pathological assessment, were all associated with an unfavorable outcome in univariable analysis. In multivariable analysis, only the anaplastic grading and presence of increased cellularity were significant determinants of unfavorable survival. The only factor adversely associated with chemosensitivity was the presence of a focal symptom at presentation.Conclusion:Overall survival is significantly longer in oligodendroglial lesions than in fibrillary astrocytic tumors. A two tier grading system using standard morphological features seems accurate in predicting outcome in these patients. The presence of a neoplastic astrocytic component does not seem to impact the outcome. No clinical, radiological or pathological factor could be identified to reliably predict chemotherapy response.
The Epidemiology of Infantile Spasms
Authors: Brna P.M. , Gordon K.E. , Dooley J.M. , Wood E.P. 
Published: December 2014

Objective:The aim of this study was to estimate population based incidence rates for infantile spasms (IS) and to study our clinical impression that the incidence of IS has recently decreased in the Canadian Provinces of Nova Scotia and Prince Edward Island.Methods:Birth cohorts from 1978 to 1998, identified through the hospital health records, EEG records and physician computerized databases, were followed for two years for the development of IS. Disease incidence rates were calculated using denominators derived from Statistics Canada's reported annual live birth rates.Results:The inclusion criteria for IS were fulfilled by 75 patients. The overall incidence of IS was 30.7/100,000 live births (95% CI 24.3, 38.8). Etiologic classification was symptomatic for 51 cases (68%), cryptogenic for 18 (24%), and idiopathic in six children (8%). Although there were more males (N=44) than females (N=31), the incidence rates were similar. There was a marked variability in annual and five-year incidence rates.Conclusion:Although the clinical characteristics of our patients were similar to other reported IS populations, the instability in IS incidence rates indicates a need for caution in interpreting smaller IS epidemiologic studies.
Parent Perceptions of the Value of Pediatric Neurology Clinic Visits
Authors: Dooley J.M. , Gordon K.E. , Wood E.P. 
Published: December 2014

Background:There is little data available on the acceptability to parents, of alternate methods of delivering care to their children. This pilot study explored parents'perceptions of conventional clinic visits and their attitudes to potential alternative methods of delivering care.Methods:Questionnaires were completed by the families of 200 consecutive children before and after a visit to a tertiary-care Pediatric Neurology Clinic in Nova Scotia, Canada.Results:Responses were received from 172 (86%) for the first questionnaire and 138 (69%) for the second. There were 59 new referrals, 76 follow-up visits and 39 were seen because of new concerns. Visiting the clinic resulted in school absenteeism for 85% of the children and lost pay for 19% of the parents. Telephone, video conference or replacement of the physician by a nurse practitioner were acceptable alternate methods of assessment for only 10%. The only factor which was associated with willingness to accept telephone as an assessment modality was an initial opinion that the visit was unnecessary.Conclusion:Escalating health care costs and limited specialist availability demand consideration of alternate methods of care delivery. Although adults seem comfortable with care outside the usual clinic setting, there is little data for the pediatric setting. The responses from the parents attending our clinic, indicate that families were happy with services offered in the conventional clinic. In contrast, only 10% found potential alternate methods acceptable. These opinions must be considered in the design of new methods of caring for children.
The Role of MRI and Nerve Root Biopsy in the Diagnosis of Neurosarcoidosis
Authors: Moore Fraser G.A. , Andermann Frederick , Richardson John , Tampieri Donatella , Giaccone Robert 
Published: December 2014

Objectives:Neurological involvement occurs in 5-15% of patients with sarcoidosis and isolated “neurosarcoidosis” occurs in less than 1% of all cases. Classical clinical presentations have been described, such as bilateral facial palsy, but often the disease presents insidiously with varied signs and symptoms. We present a patient who required biopsy of a lumbar nerve root for diagnosis of chronic, progressive neurosarcoidosis and review the literature with an emphasis on diagnosis.Methods:We have reviewed a patient who presented with signs and symptoms related to infiltration of her meninges and nerve roots by sarcoidosis. All pertinent history and physical information was taken from interviews with the patient and review of her chart. Laboratory, radiographic, and pathological investigations are presented.Results and conclusions:A high index of suspicion is required for the diagnosis of neurosarcoidosis. Gadolinium-enhanced MRI is useful but the findings are often nonspecific, and there should be a low threshold for biopsy whenever the diagnosis is considered.
Transient Hemichorea/Hemiballismus Associated with New Onset Hyperglycemia
Authors: Ifergane G. , Masalha R. , Herishanu Y.O. 
Published: December 2014

Objective:To describe three patients suffering from transient hemichorea/hemiballismus associated with hyperglycemia, review previous reports and propose a possible pathophysiological explanation for this phenomenon.Results:Our original cases and previously reported ones reveal a uniform syndrome: mostly female patients (F/M ratio of 11/2), 50-80 years old, usually with no previous history of diabetes mellitus (9/13), develop choreic or ballistic movements on one side of the body over a period of hours. Serum glucose levels are elevated. In most of the patients, a lowering of the blood sugar level reverses the movement disorder within 24-48 hours.Conclusions:We believe that the combination of a recent or old striatal lesion (causing increased inhibition of the subthalamic nucleus) and hyperglycemia (causing decreased GABAergic inhibition of the thalamus) may be responsible for the appearance of this unilateral hyperkinetic movement disorder. Undiagnosed diabetes mellitus should always be suspected in patients who develop hemiballistic or hemichoreic movements. When hyperglycemia is detected and corrected, the movement disorder usually resolves within two days and may not require symptomatic therapy with dopamine receptor antagonists.
Lactate Stress Testing in 155 Patients with Mitochondriopathy
Authors: Finsterer Josef , Milvay Erika 
Published: December 2014

Objective:Few data are available about the diagnostic yield of the lactate stress test (LST) in a large group of patients with mitochondriopathy (MCP).Methods:Serum lactate was determined once before, three times during, and once after a 15-minute, constant 30W workload on a bicycle in 62 controls, aged 17 to 84 years, 155 patients with MCP, aged 17 to 87 years, and 31 patients with neurological disorders other than MCP.Results:Lactate's upper reference limits at rest, 5, 10, 15 minutes after starting, and 15 minutes after finishing the exercise were 2.0, 2.1, 2.1, 2.1 and 1.8 mmol/l respectively. The test was regarded abnormal if more than two of the five lactate values exceeded the cut-off levels. Among the 103 patients with abnormal LST, 64 (62 %) had normal resting lactate. The sensitivity of the test was 67% and the specificity 94%.Conclusion:The LST proved to have a high sensitivity and specificity in the detection of patients with MCP, being thus a simple but powerful tool to assess the impaired oxidative metabolism in MCP patients.
The Clinical Profile of Nonmotor Fluctuations in Parkinson's Disease Patients
Authors: Gunal Dilek Ince , Nurichalichi Kerim , Tuncer Nese , Bekiroglu Nural , Aktan Sevinç 
Published: December 2014

Objective:Recently described nonmotor fluctuations may cause disability in Parkinson's disease patients. These fluctuations are generally grouped as sensory, autonomic and psychiatric. The clinical spectrum and frequency of these fluctuating symptoms are not well-described.Methods:We studied the relationship of nonmotor fluctuations with motor symptoms and determined the influence of age at disease onset, duration of disease, dosage and duration of levodopa treatment in the appearance of nonmotor fluctuations.Results:Statistical analysis showed a relationship of disease-related parameters with sensory and autonomic fluctuations but psychiatric fluctuations were only found to be associated with the duration of levodopa usage. The nonmotor fluctuations included in the study were observed during “on” periods as well as “off” periods.Conclusion:Nonmotor fluctuations had variable presentations. Moreover, their co-appearance with different types of motor fluctuations may be linked to the effect of other neurotransmitter systems acting synchronously with dopamine. Risk factors for sensory and autonomic fluctuations in patients with Parkinson's disease were early age of disease onset, longer duration and higher dose of levodopa use. Psychiatric fluctuations were only associated with higher doses of levodopa.
Tapping and Peg Insertion after Levodopa Intake in Treated and de novo Parkinsonian Patients
Authors: Müller Thomas , Benz Sabiene , Przuntek Horst 
Published: December 2014

Background:Investigators use instrumental tasks for objective assessment of parkinsonian motor disability and its drug response. To date, such studies on treated parkinsonian patients have not addressed acute and long-term effects of dopaminergic drugs.Objectives:To determine the impact of long-term dopaminergic therapy within a standardized levodopa challenge test design in combination with two repeatedly performed instrumental tasks, peg insertion and tapping, in previously treated and untreated parkinsonian patients.Results:Tapping significantly deteriorated in previously untreated, but not in treated parkinsonian patients after levodopa intake. In contrast, motor symptoms and peg insertion significantly improved in both groups of parkinsonian patients. Results of both tests differed between parkinsonian patients and matched controls.Conclusion:Worsening of cognitively less demanding tapping may result from upregulated presynaptic inhibitory feedback regulation, sedative effects of levodopa or dopamine overflow in untreated parkinsonian patients, who are sensitive to these effects in contrast to treated parkinsonian patients. Tapping is a task with autonomic repetitive performance and programming of standardised movements with a low need for cognitive effort. This autonomic functioning of attentional control and selective processing is intact in Parkinson's disease. Peg insertion depends on more complex movements and thus hypothetically on dopamine-associated cognitive processes. Therefore, impairment of peg insertion responded to dopaminergic stimulation in both groups of parkinsonian patients. Future studies on the efficacy of antiparkinsonian drugs, using instrumental tasks for objective assessment, should consider long-term impact of antiparkinsonian drug therapy and associated cognitive efforts.
Idiopathic Free-Floating Thrombus of the Common Carotid Artery
Authors: Silver Brian , Gulka Irene , Nicolle Michael , Sahjpaul Ramesh , Hachinski Vladimir 
Published: December 2014

Background:The observation of an intraluminal common carotid artery thrombus overlying a wall defect at ultrasonography or angiography is unusual. To our knowledge, there are no previous reports of a free-floating thrombus in the common carotid artery.Case Report:A 45-year-old woman who was previously healthy and on no medications presented with acute hemiparesis and aphasia. Following testing that included carotid duplex and trancranial Doppler ultrasonography, diffusion-weighted magnetic resonance imaging, and digital subtraction angiography, the patient underwent emergency open embolectomy. No underlying wall defect was seen at the time of imaging or surgery. No obvious hypercoagulable state could be identified. Her NIH Stroke Scale score improved from 26 at admission to 2 at three months and 1 at one year.Conclusions:Multimodal imaging may have improved diagnosis and management in this patient with a unique finding. The source of the thrombus remains obscure.
Single Dose versus Fractionated Stereotactic Radiotherapy for Meningiomas
Authors: Lo Simon S. , Cho Kwan H. , Hall Walter A. , Kossow Ronald J. , Hernandez Wilson L. , McCollow Kim K. , Gerbi Bruce J. , Higgins Patrick D. , Lee Chung K. , Dusenbery Kathryn E. 
Published: December 2014

Objective:To evaluate the safety and efficacy of stereotactic radiosurgery (SRS) compared to fractionated stereotactic radiation therapy (FSRT) for meningiomas treated over a seven year period.Methods and materials:Of the 53 patients (15 male and 38 female) with 63 meningiomas, 35 were treated with SRS and the 18 patients with tumors adjacent to critical structures or with large tumors were treated with FSRT. The median doses for the SRS and the FSRT groups were 1400 cGy (500- 4500 cGy) and 5400 cGy (4000-6000 cGy) respectively. Median target volumes for SRS and FSRT were 6.8 ml and 8.8 ml respectively. The median follow-up for the SRS and FSRT groups were 38 months (4.1-97 months) and 30.5 months (6.0-63 months) respectively.Results:The five-year tumor control probability (TC) for benign versus atypical meningiomas were 92.7% vs. 31% (P=.006). The three-year TC were 92.7% vs. 93.3% for SRS vs. FSRT groups respectively (P=.62). For benign meningiomas, the three-year TC were 92.9% vs. 92.3% for the SRS group (29 patients) vs. FSRT group (14 patients) respectively (P=.77). Two patients in the SRS group and one in the FSRT group developed late complications.Conclusion:Preliminary data suggest that SRS is a safe and effective treatment for patients with benign meningiomas. Fractionated stereotactic radiation therapy with conventional fractionation appeared to be an effective and safe treatment alternative for patients not appropriate for SRS. A longer follow-up is required to determine the long-term efficacy and the toxicity of these treatment modalities.