bluuebunny/crossref_metadata_2025_split huggingface dataset:
Metastatic Hemangiopericytoma Presenting as an Epidural Spinal Cord Lesion
Authors: Brass Steven David , Guiot Marie-Christine , Albrecht Steffen , Glikstein Rafael , Mohr Gérard 
Published: December 2014

Objective:Hemangiopericytoma is an aggressive vascular tumour that rarely affects the central nervous system and is even more rarely spinal in presentation. The clinical features of a patient with a recurrent extraspinal hemangiopericytoma presenting with an epidural spinal cord compression by local invasion are described, including a review of the literature on metastatic hemangiopericytoma to the spine.Methods:A case of a 53-year-old male, with a recurrent extraspinal hemangiopericytoma which metastasized to the thoracic spine five years after detection of the primary tumour is presented. A chart review was conducted where all pertinent history, physical, laboratory, and radiological data were collected. A Pub-Med search using the keyword “hemangiopericytoma” identified all reported cases documenting clinical features, treatment, recurrence and outcome with respect to metastatic hemangiopericytoma to the spine.Results:Nine patients have been reported to have metastatic hemangiopericytoma to the spine. The median patient age was 47 years and there was a slight male preference. An unusual feature of the hemangiopericytoma is the prolonged period, up to 16 years, between the diagnosis of the primary hemangiopericytoma and the metastases to the spine. All patients were treated with a combination of radiation and surgery.Conclusion:Hemangiopericytomas show a slow clinical evolution with a strong propensity to relapse long after previous treatment and thus, once identified, prolonged follow-up for recurrence is indicated. A close follow-up of these patients is required because of frequent recurrences and delayed metastases even if the primary lesion was well-controlled. Although overall uncommon, hemangiopericytoma should be kept in mind in the differential diagnosis of vascular epidural spinal cord tumours.
REM Sleep Behaviour Disorder in a Child with Tourette's Syndrome
Authors: Trajanovic Nikola N. , Voloh Inna , Shapiro Colin M. , Sandor Paul 
Published: December 2014

Purpose:To describe an association of Tourette's syndrome with rapid eye movement sleep behaviour disorder (RBD) in a prepubescent boy.Methods:A four year longitudinal single-case study.Results:The co-existence of Tourette's syndrome and RBD was confirmed after polysomnographic studies using the standard criteria. The authors propose possible overlap in the pathophysiological mechanisms underlying the two disorders.
Overview of Traumatic Brain Injury Patients at a Tertiary Trauma Centre
Authors: Guise Elaine de , Feyz Mitra , LeBlanc Joanne , Richard Sylvain-Luc , Lamoureux Julie 
Published: December 2014

ABSTRACT:Objective:The goal of this study was to provide a general descriptive and cognitive portrait of a population with traumatic brain injury (TBI) at the time of their acute care stay.Material and methods:Three hundred and forty-eight TBI patients were assessed. The following data were collected for each patient: age, level of education, duration of post-traumatic amnesia, Galveston Orientation Amnesia Test score, Glasgow Coma Scale score, results of cerebral imaging, Neurobehavioral Rating Scale score, the Functional Independence Measure cognitive score and the Glasgow Outcome Scale score.Results:The clinical profile of the population revealed a mean age of 40.2 (±18.7) and a mean of 11.5 (±3.6) years of education. Most patients presented with frontal (57.6%) and temporal (40%) lesions. Sixty-two percent had post-traumatic amnesia of less than 24 hours. Seventy percent presented with mild TBI, 14% with moderate and 15% with severe TBI. The cognitive deficits most frequently observed on the Neurobehavioral Rating Scale were in the areas of attention, memory and mental flexibility as well as slowness and mental fatigability. Most patients had good cognitive outcome on the Functional Independence Measure and scores of 2 and 3 were frequent on the GOS. Forty-five percent of the patients returned home after discharge, 51.7% were referred to in or out patient rehabilitation and 3.2% were transferred to long-term care facilities.Conclusion:Because of the specialized mandate of acute care institutions, the information provided here concerning characteristics of our TBI population is essential for more efficient decision-making and planning/programming with regards to care and service delivery.
Reducing Hardware-Related Complications of Deep Brain Stimulation
Authors: Constantoyannis Constantine , Berk Caglar , Honey Christopher R. , Mendez Ivar , Brownstone Robert M. 
Published: December 2014

ABSTRACT:Background:Deep brain stimulation (DBS) is used increasingly worldwide for the treatment of Parkinson's disease, dystonia, tremor and pain. As with any implanted system, however, DBS introduces a new series of problems related to its hardware. Infection, malfunction and lead migration or fracture may increase patient morbidity and should be considered when evaluating the risk/benefit ratio of this therapy. This work highlights several factors felt to increase DBS hardware complications.Methods:The authors undertook a prospective analysis of their patients receiving this therapy in two Canadian centres, over a four-year period.Results:One hundred and forty-four patients received 204 permanent electrode implants. The average follow-up duration was 24 months. Complications related to the DBS hardware were seen in 11 patients (7.6%). There were two lead fractures (1.4%) and nine infections (6.2%) including two erosions (1.4%). There was a significantly greater risk of infection in patients who underwent staged procedures with externalization. In patients with straight scalp incisions, the rate of infection was higher than that seen with curved incisions.Conclusion:Hardware complications were not common. A period of externalization of the electrodes for a stimulation trial was associated with an increased infection rate. It is also possible that a straight scalp incision instead of curvilinear incision may lead to an increase in the rate of infection. With a clear understanding of the accepted DBS device indications and their potential complications, patients may make a truly informed decision about DBS technology.
Pseudoephedrine-induced Hemorrhage Associated with a Cerebral Vascular Malformation
Authors: Baker Steven K. , Silva Jamie E. , Lam Ken K.S. , Baker Steven K. 
Published: December 2014

ABSTRACT:Background:Sympathomimetic-related intracerebral hemorrhage is well-documented. Most cases are associated with phenylpropanolamine use.Case Report:We report a case of intracerebral hemorrhage occurring in a middle-aged man who suffered from chronic sinusitis and had been ingesting pseudoephedrine daily for one year. This patient was previously well with no known cardiovascular risk factors. Clinical examination revealed no evidence of vasculitis nor coagulopathy and initial neuroimaging (i.e., computed tomography, angiography, magnetic resonance imaging) demonstrated no features consistent with aneurysm, arteriovenous malformation (AVM), cavernoma, nor cerebral metastases. A follow-up cerebral angiogram demonstrated a small AVM arising off a branch of the pericallosal artery and a small arteriovenous fistula arising off the costal marginal branch. The AVM was embolized without incident, however, the AVF was not accessible.Conclusions:Sympathomimetics have long been associated with intracerebral hemorrhage. Since 1979, over 30 published case reports have documented the relationship between phenylpropanolamine and stroke. Only one report links phenylpropanolamine consumption to an intracerebral hemorrhage in a patient with an AVM. There is a paucity of literature etiologically inculpating other ephedra alkaloids in the causation of intracerebral hemorrhage. This is a case of pseudoephedrine-induced intracerebral hemorrhage in a patient with an underlying vascular malformation.
Adrenomyeloneuropathy: Report of a New Mutation in a French Canadian Female
Authors: Dionne Annie , Brunet Denis , McCampbell Alexander , Dupré Nicolas 
Published: December 2014

ABSTRACT:Background:X-linked adrenoleukodystrophy is a peroxisomial disorder caused by mutations in the ABCD1 gene. Adrenomyeloneuropathy is the second most frequent phenotype (25-46%) of this disease and classically presents in adulthood with spastic paraparesis. Female heterozygotes can be symptomatic, but they are frequently misdiagnosed as having multiple sclerosis.Case report:We report a novel missense mutation in the ABCD1 gene in a 47-year-old French-Canadian female with spastic paraparesis and no confirmed family history of X-linked adrenoleukodystrophy. The mutation is located on exon 1 and causes the amino acid substitution of a valine for an alanine in a region of the protein highly conserved between mouse and man.Conclusion:Adrenomyeloneuropathy must be considered in the differential diagnosis of spastic paraparesis in men or women. This is an initial report of an ABCD1 gene mutation in the French-Canadian population, which should lead to the recognition of other cases in the future.
Canadian Association of Neuroscience Review: Respiratory Control and Behavior in Humans: Lessons from Imaging and Experiments of Nature
Authors: Moss Immanuela Ravé 
Published: December 2014

ABSTRACT:The purpose of this review is to demonstrate that respiration is a complex behavior comprising both brainstem autonomic control and supramedullary influences, including volition. Whereas some fundamental mechanisms had to be established using animal models, this review focuses on clinical cases and physiological studies in humans to illustrate normal and abnormal respiratory behavior. To summarize, central respiratory drive is generated in the rostroventrolateral medulla, and transmitted to both the upper airway and to the main and accessory respiratory muscles. Afferent feedback is provided from lung and muscle mechnoreceptors, peripheral carotid and aortic chemoreceptors, and multiple central chemoreceptors. Supramedullary regions, including cortex and subcortex, modulate or initiate breathing with volition, emotion and at the onset of exercise. Autonomic breathing control can be perturbed by brainstem pathology including space occupying lesions, compression, congenital central hypoventilation syndrome and sudden infant death syndrome. Sleep-wake states are important in regulating breathing. Thus, respiratory control abnormalities are most often evident during sleep, or during transition from sleep to wakefulness. Previously undiagnosed structural brainstem pathology may be revealed by abnormal breathing during sleep. Ondine's curse and 'the locked-in syndrome' serve to distinguish brainstem from supramedullary regulatory mechanisms in humans: The former comprises loss of autonomic respiratory control and requires volitional breathing for survival, and the latter entails loss of corticospinal or corticobulbar tracts required for volitional breathing, but preserves autonomic respiratory control.
Visual-Sensitive Epilepsies: Classification and Review
Authors: Seshia Shashi S. , Carmant Lionel 
Published: December 2014

ABSTRACT:Photosensitivity, photosensitive seizures, and photosensitive epilepsy are discussed. The International League against Epilepsy has suggested the term “visual-sensitive” replace “photosensitive”. Visual-sensitive seizures may be more common than is realized. A classification for visual-sensitive epilepsies is presented. Chromosomal and DNA testing may help to refine the classification further. A standardized approach for neurophysiologic testing, such as that proposed by European experts, is recommended. These steps should promote evidenced-based management for this group of disorders.
Inventory of Pediatric Neurology “Manpower” in Canada
Authors: Keene Daniel L. , Humphreys Peter 
Published: December 2014

ABSTRACT:Objective:To review the demographics and workload characteristics of pediatric neurology in Canada.Method:A standardized survey questionnaire was mailed out to practicing pediatric neurologists in Canada in 2001. Variables examined were age, gender, hours on call, regular hours worked per week, type of practice and projected changes in practice over next five to ten years. Results were compared to the 1994 Pediatric Neurology Manpower Survey which had used the same survey instrument.Results:Fifty-six (70%) pediatric neurologists practicing in Canada returned the survey. As was the case in 1994, no significant differences in workload were found based on age or gender. The average age of the practicing pediatric neurologist in 2001 was 51 years compared to 45 years in 1994. The proportion of physicians over 55 years in 2001 was 35% compared to 25% in 1994.Conclusions:Pediatric neurology in Canada is an aging specialty needing a significant recruitment of new members.
Clinical and Biochemical Heterogeneity in an Italian Family with CPT II Deficiency due to Ser 113 Leu Mutation
Authors: Rafay Mubeen F. , Murphy E. Gordon , McGarry J. Denis , Kaufmann Petra , DiMauro Salvatore , Tein Ingrid 
Published: December 2014

ABSTRACT:Background:Carnitine palmitoyltransferase II (CPT II) deficiency is an autosomal recessive disorder which presents with recurrent myoglobinuria. Heterozygotes are usually asymptomatic.Methods:We correlate the clinical, biochemical and molecular features of a family in which the proband is homozygous for CPT II deficiency, due to the common Ser 113 Leu mutation.Results:The 20-year-old female proband presented at age three years with episodic myalgia and myoglobinuria, elevated creatine kinase (CK) of 3600 IU/L and had a 33% residual CPT II activity in cultured skin fibroblasts. Her 25-year-old dizygotic twin brothers presented with muscle stiffness following prolonged exercise but no overt pigmenturia and had interictal CKs up to 662 IU/L. Her parents and a 13-year-old brother are asymptomatic. An elder sister, not investigated, had recurrent pigmenturia and died at eight years with myoglobinuria. Molecular analysis revealed that the proband is homozygous for the Ser 113 Leu mutation. Her parents are heterozygotes with CPT II activities of 55% to 70%. Her younger brother is normal with 83% activity. The symptomatic twin brothers are heterozygous but demonstrated unexpectedly low CPT II activities of 40%, which may explain their phenotype.Conclusion:We postulate that there may be genetic, environmental and sex hormonal factors accounting for this manifesting heterozygosity and biochemical heterogeneity in CPT II deficiency.
A Follow-up Study of Infants with Intracranial Hemorrhage at Full-Term
Authors: Jhawar Balraj S. , Ranger Adrianna , Steven David A. , Maestro Rolando F. Del 
Published: December 2014

ABSTRACT:Objective:To determine physical and cognitive outcomes of full-term infants who suffered intracranial hemorrhage (ICH) at birth.Methods:A retrospective hospital-based, follow-up study of infants treated in London, Ontario between 1985 and 1996. Follow-up was conducted by telephone interviews and clinic visits. Outcome was measured according to physical and cognitive scales. Perinatal risk factors and hemorrhage characteristics were correlated with final outcome.Results:For this study 66 infants with ICH were identified, of which seven died during the first week of life. We obtained follow-up in all but ten cases (median = 3-years; range 1.0 to 10.9 years). Overall, 57% of infants had no physical or cognitive deficits at follow-up. Death occurred most frequently among those with primarily subarachnoid hemorrhage (19%) and the most favorable outcomes occurred among those with subdural hemorrhage (80% had no disability). In univariate models, thrombocytopenia (platelet count ≤ 70 x 109/L), increasing overall hemorrhage severity, frontal location and spontaneous vaginal delivery as opposed to forceps-assisted delivery increased risk for poor outcome. In multivariate models, all these factors tended towards increased risk, but only thrombocytopenia remained significant for physical disability (OR = 7.6; 95% CI = 1.02 – 56.6); thrombocytopenia was borderline significant in similar models for cognitive disability (OR = 4.6; 95% CI = 0.9 – 23.9).Conclusion:Although forceps-assisted delivery may contribute to ICH occurrence, our study found better outcomes among these infants than those who had ICH following a spontaneous vaginal delivery. Hemorrhage in the frontal lobe was the most disabling hemorrhage location and if multiple compartments were involved, disability was also more likely to occur. However, in this report we found that the factor that was most likely to contribute to poor outcome was thrombocytopenia and this remained important in multivariate analysis.
Transient Edematous Lesions of the Splenium in Epileptic Patients
Authors: Carrara Giovanna , Ferlazzo Edoardo , Tampieri Donatella , Andermann Frederick , Melanson Denis 
Published: December 2014

ABSTRACT:Background:Transient focal lesions in the splenium of the corpus callosum (SCC) have been previously described in patients with epilepsy or without epilepsy but receiving antiepileptic drugs (AED).Case reports:Two epileptic patients were admitted to our long-term monitoring unit. Antiepileptic drugs were completely discontinued a few days later. One patient had no seizures. The other had three attacks, the last of which occurred two days before a brain magnetic resonance imaging (MRI) was performed. In both cases brain MRI showed a lesion in the SCC characterized by high signal on T2-weighted images and no enhancement after Gadolinium infusion. The patients were discharged with their pre-admission medications. A follow-up MRI five weeks later showed resolution of the SCC lesions.Conclusions:The pathogenesis of transient SCC lesions in epileptic patients is still unclear. In our patients, either the sudden AED withdrawal or the seizures activity may be presumed to be the cause, though an individual susceptibility must also be considered.
Sudden Quadriplegia after Acute Cervical Disc Herniation
Authors: Sadanand Venkatraman , Kelly Michael , Varughese George , Fourney Daryl R. 
Published: December 2014

ABSTRACT:Background:Acute neurological deterioration secondary to cervical disc herniation not related to external trauma is very rare, with only six published reports to date. In most cases, acute symptoms were due to progression of disc herniation in the presence of pre-existing spinal canal stenosis.Case report:A 42-year-old man developed weakness and numbness in his arms and legs immediately following a sneeze. On physical examination he had upper motor neuron signs that progressed over a few hours to a complete C5 quadriplegia. An emergent magnetic resonance imaging study revealed a massive C4/5 disc herniation. He underwent emergency anterior cervical discectomy and fusion. Postoperatively, the patient remained quadriplegic. Eighteen days later, while receiving rehabilitation therapy, he expired secondary to a pulmonary embolus. Autopsy confirmed complete surgical decompression of the spinal cord.Conclusions:Our case demonstrates that acute quadriplegia secondary to cervical disc herniation may occur without a history of myelopathy or spinal canal stenosis after an event as benign as a sneeze.
Hypertension and Stroke: 2005 Canadian Hypertension Educational Program Recommendations
Authors: Boulanger J.M. , Hill Michael D. ,   on behalf of the CHEP (Canadian Hypertension Educational Program)
Published: December 2014

ABSTRACT:Background:Hypertension is the most important modifiable cause of stroke. The Canadian Hypertension Educational Program, representing Canada's experts in the field of hypertension, publishes annual evidence-based recommendations on the diagnosis and treatment of hypertension.Methods:We present the 2005 Canadian Hypertension Educational Program guidelines regarding the management of hypertension in patients with stroke.Results:The diagnosis of hypertension should be expedited and can be made as early as the second visit in patients with stroke. Unless contraindicated, a combination of angiotensin-converting-enzyme (ACE) inhibitors and diuretics is the preferred therapy in these patients. A target blood pressure below 140/90mmHg for non-diabetic patients, below 130mmHg/80mmHg for diabetic patients and below 125mmHg/75mmHg for those with renal disease and proteinuria (³ 1 gram per day) should be reached. Lifestyle interventions may be as effective as medication and should be used in conjunction with medical management. Waist circumference should be less than 102cm for men and 88cm for women. There remains uncertainty about the management of high blood pressure in the context of acute stroke.Conclusions:A combination of ACE-inhibitors and diuretics is recommended in hypertensive stroke patients. Blood pressure should be maintained below 140/90 mmHg.
Highlights of the 2002 Canadian Neurological Society (CNS) Manpower Survey
Authors: Bailey Peter , Warren Sharon , Buske Lynda 
Published: December 2014

ABSTRACT:Background:The Canadian Neurological Society commissioned a manpower survey in 2002 to assess demographics, distribution, specialty interests, working conditions, job satisfaction and future plans of neurologists across the country.Methods:A survey was mailed to all known Canadian neurologists (n = 694) on two separate occasions. Further encouragement by telephone contact was undertaken. The response rate was 54%.Results:The mean age of neurologists who responded was 51 years, with 14% being women. Approximately 55% of neurologists were community-based. Seventy-six percent designated a sub-specialty interest. On average, neurologists worked 57 hours per week and the majority had significant “on-call” commitments. Job satisfaction was higher among academic neurologists when compared with community-based neurologists, and greater among men than women. A greater percentage of older neurologists were satisfied with their work than their younger colleagues. Significant attrition in the neurological work force is a major concern, since up to 20% of neurologists reported that they are likely to retire in the next five years and about 15% are likely to reduce their practice.Conclusions:This survey suggests that substantial concerns are facing Canadian neurology over the next five years. Major efforts to retain existing expertise and enhance residency training will be required to simply maintain the present quality of neurological care in Canada.