bluuebunny/crossref_metadata_2025 huggingface dataset:
Primary Prevention and Delay of Onset of AD/Dementia
Authors: Feldman Howard H. , Jacova Claudia
Published: December 2014

Prevention in Alzheimer's disease and other dementias (AD/dementia) is defined on the basis of clinical states and their expressed symptoms. Primary prevention refers to delaying the development of the full-blown state of clinically expressed disease in normal individuals. Current primary prevention research is driven by evidence of AD/dementia protective factors that have emerged from epidemiological studies. The first randomized controlled trials (RCTs) of primary AD/dementia prevention have been designed to test the efficacy and safety of NSAIDs, hormonal therapy, antihypertensive drugs and antioxidants. The experience of these trials has indicated safety concerns as a key issue and highlighted significant design challenges in this type of research. These trials have required large sample sizes and unsustainable costs. There should be consideration given in future trials to enriching study samples with risk factors to increase progression rates to AD/dementia. Innovative strategies will also be needed to recruit and retain subjects given the long follow-up periods, modest perceived benefit and the potential for the risk-benefit ratio to change during the trial. It is foreseeable that regulatory authorities will be presented with primary prevention RCTs for approval and labelling, and that criteria to evaluate such evidence still need to be developed.
A Comprehensive Analysis of MRI Research Risks: In Support of Full Disclosure
Authors: Marshall Jennifer , Martin Toby , Downie Jocelyn , Malisza Krisztina
Published: December 2014

Magnetic resonance imaging (MRI) procedures have been used for over 20 years. This modality is considered relatively safe and holds great promise. Yet, MRI has a number of risks. In order for MRI research to meet the Canadian standard of disclosure, the investigator must communicate and make note of all risks in their research protocols and consent forms. Those creating and reviewing research protocols and consent forms must take notice of the different circumstances under which MRI poses a risk. First, this paper will describe the current standard of disclosure in Canada for research participants. Second, the paper will provide a comprehensive synthesis of the known physical and psychological risks associated with MRI. Third, the paper will provide recommendations concerning areas for further investigation and risk reduction strategies. This information will thus equip researchers and research ethics boards (REBs) with the criteria needed for the composition of research protocols that meet the Canadian disclosure standard.
A Safe and Effective Method for Treatment of Chronic Subdural Haematoma
Authors: Gurelik Mustafa , Aslan Adem , Gurelik Bilge , Ozum Unal , Karadag Özen , Kars H. Zafer
Published: December 2014

Objective:

Burr-hole irrigation and burr-hole drainage without irrigation are the most popular methods for treatment of chronic subdural haematoma. It is not well known if irrigation is necessary or which method has a higher recurrence rate. We compared the recurrence rates of those two methods.

Materials and Methods:

Forty-two patients were treated by burr-hole irrigation (irrigation group), whereas 38 patients were treated by burr-hole drainage (drainage group). Recurrence rate and its relation with sex, age, haematoma localization and aetiology were investigated in both groups.

Results:

There was no significant difference between recurrence rates of the two groups. There was also no correlation between recurrence rate and age, sex, haematoma localization, or aetiology.

Conclusions:

There was no significant difference between recurrence rates of the two groups. Since the burr-hole drainage method is simpler to carry out, its use may be preferable.
The Voices of Neurosurgeons: Doctors' Non-Medical Writing
Authors: Bernstein Mark
Published: December 2014

Biomedical publishing is an integral part of medicine—both to those who produce it and those who consume it to improve the care of their patients. Non-medical writing by surgeons usually takes the form of creative non-fiction, generally reflective essays on moving and emotionally charged situations such as working in the trenches in war-time or in natural disasters, or dealing with individual patients. Such writing is both creative and cathartic for neurosurgeons, and can help educate patients thus improving the doctor-patient relationship. The purpose of this article is to encourage fellow neurosurgeons to pursue this enjoyable and valuable endeavour, to utter a call to arms so to speak.
Prevalence of Multiple Sclerosis in First Nations People of Alberta
Authors: Svenson Lawrence W. , Warren Sharon , Warren Kenneth G. , Metz Luanne M. , Patten Scott B. , Schopflocher Donald P.
Published: December 2014

Background:

Multiple Sclerosis (MS) is reported to be uncommon among North American aboriginals despite frequent intermarriage with people of European ancestry, but few population-based studies have been conducted. The purpose of this study was to determine the prevalence of MS among First Nations aboriginal people in Alberta, Canada compared to the general population.

Methods:

All hospital in-patient and physician fee-for-service records between 1994 and 2002 where a diagnosis of MS was mentioned were extracted from government health databases in the province of Alberta. First Nations people can be identified since the federal government (Health Canada) pays health care insurance premiums on their behalf. Multiple Sclerosis prevalence per 100,000 population for both First Nations people and the general population of Alberta were calculated for each year during this time span.

Results:

Among First Nations in Alberta, MS prevalence was 56.3 per 100,000 in 1994 and 99.9 per 100,000 in 2002, an increase of 43.6%. In 2002 prevalence was 158.1 and 38.0 for females and males respectively, a female to male ratio of 4.2:1. Multiple Sclerosis prevalence among the general population of Alberta was 262.6 per 100,000 in 1994 and 335.0 per 100,000 in 2002, an increase of 21.6%. In 2002 prevalence was 481.5 and 187.5 for females and males respectively, a female to male ratio of 2.6:1. Peak prevalence for both First Nations and general population females in 2002 was age 50-59, also 50-59 for both First Nations and general population males.

Conclusion:

While MS prevalence in First Nations people is lower than in the general population of Alberta, it is not rare by worldwide standards.
The Incidence Trends of Primary Brain Tumors in Saskatchewan from 1970 to 2001
Authors: Pirouzmand F. , Sadanand V.
Published: December 2014

Objective:

There has been a paucity of information on the epidemiology of primary brain tumors (BTs) in Canada. This study documents epidemiology of primary BTs in Saskatchewan over three decades to define their current state, changing pattern over years and relative distribution in two geographically defined areas of the province.

Methods:

Data on all primary BTs from 1970 to 2001 from the Brain Tumor Registry in Saskatchewan was collected. The aggregate data on primary BTs including the time-series for incidence, age, geographic location and sex were statistically analyzed using SPSS 13. Poisson regression was used to model the incidence as a function of decade of diagnosis and age at the time of diagnosis.

Results:

The average annual incidence of primary BTs was 11.1 per 100,000 person-years (male 12.5 per 100.000 and female 9.8 per 100.000). Males constituted 54.5% of all these tumors. The age distribution of tumors was bimodal with peaks at 5 years and 65 years. During this time, the incidence of primary BTs has increase predominantly in non-malignant types. No difference was found in the rate of all the diagnosed primary BTs combined, meningioma and lymphoma between the northern part (Regina) and southern part (Saskatoon) of the province.

Conclusions:

The incidence of BTs in Saskatchewan is more than previously reported in Canada. There is a temporal trend in increasing incidence of some of the BTs predominantly in the non-malignant types. No spatial difference in the incidence of primary BTs was shown in this province. These data will provide useful information to guide the future studies on BTs changing patterns, possible etiologies and efficient resource allocation for management of these diseases.
Possible Anticipation in Hereditary Spastic Paraplegia Type 4 (SPG4)
Authors: Reddy P. Leema , Seltzer William K. , Grewal Raji P.
Published: December 2014

Objective:

We report a multigenerational family with uncomplicated hereditary spastic paraplegia type 4 and apparent anticipation. Genetic analysis of the proband revealed a frame shift mutation (5 base pair deletion) in exon 9 of the SPG4 gene encoding the spastin protein. We hypothesized that this deletion mutation may be dynamic and variability in the size of the deletion could account for the anticipation.

Methods:

Clinical and genetic analysis of this family and the deletion mutation.

Results:

In this family, the age of onset, which ranges from 3 to 50 years shows an average decrease in the age of onset of 21.8 years per transmission over three generations. Genetic analysis of multiple family members indicates that all affected members carry the same c.1340_1344deITATAA mutation and that it is not dynamic.

Conclusion:

In this family, other molecular mechanisms may contribute to development of anticipation.
SPG4 Founder Effect in French Canadians with Hereditary Spastic Paraplegia
Authors: Meijer Inge A. , Dupré Nicolas , Brais Bernard , Cossette Patrick , St-Onge Judith , Rioux Marie-France , Benard Melanie , Rouleau Guy A.
Published: December 2014

Background:

The most common cause of autosomal dominant Hereditary Spastic Paraplegia (HSP) is mutations in the SPG4 gene. We have previously identified novel SPG4 mutations in a collection of North American families including the c.G1801A mutation present in two families from Quebec. The aim of this study is to estimate the frequency of the c.G1801A mutation in the French Canadian (FC) population and to determine whether this mutation originates from a common ancestor.

Methods:

We collected and sequenced exon 15 in probands of 37 families. Genotypes of markers flanking the SPG4 gene were used to construct haplotypes in five families. Clinical information was reviewed by a neurologist with expertise in HSP.

Results:

We have identified three additional unrelated families with the c.G1801A mutation and haplotype analysis revealed that all five families share a common ancestor. The mutation is present in 7% of all our FC families and explains half of our spastin linked FC families. The phenotype associated with the c.G1801A genotype is pure HSP with bladder involvement.

Conclusion:

In this study we have determined that the relative frequency of the c.G1801A mutation in our FC collection is 7%, and approximately 50% in the spastin positive FC group. This mutation is the most common HSP mutation identified in this population to date and is suggestive of a founder effect in Quebec.
The Role of Polyamine Metabolism in Neuronal Injury Following Cerebral Ischemia
Authors: Kim Grace H. , Komotar Ricardo J. , McCullough-Hicks Margy E. , Otten Marc L. , Starke Robert M. , Kellner Christopher P. , Garrett Matthew C. , Merkow Maxwell B. , Rynkowski Michal , Dash Kelly A. , Connolly E. Sander
Published: December 2014

Stroke is a leading cause of morbidity and mortality in the US, with secondary damage following the initial insult contributing significantly to overall poor outcome. Prior investigations have shown that the metabolism of certain polyamines such as spermine, spermidine, and putrescine are elevated in ischemic parenchyma, resulting in an increase in their metabolite concentration. Polyamine metabolites tend to be cytotoxic, leading to neuronal injury in the penumbra following stroke and expansion of the area of infarcted tissue. Although the precise mechanism is unclear, the presence of reactive aldehydes produced through polyamine metabolism, such as 3-aminopropanal and acrolein, have been shown to correlate with the incidence of cerebral vasospasm, disruption of oxidative metabolism and mitochondrial functioning, and disturbance of cellular calcium ion channels. Regulation of the polyamine metabolic pathway, therefore, may have the potential to limit injury following cerebral ischemia. To this end, we review this pathway in detail with an emphasis on clinical applicability.
Period Prevalence of Epilepsy in Children in BC: A Population-Based Study
Authors: Schiariti Veronica , Farrell Kevin , Houbé Jill S. , Lisonkova Sarka
Published: December 2014

Background:

Most estimates of the prevalence of seizure disorders in Canada derive from national surveys which differ in sampling and case-finding methods. This study used health care utilization data to make a population-based estimate of the prevalence of epileptic seizures and of epilepsy in children in British Columbia (BC).

Methods:

All BC residents between 0-19 years-of-age in 2002-3 enrolled in the Medical Services Plan were included. Epileptic seizures were defined using ICD-9 codes; health care utilization data was obtained from BC Linked Health Database. The period prevalence of epileptic seizures and of epilepsy was determined by age, urban/rural region and socioeconomic status.

Results:

8,125 of 1,013,816 children were identified as having an epileptic seizure of which 5,621 were classified as epilepsy - 5.5 per 1,000 children (95% CI: 5.4-5.7). The prevalence of epilepsy in infants and preschoolers was higher than that reported in the literature. A higher prevalence of epilepsy was observed also among those with low socioeconomic status. A higher prevalence of epilepsy was observed in those health regions with a higher proportion of First Nations and a lower prevalence was observed in health regions with a higher proportion of visible minorities.

Conclusions:

Age-specific prevalence rates in BC children for epilepsy, determined from population-based administrative records, were similar to published data except in children under five years. We found a gradient of increased prevalence with decreased level of income. Prevalence rates based on utilization data have the potential to guide program planning for children with epileptic seizures.
Arterial Onyx Embolisation of Intracranial DAVFs with Cortical Venous Drainage
Authors: Chew Jason , Weill Alain , Guilbert François , Raymond Jean , Audet Marie-Eve , Roy Daniel
Published: December 2014

Purpose:

To present our experience with the endovascular management of intracranial dural arteriovenous fistulas with direct cortical venous drainage by trans-arterial embolisation using Onyx.

Materials & Methods:

Between January 2004 and April 2008, 12 consecutive high grade intracranial dural arteriovenous fistulas (Cognard type III (eight patients) or IV (three patients)) were treated by trans-arterial embolisation with Onyx. The majority of cases were treated by Onyx embolisation alone. One case had additional embolisation with n-butyl-2-cyanoacrylate at the same session. Imaging follow-up was obtained in all but one patient (mean 3.6 months).

Results:

Nine patients had a technical success at the end of the embolisation procedure with complete angiographic exclusion of the fistula. Two patients had a small residual fistula at the end of embolisation, one of which had residual mild cortical venous drainage. Both were stable at follow-up angiography. One patient had a residual fistula supplied by the ophthalmic artery, which was thought to be unsafe to embolise and was sent for surgery, which was curative. In one patient the microcatheter ruptured, with a fragment of the distal microcatheter left in the occipital artery. No clinical complications were observed in this series at clinical follow-up (mean 3.3 months). Two patients were noted to have significant radiation dose.

Conclusion:

Endovascular management of intracranial dural arteriovenous fistulas with direct venous cortical drainage by trans-arterial Onyx embolisation is a safe and effective treatment according to our experience. Fluoroscopy times and radiation dose may be a concern.
Percutaneous Muscle Biopsies: Review of 900 Consecutive Cases at London Health Sciences Centre
Authors: Derry Kendra L. , Nicolle Michael N. , Keith-Rokosh Julia A. , Hammond Robert R.
Published: December 2014

Objective:

In the present study we review our experience with 900 consecutive percutaneous muscle biopsies over the period 1993 to 2007. We examined the advantages and limitations of the procedure, biopsy site preferences, diagnostic range, frequency of diagnoses and quality of histopathology. Demographics, referral patterns and patients' perceptions of the procedure were also assessed.

Methods:

Cases were identified through the London Health Sciences Centre Department of Pathology database. Standard biopsy procedures were followed using a manual trocar style instrument. With a neuropathology technologist in attendance at all biopsies, biopsies were oriented in the fresh state and snap frozen.

Results:

Most referrals for muscle biopsy were from neuromuscular neurologists. The procedure was found to be efficient, well-tolerated and produced high quality specimens in all diagnostic categories. No major complications occurred. Failure to obtain an adequate tissue sample, although uncommon (<2%), was usually due to marked obesity, edema or muscle wasting. Bleeding at the site was rarely problematic and no wound infections were reported.

Conclusions:

Needle muscle biopsies represent an efficient alternative to open biopsies when peripheral nerve sampling is not required and when large tissue samples are not needed for extensive biochemical analyses.
Resiniferatoxin Mediated Ablation of TRPV1+ Neurons Removes TRPA1 as Well
Authors: Pecze László , Pelsőczi Péter , Kecskés Miklós , Winter Zoltán , Papp András , Kaszás Krisztián , Letoha Tamás , Vizler Csaba , Oláh Zoltán
Published: December 2014

Objectives:

Resiniferatoxin, the most potent agonist of inflammatory pain/vanilloid receptor/cation channel (TRPV1) can be used for neuron subtype specific ablation of pain generating cells at the level of the peripheral nervous system by Ca2+-excytotoxicity. Molecular neurosurgery is an emerging technology either to alleviate severe pain in cancer or treat/prevent different local neuropathies. Our aim was determining sensory modalities that may be lost after resiniferatoxin treatment.

Methods:

Newborn or adult mice were treated with resiniferatoxin, then changes in chemical and heat sensitivity were correlated with alterations of the cell composition of sensory ganglions.

Results:

Only mice treated at adult age became less sensitive to heat stimuli, while both treatment groups lost sensitivity to specific vanilloid agonists of TRPV1 and, interestingly, to allyl-isothiocyanate, a selective agonist of TRPA1. Our in vivo and post mortem analytical results confirmed that TRPV1 and TRPA1 function together and resiniferatoxin-mediated neurosurgery removes both sensor molecules

Discussion:

In adult mice resiniferatoxin causes: i) desensitization to heat and ii) sensitization to cold. Cold hyperalgesia, an imbalance in thermosensation, might be conferred by a prominent cold receptor that is expressed in surviving resiniferatoxin-resistant sensory neurons and compensates for pain signals lost with TRPA1 and TRPV1 double positive cells in the peripheral nervous system.
Cerebral Proliferative Angiopathy
Authors: Eesa Muneer , Sharma Pranshu , Goyal Mayank
Published: December 2014

A 31-year-old male presented with transient numbness of the right hand, progressing to involve the forearm. The symptoms lasted for around 30 minutes without any associated weakness or positive motor phenomena. An evaluation in the emergency room revealed no focal deficits.Acomputer tomogram (CT) scan performed in the emergency room suggested a few abnormal vascular structures in relation to the left hemisphere, with no evidence of hemorrhage. The patient was referred for magnetic resonance imaging (MRI) and conventional angiography.

The MRI showed a diffuse network of densely enhancing vascular spaces involving the left middle cerebral artery (MCA) and anterior cerebral artery (ACA) territories without a clear nidus or abnormal parenchymal signal change (Figure 1). There was no involvement of the basal ganglia or thalami. Given the size and extent of the abnormality, there was a relative paucity of draining vessels, which were only moderately enlarged.
Primary Sjögren's Syndrome Associated Neuropathy
Authors: Mellgren Svein Ivar , Göransson Lasse G , Omdal Roald
Published: December 2014

Primary Sjögren's syndrome (PSS) mainly affects exocrine glands and is clinically characterized by keratoconjunctivitis sicca and xerostomia. Among several possible extraglandular manifestations, involvement of the peripheral nervous system may occur with reported frequencies from 10% to 60%. Peripheral nerve manifestations constitute sensory neuropathy, including sensory ganglioneuronopathy, sensorimotor, including polyradiculoneuropathy and demyelinating neuropathy, motor neuropathy, multiple mononeuropathy, trigeminal and other cranial neuropathies, autonomic neuropathy, and mixed patterns of neuropathy. Knowledge of the neurological manifestations of PSS is hampered by evolving classification criteria of PSS over the years, and by use of highly selected patient populations on the basis of a primary neurological diagnosis. Sural nerve biopsy may show vascular or perivascular inflammation of small epineurial vessels (both arterioles and venules) and in some cases necrotizing vasculitis. Loss of myelinated nerve fibers is common and loss of small diameter nerve fibers occurs. Pathology in cases of sensory ganglioneuronopathy consists of loss of neuronal cell bodies and infiltration of T cells. Peripheral neuropathy in PSS often is refractory to treatment although newer biological agents may provide more effective treatment options. Current treatment strategies used in autoimmune neuropathies may be tried depending upon characteristics of the neuropathy and results obtained by a thorough clinical and laboratory investigation.
Acute Glomerulonephritis Presenting With PRES: A Report of 4 Cases
Authors: Wirrell E C , Hamiwka L D , Hamiwka L A , Grisaru S , Wei X
Published: December 2014

Objective:

Posterior reversible encephalopathy syndrome (PRES) occurs most commonly in the setting of known hypertension or use of immunosuppressive agents.

Design and Methods:

We report four previously-well children who presented acutely with altered mentation, seizures and visual disturbances and were diagnosed with PRES.

Results:

Only one child had a history of gross hematuria prior to the seizure. All four were discovered to be hypertensive only after onset of their neurological symptoms, and were subsequently diagnosed with glomerulonephritis. All four had rapid resolution of neurological symptoms with adequate treatment of hypertension.

Conclusions:

Blood pressure must be measured promptly in all children presenting with these symptoms. If elevated, the diagnosis of PRES should be strongly considered and a workup for renal disease pursued.
Multimodal Evoked Potentials of Kennedy's Disease
Authors: Lai Tsu-Hsien , Soong Bing-Wen , Chen Jen-Tse , Chen Yen-Yu , Lai Kuan-Lin , Wu Zin-An , Liao Kwong-Kum
Published: December 2014

Background:

Kennedy's disease (KD) is an X-linked recessive polyglutamine disease. Traditionally, it is a lower motor neuron syndrome with additional features such as gynecomastia and tremor. Sensory symptoms are minimal if ever present. We used multimodal evoked potential (EPs) tests to study the distribution of the involvement of the disease.

Methods:

Visual, brainstem auditory, somatosensory and motor EPs were studied in six KD patients. All of them had typical presentations and had been proved genetically.

Results:

Abnormal findings were noted as follows: prolonged peak latencies of visual EPs, increased hearing threshold level, inconsistent brainstem auditory EPs, decreased amplitudes of cortical potentials of somatosensory EPs, and increased motor threshold to transcranial magnetic stimulation.

Conclusions:

Our multimodal EP studies showed that KD involved multiple levels of the nervous system. It implies the widespread effects of the mutant androgen receptors.
LRRK2 Screening in a Canadian Parkinson's Disease Cohort
Authors: Grimes D. A. , Racacho L. , Han F. , Panisset M. , Bulman D. E.
Published: December 2014

Background:

Mutations in the leucine-rich repeat kinase 2 gene (LRRK2) have become the most common known cause for developing Parkinson's disease. The frequency of mutations described in the literature varies widely depending on the population studied with most reports focusing only on screening for the most common G2019S mutation in exon 41.

Methods:

In this study seven exons (19, 24, 25, 31, 35, 38, and 41) in LRRK2 where mutations have been reported were screened in 230 unselected Parkinson's disease patients using denaturing high-performance liquid chromatography.

Results:

The sequencing of samples with heteroduplex profiles revealed five novel and two known intronic sequence variants. In our cohort, we were unable to detect any of the known mutations in these exons or identify novel mutations within the LRRK2 gene.

Conclusions:

Therefore, despite the availability of diagnostic LRRK2 genetic testing it is unlikely to yield a positive result in this population.
Coincidence vs Cause: Cure in Three Glioblastoma Patients Treated with Brachytherapy
Authors: Dehdashti Amir R. , Sharma Sunjay , Laperriere Normand , Bernstein Mark
Published: December 2014

Background:

Very long term survival after diagnosis of malignant glioma has been described in individual case reports. Survival of more than 10 years is extremely rare, especially when identified in 3 out of 71 patients assigned to one arm of a randomized controlled trial.

Patients:

Three patients survived 11, 16, and 18 years following the diagnosis of glioblastoma and treatment with surgery, conventional fractionated radiation, and high-activity iodine-125 boost brachytherapy as part of a randomized controlled trial.

Conclusion:

Despite this apparent cause and effect relationship, statistical analysis shows no relationship between these cures and treatment with brachytherapy. Cure of glioblastoma remains rare.
Oxidative Stress after Subarachnoid Hemorrhage in gp91phox Knockout Mice
Authors: Liu Shimin , Tang Jiping , Ostrowski Robert P , Titova Elena , Monroe Cara , Chen Wanqiu , Lo Wendy , Martin Robert , Zhang John H
Published: December 2014

Background:

Oxidative stress largely contributes to early brain injury after subarachnoid hemorrhage (SAH). One of the major sources of reactive oxygen species is NADPH oxidase, upregulated after SAH. We hypothesized that NADPH oxidase-induced oxidative stress plays a major causative role in early brain injury after SAH.

Methods:

Using gp91phox knockout (ko) and wild-type (wt) mice, we studied early brain injury in the endovascular perforation model of SAH. Mortality rate, cerebral edema, oxidative stress, and superoxide production were measured at 24 h after SAH. Neurological evaluation was done at 23 h after SAH surgery.

Results:

Genotyping confirmed the existence of a nonfunctional gp91phox gene in the ko mice. CBF measurements did not show differences in SAH-induced acute ischemia between ko and wt mice. SAH caused a significant increase of water content in the ipsilateral hemisphere as well as an increase of Malondialdehyde (MDA) levels and superoxide production. There were no significant differences in post-SAH mortality rate, brain water content and the intensity of the oxidative stress between knockout and wild type groups of mice.

Conclusions:

Our results suggest that gp91phox is not critically important to the early brain injury after SAH. An adaptive compensatory mechanism for free radical production in knockout mice is discussed.
Canadian Minor Hockey Participants’ Knowledge about Concussion
Authors: Cusimano Michael D. , Chipman Mary L. , Volpe Richard , Donnelly Peter
Published: December 2014

Background and Objectives:

In Canada and the USA, ice hockey is a cause of traumatic brain injury. Post-concussive symptoms are the most important feature of the diagnosis of concussion in sports and it is recommended that athletes not return to play while still symptomatic. Lack of knowledge of concussions could therefore be one of the main detriments to concussion prevention in hockey. The purpose of this research is to describe what minor league hockey players, coaches, parents and trainers know about concussion and its management.

Methods:

A questionnaire to assess concussion knowledge and return to play guidelines was developed and administered to players at different competitive levels (n = 267), coaches, trainers and parents (total adults n = 142) from the Greater Toronto Area.

Results:

Although a majority of adults and players could identify mechanisms responsible for concussion, about one-quarter of adults and about a quarter to a half of children could not recall any symptoms or recalled only one symptom of a concussion. A significant number of players and some adults did not know what a concussion was or how it occurred. Almost half of the players and a fifth of the adults incorrectly stated that concussion was treated with medication or physical therapy. Nearly one quarter of all players did not know if an athlete experiencing symptoms of concussion should continue playing.

Conclusions:

This study demonstrated that a significant number of people held misconceptions about concussion in hockey which could lead to serious health consequences and creates a need for better preventive and educational strategies.
Primary Stenting for Recurrent Stenosis Following Carotid Endarterectomy
Authors: Bussière Miguel , Pelz David M. , Lownie Stephen P.
Published: December 2014

Background:

Carotid angioplasty and stenting is an accepted alternative treatment for severe restenosis following carotid endarterectomy. Balloons may not be required to effectively treat these lesions, given their altered histopathology compared to primary atherosclerotic plaque and tendency to be less calcified. Primary stenting using self-expanding stents alone may, therefore, be a safe and effective treatment for restenosis post-carotid endarterectomy.

Methods:

We review our experience in the treatment of 12 patients with symptomatic severe restenosis following carotid endarterectomy with primary stent placement alone.

Results:

Self-expanding stent placement alone reduced the mean internal carotid artery stenosis from 85% to 29%. Average peak systolic velocity determined at the time of ultrasonography decreased from 480 cm/s at initial presentation to 154 cm/s post-stent deployment and further decreased to 104 cm/s at one year follow-up. The stented arteries remained widely patent with no evidence of restenosis. A single peri-procedural ipsilateral transient ischemic event occurred. There were no cerebral or cardiac ischemic events recorded at one year of follow-up.

Conclusions:

In this series, primary stent placement without use of angioplasty balloons was a safe and effective treatment for symptomatic restenosis following carotid endarterectomy.
Neuromuscular Function in Survivors of the Acute Respiratory Distress Syndrome
Authors: Angel Michael J. , Bril Vera , Shannon Patrick , Herridge Margaret S.
Published: December 2014

Background:

Survivors of acute respiratory distress syndrome (ARDS) report generalized weakness and reduced exercise tolerance up to two years following discharge from the intensive care unit (ICU). Persistent neuromuscular complications of ARDS may contribute to the functional disability observed in these patients.

Methods:

Sixteen ARDS survivors underwent comprehensive neurological evaluation and standardized electrodiagnostic testing 6 to 24 months after ICU discharge. Four of these patients agreed to open muscle biopsy.

Results:

Seven of sixteen patients had clinically significant focal compressive mononeuropathies. Electrodiagnostic testing failed to reveal any changes attributable to critical illness polyneuropathy or myopathy. All four muscle biopsies were abnormal, and although the pathological features were structurally non-specific, the presence of an acquired myopathy remains possible. Four patients had persistent mixed sensory complaints but had normal electrodiagnostic evaluation.

Conclusions:

The high frequency of mononeuropathies highlights the need for vigilance in daily ICU care. The findings also suggest that complaints of weakness and reduced exercise capacity in ARDS survivors may be related to combined effects of compressive neuropathies and generalized longstanding structural changes in muscle and may support an organic basis for longterm functional disability.
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.
Salivary Gland-Like Tumor of the Sella
Authors: van Furth Wouter , Smyth Harley S. , Horvath Eva , Kovacs Kalman , Salehi Fateme , Cusimano Michael D.
Published: December 2014

Tumors arising in the sellar region are predominantly benign, non-invasive pituitary adenomas. Invasive pituitary adenomas (approximately 35 % of the pituitary adenomas) and pituitary carcinomas (0.1 – 0.5 %) arise from the same cells in the pituitary gland as the benign tumors, but have different pathology and biological behavior. A wide variety of nonadenomatous lesions involve the sellar region. These include tumors that arise specifically from sellar and suprasellar structures, as well as those that are also found elsewhere in the CNS (e.g. meningioma, glioma, metastasis, etc.). Typical nonadenomatous tumors that originate in the sellar region are craniopharyngioma and Rathke’s cleft cyst. The purpose of this paper is to describe another nonadenomatous tumor specific to the sellar region, a salivary gland-like tumor, and review the literature on this subject. This case report will also illustrate the management decisions regarding a patient with multiple medical issues and an aggressive sellar tumor.
A Neuromuscular Approach to Statin-Related Myotoxicity
Authors: Baker Steven K. , Samjoo Imtiaz A.
Published: December 2014

Approximately 95% of statin-treated patients tolerate this form of cholesterol management without any adverse effects. However, given their efficacy in reducing low density lipoproteins and cardiovascular events large numbers of patients are selected for statin therapy. Therefore muscle complications are, in fact, quite common. Limited understanding of the underlying pathophysiology has hampered physicians' ability to identify patients at risk for developing statin myotoxicity. A growing number of published case reports/series have implicated statins in the exacerbation of both acquired and genetic myopathies. A clinical management algorithm is presented which outlines a variety of co-morbidities which can potentiate the adverse effects of statins on muscle. In addition, a rational approach to the selection of those patients most likely to benefit from skeletal muscle biopsy is discussed. Ongoing work will define the extent to which statin-intolerant patients represent carriers of recessive metabolic myopathies or pre-symptomatic acquired myopathies. The expanding importance of pharmacogenomics will undoubtedly be realized in the field of statin myopathy research within the next few years. Such critical information is needed to establish more definitive management and diagnostic strategies.
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.