bluuebunny/crossref_metadata_2025 huggingface dataset:
Headache as a Predictive Factor of Severe Systolic Hypertension in Acute Ischemic Stroke
Authors: Hong Yoon-Ho , Lee Yong-Seok , Park Seong-Ho
Published: December 2014

Background:

Elevation of blood pressure (BP) is common in acute cerebral infarction, with several studies reporting a high plasma catecholamine level or previous hypertension as a contributory factor. However, more comprehensive studies on associated clinical parameters are lacking. Our main aim in undertaking this study was to correlate clinical variables associated with a BPelevation in acute ischemic stroke.

Methods:

Consecutive patients who were admitted to the emergency room and diagnosed with an acute cerebral infarction within 24 hours after the onset of symptoms were investigated. A BP elevation was defined as a high systolic (³200mmHg) or diastolic (³110 mmHg) pressure. The mean systolic and diastolic BP were compared between the different stroke subtypes, lesion locations (carotid vs. vertebrobasilar), and hemispheric sides. The frequency of symptoms, risk factors, location of the infarct, stroke severity, vascular status and laboratory abnormalities were analyzed in order to build a regression model.

Results:

One hundred thirty-one patients were recruited (M:F=60:71, mean age 66±12 years) and an elevated BP was identified in 33 patients (25.2%). The mean systolic and diastolic BP did not differ significantly between the stroke subtypes, lesion locations, and hemispheric sides. According to univariate logistic regression, an elevated systolic BP correlated with headache (p=0.01) and underlying hypertension (p=0.02) while an elevated diastolic BP correlated with underlying hypertension (p=0.01). Multivariate logistic regression analysis revealed previous hypertension (OR 5.21, 95% CI 1.40-19.37) and headache (OR 4.09, 95% CI 1.44-11.66) to be independent predictors of an elevated systolic BP.

Conclusions:

Headache itself is closely associated with severe systolic BP elevation in acute ischemic stroke. Whether treatment of elevated BP improves headache and clinical outcome is not yet known, necessitating future controlled studies.
Respiratory Pattern Changes in Sleep in Children on Vagal Nerve Stimulation for Refractory Epilepsy
Authors: Nagarajan Lakshmi , Walsh Peter , Gregory Pauline , Stick Stephen , Maul Jennifer , Ghosh Soumya
Published: December 2014

Background:

An altered breathing pattern in sleep, over two to three weeks, reported by the parents of a child on Vagal Nerve Stimulation (VNS) therapy for refractory epilepsy, prompted a sleep study in him. His polysomnography (PSG) revealed respiratory irregularity concordant with VNS activation. Dyspnoea is a well recognised and reported side effect of the VNS. However there are only a few studies looking at respiration in sleep with VNS. We therefore undertook PSGs in seven other children on VNS.

Methods:

Sleep studies were undertaken, in accordance with standard clinical practice. Sleep and apnoeas and hypopneas were scored in accordance with conventional criteria. Respiratory pattern changes in sleep (RPCS) with VNS were looked for.

Results:

Respiratory pattern changes in sleep were seen during PSG in seven of eight children on VNS for refractory epilepsy. Decreased effort and tidal volume occurred in seven children, concordant with VNS activation. In one child, this was associated with a fall in respiratory rate, in the other six children with an increase. No study showed an apnoea/hypopnoea index in the abnormal range. The RPCS were not associated with significant hypoxia or hypercapnoea.

Conclusion:

Our results suggest that RPCS occur in most children with VNS. This is not surprising in view of the significant influence vagal afferents have on respiratory control centres. The RPCS did not appear to have a clinical impact in our group. However further investigations are suggested to explore this phenomenon, especially in patients with sleep apnoea syndromes or compromised respiratory function.
Variable Phenotype in a P102L Gerstmann-Sträussler-Scheinker Italian Family
Authors: Michele Giuseppe De , Pocchiari Maurizio , Petraroli Rossella , Manfredi Mario , Caneve Giorgio , Coppola Giovanni , Casali Carlo , Saccà Francesco , Piccardo Pedro , Salvatore Elena , Berardelli Alfredo , Orio Marcello , Barbieri Fabrizio , Ghetti Bernardino , Filla Alessandro
Published: December 2014

Background:

Gerstmann-Sträussler-Scheinker disease is an autosomal dominant prion disease. The clinical features include ataxia, dementia, spastic paraparesis and extrapyramidal signs.

Methods:

We report a new large Italian family affected by Gerstmann-Sträussler-Scheinker disease.

Results:

The four generation pedigree includes 11 patients. The mean age at onset ± SD was 41.4 ± 16.2 years. Mean disease duration to death in four patients was 5.5 ± 1.7 years. Two clinical patterns were evident: cognitive impairment with scarce neurological features or ataxia followed by cognitive impairment. Molecular analysis showed P102L mutation in PRNP gene.

Conclusion:

Three Italian families have been reported to date. The variable phenotype has already been reported, and does not appear related to the codon 129 polymorphism.
hnRNP A1 and A/B Interaction with PABPN1 in Oculopharyngeal Muscular Dystrophy
Authors: Fan Xueping , Messaed Christiane , Dion Patrick , Laganiere Janet , Brais Bernard , Karpati George , Rouleau Guy A.
Published: December 2014

Background:

Oculopharyngeal muscular dystrophy (OPMD) is an adult-onset disorder characterized by progressive ptosis, dysphagia and proximal limb weakness. The autosomal dominant form of this disease is caused by short expansions of a (GCG)6repeat to (GCG)8-13in the PABPN1 gene. The mutations lead to the expansion of a polyalanine stretch from 10 to 12-17 alanines in the N-terminus of PABPN1. The mutated PABPN1 (mPABPN1) induces the formation of intranuclear filamentous inclusions that sequester poly(A) RNA and are associated with cell death.

Methods:

Human fetal brain cDNA library was used to look for PABPN1 binding proteins using yeast two-hybrid screen. The protein interaction was confirmed by GST pull-down and co-immunoprecipitation assays. Oculopharyngeal muscular dystrophy cellular model and OPMD patient muscle tissue were used to check whether the PABPN1 binding proteins were involved in the formation of OPMD intranuclear inclusions.

Results:

We identify two PABPN1 interacting proteins, hnRNP A1 and hnRNP A/B. When co-expressed with mPABPN1 in COS-7 cells, predominantly nuclear protein hnRNP A1 and A/B co-localize with mPABPN1 in the insoluble intranuclear aggregates. Patient studies showed that hnRNP A1 is sequestered in OPMD nuclear inclusions.

Conclusions:

The hnRNP proteins are involved in mRNA processing and mRNA nucleocytoplasmic export, sequestering of hnRNPs in OPMD intranuclear aggregates supports the view that OPMD intranuclear inclusions are “poly(A) RNA traps”, which would interfere with RNA export, and cause muscle cell death.
Treadmill Training Effects on Neurological Outcome After Middle Cerebral Artery Occlusion in Rats
Authors: Yang Yea-Ru , Wang Ray-Yau , Wang Paulus Shyi-Gang , Yu Shang-Ming
Published: December 2014

Background:

Treadmill training is used for promoting rhythmical vigorous walking and for task-related training in patients with stroke. The neurological impact of treadmill training has not been established. The present investigation is aimed at (1) examining neurological changes over a four-week period after middle cerebral artery occlusion (MCAO) in rats and (2) assessing the impact of one-week, two-week and four-week treadmill training in MCAO rats.

Methods:

Male Sprague-Dawley rats were subjected to 60-minute right MCAO. All rats were randomly assigned to one of seven groups. Infarct volume and neurological score were measured.

Results:

Rats sacrificed 24 hours post MCAO had the largest infarct volumes (171.4 ± 14.4 mm3) and the highest neurological score (median: 2, range: 1-3). We noted that without treadmill training, infarct sizes and neurological score diminished with time. Treadmill training for at least one week further reduced infarct volume and significantly improved neurologic function in MCAO rats.

Conclusion:

Treadmill training after focal cerebral ischemia significantly improves neurological outcome in MCAO rats. Treadmill training may be beneficial for ischemic brain recovery.
Mefenamate, an Agent that Fails to Attenuate Experimental Cerebral Infarction
Authors: Kelly John J. , Auer Roland N.
Published: December 2014

Background:

Blockade of nonselective cation channels is a potential therapeutic approach that has not been attempted in cerebral ischemia, in spite of the ability of these channels to allow cellular calcium influx into neurons. Fenamates are a class of molecules that block these channels, and many congeners are also anti-inflammatory and free radical scavenging. These three mechanisms may contribute to brain damage in ischemia.

Methods:

Pretreatment or posttreatment with mefenamate (30 mg/kg) was evaluated in a temperature-controlled rat transient focal ischemia model. Quantitative histopathology on 26 coronal sections allowed determination of tissue necrosis and tissue atrophy at one week survival.

Results:

Neither pre- nor postischemic administration of a dose previously shown effective in preventing epileptic neuronal necrosis was found to reduce necrosis in cortex, nor in any subcortical structures.

Conclusions:

We conclude that nonselective cation channel blockade with mefenamate affords no neuroprotection in this model. Publication bias against negative studies exists in the literature, but we here report negative findings due to the multiple potentially positive actions of the drug. Closer examination of the effects of the molecule, however, reveals several potentially negative effects as well. We conclude there may be inherent weakness in pharmacologic monotherapy, even with molecules having protean potentially beneficial effects. This conclusion seems to have been borne out by the results of recent clinical trials.
Intraspinal Transplantation of hNT Neurons in the Lesioned Adult Rat Spinal Cord
Authors: Christie Sean Dennis , Sadi Damaso , Mendez Ivar
Published: December 2014

Background:

The role of neural transplantation as a restorative strategy for spinal cord injury continues to be intensely investigated. Ideally, the tissue source for transplantation must be readily available, free of disease and able to survive and mature following implantation into the adverse environment created by the injury. We have studied the use of a commercially available cell line of cultured human neurons (hNT neurons) as a tissue source for neural transplantation in spinal cord injury.

Methods:

Following a left lateral thoracic hemisection, 54 immunosuppressed, female Wistar rats were randomly allocated into different treatment groups; hemisection only or hemisection and hNT cell transplantation (via a bridge, double or triple graft). Grafting occurred three days after spinal cord injury. After thirteen weeks the animals were sacrificed and tissue sections were stained with human neuron specific enolase and human specific neural cell adhesion molecule.

Results:

Immunohistochemical evidence of graft survival was displayed in 66.7% of the surviving, grafted animals. Fibre outgrowth, greatest in the bridge and triple grafts, was observed in both rostral and caudal directions essentially bridging the lesion. Double grafts were smaller, displaying less fibre outgrowth, which did not cross the lesion. Long fibre outgrowth was evident up to 2 cm from the graft as assessed by tracing and immunohistochemical studies.

Conclusion:

Bridge and triple grafts displayed greater growth and enabled the hNT graft to essentially bridge the lesion. This suggests that hNT neurons have the potential to structurally reconnect the proximal and distal spinal cord across the region of injury.
Fabry’s Disease Presenting as Stroke in a Young Female
Authors: Giacomini Paul S. , Shannon Patrick T. , Clarke Joe T.R. , Jaigobin Cheryl
Published: December 2014

Background:

Fabry’s disease is an X-linked disorder, caused by a deficiency of the lysosomal enzyme α-galactosidase A which results in the accumulation of the glycosphingolipid, ceramide trihexose in the vascular endothelium and can lead to cerebral infarction. Male hemizygotes are generally more severely affected than heterozygote females. Clinical disease in females is thought to be due to unequal X chromosome inactivation.

Case:

A 19-year-old woman, who was previously well, presented with neurological deficits secondary to basal ganglia and pontine infarction. Extensive cardiac, arterial and hematologic investigations did not identify the etiology of her stroke. Muscle biopsy revealed endothelial lysosomal aggregates most consistent with a diagnosis of Fabry’s disease. The diagnosis was confirmed on the basis of molecular genotype analysis.

Discussion:

Inherited causes of stroke such as Fabry’s disease should be considered in young patients with stroke if an etiologic diagnosis is not reached after complete investigations. Muscle biopsy can assist with the diagnosis and guide further investigations. This report summarizes the biochemical and histological features of Fabry’s disease and the associated genetic abnormalities.
Renal Cell Carcinoma Metastatic to the Choroid Mimicking Intraventricular Meningioma
Authors: Quinones-Hinojosa Alfredo , Chang Edward F. , Khan Saad A. , Lawton Michael T. , McDermott Michael W.
Published: December 2014

Background:

Metastatic renal cell carcinoma to the choroid plexus is a rare condition and can be easily confused with meningioma.

Methods:

A 61-year-old female presented with progressive neurologic deterioration and MRI findings of obstructive hydrocephalus and a homogeneously contrast enhancing 3 cm oval mass in the trigone of the left lateral ventricle.

Results:

Despite radiologic, intraoperative gross features, and frozen pathology all consistent with meningioma, the final pathology revealed metastatic renal cell carcinoma.

Conclusion:

Renal cell carcinoma metastatic to the choroid plexus can mimic intraventricular meningioma. We present a review of the literature and comparison of the radiological features of meningiomas and metastatic renal cell carcinoma. We also discuss the use of an under-utilized technique, the contralateral transcallosal approach, in the surgical treatment of this intraventricular lesion.
The Electrodiagnosis of Ulnar Nerve Entrapment at the Elbow
Authors: Kern Ralph Z.
Published: December 2014

Entrapment of the ulnar nerve at the elbow is the second most common focal peripheral neuropathy. Recent advances have facilitated the electrodiagnosis of this common nerve entrapment. The goals of electrodiagnosis are to localize ulnar nerve dysfunction, confirm that the disturbance is confined to the ulnar nerve, and assess the severity of ulnar nerve dysfunction. The goal of this review is to highlight the important advances in anatomy, neurophysiology and methodology that impact upon the electrodiagnosis of entrapment of the ulnar nerve at the elbow, illustrate the limits of electrodiagnosis, and discuss methodological issues that may be the subject of further study. Careful attention to elbow position, temperature, and conservative estimates of conduction block should be part of common practice. Awareness of anatomical variations in structural anatomy, anomalous innervation and fascicular arrangement of ulnar nerve fibers are required to interpret electrodiagnostic studies accurately. The most reliable finding is slowing of the ulnar across-elbow motor nerve conduction velocity to less than 50 m/sec while recording from the abductor digiti minimi muscle, and should be carefully interpreted in the presence of a polyneuropathy or other neurogenic process. Alternative techniques such as relative ulnar slowing in different ulnar nerve segments, use of alternative muscles, sensory and mixed nerve techniques provide complementary information, and like all nerve conduction studies are highly operator-dependent and should be used on a case by case basis. Recent studies have focused the electromyographer's attention on the use of shorter across-elbow segments (2-5 cm). This may offer a reasonable trade-off between sensitivity and measurement error and may result in improved electrodiagnosis.
Cell-Cycle Gene Expression in Lovastatin-Induced Medulloblastoma Apoptosis
Authors: Wang Wei , Macaulay Robert J.B.
Published: December 2014

Background:

3-hydroxy-3-methylglutaryl coenzyme A (HMG-CoA) reductase is a key rate-limiting enzyme in the mevalonate pathway, which generates precursors both for cholesterol biosynthesis and for the production of nonsteroidal mevalonate derivatives that are involved in a number of growth-regulatory processes. We have reported that lovastatin, a competitive inhibitor of HMG-CoA reductase, not only inhibits medulloblastoma proliferationin vitro, but also induces near-complete cell death via apoptosis. The mechanism of this phenomenon is unclear. Possible involvement of changes in expression of certain cell-cycle related genes led us to study some of them in more detail.

Methods:

Medulloblastoma cell lines were exposedin vitroto lovastatin, and the effects of gene expression changes were studied using RT-PCR, antisense oligonucleotide, DNA electrophoresis and Western blotting analysis.

Results:

1) Levels of totalRasgene mRNA and individualRasgene mRNA are stable in lovastatin treatment in all examined medulloblastoma cell lines. 2) Blockingc-mycgene over-expression does not enhance medulloblastoma cell sensitivity to lovastatin. 3) Following lovastatin treatment,p16expression exhibits no change, but pronounced increases of p27KIP1protein are observed in all examined cell lines. Lovastatin induces pronounced increases of p21WAF1protein only in Daoy and UW228, but not in D283 Med and D341 Med. 4) Following lovastatin treatment, increased p53 protein is detected only in D341 Med, and bax protein is unchanged in all cell lines.

Conclusion:

Lovastatin-induced growth inhibition and apoptosis in medulloblastoma are not dependent on the regulation ofRasandc-mycgene expression, but may be mediated byp27KIP1gene expression. Lovastatin-induced apoptosis in medulloblastoma is probably p53 independent, butp53andp21WAF1gene expression may also mediate anti-proliferative effects of lovastatin on specific medulloblastoma cell lines.
Early Arrest of Mental and Motor Development Associated with Bilateral Hippocampal Anoxic Damage
Authors: Fisher C.M.
Published: December 2014

Background:

Clinical-pathological study of the case of a woman with amentia from birth.

Case history:

A 45-year-old woman was institutionalized since the age of two and showed no change in 43 years. She did not stand, walk, talk, use her hands or comprehend. She was placed in a chair each day. She lacked control of bladder and bowel. She showed no mental capability.

Case pathology:

The brain weighed 1250 gm and was normally developed. There were two anoxic-ischemic regions of damage. The hippocampus showed extensive damage bilaterally and the cerebellum showed watershed infarction bilaterally.

Conclusion:

A perinatal anoxic-ischemic episode is suspected. Hippocampal damage was the only finding that might explain the failure of mental and motor development. If this hypothesis is correct, the function of the human hippocampus is broader than currently accepted.
Sarcomatous Change After Sellar Irradiation in a Growth Hormone-Secreting Pituitary Adenoma
Authors: Prabhu Sujit S. , Aldape Kenneth D. , Gagel Robert F. , Benjamin Robert S. , Trent Jonathan C. , McCutcheon Ian E.
Published: December 2014

Background:

Although the benefits of radiotherapy for pituitary adenomas are well-documented, post-irradiation sarcomas of the sella are rarely seen, with only 20 cases (mainly of fibrosarcoma) reported in the medical literature.

Method:

We describe a case of post-irradiation sarcoma five years after surgery followed by external-beam irradiation for an extensive and locally invasive growth hormone-secreting tumor. The patient was subsequently given pegvisomant, an antagonist of growth hormone receptor, to control symptoms of growth hormone excess.

Results:

The patient underwent transsphenoidal resection of the recurrent tumor, followed by adjuvant chemotherapy. This led to significant relief in the patient's symptoms including radiological evidence of tumor shrinkage, but the tumor regrew when, owing to dose-limiting toxicity, chemotherapy was stopped.

Conclusion:

Post-irradiation sarcomas of the pituitary are well-recognized but rare. They should be suspected in patients following sellar irradiation who show abrupt onset of new symptoms and appropriate radiological findings, and such tumors may respond to cytotoxic chemotherapy.
Distal Mycotic Aneurysm of the AICA Mimicking Intracanalicular Acoustic Neuroma
Authors: DiMaio Salvatore , Mohr Gérard , Dufour Jean-Jacques , Albrecht Steffan
Published: December 2014

Background:

Among cases of cerebellopontine angle lesions, vascular lesions involving the internal auditory canal are extremely rare. We report a distal fusiform mycotic pseudoaneurysm of the anterior inferior cerebellar artery (AICA) that simulated an acoustic neuroma on presentation.

Methods:

A 60-year-old woman was investigated for recent onset of acute dizziness. Laboratory and radiographic investigations are presented, as well as the surgical management of the patient and pathological examination of the aneurysm.

Conclusion:

An exceptionally rare case of distal mycotic intracanalicular pseudoaneurysm of the AICA with intraluminal thrombus and fusiform anatomy is described. In our review of the literature (1966-present), only five other intracanalicular AICA-aneurysms were encountered, none of which were infectious in etiology. The possible pathophysiologic mechanisms of distal AICA-aneurysms are discussed along with the currently available literature.
The Fluctuating Parkinsonian Patient - Clinical and Pathophysiological Aspects
Authors: Blanchet Pierre J.
Published: December 2014

Although levodopa-related motor response complications remain challenging from a pathophysiological and therapeutic standpoint, major advances have been made in the last decade, supporting the development of several promising drugs. Eventually, these drugs may help us to prevent, alleviate, or even “deprime” these frequent and disabling complications. Knowledge of the basic mechanisms and hypotheses underlying this fascinating conversion in the parkinsonian brain allows neurologists to understand the rationale behind emerging treatment strategies.
Stereotactic Neurosurgery for Movement Disorders
Authors: Abosch Aviva , Lozano Andres
Published: December 2014

Stereotactic neurosurgery for the treatment of movement disorders focuses primarily on the treatment of Parkinson's disease (PD), essential tremor (ET), and dystonia. The surgical targets in use are the subthalamic nucleus (STN) and the globus pallidus internus (GPi) for PD, GPi for dystonia, and ventralis intermedius (Vim) nucleus of the thalamus for ET. Following target selection, procedures include the generation of lesions or the placement of deep brain stimulating electrodes in the selected target. Additionally, transplantation has been used in the treatment of PD. The indications, outcomes, and risks of the various procedures are reviewed.
Psychogenic Movement Disorders
Authors: Miyasaki Janis M. , Sa Daniel S. , Galvez-Jimenez Nestor , Lang Anthony E.
Published: December 2014

Psychogenic movement disorders (PMD) are challenging to diagnose and to treat. Since the nineteenth century, PMDs were recognized and described in painstaking detail. In the modern neurology clinic, PMDs may comprise 2-25% of the patient population. Recognition of the various types of PMDs, differentiation from organic illness and an approach to PMDs are described in this article.
Prevalence of Reported Migraine Headaches in Canadian Adolescents
Authors: Gordon K.E. , Dooley J.M. , Wood E.P.
Published: December 2014

Objective:

To examine self-reported prevalence data for migraine among adolescent Canadians and to explore how reported migraine treatment varies by age.

Methods:

We analyzed the microdata files of the Canadian National Population Health Survey (1996-1997). Respondents reported whether they had “migraine headaches diagnosed by a health professional”. They also reported whether they received “any treatment or medication for migraine headaches”, with treatments subdivided into drug, diet or “other”.

Results:

99.9% of 173,216 eligible respondents reported whether they had migraine headaches. Migraine was reported by 2.4% of Canadian youth aged 12-14 years and by 5.0% of 15-19 year-olds compared to 7.2% of adults aged 20 years of age (p< 0.0001, chi-square). Active treatment was used by 51.0% - higher by females (53.1%) than males (44.7%) (p<0.0001 chi-square). Treatment was used by 45.1% of 12-14 year-olds, by 45.7% of 15-19 year-olds and by 51.5% of those 20 years (p=0.0027). The nature of the active treatment choice (drug, diet or other) did not significantly vary within the age groups studied.

Conclusion:

We present robust estimates of self-report diagnosed migraine prevalence, derived from a large nationally representative population survey. Estimates of the prevalence of active treatment for migraine provide insight into the burden of migraine within this population.
Pallidal Deep Brain Stimulation in Cervical Dystonia: Clinical Outcome in Four Cases
Authors: Eltahawy H.A. , Saint-Cyr J. , Poon Y.Y. , Moro E. , Lang A.E. , Lozano A.M.
Published: December 2014

Objective:

Report on the clinical results following bilateral globus pallidus interna deep brain stimulation in four patients (one female and three males) with severe cervical dystonia, mean age 48 years (range 37-67).

Methods:

All four patients had failed extensive medical and botulinum toxin treatment. The mean duration of the disease was nine years (range 4-15 years). Patients were assessed pre and postoperatively using the Toronto Western Spasmodic Torticollis Rating Scale (TWSTRS). Preoperatively, the mean TWSTRS total score was 43.2 (range 28-60.5). Posteroventral pallidal deep brain stimulators were inserted using MRI and microelectrode recording guidance. Last follow-up was 15 months for the four patients.

Results:

Mean reduction in the TWSTRS total scores at last follow- up was 73% (range 61- 85%). Improvement in pain occurred soon after deep brain stimulation surgery. Motor improvement was delayed and prolonged over several months. Frequent adjustment in the stimulation parameters was necessary in the first three months.

Conclusion:

Bilateral pallidal stimulation is effective in management of selected cases of intractable cervical dystonia.
Electrocardiogram Artifacts Caused by Deep Brain Stimulation
Authors: Constantoyannis Constantine , Heilbron Brett
Published: December 2014

Background:

Deep brain stimulation (DBS) is increasingly used to treat a variety of neurological conditions (e.g. movement disorders and chronic pain). This prospective study was designed to detect electrocardiogram (ECG) artifacts induced by deep brain stimulation and to investigate which factors (patient disease, electrode position within the brain or type of stimulation) produced these artifacts.

Methods:

Twelve patients (four women, eight men) with deep brain stimulators were enrolled in the study. Patients were selected to represent the common indications for DBS (Parkinson's disease, tremor, dystonia), the common electrode locations (pallidum, thalamus, subthalamic nucleus) and the two types of stimulation (monopolar, bipolar). Patients had one ECG with the DBS turned 'on'and another with the DBS turned 'off'. The ECGs were then randomized and read by a cardiologist blinded to the status of the patient and DBS and artifacts were noted to be either present or absent.

Results:

The six patients using monopolar stimulation all had artifacts on their electrocardiograms. These artifacts were severe enough to interfere with ECG interpretation. There were no artifacts detected in the six patients using bipolar stimulation. Electrode location and patient disease appeared to have no effect on ECG artifact.

Conclusion:

Deep brain stimulation can cause ECG artifacts when monopolar settings are used. These artifacts are not present with bipolar settings or when the DBS is turned 'off'. Knowledge of these potential ECG artifacts and how to avoid them is essential to facilitate accurate ECG interpretation.
Collision Frequency in Elite Hockey on North American versus International Size Rinks
Authors: Wennberg Richard
Published: December 2014

Objective:

Body impact or collision is the risk factor underlying all sports-related concussions. This study sought to determine whether collision rates in elite hockey differ between games played on North American size rinks as compared to games played on larger international size ice surfaces.

Methods:

Videotapes of games from the 2001 and 2002 National Hockey League Stanley Cup finals, World Junior championships and the 2002 Winter Olympics were analyzed, with all collisions counted and separated into various categories (player/player bodycheck, player/player into boards, player/boards, player/ice, head/stick, head/puck). Further subdivisions included collisions involving the head directly or indirectly. Twenty-two games were analyzed, 11 played on the small ice and 11 on the big ice.

Results:

Significantly more collisions of all types (in all categories and subdivisions within categories) were found to occur on the smaller North American ice surface (P value differences from 0.01 to 0.00001).

Conclusion:

The results of this study showed significantly fewer collisions of all types in elite hockey games played on the international size ice surface. The comparison groups studied here did differ in some aspects other than ice size and so replication of the findings with even more closely matched groups will be needed before definitive conclusions can be drawn. However, if these findings are replicable, it would suggest that a change to uniform usage of the larger international rinks, with no rule changes or other alterations in the game, could provide direct primary prevention to reduce the number of collisions, and, by extension, concussions, that occur in the sport.
Neurologic Course, Endocrine Dysfunction and Triplet Repeat Size in Spinal Bulbar Muscular Atrophy
Authors: Sinnreich Michael , Sorenson Eric J. , Klein Christopher J.
Published: December 2014

Objective:

To study the role of diabetes, gynecomastia and CAG triplet repeat size as disease modifying factors of neurologic expression in spinal bulbar muscular atrophy (SBMA, Kennedy's disease).

Methods:

Twenty unrelated SBMApatients with confirmatory genetic testing were reviewed. Patterns of neurologic involvement were assessed (e.g. bulbar, asymmetric, proximal, distal, motor and sensory). Slopes of disease progression were calculated from serial quantified neurologic examinations. Patterns of neurologic involvement and course were correlated to the presence of diabetes, gynecomastia and triplet repeat size.

Results:

Diabetes or glucose impairment occurred in nine and 11 had gynecomastia. Patterns of neurologic involvement and rates of progression did not correlate with these endocrine diseases or triplet repeat sizes. Correlation was seen between number of CAG repeats and age of onset weakness (r = -0.53, r2 = 29%, p = 0.01).

Conclusion:

The specific neurotoxic effect of expanded CAGs appears limited to age of onset weakness in SBMA. Although significant, only 29% of the variability in onset age could be accounted for by polyglutamine size suggesting the importance of other unidentified factors. In this series diabetes or glucose impairment was more common than previously reported and, like gynecomastia, did not correlate with size of triplet repeats, severity or patterns of neurologic involvement. Modifying factors other than diabetes, gynecomastia or triplet repeat size are suggested in disease expression.
Exacerbation of Pre-existing Epilepsy by Mild Head Injury: a Five Patient Series
Authors: Tai P.C. , Gross D.W.
Published: December 2014

Objective:

While the risk of developing seizures following a mild head injury has been reported and is thought to be low, the effect of mild head injury on patients with a pre-existing seizure disorder has not been reported. We present a series of cases where a strong temporal relationship between mild head injury and worsening of seizure frequency was observed.

Methods:

Five cases were identified and reviewed in detail. Information was derived from clinic and hospital charts with attention to the degree of injury, pre- and postinjury seizure patterns and frequency.

Results:

One patient has primary generalized epilepsy and four have localization related epilepsy. Prior to the head injury, three of the patients were seizure free (range: two to 24 years). The patients suffered from mild head injuries with no or transient loss of consciousness and no focal neurological deficits. In all cases, the patients experienced a worsening of seizure control within days of the injury. In one case, the patient's seizure pattern returned to baseline one year after the accident, while in the remaining four cases, the patients continue to have medically refractory seizures.

Conclusion:

A close temporal relationship between mild head injury and a worsening of seizure control was observed in five patients with epilepsy. Although further study is required, this observation suggests that a head injury that would be considered benign in the general population can have serious consequences such as recurrence of seizures and medical intractability in patients with epilepsy.
Lymphocytic Hypophysitis with a Long Latent Period Before Development of a Pituitary Mass
Authors: Wong Rene W.G. , Ooi Teik Chye , Benoit Brien , Zackon David , Jansen Gerard , Telner Adam
Published: December 2014

Background:

Lymphocytic hypophysitis is an autoimmune condition that commonly presents in women of childbearing age as hypopituitarism and a sellar mass.

Case report:

A 66-year-old woman presented with anterior pituitary dysfunction. Computed tomography imaging revealed a small hypodensity that was not felt to be the cause of the pituitary dysfunction. Eight years later, her vision rapidly deteriorated and MRI showed a pituitary mass lesion causing optic chiasm compression. Histological examination of the partially resected gland revealed evidence of lymphocytic hypophysitis.

Conclusion:

Our patient is an example of the variable presentation and course of lymphocytic hypophysitis. Such a long latent period between the initial presentation of adenohypophysial hypofunction and optic chiasm compression due to an enlarging pituitary mass has not been reported.
Intraparenchymal Supratentorial Neurenteric Cyst
Authors: Kachur Edward , Ang Lee-Cyn , Megyesi Joseph F.
Published: December 2014

Background:

Neurenteric cysts are congenital cysts of the central nervous system that are believed to be of endodermal origin. In this report we present the unique case of a supratentorial neurenteric cyst that is contained entirely within the brain parenchyma.

Methods:

Apatient presented with an intraparenchymal cystic lesion that was subsequently identified as a neurenteric cyst. This lesion is reviewed in light of the available literature.

Case report:

A 35-year-old female presented with a one year history of progressive headaches and seizure-like episodes. Her examination revealed no deficits. Magnetic resonance imaging showed a 4 cm x 4 cm x 4 cm cystic lesion within the parenchyma of the right frontal lobe. A right frontal craniotomy and complete excision of the cystic lesion was performed. Pathologic examination confirmed that it was a neurenteric cyst. Postoperatively the patient's symptoms improved.

Conclusion:

Review of the literature revealed this to be the first case of a surgically excised, pathologically confirmed supratentorial neurenteric cyst, contained entirely within the brain parenchyma.
Predicting Final Disposition after using the Orpington Prognostic Scor
Authors: Wright C.J. , Swinton L.C. , Green T.L. , Hill M.D.
Published: December 2014

Background:

Prediction of outcome after stroke is important for triage decisions, prognostic estimates for family and for appropriate resource utilization. Prognostication must be timely and simply applied. Several scales have shown good prognostic value. In Calgary, the Orpington Prognostic Score (OPS) has been used to predict outcome as an aid to rehabilitation triage. However, the OPS has not been assessed at one week for predictive capability.

Methods:

Among patients admitted to a sub-acute stroke unit, OPS from the first week were examined to determine if any correlation existed between final disposition after rehabilitation and first week score. The predictive validity of the OPS at one week was compared to National Institute of Health Stroke Scale (NIHSS) score at 24 hours using logistic regression and receiver operator characteristics analysis. The primary outcome was final disposition after discharge from the stroke unit if the patient went directly home, or died, or from the inpatient rehabilitation unit.

Results:

The first week OPS was highly predictive of final disposition. However, no major advantage in using the first week OPS was observed when compared to 24h NIHSS score. Both scales were equally predictive of final disposition of stroke patients, post rehabilitation.

Conclusion:

The first week OPS can be used to predict final outcome. The NIHSS at 24h provides the same prognostic information.
Infection Rates in Standard vs. Hydrogel Coated Ventricular Catheters
Authors: Kaufmann Anthony M. , Lye Tara , Redekop Gary , Brevner Angela , Hamilton Mark , Kozey Michelle , Easton David
Published: December 2014

Background:

Infection related to external ventricular drain (EVD) use is a common neurosurgical complication. Modified catheters with a hydrophilic surface may impede bacterial adherence and thereby reduce catheter related cerebrospinal fluid (CSF) infection.

Methods:

A prospective randomized clinical trial compared the occurrence of CSF infection related to use of either standard silastic or hydrogel coated EVD catheters (Bioglide®, Medtronic). Enrolment was available to all adult neurosurgery patients undergoing placement of a first EVD, at three university centers. The catheters were presoaked in a low concentration of bacitracin solution for 5-10 minutes prior to insertion. Bacterial infection was defined by heavy growth in a single CSF sample or light / medium growth in two consecutive samples. A secondary analysis was also conducted for “probable” CSF infection, including patients started on antibiotics after light / medium growth in a single CSF sample. Statistical analyses included Kaplan-Meier survival curve estimates accompanied by Log Rank and Breslow tests.

Results:

There were 158 randomized patients available to assess for EVD related infection of CSF. The two study groups had similar clinical characteristics including average duration of EVD use (8±4 days). Definite CSF infection occurred in seven and probable infection in another six (8% total). Infection incidence rose steadily from day 2 (1%) to day 11 (11%). There was no difference of daily occurrence of EVD infection between the two catheter types.

Conclusion:

Infection remains a common hazard in the use of EVD, and we found no reduction of infection using the hydrogel-coated catheters when presoaked in low concentration bacitracin solution.
A Mathematical Model for Prediction of Drug Molecule Diffusion Across the Blood-Brain Barrier
Authors: Burns Jonathan , Weaver Donald F. , Burns Jonathan , Weaver Donald F.
Published: December 2014

Background:

Predicting the ability of drugs to enter the brain is a longstanding problem in neuropharmacology. The first step in creating a much-needed computational algorithm for predicting whether a drug will enter brain is to devise a rigorous mathematical model.

Methods:

Employing two experimental measures of blood-brain barrier (BBB) penetrability (brain/plasma ratio and the brain-uptake index) and 14 theoretically derived biophysical predictors, a mathematical model was developed to quantitatively correlate molecular structure with ability to traverse the BBB.

Results:

This mathematical model employs Stein's hydrogen bonding number and Randic's topological descriptors to correlate structure with ability to cross the BBB. The final model accurately predicts the ability of test molecules to cross the BBB.

Conclusion:

A mathematical method to predict blood-brain barrier penetrability of drug molecules has been successfully devised. As a result of bioinformatics, chemoinformatics and other informatics-based technologies, the number of small molecules being developed as potential therapeutics is increasing exponentially. A biophysically rigorous method to predict BBB penetrability will be a much-needed tool for the evaluation of these molecules.
The Behavioural Neurology Assessment
Authors: Darvesh S. , Leach L. , Black S. E. , Kaplan E. , Freedman M.
Published: December 2014

Background:

We present information regarding the standardization, reliability and clinical validity of two versions of the Behavioural Neurology Assessment (BNA). The BNA-Long Form consists of 24 subtests within separate domains: Attention, Memory, Language, Visuospatial Function, Executive Function, and Praxis. The BNA-Short Form consists of 13 subtests within the domains of Attention, Memory, Naming, Visuospatial Function and Executive Function. In addition to individual domain indices, a Grand Total score was calculated for both BNA versions.

Objective:

To standardize the administration and scoring and validate the BNA for detection of dementia.

Methods:

Standardized normative data were obtained on 115 healthy subjects ranging in age from 50 to 95. Test-retest stability was obtained on 19 subjects and clinical validity was investigated by administering the BNA and Mini-Mental Status Examination (MMSE) to 29 patients with dementia and 29 age-matched healthy subjects (controls).

Results:

Age had a significant effect on all but the Visuospatial and Praxis indices of the BNA-Long Form and an effect on Naming and Grand Total score of the Short-Form. Internal consistency (Cronbach's coefficient a) was .87 and .67 for the Long and Short Forms (.95 and .96 for dementia and control groups combined). Test-retest stability was acceptable. Grand Total indices of both BNA versions showed significant, positive correlations with the MMSE. Both BNA versions had superior sensitivity to dementia relative to the MMSE (.93 versus .79). Specificity was equivalent to the MMSE (.93 versus .97).

Conclusions:

Positive predictive values of the BNA and MMSE are equivalent but the BNA provides superior negative predictive value.
Ligamentum Flavum Cysts Causing Incapacitating Lumbar Spinal Stenosis
Authors: DiMaio Salvatore , Marmor Eric , Albrecht Stephan , Mohr Gérard
Published: December 2014

Background:

Cysts of the ligamentum flavum are rare and unusual causes of spinal compression.

Methods:

We report our experience of four cases of ligamentum flavum cysts occurring in the lumbar spine and discuss some of the possible etiologies and pathophysiologic mechanisms according to the available literature.

Conclusion:

This entity is clearly different from the synovial facet-joints or ganglion cysts.
Primary Central Nervous System Angiitis in a 10-year-old Girl
Authors: Rafay Mubeen F. , Sutcliffe Trenna L. , Shroff Manohar , Hawkins Cynthia , Weiss Shelly , Benseler Susanne M. , deVeber Gabrielle
Published: December 2014

Primary angiitis of central nervous system (PACNS) is well reported, however its occurrence in the pediatric population is infrequent. We describe the clinical, neuroimaging and histopathological features of PACNS in a young girl.

A ten year-old, previously healthy girl presented with a threeweek history of progressive left hemiparesis and facial weakness. Other findings included left hemineglect, impaired concentration and memory. She had no evidence of systemic disease. Head computed tomography (CT), with and without contrast, revealed no abnormalities. Magnetic resonance imaging (MRI) demonstrated multifocal, bilateral signal abnormalities within basal ganglia, thalami, right frontal cortex and subcortical white matter on T2 and FLAIR images. However, these areas did not show restricted diffusion on diffusion weighted imaging (DWI). Cerebral angiography revealed normal intracranial vessels (Figure 1). Neuroimaging, three weeks later,
showed progression of these abnormalities. The areas of abnormal signal showed heterogeneous and nodular enhancement on gadolinium enhanced T1 weighted images
(Figure 2). Rheumatologic, metabolic, prothrombotic and cerebral spinal fluid investigations were normal.
Intra-arterial Thrombolysis of Embolic Middle Cerebral Artery Using Collateral Pathways
Authors: Kole Max K. , Pelz David M. , Lee Donald H. , Jain Vivek , Spence J. David , Lownie Stephen P.
Published: December 2014

Background and purpose:

Cervical internal carotid artery (ICA) occlusion associated with middle cerebral artery (MCA) embolic occlusion requires prompt revascularization to prevent devastating stroke. With the advent of endovascular techniques for chemical and mechanical thrombolysis, the clinical outcome of patients with major arterial occlusions will improve. Finding the most expedient pathway to the site of end organ occlusion for thrombolysis is important.

Methods:

We present two cases of acute stroke secondary to thrombotic occlusion of the cervical ICA associated with MCA embolic occlusion treated with intra-arterial thrombolysis via catheter navigation through the posterior communicating artery to the site of MCA arterial occlusion. No attempt was made to transverse the occluded ICA.

Results:

Near complete restoration of flow was achieved in one patient and minimal vessel reopening was observed in the other patient. Both patients had good outcomes.

Conclusion:

Intraarterial thrombolysis via Circle of Willis collaterals such as the posterior communicating artery for the treatment of acute thrombotic occlusion of the cervical internal carotid artery associated with embolic occlusion of the middle cerebral artery is a therapeutic option. This treatment option avoids the potential complications of navigating through an occluded proximal internal carotid artery and may expedite reopening of the MCA.
Clinical Stringency Greatly Improves Mutation Detection in Rett Syndrome
Authors: Gauthier Julie , Amorim Giovana de , Mnatzakanian Gevork N. , Saunders Carol , Vincent John B. , Toupin Sylvie , Kauffman David , St-Onge Judith , Laurent Sandra , Macleod Patrick M. , Minassian Berge A. , Rouleau Guy A.
Published: December 2014

Background:

Rett syndrome (RTT) is a severe neurodevelopmental disorder of girls, caused by mutations in the X-linked MECP2 gene. Worldwide recognition of the RTT clinical phenotype in the early 1980's allowed many cases to be diagnosed, and established RTT as one of the most common mental retardation syndromes in females. The years since then led to a refinement of the phenotype and the recent elaboration of Revised Diagnostic Criteria (RDC). Here, we study the impact of the presence versus the absence of the use of diagnostic criteria from the RDC to make a diagnosis of RTT on MECP2 mutation detection in Canadian patients diagnosed and suspected of having RTT.

Methods:

Using dHPLC followed by sequencing in all exons of the MECP2 gene, we compared mutation detection in a historic cohort of 35 patients diagnosed with RTT without the use of specific diagnostic criteria to a separate more recent group of 101 patients included on the basis of strict fulfillment of the RDC.

Results:

The MECP2 mutation detection rate was much higher in subjects diagnosed using a strict adherence to the RDC (20% vs. 72%).

Conclusions:

These results suggest that clinical diagnostic procedures significantly influence the rate of mutation detection in RTT, and more generally emphasize the importance of diagnostic tools in the assessment of neurobehavioral syndromes.