bluuebunny/crossref_metadata_2025 huggingface dataset:
Diffusion Tensor Imaging Abnormalities in Focal Cortical Dysplasia
Authors: Gross Donald W , Bastos Alexandre , Beaulieu Christian
Published: December 2014

Purpose:

Focal cortical dysplasia (FCD) is one of the most common underlying pathologic substrates in patients with medically intractable epilepsy. While magnetic resonance imaging (MRI) evidence of FCD is an important predictor of good surgical outcome, conventional MRI is not sensitive enough to detect all lesions. Previous reports of diffusion tensor imaging (DTI) abnormalities in FCD suggest the potential of DTI in the detection of FCD. The purpose of this study was to study subcortical white matter underlying small lesions of FCD using DTI.

Methods:

Five patients with medically intractable epilepsy and FCD were investigated. Diffusion tensor imaging images were acquired (20 contiguous 3mm thick axial slices) with maps of fractional anisotropy (FA), trace apparent diffusion coefficient (trace/3 ADC), and principal eigenvalues (ADC parallel and ADC perpendicular to white matter tracts) being calculated for each slice. Region of interest analysis was used to compare subcortical white matter ipsilateral and contralateral to the lesion.

Results:

Three subjects with FCD associated with underlying white matter hyperintensities on T2 weighted MRI were observed to have increased trace/3 ADC, reduced fractional anisotropy and increased perpendicular water diffusivity which was greater than the relative increase in the parallel diffusivity. No DTI abnormalities were identified in two patients with FCD without white matter hyperintensities on conventional T2-weighted MRI.

Conclusions:

While DTI abnormalities in FCD with obvious white matter involvement are consistent with micro-structural degradation of the underlying subcortical white matter, DTI changes were not identified in FCD lesions with normal appearing white matter.
The Impact of a Stroke Prevention Clinic in Diagnosing Modifiable Risk Factors for Stroke
Authors: Mouradian Mikael S. , Hussain Muhammad S. , Lari Harris , Salam Abdul , Senthilselvan Ambikaipakan , Dean Naeem , Shuaib Ashfaq
Published: December 2014

Objective:

To evaluate the referral patterns of patients to a stroke prevention clinic (SPC) and to test the adequacy of pre-referral diagnosis and management of modifiable risk factors for stroke.

Methods:

We collected prospective data on consecutive patients referred to the SPC at University of Alberta Hospital in Edmonton, Alberta, Canada. Outcome measures included: alternate diagnoses to stroke or transient ischemic attack (TIA), uncontrolled or undiagnosed hypertension, hyperlipidemia and diabetes, therapies, and investigations leading to carotid endarterectomy.

Results:

Two thousand and eleven patients were referred to SPC. Nearly 25% of the referrals originated from the emergency room and the rest from general physicians. Of the referrals, 68.7% were confirmed as TIA or stroke at the SPC. Among 1381 patients with TIA or stroke, 736 had history of hypertension. Uncontrolled hypertension was found in 265 patients (36.0% of those with hypertension: 95% CI: 32.5–39.5) while undiagnosed hypertension was found in 103 (15.9% of those without hypertension: 95%CI: 13.14-18.79). History of hyperlipidemia was present in 451 patients (32.6%) and 356 (78.9%: 95% CI: 75.2-82.69) of these patients were not at target for secondary prevention. Among 930 patients without history of hyperlipidemia, 739 (79.5%: 95% CI: 76.8-82.1) were diagnosed with hyperlipidemia through the SPC. Fasting blood glucose levels above 7.1 mmol/L in patients with and without history of diabetes were 221 (79.2%: 95% CI: 74.5-83.9) and 66 (6%: 95%CI: 4.6-7.4) respectively.

Conclusions:

Management of risk factors for stroke needs improvement. SPCs should consider actively managing the classical modifiable risk factors of stroke.
Tension Pneumocephalus - The Mount Fuji Sign
Authors: Beiko Jason , McDonald Patrick
Published: December 2014

A 69-year-old female presented to the emergency department after becoming unresponsive at home following a progressive decrease in her level of consciousness (LOC) (See Figure 1). An urgent computerized tomography (CT) scan of the head demonstrated large bilateral chronic subdural hematomas (SDH’s). Due to the resulting mass effect she was brought urgently to the operating room where the SDH’s were evacuated via anterior and posterior burr holes placed bilaterally. No complications were encountered during the procedure. Prior to skin closure bilateral subdural catheters were placed in the posterior burr hole sites and left under closed suction.
Prognostic Indicators in an Aggressive Pituitary Crooke's Cell Adenoma
Authors: Kovacs K. , Diep C.C. , Horvath E. , Cusimano M. , Smyth H. , Coire C. , Lombardero M. , Scheithauer B.W. , Lloyd R.V.
Published: December 2014

Objective:

To investigate prognostic indicators in an aggressive Crooke's cell adenoma of the pituitary.

Methods:

The surgically removed tumor was studied by histology, immunohistochemistry and transmission electron microscopy.

Results:

An aggressive invasive sellar tumor removed by repeated surgeries from a 43-year-old woman with pituitary related Cushing's disease was classified as a Crooke's cell adenoma of the pituitary. The application of several cell proliferation markers confirmed the aggressive nature of the tumor.

Conclusions:

The investigation of the present case provides additional evidence that pituitary Crooke's cell adenomas may possess aggressive behavior.
Progress in Clinical Neurosciences: Frontotemporal Dementia-Pick's Disease
Authors: Kertesz Andrew
Published: December 2014

Frontotemporal dementia (clinical Pick's disease) is a relatively common, but underdiagnosed degenerative disease in the presenium. Estimated prevalence ranges from 6-12% of dementias. The behavioural, aphasic and extrapyramidal presentations are labeled FTD-behavioural variant, Primary Progressive Aphasia (PPA) and Corticobasal Degeneration/Progressive Supranuclear Palsy (CBD/PSP). The diagnostic features and course of each are described and their overlap in the evolution of the illness is emphasized. The neuropathology ranges from the most common tau negative ubiquitin positive amyotrophic lateral sclerosis (ALS) type inclusions to the tau positive classical Pick bodies and more or less distinct changes of PSP and CBD. The genetics of the relatively frequent tau mutations and the yet unsolved problem of tau negative families are discussed. The tau negative cases tend to be associated with the behavioural presentation and semantic dementia and the tau positive ones with PPA and the CBD/PSP syndrome. However the overlap is too great to split the disease. A glossary to navigate the proliferating terminology is included.
Triphasic Waves Versus Nonconvulsive Status Epilepticus: EEG Distinction
Authors: Boulanger Jean-Martin , Deacon Charles , Lécuyer Diane , Gosselin Sylvie , Reiher Jean
Published: December 2014

Background:

Triphasic waves (TWs) and generalized nonconvulsive status epilepticus (GNCSE) share morphological features that may create diagnostic ambiguity.

Objective::

To describe electroencephalographic differences between TWs and GNCSE.

Methods:

We retrospectively compared the electroencephalograms (EEGs) of two groups of patients presenting with decreased level of consciousness; those with TWs associated with metabolic encephalopathy and those with GNCSE. We studied the following: demographics, etiology and EEG morphological features. All EEGs were classified blindly (TWs or GNCSE) by two expert EEGers. Agreement between experts and concordance with clinical diagnosis were measured.

Results:

We analysed 87 EEGs (71 patients) with TWs and 27 EEGs (13 patients) with GNCSE. Agreement between experts and concordance with clinical diagnosis were excellent. When compared to TWs, epileptiform discharges associated with GNCSE had a higher frequency (mean=2.4Hz vs 1.8Hz) (p<0.001), a shorter duration of phase one (p=0.001), extra-spikes components (69% vs 0%) (p<0.001) and less generalized background slowing (15.1% vs 91.1%) (p<0.001). Amplitude predominance of phase two was common with TWs (40.8% vs 0%) (p=0.01). Lag of phase two was absent in all cases of GNCSE but present in 40.8% of patients with TWs. Noxious or auditory stimulation frequently increased the TWs (51%) while it had no effect on the epileptiform pattern (p=0.008).

Conclusion:

Certain EEG morphological criteria and the response to stimulation are very helpful in distinguishing TWs from GNCSE.
Subdural Effusions in the Posterior Fossa Associated with Spontaneous Intracranial Hypotension
Authors: Watanabe Arata , Horikoshi Toru , Uchida Mikito , Koizumi Hidehito , Yamazaki Hiromichi , Kinouchi Hiroyuki
Published: December 2014

Background:

Misdiagnosis of spontaneous intracranial hypotension remains a problem, despite increasing recognition.

Methods:

Three patients with spontaneous intracranial hypotension presented with typical findings on lumbar puncture, magnetic resonance (MR) imaging, and radioisotope cisternography. All patients showed subdural effusions in the posterior fossa on axial T2-weighted MR imaging. Axial MR images of 112 patients with other conditions were also screened for this finding.

Results:

One of three patients had typical orthostatic headache, and the other two had continuous headache. The finding of subdural effusions in the posterior fossa on axial T2-weighted MR imaging disappeared after treatment. Similar findings were found in 14 of 112 patients with other conditions. Most of the patients were over 60 years old or had dementia or previous radiation therapy.

Conclusion:

Subdural effusions in the posterior fossa can be identified by T2-weighted axial MR imaging, and are useful for the diagnosis of spontaneous intracranial hypotension and for verifying the effectiveness of treatment.
Acute Anterior Circulation Stroke: Recanalization Using Clot Angioplasty
Authors: Lum Cheemun , Stys Peter K. , Hogan Matthew J. , Nguyen Thanh B. , Srinivasan Ashok , Goyal Mayank
Published: December 2014

Background and Purpose:

Different strategies have been employed to recanalize acutely occluded middle cerebral and internal carotid arteries (ICA) in the setting of acute stroke including intravenous and intra-arterial tPA. However, pharmaceutical thrombolysis alone, may not be effective in patients with a large amount of clot volume (complete M1, terminal internal carotid artery). We report our initial experience with endovascular clot disruption using a soft silicone balloon in addition to intravenous or intraarterial thrombolysis with tPA.

Methods:

This is a retrospective review of nine patients with symptoms of acute stroke from clot in the middle cerebral or internal carotid territories who were treated with intracranial balloon angioplasty. All patients presented with symptoms of acute anterior circulation stroke less than six hours from onset. Patients in whom computed tomography (CT) angiography confirmed the presence of large vessel clot (terminal ICA, M1 or proximal M2) were included in the study. A CT perfusion was performed providing maps of cerebral blood volume, flow and mean transit time. If the patient presented less than three hours from onset then intravenous tissue plasminogen activator (tPA) was also administered. Intra-arterial tPA was delivered into the clot. If the volume of clot was judged to be significant by the treating neurointerventionist, then a limited trial of tPA was administered intraarterially followed by balloon angioplasty of persistant clot. The time from imaging to vessel recanalization was recorded. Clinical outcomes were assessed using the modified Rankin scale and Barthel Index.

Results:

Diagnostic CT perfusion studies were performed in 7 (78%), all of which showed a significant amount of salvageable tissue as judged by the treating neurointerventionist and neurologist. Recanalization (TIMI 2 or 3) was possible in 8 (89%). There were no cases of symptomatic intracranial hemorrhage and 2 (22%) asymptomatic hemorrhages. The average time from performance of the initial emergency CT to vessel recanalization was 2.1 hours with mean time from symptom onset to vessel recanalization of 4.1 hours. Five (56%) patients had good outcomes, 1 (11%) had mild and 3 (33%) had moderate to severe disability.

Conclusion:

Clot angioplasty can potentially shorten recanalization times in well-selected patients and can be an effective complimentary procedure in patients with tPA resistant clot. Angioplasty can be performed with a very low complication rate using the technique described and may be associated with good outcomes.
Demographics and Clinical Features of Patients Referred to Headache Specialists
Authors: Jelinski Susan E. , Becker Werner J. , Christie Suzanne N. , Giammarco Rose , Mackie Gordon F. , Gawel Marek J. , Eloff Arnolda G. , Magnusson Jane E.
Published: December 2014

Objective:

To examine demographic characteristics and clinical features of headache patients referred to neurologists specializing in headache in Canada.

Methods:

Demographic and clinical data were collected at the time of consultation for 865 new headache patients referred to five headache-specialty clinics in Canada. The Headache Impact Test (HIT-6) and Migraine Disability Questionnaire (MIDAS) were used to measure headache impact and disability. Data were analyzed as part of the Canadian Headache Outpatient Registry and Database (CHORD) Project.

Results:

The average age of the patients was 40 years and the majority were female (78%). Most were employed either full time (49%) or part time (13%). The majority of patients were diagnosed with either migraine or tension-type headache (78%). Over a third of patients experienced headache every day, and half had experienced a headache in the previous month which was of severe intensity. Most (80%) scored in the “very severe” category of the HIT-6 and over half (55%) were severely disabled as measured by the MIDAS.

Conclusion:

Patients referred to headache specialists in Canada are severely disabled by their headache disorders. These patients are in the most productive phase of their lives in terms of age and employment. It is important to provide the best available treatment to headache patients in order to minimize the disability and impact of their headache disorders.
Rasmussen's Encephalitis in a 58-Year-Old Female: Still a Variant?
Authors: Hunter Gary R. W. , Donat Jeffrey , Pryse-Phillips William , Harder Sheri , Robinson Christopher A.
Published: December 2014

Background:

We report the case of a 58-year-old female with clinical, radiological, and histopathological evidence of Rasmussen's encephalitis, representing the oldest confirmed case to date.

Case Summary:

The patient presented with complex partial seizures characterized by numbness of the left face and staring spells. These progressed to a state of epilepsia partialis continua with jerking of the left face, as well as severe cognitive impairment and loss of all communication. The patient responded well to Intravenous Immunoglobulin (IVIG) therapy despite early complications and with ongoing treatment is living independently with minimal cognitive impairment.

Conclusions:

This represents the oldest confirmed case of Rasmussen's encephalitis and suggests that this diagnosis should be considered in patients of any age with an appropriate clinical picture. We recommend IVIG as a first line therapy for adult cases of Rasmussen's encephalitis.
Canadian Association of Neurosciences Review: Learning at a Snail's Pace
Authors: Parvez Kashif , Rosenegger David , Orr Michael , Martens Kara , Lukowiak Ken
Published: December 2014

While learning and memory are related, they are distinct processes each with different forms of expression and underlying molecular mechanisms. An invertebrate model system,Lymnaea stagnalis, is used to study memory formation of a non-declarative memory. We have done so because: 1) We have discovered the neural circuit that mediates an interesting and tractable behaviour; 2) This behaviour can be operantly conditioned and intermediate-term and long-term memory can be demonstrated; and 3) It is possible to demonstrate that a single neuron in the model system is a necessary site of memory formation. This article reviews howLymnaeahas been used in the study of behavioural and molecular mechanisms underlying consolidation, reconsolidation, extinction and forgetting.
Intractable SUNCT Cured After Resection of a Pituitary Microadenoma
Authors: Leroux Elizabeth , Schwedt Todd J. , Black David F. , Dodick David W.
Published: December 2014

Background:

SUNCT is a rare primary headache disorder that is associated with activation of the posterior hypothalamus and often poorly responsive to medication. Recently, a relationship between between pituitary microadenoma and SUNCT has been suggested, and reports of both amelioration and exacerbation by dopamine-agonists have been published. These findings suggest a functional role for the hypothalamic-pituitary axis in SUNCT.

Methods:

We report the long-term 4 year follow-up of a 35 year-old patient with a 14-year history of medically and surgically intractable SUNCT who experienced immediate and complete resolution of symptoms after resection of a 6 mm pituitary microadenoma.

Results:

This patient was first seen at the age of 28 years with a 10-year history of attacks of right retro-orbital pain satisfying the IHS criteria for SUNCT. Many medical and surgical treatments were attempted without success. An MRI demonstrated a 6 mm microadenoma without compression of surrounding structures. A trial of bromocriptine caused marked exacerbation of his pain. The patient underwent a trans-sphenoidal resection of the pituitary lesion. SUNCT attacks worsened for the first 24h post-operatively, then disappeared. He has been completely headache-free, without medication, for the past 43 months with the last follow-up being January 2006.

Conclusion:

This case emphasizes the relationship between pituitary microadenomas and SUNCT, supports the role of the hypothalamic-pituitary axis in the genesis of SUNCT, and illustrates the importance of careful imaging of the pituitary region in patients with SUNCT.
Anterior Spinal Artery Infarction at the Cervicomedullary Junction
Authors: Gofton Teneille E. , Barber Philip A.
Published: December 2014

An 81-year-old right handed man presented with bilateral leg weakness. The patient had been previously independent and was medicated with warfarin for atrial flutter. He had longstanding type 2 diabetes, controlled by oral hypoglycemic medication. At presentation, he reported sudden onset left sided weakness with accompanying neck pain at 22h00 the previous evening. Pertinent findings on neurological examination included a left pronator drift and profound left leg weakness with unsustained antigravity strength. Deep tendon reflexes were brisk throughout but absent at the ankles and there was a left extensor plantar response. Cranial nerve and sensory examinations were normal.
Diagnostic Criteria of Dementia
Authors: Bouchard Rémi W.
Published: December 2014

In the past two decades there has been a tremendous effort among clinicians and searchers to improve the diagnostic criteria of the dementias on the basis of the differential neurological and neuropsychological profiles. This was an obligatory requirement for clinical trials and the development of treatments. Over the years it became rapidly evident that the cohorts of patients in studies had some degree of heterogeneity, making it difficult to interpret the results of some studies, particularly in the vascular dementias and the mild cognitive impairment (MCI) group. For example, many sub-types of the vascular group were included in clinical trials, such as the cortical strokes, the lacunar states and the diffuse white matter disease cases, and some of the patients might have had also mixed pathology. In addition, the standard DSM IV criteria for dementia no longer represent our present knowledge of the clinical profile of some of the dementias such as vascular dementia (VaD) and fronto-temporal dementia where the memory impairment is not necessarily the first requirement. To improve the validity of clinical trials and eventually help developing more appropriate treatments, we revised the present diagnostic criteria and made recommendations for some changes in the context of the 2nd Canadian Conference on the Development of Antidementia Therapies, held in 2004 and reviewed in the light of the recent literature as of early 2006. It is expected that in the near future, these dementia criteria for clinical trials will have to be revised again in order to include specific subtypes of the dementias as well as biomarkers, structural and functional imaging.
Assessment of Behavioural and Psychological Symptoms Associated with Dementia
Authors: Conn David , Thorpe Lilian
Published: December 2014

Neuropsychiatric symptoms (mood, psychotic, and behavioural) are very common in dementia and do not necessarily correlate well with other measures of cognition. However, these symptoms are of great importance, as they are a major source of excess disability, patient distress and caregiver burden and have great impact on the level of care required, and the associated costs. This paper is a review of the most useful outcome measures for behaviour and mood symptoms. Investigators who require a comprehensive instrument to measure neuropsychiatric symptoms in studies of patients with dementia should consider using the Neuropsychiatric Inventory (NPI), the Behavior Rating Scale for Dementia of the Consortium to Establish a Registry for Alzheimer's Disease (CERADBRSD) or, possibly, the Behavioral Pathology in Alzheimer's Disease Scale (BEHAVE-AD). The Cornell Scale for Depression in Dementia and the Dementia Mood Assessment Scale (DMAS) are recommended for evaluating depressive symptoms and the Cohen-Mansfield Agitation Inventory (CMAI) is very useful for evaluating the full range of agitation symptoms.
Biological Markers in Alzheimer's Disease
Authors: Bailey Peter
Published: December 2014

abstract

Biomarkers are required to improve our diagnostic sensitivity and specificity and to monitor the biological activity of the Alzheimer’s disease (AD) in terms of the burden of neural involvement and the tempo of disease progression. Biomarkers will initially supplement our more traditional neuropsychological and imaging markers but may eventually evolve into useful surrogate endpoints in AD research. These markers may also provide important mechanistic clues to the pharmacological action of anti-dementia compounds. At this point, the combination of elevated cerebrospinal fluid phosphorylated TAU (CSF p-TAU) proteins and low CSF A²1-42are the only biomarkers with the sensitivity and specificity to serve as useful diagnostic biomarkers capable of distinguishing AD from other dementias in the early stages. Advances in non CSF tests is urgently required. Markers assessing the progression of disease do not necessarily require the same high disease specificity as diagnostic markers, but need to be sensitive to changes in disease state. At present, candidate markers fall under four main biological rationales: 1. Specific markers of AD neuropathology; 2. Non-specific markers of neural degeneration; 3. Markers of oxidative stress; 4. Markers of neural inflammation. It is foreseeable that a panel of such markers might prove advantageous. It will be important to develop “non-invasive “ markers utilizing readily obtainable tissue samples such as serum or urine to monitor disease progression (or hopefully regression). Repeated sampling would allow for comparison with traditional neuropsychological and imaging measures. The assays themselves will need to be reproducible, reliable and relatively inexpensive. Unfortunately, these biomarkers are in the formative stages of testing and results at present are inconclusive. To facilitate biomarker development in the future it would be highly advantageous to begin to collect and store biological specimens as an adjunct to current research in AD.
Clinical Equipoise and Treatment Decisions in Cervical Spondylotic Myelopathy
Authors: Benatar Michael
Published: December 2014

Objective:

The primary objective of this study is to evaluate clinician attitudes towards the treatment of cervical spondylotic myelopathy (CSM) in order to determine whether clinical equipoise exists for a segment of this patient population. The secondary objective is to examine the factors that influence treatment decisions.

Methods:

Cross-sectional internet-based survey of neurologists, neurosurgeons and orthopedic surgeons.

Results:

Between 40-60% of respondents recommended surgery for (1) patients with minimal or no symptoms, but incidentally discovered increased T2 signal within the cervical cord on MRI, (2) patients with mild symptoms and indentation of the cervical cord but without increased T2 signal and (3) those with at least moderately severe clinical findings accompanied by MRI showing effacement of the thecal sac but without indentation of the cord or increased T2 signal. The severity of the radiological abnormalities most strongly influence treatment decisions.

Conclusion:

We conclude that clinical equipoise does exist for certain groups of patients with CSM, suggesting that a randomized controlled trial could be performed in this population.
The G2019S LRRK2 Mutation is Rare in Korean Patients with Parkinson's Disease
Authors: Cho Jin-Whan , Kim Sung-Yeon , Park Sung-Sup , Kim Han-Jun , Ahn Tae-Beom , Kim Jong-Min , Jeon Beom-Seok
Published: December 2014

Background:

A number of causative mutations such as a-synuclein, parkin, UCHL1, Pink-1, DJ-1 have been identified in Parkinson's disease (PD). They are usually found in the familial cases. One mutation of great interest is the G2019S mutation in the LRRK2 gene, which has been reported in both familial and sporadic PD. Its prevalence has been reported to vary markedly among different races. We examined the prevalence of the G2019S mutation in the Korean PD population for genetic study planning.

Methods:

We conducted a genetic analysis of the G2019S mutation by standard PCR and restriction digestion method. 453 PD patients were studied, 34% of whom had an age at onset of <50 years and 3.8% had a positive family history.

Results:

None of the 453 study subjects carried the G2019S mutation.

Conclusion:

Our result confirms previous reports that the G2019S mutation is rare among PD patients in the Asian population. This result supports the notion that the prevalence of this LRRK2 mutation is population specific, and that there may be a founder effect within western populations.
Axial Signs and Magnetic Resonance Imaging Correlates in Parkinson's Disease
Authors: Acharya Hernish J. , Bouchard Thomas P. , Emery Derek J. , Camicioli Richard M.
Published: December 2014

Background:

Age-related brain changes may contribute to axial features in Parkinson's disease (PD).

Objectives:

To determine if ventricular volume and white matter high signal changes (WMC) are related to motor signs in PD and controls independent of age.

Methods:

Patients were rated with the Unified Parkinson's Disease Rating Scale (subscore A: tremor, rigidity, bradykinesia, and facial expression; subscore B: speech and axial impairment). Steps and time taken to walk 9.144 meters were measured. Total ventricular volume (TVV) and intracranial volume (ICV) were measured on T1-weighted MRI using manual tracing software. WMC were rated on axial T2-weighted, dual-echo or FLAIR MR images using a visual scale.

Results:

TVV (cm3) (PD: 36.48 ± 15.93; controls: 32.16 ± 14.20, p = 0.21) and WMC did not differ between groups (PD: 3.7 ± 4.2; controls: 3.2 ± 3.1, p = 0.55). Age correlated positively with ICV-corrected TVV and WMC in PD (cTVV: r = 0.48, p = 0.003; WMC: r=0.42, p=0.01) and controls (cTVV: r = 0.31, p = 0.04; WMC: r=0.44, p=0.003). Subscore B (r = 0.42, p = 0.01) but not subscore A (r = 0.25, p = 0.14) correlated with cTVV in PD. Steps and walking time correlated with cTVV and WMC in PD; cadence correlated with cTVV and steps with WMC in controls. Age-adjustment eliminated correlations.

Conclusion:

Subscore B, but not subscore A correlated positively with ventricular volume in PD, though this association was accounted for by age. Age-related brain change super-imposed on PD may contribute to axial features.
Tumor Effects on Cerebral White Matter as Characterized by Diffusion Tensor Tractography
Authors: Wei Corie W. , Guo Gang , Mikulis David J.
Published: December 2014

Background:

Diffusion tensor MRI fiber tractography (DTT) is the first non-invasive in vivo technique for delineating specific white matter (WM) tracts. In cerebral neoplasm, DTT can be used to illustrate the relationship of the tumor with respect to adjacent WM trajectories.

Methods:

Fiber tractography was used in this study to assess tumor-induced changes in WM trajectories in three cases of cerebral neoplasm: glioblastoma multiforme, meningioma, and anaplastic astrocytoma.

Results:

Three patterns of WM alteration were identified: 1) disruption, 2) displacement, and 3) infiltration. Tumor disruption of WM tracts was observed in glioblastoma multiforme, which terminated fibers crossing the corpus callosum. In meningioma, DTT illustrated bulk displacement of the corticospinal tract in the affected hemisphere as well as preservation of the deviated axons. In anaplastic astrocytoma, fiber tracking demonstrated disruption of WM tracts at the tumor origin as well as intact axons through areas of tumor infiltration.

Conclusions:

Fiber tracking results correlated with the clinical and histopathological features of the tumor. Larger case series will be required to determine if fiber tracking can add accuracy to existing imaging methods for grading tumors.
Recurrent Stroke/TIA in Cryptogenic Stroke Patients with Patent Foramen Ovale
Authors: Casaubon Leanne , McLaughlin Peter , Webb Gary , Yeo Erik , Merker Darren , Jaigobin Cheryl
Published: December 2014

Background:

Patent foramen ovale (PFO) is present in 40% of patients with cryptogenic stroke and may be associated with paradoxical emboli to the brain. Therapeutic options include antiplatelet agents, anticoagulation, percutaneous device and surgical closure. We assessed the hypothesis that there are differences in rates of recurrent TIA or stroke between patients in the four treatment groups.

Methods:

Patients presenting from January 1997 with cryptogenic stroke or TIA and PFO were followed prospectively until June 2003. Treatment choice was made on an individual case basis. The primary outcome was recurrent stroke. The secondary outcome was a composite of stroke, TIA, and vascular death.

Results:

Baseline. Our cohort consisted of 121 patients; 64 (53%) were men. Median age was 43 years. Sixty-nine percent presented with stroke and 31% with TIA. One or more vascular risk factor was present in 40%. Atrial septal aneurysm (ASA) was present in 24%. Treatment consisted of antiplatelet agents (34%), anticoagulation (17%), device (39%) and surgical closure (11%). Follow-up. Recurrent events occurred in 16 patients (9 antiplatelet, 3 anticoagulation, 4 device closure); 7 were strokes, 9 were TIA. Comparing individual treatments there was a trend toward more strokes in the antiplatelet arm (p=0.072); a significant difference was seen for the composite endpoint (p=0.012). Comparing closure versus combined medical therapy groups, a significant difference was seen for primary (p=0.014) and secondary (p=0.008) outcomes, favoring closure. Age and pre-study event predicted outcome.

Conclusion:

Patent foramen ovale closure was associated with fewer recurrent events. Complications of surgical and device closure were self-limited.
Health Status and Health Care Utilization of Multiple Sclerosis in Canada
Authors: Pohar Sheri L. , Jones C. Allyson , Warren Sharon , Turpin Karen V.L. , Warren Kenneth
Published: December 2014

Background:

Persons with multiple sclerosis (MS) represent a small segment of the population, but given the progression of the disease, they experience substantial physical, psychosocial and economic burdens.

Objective:

The primary aim was to compare demographic characteristics, health status, health behaviours, health care resource utilization and access to health care of the community dwelling populations with and without MS.

Methods:

Cross-sectional survey using data from the Canadian Community Health Survey (CCHS 1.1). Adjusted analyses were performed to assess differences between persons with MS and the general population, after controlling for age and sex. Normalized sampling weights and bootstrap variance estimates were used.

Results:

Respondents with MS were 7.6 times (95% CI: 5.4, 10.7) more likely to have health-related quality of life scores that reflected severe impairment than respondents without MS. Respondents with MS were 12.2 times (95% CI: 8.6, 17.2) to rate their health as ‘poor’ or ‘fair’ than the general population. Urinary incontinence and chronic fatigue syndrome were 18.7 times (95% CI: 12.5, 28.2) and 21.9 times (95% CI: 11.9, 40.3), more likely to be reported by respondents with MS than those without. Differences between the two populations also existed in terms of health care resource utilization and access and health behaviours.

Conclusion:

Large discrepancies in health status and health care utilization existed between persons with MS who reside in the community and the general population according to all indicators used. Health care needs of persons with MS were also not met.
Motor Neuron Degeneration in a 20-Week Male Fetus: Spinal Muscular Atrophy Type 0
Authors: Sarnat Harvey B. , Trevenen Cynthia L.
Published: December 2014

Background:

Neuropathological changes in degenerating motor neurons are well documented in the term neonate with spinal muscular atrophy, but not at midgestation.

Methods:

Postmortem neuropathological examination was performed in a 20-week male fetus with a hypoplastic left cardiac anomaly.

Results:

Selective degeneration of spinal and hypoglossal motor neurons was an incidental finding. Degenerating motor neurons were not immunoreactive with neuronal nuclear antigen (NeuN) or neuron-specific enolase (NSE), as were the normal motor neurons. Synaptophysin reactivity was reduced around the soma of degenerating normal motor neurons. Ubiquitin and tau were expressed in degenerating motor neurons. Gliosis, inflammation and microglial activation were lacking in the ventral horns of the spinal cord. Laryngeal striated muscle was unaltered for age. No cerebral malformations or hypoxic-ischaemic changes were found.

Conclusion:

This case represents an early motor neuronal degeneration and corresponds to the recently described “type 0” spinal muscular atrophy. Lack of contractures is attributed to the early fetal age, since most muscular growth occurs in the second half of gestation.
Neurocardiogenic Syncope: Frequency and Consequences of its Misdiagnosis as Epilepsy
Authors: Josephson Colin B. , Rahey Susan , Sadler R. Mark
Published: December 2014

Background:

Neurocardiogenic syncope (NCS) can be mistaken as a seizure. We reviewed the frequency and diagnostic consequences of this misdiagnosis.

Methods:

A retrospective review of outpatient adult epilepsy clinic charts (QEII health Sciences Centre, Halifax, NS) was conducted to identify NCS patients referred with a provisional diagnosis of seizures. Charts were reviewed in detail with an emphasis on the consequences of misdiagnosis.

Results:

Of 1506 consecutive referrals to the epilepsy clinic, 194 (12.9%) ultimately had a clinical diagnosis of NCS. Mean age was 38 +/- 16 years (mean age of syncopal onset was 28 +/- 16 years). Two-thirds of referrals were from primary care physicians (including emergency departments) and 18% from neurologists. Thirty-five percent were prescribed antiepileptic drugs (AEDs) prior to referral with eight patients (4.1%) experiencing hypersensitivity reactions. Three of five women had adverse pregnancy outcomes while taking AEDs. One-third of patients had restrictions placed on their driving privileges while 11 patients (5.7%) had their employment interrupted. Diagnostic modalities used in the work-up included EEG (90%), CT head (51%), and MRI head (15%).

Conclusions:

NCS is commonly misdiagnosed as epilepsy. Some patients had an incorrect diagnosis for > 10 years. Patients with this misdiagnosis are often excessively investigated, inappropriately treated, and have unnecessary restrictions placed on driving and employment.
Treating Newly Diagnosed Epilepsy: The Canadian Choice
Authors: Burneo J. G. , McLachlan R. S.
Published: December 2014

Background:

Choosing an antiepileptic medication to treat a patient with epilepsy can be a complicated process during which the treating physician must base her or his decision on efficacy and safety of each of many available drugs. The lack of comparative studies between medications is one of the reasons.

Methods:

We conducted a survey on the management of newly diagnosed epilepsy in adult patients. The surveyed were adult and pediatric neurologists with a subspecialty interest in epilepsy who were working in academic institutions or private practice across Canada. Scenarios presented were grouped in categories according to the epilepsy syndrome (absence epilepsy, juvenile myoclonic epilepsy, undetermined idiopathic generalized epilepsy, symptomatic or cryptogenic partial epilepsy, and unclassified epilepsy), the patient's gender and age. First and second step in medical treatment for status epilepticus were surveyed as well.

Results:

Forty one of 64 experts responded the survey (responder rate of 66%). The results revealed a consensus among Canadian epileptologists that the first choice of antiepileptic medication in generalized epilepsies was between valproate in men (chosen by 88% of respondents) and lamotrigine in women. In localization-related epilepsies, carbamazepine was the preferred drug of choice (chosen by 90% of respondents). In the treatment of status epilepticus, an initial intravenous dose of lorazepam (95% of respondents), followed by a second dose of lorazepam or intravenous phenytoin in case the initial dose of lorazepam failed, were the treatments preferred.
Wallerian-Like Degeneration After Ischemic Stroke Revealed by Diffusion - Weighted Imaging
Authors: Sylaja P. N. , Goyal M. , Watson T. , Hill M. D.
Published: December 2014

A 22-year-old female was seen in the emergency within one hour of acute onset of right sided headache followed by weakness of the left side of body. On neurological examination, she was mildly drowsy, had forced right gaze deviation, dysarthria, left hemiplegia and left hemisensory loss. Computed tomography (CT) scan revealed early ischemic changes in the right middle cerebral artery (MCA) territory. The CT angiography done showed evidence of dissection of the supraclinoid segment of the right internal carotid artery with reduced flow distally into the MCA, which was confirmed by a conventional angiogram. In view of the intracranial carotid dissection, the patient was not treated with intravenous tissue plasminogen activator. Magnetic resonance imaging (MRI) of the brain done on the next day revealed evidence of acute ischemic lesions in the right MCA and anterior cerebral artery territory on diffusion-weighted imaging (DWI), with normal brainstem. [Figure 1] A repeat MRI performed 13 days after ictus showed hyperintense signal on DWI in the right cerebral peduncle which was hypointense on apparent diffusion coefficient (ADC) map suggestive of Wallerian-like degeneration. [Figure 2] The signal changes were less conspicuous on T2-weighted images. She had antigravity strength in the left leg but remained weak in her left arm at one month.
Neuromonitoring During Surgery for Paediatric Spinal Deformity in Canada (2007)
Authors: Norton Jonathan , Hedden Douglas
Published: December 2014

Background:

Neuromonitoring during paediatric (and adult) spinal deformity surgery helps to reduce the risk of both permanent and short term neurological damage. A shortage of neurophysiologists and technicians limits the availability of this service. Not all surgeons believe neuromonitoring offers neuroprotection during spinal surgery. This study aimed to document the degree to which paediatric patients undergoing spinal deformity correction surgery have their spinal cord function monitored.

Methods:

A questionnaire was sent electronically to all of the surgical members of the Canadian Paediatric Spinal Deformity Study Group.

Results:

Results were received from 9/9 centres indicating that monitoring was performed in 7/9 centres, with one further centre awaiting staffing. Whilst half of those centres that do monitor only monitor sensory and motor evoked potentials, the remaining centres also use EMG and EEG to assess the state of the patient intraoperatively.

Conclusions:

Despite a shortage of staff, most paediatric spinal deformity surgeons in Canada who wish to, are able to neurophysiologically monitor their surgical cases. Neuromonitoring appears to be becoming a standard of care, at least for paediatric spinal deformity surgery. There is an urgent need for the establishment of national standards for both technologists and interpreters, as well as training programmes for both these groups.
Trigeminal Neuralgia: Outcomes after Gamma Knife Radiosurgery
Authors: Knafo Henri , Kenny Brendan , Mathieu David
Published: December 2014

Background:

Trigeminal neuralgia (TN) often remains difficult to treat despite multiple available medications, and can severely impact on the quality of life of affected patients. Gamma knife radiosurgery has recently emerged as a minimally-invasive alternative to surgery for patients suffering from drug-resistant TN. The goal of this study was to report the short-term efficacy of gamma knife radiosurgery for TN and assess its impact on the quality of life of patients treated in the first 18 months of our experience.

Methods:

Patients with medically-refractory TN or with unacceptable drug side effects were considered for radiosurgery. A maximum dose of 80 Gy was administered to the affected nerve using a single 4-mm isocenter. Follow-up assessments were made at 2, 4 and 6 months, with evaluation of pain relief, drug reduction and quality of life. Factors impacting treatment response were assessed using Cox regression analysis.

Results:

A total of 67 patients were treated. Significant pain relief was seen in 77.6% of patients, including 32.6% who became pain-free. Patients were able to discontinue all medications in 34.3% or reduce drug intake by more than 50% in an additional 28.4% of cases. No variable was found to predict pain relief although older age (>66 years) approached statistical significance. Sensory side effects were seen in 14.9% of patients. Quality of life improved in the majority of patients after radiosurgery.

Conclusions:

Gamma knife radiosurgery is a safe and effective management alternative for trigeminal neuralgia, providing good or excellent pain relief and improvement in quality of life in the majority of patients with few side effects.
Central Nervous System Imaging in Mitochondrial Disorders
Authors: Finsterer Josef
Published: December 2014

Imaging of central-nervous-system (CNS) abnormalities is important in patients with mitochondrial disorders (MCDs) since the CNS is the organ second most frequently affected in MCDs and some of them are potentially treatable. Clinically relevant imaging techniques for visualization of CNS abnormalities in MCDs are computed tomography, magnetic resonance imaging, and MR-spectroscopy. The CNS abnormalities in MCDs visualized by imaging techniques include stroke-like lesions with cytotoxic or vasogenic edema, laminar cortical necrosis, basal ganglia necrosis, focal or diffuse white matter lesions, focal or diffuse atrophy, intra-cerebral calcifications, cysts, lacunas, hypometabolisation, lactacidosis, hemorrhages, cerebral hypo- or hyperperfusion, intra-cerebral artery stenoses, or moyamoya syndrome. The CNS lesions may proceed with or without clinical manifestations, why neuroimaging should be routinely carried out in all MCDs to assess the degree of CNS involvement. Some of these lesions may remain unchanged for years, some may show contiguous spread and progression, but some may even disappear, spontaneously or in response to medication. Dynamics of Stroke-like lesions may be positively influenced by L-arginine, dichloracetate, steroids, edavarone, or antiepileptics. Symptomatic treatment of CNS abnormalities in MCD patients may positively influence their outcome.
Precentral Knob Corresponds to the Primary Motor and Premotor Area
Authors: Shinoura N. , Suzuki Y. , Yamada R. , Tabei Y. , Saito K. , Yagi K.
Published: December 2014

Purpose:

Cortical mapping during awake surgery assesses intraoperative neurological change in response to electrical stimulation to provide direct information regarding the anatomical localization of the primary motor area (M1). The goal of the present study was to analyze the reliability of the identification of the precentral knob in the axial image of magnetic resonance imaging or functional MRI (fMRI) for the detection of M1.

Materials and Methods:

Among patients with brain tumors within or near M1 in whom awake surgery was employed from April 2004 through March 2007, 14 cases were analyzed in which either the M1 or premotor area (PMA) was successfully detected by mapping during awake surgery.

Results:

The precentral knob was localized to the PMA in 4 cases and to M1 in 10 cases. By contrast, the gyrus activated by hand clenching in fMRI on the affected side at least partially corresponded to M1 in all cases, while those on the unaffected side corresponded to M1 in 12 of 12 cases.

Conclusion:

These results indicate that the precentral knob corresponds to PMA as well as to M1, whereas the gyrus activated in fMRI corresponds to M1 on the affected and unaffected side.
Standardization and Detailed Characterization of the Syngeneic Fischer/F98 Glioma Model
Authors: Mathieu David , Lecomte Roger , Tsanaclis Ana Maria , Larouche Annie , Fortin David
Published: December 2014

Introduction:

Adequate animal glioma models are mandatory for the pursuit of preclinical research in neuro-oncology. Many implantation models have been described, but none perfectly emulate human malignant gliomas. This work reports our experience in standardizing, optimizing and characterizing the Fischer/F98 glioma model on the clinical, pathological, radiological and metabolic aspects.

Materials and methods:

F98 cells were implanted in 70 Fischer rats, varying the quantity of cells and volume of implantation solution, and using a micro-infusion pump to minimize implantation trauma, after adequate coordinates were established. Pathological analysis consisted in hematoxylin and eosin (H&E) staining and immunohistochemistry for GFAP, vimentin, albumin, TGF-b1, TGF-b2, CD3 and CD45. Twelve animals were used for MR imaging at 5, 10, 15 and 20 days. Corresponding MR images were compared with pathological slides. Two animals underwent 18F-FDG and 11C-acetate PET studies for metabolic characterization of the tumors.

Results:

Implantation with 1x104 cells produced a median survival of 26 days and a tumor take of 100%. Large infiltrative neoplasms with a necrotic core were seen on H&E. Numerous mitosis, peritumoral infiltrative behavior, and neovascular proliferation were also obvious. GFAP and vimentin staining was positive inside the tumor cells. Albumin staining was observed in the extracellular space around the tumors. CD3 staining was negligible. The MR images correlated the pathologic findings. 18F-FDG uptake was strong in the tumors.

Conclusion:

The standardized model described in this study behaves in a predictable and reproducible fashion, and could be considered for future pre-clinical studies. It adequately mimics the behavior of human malignant astrocytomas.