bluuebunny/crossref_metadata_2025 huggingface dataset:
Management of Optic Neuritis in Canada: Survey of Ophthalmologists and Neurologists
Authors: Atkins Edward J. , Drews-Botsch Carolyn D. , Newman Nancy J. , Calvetti Olivier , Swanson Seegar , Biousse Valérie
Published: December 2014

ABSTRACT

Background:

Acute isolated optic neuritis is often the first manifestation of multiple sclerosis (MS), and its management remains controversial. Over the past decade, with the advent of new disease-modifying agents, management of isolated optic neuritis has become more complicated.

Objectives:

To evaluate the current practice patterns of Canadian ophthalmologists and neurologists in the management of acute optic neuritis, and to evaluate the impact of recently published randomized clinical trials.

Design:

Mail survey.

Methods:

All practicing ophthalmologists and neurologists in Canada were mailed a survey evaluating the management of isolated acute optic neuritis and familiarity with recent clinical trials. Surveys for 1158 were mailed, and completed surveys were collected anonymously through a datafax system. Second and third mailings were sent to non-respondents 6 and 12 weeks later.

Results:

The final response rate was 34.5%. Although many acute optic neuritis patients initially present to ophthalmologists, neurologists are the physicians primarily managing these patients. Ordering magnetic resonance imaging, and treating with high dose intravenous steroids has become the standard of care. However, 15% of physicians (14% of ophthalmologists and 16% of neurologists) continue to prescribe low dose oral steroids, and steroids are being given for reasons other than to shorten the duration of visual symptoms by 73% of ophthalmologists and 50% of neurologists. More neurologists than ophthalmologists are familiar with recent clinical trials involving disease-modifying agents.

Conclusion:

Although the management of acute optic neuritis has been evaluated in large clinical trials that were published in major international journals, some ophthalmologists and neurologists are not following evidence-based recommendations.
Pilot Study of Minocycline in Relapsing-Remitting Multiple Sclerosis
Authors: Zhang Yunyan , Metz Luanne M , Yong V Wee , Bell Robert B , Yeung Michael , Patry David G , Mitchell J Ross
Published: December 2014

Background:

Current multiple sclerosis (MS) treatment is only partially effective and not all patients respond well. The goal in this study was to evaluate minocycline for its safety, tolerability, and MRI impact as a potential therapy over 36 months after a three month run-in in ten relapsing-remitting (RR) MS patients.

Methods:

Clinical assessments were at three month intervals until six months, then at six month intervals. Three Tesla MRI was performed monthly during the run-in and first six months of treatment, then at 12, 24, and 36 months.

Results:

Treatment was safe and well tolerated. Annualized relapse rate was 1.2 during the run-in and 0.25 during treatment. The proportion of active scans was lower during the first six months of treatment (5.6%, p<0.001) and during the extension (8.7%, p= 0.002) than during the run-in (47.5%). Consistent with these outcomes, mean T2 lesion volume remained stable over three years and percent brain volume change was reduced during year three (-0.37%) of minocycline treatment.

Conclusions:

This trial is limited by small sample and no control group but suggests that minocycline is safe and potentially beneficial in RRMS. This supports further investigation of its efficacy.
Ophthalmoscopy: A 7-Step Program
Authors: Dooley Joseph M. , Gordon Kevin E.
Published: December 2014

Background:

Fundoscopy is viewed as a difficult or impossible task by many students and physicians. We have used a novel seven-step approach to teach trainees to use the ophthalmoscope. The technique is based on the premise that success is most easily achieved if the necessary motor skills are mastered first. A step by step approach will enable others to teach their trainees to attain the ability to routinely view the fundi of their pediatric patients.

Methods:

Step 1 involves examination of the trainee’s fundi to ensure there is no impediment to their success. In Step 2 the student examines the teacher. This identifies major errors. The next step teaches the trainee how to hold the ophthalmoscope. Step 4 gets the learner to read a journal article through the ophthalmoscope. In Step 5 they examine the teacher’s eyes again and with a little help they are always successful. In the last two steps an older patient is first examined and finally the student examines a young child.

Conclusion:

This method differs from most other approaches by leaving the cognitive component of ophthalmoscopy until the student is comfortable with handling the instrument. It has been uniformly successful among our students and residents.
Stroke Prevention and Sodium Restriction
Authors: Campbell Norman RC , Spence J David
Published: December 2014

Stroke prevention is extremely cost-effective. The Ontario Task Force for the Coordinated Stroke Strategy for Ontario received from Dr. Muhammad Mamdani of the Institute for Clinical Evaluative Sciences the estimate that stroke prevention is approximately ten times more cost-effective than treating acute stroke with tPA.1 Hackam and Spence2 have calculated that in the highest-risk patients it would be possible to reduce the risk of stroke by more than 90% by a combination of interventions (including blood pressure control, smoking cessation, diet, treatment of diabetes, lipid-lowering therapy, and for patients in whom it is indicated, anticoagulation for atrial fibrillation or carotid endarterectomy). Dr Mukul Sharma (personal communication) has shown that stroke prevention is a dominant strategy in health economic terms; i.e. that it improves outcomes while reducing net costs. Effective control of hypertension has the potential to reduce stroke by half.3,4 Because hypertension is so prevalent, it accounts for the greatest population attributable risk for stroke, and thus represents the greatest opportunity to reduce stroke.
Quality of Life and Memory after Vagus Nerve Stimulator Implantation for Epilepsy
Authors: McGlone Jeannette , Valdivia Ivan , Penner Marsha , Williams Jaime , Sadler R. Mark , Clarke David B.
Published: December 2014

Objective:

This prospective, case control study evaluates quality of life (QOL), depressive affect, and memory outcomes of epilepsy patients implanted with a vagus nerve stimulator (VNS).

Methods:

Three groups of patients with epilepsy underwent assessment on two occasions: 1) patients with a VNS were tested before and 12 months after implantation (n = 16); 2) patients who underwent cerebral resective surgery were tested pre- and post-operatively (n = 10); and 3) patients under medical management (n = 9). Group means were compared on the QOLIE-89, Geriatric Depression Scale, Wechsler Memory Scale - III, and the Memory Observation Questionnaire. Secondary analyses calculated the reliable change index, providing information on change beyond measurement error and chance.

Results:

Mean ratings of QOL, depression, and memory complaints and objective memory scores remained stable or improved in all the groups. The QOL improved more after cerebral resective surgery than VNS or medication controls, but the VNS and medication control groups did not differ. In the VNS group, QOL was not related to seizure reduction. The percentage of cases showing real change in memory was equivalent across groups, except in one of eight indices (i.e., verbal recognition memory).

Conclusions:

This first case controlled design found that vagus nerve stimulation as an adjunctive therapy for seizure control did not change QOL, depressive affect, or objective memory scores over one-year more so than medical management alone. We point out the need for larger case control, non-industry funded investigations.
L’absence épileptique de l’enfance dont le controle nécessite plus d’un medicament.
Authors: Nadler B. , Shevell M. I.
Published: December 2014

Introduction:

Absence epilepsy is the most common primary generalized epilepsy syndrome encountered in pediatric practice. Treatment is pharmacologically specific and usually successful with a single medication. The objective of this study was to identify any clinical or electroencephalographic features at initial presentation in a consecutive cohort of children with absence epilepsy that may be associated with the need for a second medication.

Methods:

A computerized pediatric neurology database (1991-2007 inclusive) was retrospectively searched for all patients with typical absence seizures, 3 Hz spike and wave on EEG and no apparent symptomatic etiology who were over the age of two years at seizure onset with at least one year of follow-up. All such children were then divided into two groups; a) those requiring a single medication for seizure control (Group 1), and b) those requiring two medications for seizure control despite optimal management with the initial medication as determined by serum drug monitoring (Group 2). Clinical and electrographic features evident at diagnosis were then contrasted between Group 1 and 2.

Results:

Seventy-five children with absence seizures were initially identified with 52 meeting the study’s inclusion and none of the exclusion criteria. Of these 52 children, 43 required a single medication for seizure control (Group 1), while 9 required two or more medications for seizure control (Group 2). A significant difference (p<0.05) was apparent between Group 1 and 2 with respect to gender (16/43 males vs 8/9 males) and mean age of diagnosis (8.19 years +/− 3.00 vs 6.06 years +/− 2.22). Age of onset of seizures, interval duration of seizures prior to treatment initiation, duration of seizures, presence of automatisms, family history, presence of co-morbid conditions and EEG findings were not found to be significantly different between the two Groups.

Conclusions:

Male gender and an earlier age of diagnosis is associated with the need for two medications for seizure control in children with absence epilepsy. This observation may suggest the need for more intensive early programmatic follow-up for young male children with newly diagnosed absence epilepsy to effect more rapid attainment of seizure control.
Can Clinical Data Predict Progression to Dementia in Amnestic Mild Cognitive Impairment?
Authors: Fellows Lesley , Bergman Howard , Wolfson Christina , Chertkow Howard
Published: December 2014

Background:

To determine whether clinical data obtained by history and physical examination can predict eventual progression to dementia in a cohort of elderly people with mild cognitive impairment.

Methods:

A prospective, longitudinal study of a cohort of elderly subjects with amnestic Mild Cognitive Impairment (MCI). Ninety subjects meeting the criteria for amnestic MCI were recruited and followed annually for an average of 3.3 years. Main outcome measure was the development of dementia determined by clinical assessment with confirmatory neuropsychological evaluation.

Results:

Fifty patients (56%) developed dementia on follow-up. They were older, had lower Mini-mental status exam (MMSE) scores and a shorter duration of symptoms at the time of first assessment. Multivariate logistic regression analysis identified age at symptom onset as the only clinical parameter which distinguished the group that deteriorated to dementia from the group that did not. The odds ratio for age was 1.1 (confidence interval 1.04 - 1.18).

Conclusions:

Patients presenting with amnestic MCI insufficient for the diagnosis of dementia are at high risk of developing dementia on follow-up. In our cohort, 56% were diagnosed with dementia over an average period of 5.9 years from symptom onset. The only clinical predictor for the eventual development of dementia was older age at symptom onset. Clinical features alone were insufficient to predict development of dementia.
RAVLT and Nonverbal Analog: French Forms and Clinical Findings
Authors: Sziklas Viviane , Jones-Gotman Marilyn
Published: December 2014

Background:

Objective clinical evaluation of memory frequently requires serial testing but the issue of whether multiformed tests are equivalent and can be used interchangeably is seldom examined. An added problem in bilingual Canadian settings is the extent to which it is appropriate to measure French speakers’ performance on translations of English tests. The present work used the Rey Auditory Verbal Learning Test (RAVLT) and a nonverbal analog, the Aggie Figures Learning Test (AFLT), to examine whether a) different forms of the same test are equivalent, b) performance on the two tests is comparable, c) two language groups perform similarly, and d) the RAVLT can detect dysfunction in patients with temporal lobe epilepsy (TLE).

Methods:

We compared three French versions of the RAVLT and three forms of the AFLT in 114 healthy francophone adults. We subsequently compared the performance of the same francophone subjects to a previously obtained sample of anglophones on both tests, and then administered the RAVLT to anglophone or francophone patients with TLE.

Results:

For both tasks the three forms were equivalent and performance on the RAVLT was comparable to that on the AFLT. Francophone subjects performed slightly worse on the RAVLT compared to anglophones but performance of the two language groups did not differ on the AFLT. Finally, left TLE patients were impaired compared to right on the RAVLT, but no performance differences were observed across the two language groups in the patient sample.

Conclusions:

The RAVLT and AFLT are useful tools for examination of learning and memory in French and English speaking populations. On the RAVLT, the lesion effect in patients is not affected by differences in performance between language groups.
Diagnostic Accuracy of Neurological Problems in the Emergency Department
Authors: Moeller Jeremy J , Kurniawan Joelius , Gubitz Gordon J , Ross John A , Bhan Virender
Published: December 2014

Background:

Previous studies describe significant rates of misdiagnosis of stroke, seizure and other neurological problems, but there are few studies examining diagnostic accuracy of all emergency referrals to a neurology service. This information could be useful in focusing the neurological education of physicians who assess and refer patients with neurological complaints in emergency departments.

Methods:

All neurological consultations in the emergency department at a tertiary-care teaching hospital were recorded for six months. The initial diagnosis of the requesting physician was recorded for each patient. This was compared to the initial diagnosis of the consulting neurologist and to the final diagnosis, as determined by retrospective chart review.

Results:

Over a six-month period, 493 neurological consultations were requested. The initial diagnosis of the requesting physician agreed with the final diagnosis in 60.4% (298/493) of cases, and disagreed or was uncertain in 35.7% of cases (19.1% and 16.6% respectively). In 3.9% of cases, the initial diagnosis of both the referring physician and the neurologist disagreed with the final diagnosis. Common misdiagnoses included neurocardiogenic syncope, peripheral vertigo, primary headache and psychogenic syndromes. Often, these were initially diagnosed as stroke or seizure.

Conclusions:

Our data indicate that misdiagnosis or diagnostic uncertainty occurred in over one-third of all neurological consultations in the emergency department setting. Benign neurological conditions, such as migraine, syncope and peripheral vertigo are frequently mislabeled as seizure or stroke. Educational strategies that emphasize emergent evaluation of these common conditions could improve diagnostic accuracy, and may result in better patient care.
A Case of Foreign Accent Syndrome Resulting in Regional Dialect
Authors: Naidoo Raveeni , Warriner Erin M. , Oczkowski Wieslaw J. , Sévigny Alexandre , Humphreys Karin R.
Published: December 2014

Background:

Foreign Accent Syndrome (FAS) is a rare acquired syndrome following neurological damage that results in articulatory distortions that are commonly perceived as a “foreign” accent. The nature of the underlying deficit of FAS remains controversial. We present the first reported Canadian case study of FAS following a stroke. We describe a stroke patient, RD, who suffered an acute infarction to the left internal capsule, basal ganglia and frontal corona radiata. She was diagnosed as having FAS without any persistent aphasic symptoms. Family, friends, and health care professionals similarly described her speech as sounding like she had a Canadian East Coast accent, a reported change from her native Southern Ontario accent.

Method:

An investigation of this case was pursued, incorporating neuroimaging, neuropsychological and speech pathology assessments, and formalized linguistic analyses.

Results:

Linguistic analyses confirmed that RD’s speech does in fact have salient aspects of Atlantic Canadian English in terms of both prosodic and segmental characteristics. However, her speech is not entirely consistent with an Atlantic Canadian English accent.

Interpretation:

The fact that RD’s speech is perceived as a regional variant of her native language, rather than the “generic foreign accent” of FAS described elsewhere, suggests that the perceived “foreignness” in FAS is not primarily due to dysfluencies which indicate a non-native speaker, but rather due to very subtle motor-planning deficits which give rise to systemic changes in specific phonological segments. This has implications for the role of the basal ganglia in speech production.
Acute Electrical Stimulation of Nucleus Ambiguus Enhances Immune Function in Rats
Authors: Mei Ying-Wu , Yang Zhan-Qing , Wang Wei , Song De-Guang , Deng Xu-Ming , Liu Ju-Xiong
Published: December 2014

Background:

Up to now, many “immunoactive” brain areas have been identified, such a hypothalamic nuclei, brain reward system; but the nucleus ambiguous (Amb), a nucleus nervi vagis of medulla oblongata, was less well studied in neuroimmunomodulation.

Methods:

In order to obtain more profound comprehension and more knowledge on Amb, we studied the effect of acute electrical stimulation of Amb on thymus and spleen activity in rat. A stimulator was applied to stimulate the Amb of the anaesthetic rats using the parameter at 100μAx5ms x100 Hz every 1s for 1 min. The levels of TGF-β and thymosin-β4 mRNA in thymus, the release of IL-2 and IL-6 at splenocyte in vitro and splenic lymphocyte proliferation were measured at hour 0.5,1,2,3 following the electrical stimulation.

Results:

The results showed that concanavalin A (Con A)-induced splenic lymphocyte proliferation and the release of IL-2 and IL-6 were all significantly enhanced at 0.5, 1, and 2 h following effective Amb stimulation as compared to in the control group. However, as compared to in the control group, the levels of TGF-β and thymosin-β4 mRNA in the thymus were both remarkably reduced at 0.5, 1, and 2 h following effective Amb stimulation.

Conclusions:

These findings reveal that the Amb participates in the modulation of animal immune functions.
Tasks of Attention Augment Rigidity in Mild Parkinson Disease
Authors: Mendon¸a Dan A. , Jog Mandar S.
Published: December 2014

Objective:

The purpose of this study was to determine whether tasks involving effortful attention would cause augmentation of rigidity in patients with mild Parkinson disease.

Methods:

In 17 subjects with mild Parkinson disease, rigidity in a single arm was assessed during various experimental conditions by a blinded movement disorders neurologist. Rigidity was scored separately at the wrist and the elbow using an ordinal scale. In three of the conditions, sustained attention was directed toward visual, auditory or movement-related stimuli. Two varieties of Froment maneuver served as positive controls: contralateral hand opening-closing or ipsilateral foot tapping. In addition, rigidity was assessed twice with subjects resting. The examiner was unaware of the sequence of experimental conditions and this was changed for each subject. Mean rigidity scores for the various experimental conditions were compared against the baseline state (an average of both trials with the patient resting) using a repeated measures ANOVA and post-hoc Tukey-Kramer multiple comparisons test.

Results:

Rigidity was significantly increased from baseline with each of the attentional tasks (p <0.01 to p <0.001) and also with the two Froment maneuvers (p <0.001). Rigidity augmentation with contralateral hand opening-closing was significantly greater than with any of the attentional tasks (p <0.05 to p <0.001).

Conclusion:

Tasks of effortful attention did appear to augment rigidity in patients with mild Parkinson disease. We speculate that the greater augmentation seen with the Froment maneuver could have an anatomic basis.
Recombinant Factor VIIa plus Surgery for Intracerebral Hemorrhage
Authors: Sutherland Christina S. , Hill Michael D. , Kaufmann Anthony M. , Silvaggio Joseph A. , Demchuk Andrew M. , Sutherland Garnette R.
Published: December 2014

Background:

Hyperacute surgical evacuation of intracerebral hemorrhage is associated with a high rebleeding rate. The peri-operative administration of rFVIIa to patients with intracerebral hemorrhage may decrease the frequency of post-operative hemorrhage, and improve outcome.

Methods:

Patients receiving recombinant activated factor VII (rFVIIA) therapy immediately prior to acute surgery were collected at two centres. The intracerebral hemorrhage (ICH) score and ICH Grading Scale were determined, as was long-term outcome using the modified Rankin Scale. Residual/ recurrent clot was evaluated by comparing pre-operative to post-operative CT scans.

Results:

Fifteen patients with intracerebral hemorrhage received 40-90 μg/kg of rFVIIa and underwent surgical hematoma evacuation at a median time of five hours following symptom onset. Median pre-operative clot volume was 60 ml, decreasing to 2 ml post-operatively. There were no thromboembolic adverse events. Thirteen patients survived, 11 (73%) were independent, and two (13%) had a moderate to severe disability. These outcomes were significantly better than expected based on the median ICH score (40% mortality) and based on median ICH Grading Scale (18% good outcome).

Conclusions:

The pre or peri-operative administration of rFVIIa resulted in minimal residual or recurrent hematoma volume and may be an important adjunct to surgery in patients with intracerebral hemorrhage.
Inflammatory Cerebrospinal Fluid in Sporadic Creutzfeldt-Jakob Disease
Authors: Bui Esther , Ehrensperger Eric , Sahlas Demetrios J. , Murray Brian J. , Bergeron Catherine , Glikstein Rafael S. , Aviv Richard , Schipper Hyman M.
Published: December 2014

Background:

Sporadic Creutzfeldt-Jakob disease (CJD) is a fatal, transmissible spongiform encephalopathy characterized by rapidly progressive dementia, myoclonus, ataxia and akinetic mutism. The underlying mechanism is believed to be a conformational change of a native prion protein which characteristically fails to provoke an immune response. Commensurate with the latter, cerebrospinal fluid (CSF) classically exhibits a non-inflammatory profile.

Cases:

We report two patients with pathologically-proven sporadic CJD presenting with a significant CSF pleocytosis.

Conclusion:

Although uncommon, the presence of an inflammatory CSF profile should not exclude the diagnosis of sporadic CJD.
Comparison of Post-Operative Lordosis with the PEEK Cage and the Cervical Plate
Authors: Wilkinson Jeffrey S. , Mann Sumeer A. , Stoneham Grant W. , Hentschel Stephen , Fourney Daryl R.
Published: December 2014

Objective:

The maintenance of post-operative lordosis has been shown to be a key factor in decreasing adjacent level disc stress. Previous studies of the PEEK (polyetherether ketone) cage have used intervertebral bony fusion as the primary measure of surgical success; however, little is known about its effects on spinal curvature. Our objective was to compare the PEEK cage to the cervical plate with respect to the maintenance of cervical lordosis at one year. Secondary outcomes included fusion and complication rates.

Methods:

We performed a retrospective study of patients who underwent ACDF (anterior cervical discectomy and fusion) by two different methods; 13 patients were treated with the PEEK cage, and 22 with allograft and plating.

Results:

Patient and treatment characteristics were similar in both groups. Average global lordotic curvature (C2-C7) was increased by 1.7 degrees for the PEEK cage and decreased by 1.6 degrees for the plate after an average follow-up of 12.46 and 14.95 months, respectively. Regional lordosis for the PEEK cage and plate was decreased by 2.5 and 2.1 degrees, respectively for the same time period. These differences did not achieve statistical significance. Bony fusion was observed in all patients. One patient in each group developed persistent mild dysphagia.

Conclusions:

The PEEK cage is comparable to the anterior cervical plate in the maintenance of post-operative cervical lordosis.
Preservation of Language in the Ataxic Infant in a Case of Cerebellar Agenesis
Authors: Jacob Francois Dominique , Goez Helly R.
Published: December 2014

An eight-month-old male Caucasian infant presented with ataxia and titubations, which had been noticed shortly after birth. He was born to healthy non-consanguineous parents after an unremarkable pregnancy. There was no maternal use of drugs, alcohol or tobacco. A prenatal ultrasound at 36 weeks conceptual age (performed at the request of the parents since the 20 week ultrasound had not been performed) revealed an enlarged posterior fossa, no cerebellar tissue, a normal corpus callosum and no evidence of hydrocephalus. He was delivered via spontaneous vaginal delivery at 37 weeks gestation without complications. Birth weight was 3630g and the neonatal course was unremarkable. Developmentally, at eight months-of-age he could not sit. A formal developmental assessment at 19 months-of-age revealed gross and fine motor delay. He was pulling to stand and was cruising but was not walking independently. He was able to stack two cubes. He was very social, highly imitative and used over 50 words. His words were clear and there was no dysarthria. His receptive and expressive language skills were deemed to be at the 18-21 month level. Family history was non-contributory. Physical examination at 19 months-of-age revealed weight and height between the 10-25 percentiles and head circumference at the 25th percentile. Head titubation was present throughout the examination. Extraocular movements were full, with bilateral intermittent esotropia. Upon fixing of the head, horizontal nystagmus and saccadic intrusions on smooth pursuit exam were noted. Fundoscopic examination was normal. He had mild truncal hypotonia, with appropriate strength for his age.
Spinal Cord Infarction from an Unstable Aortic Plaque
Authors: Desai Jamsheed A. , Gambarotta Nicola
Published: December 2014

A 78 year-old woman presented with acute onset low back pain with radiation into the right T12-L2 dermatomes, progressive flaccid paraparesis, urinary incontinence and bilateral lower extremity paresthesias. Examination revealed flaccid paraparesis, and a T12 sensory level to pin with intact vibration and proprioception. Magnetic resonance imaging (MRI) (Figure 1) of the spinal cord demonstrated T2 hyperintensity and restricted diffusion from T12 to the conus medullaris. Computed tomogram (CT) aortogram (Figure 2) highlighted an ulcerated plaque and thrombus at the approximate level of the artery of Adamkiewicz. These findings were consistent with a spinal cord infarct of the cord from T12-L2. The patient was treated with Clopidogrel and IV heparin (x two days) and was transferred for spinal cord rehabilitation.
A History of Neurosurgery in Canada
Authors: Weir Bryce
Published: December 2014

Canada existed for more than half a century before there were glimmerings of modern neurosurgical activity. Neurosurgery had advanced significantly in Europe and the United States prior to its being brought to Toronto and Montreal from American centers. The pioneers responsible for the rapid evolution in practice, teaching and research are described. The interplay of scientific, professional, demographic and economic forces with general historical trends has produced dramatic changes in the way that neurosurgery is now practiced.
Stereotactic Radiotherapy: An Emerging Treatment for Spinal Metastases
Authors: Dahele Max , Fehlings Michael G. , Sahgal Arjun
Published: December 2014

Aim:

The purpose of this concise update is to describe the emerging treatment of stereotactic body radiotherapy (SBRT) for spinal metastases.

Rationale:

Spinal metastases are common and can present complex clinical challenges that conventional treatment cannot always meet satisfactorily. Examples include a history of prior irradiation at the same site or radio-resistant tumor histology. Stereotactic body radiotherapy makes it possible to deliver high doses of radiation with the aim of improving tumor control and palliation. It is increasingly being offered to selected patients including those requiring re-irradiation and post-operative treatment.

Conclusion:

It is important that specialists managing patients with spinal metastases are aware of the potential advantages of SBRT and how this can complement and extend existing treatment approaches, including spinal decompression and stabilization.
Patients' Perceptions of Carpal Tunnel and Ulnar Nerve Decompression Surgery
Authors: Khu Kathleen Joy , Bernstein Mark , Midha Rajiv
Published: December 2014

Background:

Carpal tunnel syndrome and ulnar nerve entrapment at the elbow are the most common entrapment neuropathies seen in adults. Surgery for nerve decompression is a safe and effective treatment option, and is usually performed under local anesthesia and as an outpatient procedure. This study aimed to explore patients' satisfaction and other aspects of the overall experience with this type of surgery.

Methods:

Qualitative research methodology was used. Semi-structured, open-ended interviews were conducted with 30 adult patients who had undergone carpal tunnel release or ulnar nerve decompression at the elbow 6-24 months prior. Interviews were digitally audio recorded and transcribed, and the data subjected to thematic analysis.

Results:

Four overarching themes emerged from the data: (1) most patients did not perceive their condition to be serious; (2) patients were satisfied with the overall surgical experience; (3) the outcome was more important to patients than the process; and (4) majority of patients had a realistic expectation of outcomes.

Conclusions:

Patients had a positive experience with carpal tunnel and ulnar nerve decompression surgery, although their level of satisfaction was dependent on the surgical outcome. Areas requiring improvement, specifically information about post-operative care and expectations of recovery, will be implemented in the future care of patients.
Contemporary Treatment Strategy for Spinal Metastasis: The “LMNOP” System
Authors: Paton Gillian R. , Frangou Evan , Fourney Daryl R.
Published: December 2014

The choice of treatment for spinal metastasis is complex because (1) it depends on several inter-related clinical and radiologic factors, and (2) a wide range of management options has evolved in recent years. While radiation therapy and surgery remain the cornerstones of treatment, radiosurgery and percutaneous vertebral augmentation have also established a role. Classification systems have been developed to aid in the decision-making process, and each has different strengths and weaknesses. The comprehensive scoring systems developed to date provide an estimate of life expectancy, but do not provide much advice on the choice of treatment. We propose a new decision model that describes the key factors in formulating the management plan, while recognizing that the care of each patient remains highly individualized. The system also incorporates the latest changes in technology. The LMNOP system evaluates the number of spinal Levels involved and the Location of disease in the spine (L), Mechanical instability (M), Neurology (N), Oncology (O), Patient fitness, Prognosis and response to Prior therapy (P).
Quality of Life Following Hemicraniectomy for Malignant MCA Territory Infarction
Authors: Weil Alexander G. , Rahme Ralph , Moumdjian Robert , Bouthillier Alain , Bojanowski Michel W.
Published: December 2014

Objective:

Decompressive hemicraniectomy (DH) has been shown to reduce mortality in patients with malignant middle cerebral artery (MCA) territory infarction. However, many patients survive with moderate-to-severe disability and controversy exists as to whether this should be considered good outcome. To answer this question, we assessed the quality of life (QoL) of patients after DH for malignant MCA territory infarction in our milieu.

Methods:

The outcome of all patients undergoing DH for malignant MCAterritory infarction between 2001 and 2009 was assessed using retrospective chart analysis and telephone follow-up in survivors. Functional outcome was determined using Glasgow outcome scale, modifed Rankin scale (mRS), and Barthel index (BI). The stroke impact scale was used to assess QoL.

Results:

There were 14 patients, 6 men and 8 women, with a mean age of 44 years (range 27-57). All patients had reduced level of consciousness preoperatively. Five had dominant-hemisphere stroke. Median time to surgery was 45 hours (range 1- 96). Two patients died and one was lost to follow-up. Of 11 survivors, 7 (63.6%) had a favorable functional outcome (mRS<4). No patient was in persistent vegetative state. Despite impaired QoL, particularly in physical domains, the majority of interviewed patients and caregivers (7 of 8), including those with dominant-hemisphere stroke, were satisfied after a median follow-up of 18 months (range 6-43).

Conclusion:

Most patients report satisfactory QoL despite significant disability even in the face of moderate-to-severe disability and dominant-hemsiphere stroke. Dominant-hemisphere malignant MCA territory infarction should not be considered a contraindication to DH.
A Population-Based Study of Dystrophin Mutations in Canada
Authors: Mah Jean K. , Selby Kathryn , Campbell Craig , Nadeau Amelie , Tarnopolsky Mark , McCormick Anna , Dooley Joseph M. , Kolski Hanna , Skalsky Andrew J. , Smith R. Garth , Buckley David , Ray Peter N. , Yoon Grace
Published: December 2014

Introduction:

We carried out a population-based study of dystrophin mutations in patients followed by members of the Canadian Paediatric Neuromuscular Group (CPNG) over a ten-year period.

Objectives:

We aimed to describe the changes in diagnostic testing for dystrophinopathy and to determine the frequency of dystrophin mutations from 2000 to 2009.

Methods:

De-identified data containing the clinical phenotypes, diagnostic methods, and mutational reports from dystrophinopathy patients followed by CPNG centres from January 2000 to December 2009 were analyzed using descriptive statistics.

Results:

773 patients had a confirmed diagnosis of dystrophinopathy based on genetic testing (97%), muscle biopsy (2%), or family history (1%). 573 (74%) had complete deletion/duplication analysis of all 79 exons or whole gene sequencing, resulting in 366 (64%) deletions, 64 (11%) duplications, and 143 (25%) point mutations. The percentage of patients who were diagnosed using currently accepted genetic testing methods varied across Canada, with a mean of 63% (SD 23). 246 (43%) mutations involved exons 45 to 53. The top ten deletions (n=147, 26%) were exons 45-47, 45-48, 45, 45-50, 45-55, 51, 45-49, 45-52, 49-50, and 46-47. 169 (29%) mutations involved exons 2 to 20. The most common duplications (n=29, 5.1%) were exons 2, 2-7, 2-17, 3-7, 8-11, 10, 10-11, and 12.

Conclusion:

This is the most comprehensive report of dystrophin mutations in Canada. Consensus guidelines regarding the diagnostic approach to dystrophinopathy will hopefully reduce the geographical variation in mutation detection rates in the coming decade.
Meta-Analysis of Randomized Trials on First Line and Adjunctive Levetiracetam
Authors: Lo Benjamin W. Y. , Kyu Hmwe H. , Jichici Draga , Upton Adrian M. , Akl Elie A. , Meade Maureen O.
Published: December 2014

Context:

New evidence suggests that levetiracetam may be as effective as traditional agents, with better safety profile.

Objective:

To synthesize evidence regarding efficacy and tolerability of levetiracetam as first line, adjunctive or prophylactic antiepileptic agent.

Study Selection & Data Extraction:

Eligible studies were randomized controlled trials (RCTs) of levetiracetam used in adults with epilepsy. MEDLINE, EMBASE, CENTRAL, CINHAL, PAPERSFIRST, PROCEEDINGSFIRST, PROQUEST and conference proceedings identified studies (to September 30, 2010). Two investigators independently selected, appraised studies, collected and analyzed data.

Results:

Of ten eligible randomized trials, eight investigated adjunctive levetiracetam for refractory seizures, one as monotherapy for newly diagnosed seizures, one as monotherapy for prophylaxis. Eight RCTs of adjunctive levetiracetam were of moderate quality (GRADE criteria), with two showing lack of allocation concealment. Meta-analyses showed adjunctive levetiracetam was more effective than placebo in achieving at least 50% reduction of seizure frequency, when added to baseline antiepileptic regimen (pooled RR 2.15 [1.65,2.82], I2 = 45%, p value (heterogeneity) = 0.08, p value (overall effect) < 0.01). Likelihood of serious adverse events necessitating withdrawal from study was not significantly different between levetiracetam and control (pooled RR 1.37 [0.88,2.13], I2 = 0%, p value (heterogeneity) = 0.84, p value (overall effect) = 0.17). Subgroup analyses suggested similar effects across different dosages. Sensitivity analysis of studies with adequate concealment showed similar effects.

Conclusions:

Levetiracetam is an effective adjunctive agent for refractory epilepsy. More studies are needed to establish whether it is effective as monotherapy for newly diagnosed seizures, and for prophylaxis in traumatic brain injury.
Misdiagnosis of Epileptic Seizures as Manifestations of Psychiatric Illnesses
Authors: Mirsattari Seyed M. , Gofton Teneille E. , Chong Derek J.
Published: December 2014

Background:

Epileptic seizures may be misdiagnosed if they manifest as psychiatric symptoms or seizures occur in patients with known psychiatric illness.

Methods:

We present clinical profiles of six patients with epilepsy (three male, mean age 39 ± 12 years) that presented with prominent psychiatric symptoms.

Results:

Two patients had pre-existing psychiatric illnesses. Three patients were initially diagnosed with panic attacks, two with psychosis, and one with schizophrenia. Five patients had temporal lobe epilepsy (TLE) while the sixth patient was subsequently found to have absence status epilepticus (SE). Cranial computed tomogram (CT) including contrast study was unremarkable in five patients and showed post-traumatic changes in one patient. Cranial magnetic resonance imaging (MRI) revealed dysembryoplastic neuroepithelial tumour (DNET) in one patient, cavernous hemangioma in one, and post-traumatic changes plus bilateral mesial temporal sclerosis in another patient but it was normal in two TLE patients. Routine electroencephalography (EEG) revealed absence SE in one patient but it was non-diagnostic in the TLE patients. Video-EEG telemetry in the epilepsy monitoring unit (EMU) was necessary to establish the diagnosis in four TLE patients. None of the patients responded to medications aimed at treating psychiatric symptoms alone. Two patients required surgery while the other four required treatment with anti-epileptic drugs. All the patients had favorable response to the treatment of their epilepsy.

Conclusions:

This case series illustrates that epileptic patients may experience non-convulsive seizures that might be mistaken as primary psychiatric illnesses. In this subset of patients, evaluation by an epileptologist, MRI of the brain, and/or video-EEG telemetry in an EMU was necessary to confirm the diagnosis of epilepsy if routine EEGs and cranial CT are normal.
Metronidazole-Induced Encephalopathy: Case Report and Review of MRI Findings
Authors: Desai Jamsheed A. , Dobson Jessica , Melanson Michel , Pari Giovanna , Jin Albert Yongwon
Published: December 2014

A 74-year-old man presented with a four week history of behavioural disturbances, upper and lower extremity numbness and impaired balance. He had been treated with metronidazole for six months for osteomyelitis of the right hallux. Examination revealed encephalopathy, and glove-and-stocking sensory loss to pinprick with reduced vibration threshold at the toe. The gait was wide based and ataxic. Nerve conduction studies showed a large fibre sensory-motor axonal polyneuropathy. Magnetic resonance imaging (MRI) revealed a solitary restricted diffusion lesion in the splenium of the corpus callosum (Figure A, B) with subtle prolongation of T2 (Figure C). The radiographic differential diagnosis included hypoglycaemia, viral encephalitis, antiepileptic drug toxicity/withdrawal and metronidazole toxicity. The combination of the imaging finding with the history of prolonged metronidazole use suggested metronidazole induced encephalopathy.
Asphyxiation Causing Distinctive Basal Ganglia Injury and Generalized Dystonia
Authors: Wiltshire Katie M. , Myers Kenneth , Wei Xing-Chang , Mohamed Ismail
Published: December 2014

A previously healthy five year-old boy was seen after intermittent strangulation attempts over the course of two days. Details of the event are vague due to lack of reliable adult witnesses. There was no indication of toxic exposure. General examination on arrival to the emergency department revealed an alert boy with bruising of the neck consistent with multiple strangulation attempts. There was no evidence of heart, kidney, or liver involvement to suggest systemic hypoperfusion or organ failure. Investigations done on arrival showed no acidosis, and a capillary blood gas 48 hours later revealed a normal carboxyhemoglobin. Although the initial neurologic exam was near normal, severe generalized dystonia developed over the next four weeks, requiring intrathecal baclofen treatment.
Glioblastoma: Patterns of Recurrence and Efficacy of Salvage Treatments
Authors: Oh Jiwon , Sahgal Arjun , Sanghera Paul , Tsao May N. , Davey Phil , Lam Kelvin , Symons Sean , Aviv Richard , Perry James R.
Published: December 2014

Background:

It is controversial if distant recurrence of glioblastoma is more common after temozolomide (TMZ) concurrent with radiotherapy (RT). Optimal therapy for patients with recurrent disease after RT/TMZ is unclear. Our purpose was to evaluate recurrence patterns in glioblastoma and the effect of treatment at recurrence upon survival.

Methods:

We performed a retrospective review of 67 patients with newly diagnosed glioblastoma treated with RT/TMZ between 2003-2007. Statistical analyses included Kaplan-Meier method for survival, and multivariate Cox proportional hazards model for the effect of salvage treatment on survival.

Results:

58 patients (86.6%) recurred locally; 9 patients (13.4%) had a distant non-contiguous focus of new disease. Median survival(MS) was 17 months; median time-to-progression(TTP) 6.8 months. The local and distant groups had comparable prognostic factors. There was no difference in MS(p=0.35) or TTP(p=0.95) by location of recurrence. At relapse, 26 patients(38.8%) received continuous, dose-intense TMZ, 24(35.8%) other therapy(4.5% RT; 20.9% lomustine+/-procarbazine; 4.5% etoposide; 1.5% conventional TMZ; 4.5% TMZ then lomustine), and 17(25.4%) were untreated. Dose-intense TMZ was associated with prolonged MS compared to all other patients(21.5 months vs. 12.4 months, p=0.019, HR=3.86, 95%CI: 1.81-8.22) and similar to MS with other chemotherapy regimens(18.8 months, p=0.40, HR=1.30, 95% CI: 0.65-2.61).

Conclusion:

The pattern of recurrence of glioblastoma treated with RT/TMZ was predominantly local. Second-line treatment with continuous dose-intense TMZ may prolong survival in patients with recurrent glioblastoma. Overall survival is similar to other conventional salvage regimens; however TMZ may be better tolerated. This study is limited by its retrospective nature and potential selection bias. Prospective controlled studies are needed.
Amyloid β-related Angiitis of the Central Nervous System: Report of 3 Cases
Authors: Rigby Heather , Easton Alexander , Bhan Virender
Published: December 2014

Objective:

Amyloid-β (Aβ) related angiitis (ABRA) is a recently described clinicopathological entity characterized by cerebrovascular Aβ deposition and arteritis. Cerebral Aβ deposition is commonly present in cerebal amyloid angiopathy (CAA) and Alzheimer's disease (AD) but is rarely associated with inflammatory infiltration of vessel walls. Our objective is to help clarify the clinical spectrum, radiographic findings, response to treatment, and outcomes of ABRA. The neuropathological relationship between ABRA, cerebral amyloid angiopathy, and Alzheimer's disease is discussed.

Methods:

We present three cases of ABRA managed at a tertiary care centre.

Results:

All three patients presented with seizures and cognitive dysfunction; one had multifocal neurologic findings. Brain biopsies revealed inflammatory arteritis with Aβ deposits in the vessel walls. All were treated with steroids and cyclophosphamide. Two had favorable outcomes and one stabilized but with severe residual neurologic disability.

Conclusions:

ABRA is an unusual but likely under-recognized and potentially treatable disorder. As in other reported cases, our findings suggest that many patients respond favorably to immunosuppressive therapy. We believe that all biopsy specimens consistent with primary angiitis of the central nervous system (CNS) should be further examined for vascular Aβ deposition.
Gamma Knife for Cerebral Arteriovenous Malformations at a Single Centre
Authors: Zeiler F. A. , McDonald P. J. , Kaufmann A. , Fewer D. , Butler J. , Schroeder G. , West M.
Published: December 2014

Background:

We report the results of a consecutive series of patients treated with Gamma Knife (GK) Surgery for cerebral arteriovenous malformations (AVMs).

Methods:

We retrospectively reviewed 69 patients treated with GK for cerebral AVMs between November 2003 and April 2009, recording clinical data, treatment parameters, and AVM obliteration rates in order to assess our effectiveness with GK in treating these lesions.

Results:

Ten patients were lost to follow-up. Presentations included: seizure (24), hemorrhage (18), persistent headache (12), progressing neurological signs (10), and incidental (9). In 24 patients (34.8%) treatment planning consisted of digital subtraction angiography (DSA), magnetic resonance imaging (MRI), and computed tomogram (CT) angiography (CTA). Currently we rely predominantly on CTA and/or MRI scanning only. Fourty-one patients have been followed for a minimum of 3 years; average age 40.9yr., 58.5% males. Average dose at the 50% isodose line was 20.3 Gy (range 16 to 26.4 Gy). Obliteration was observed in 87.8% by MRI, CT, or DSA. Not all obliteration was confirmed by DSA. Complications occurred in 12 of 59 (20.3%) patients, and in 11 of 41 (26.8%) with 3 year follow-up. Major (temporary) complications for the 59 included symptomatic cerebral edema (7), seizure (2), and hemorrhage (1). Major permanent complications occurred in one patient suffering a cranial nerve V deafferentation, and in two patients suffering a hemorrhage.

Conclusion:

GKS for cerebral AVM's offers an effective and safe method of treatment, with low permanent complication rate.