bluuebunny/crossref_metadata_2025 huggingface dataset:
Malignant Teratoid/Rhabdoid Tumour: Long-Term Survival
Authors: Fisher Barbara J. , Dennis Kristopher E.B. , Ang Lee-Cyn
Published: December 2014

The patient is a previously healthy 19-month-old child who became symptomatic in October 1998 with right hand weakness and cessation of new words with progression of right sided weakness and decreased sensation over the next month. The computed tomography (CT) and magnetic resonance imaging (MRI) scans demonstrated a large, deep, left frontal periventricular enhancing mass with several cystic areas. A left frontoparietal craniotomy was performed and the tumour was grossly resected. The pathological diagnosis was AT/RT. Grossly, the tumour was composed of firm, pink-tan hemorrhagic tissue. Sections showed a densely cellular, infiltrating neoplasm with areas of necrosis (some calcified), fascicular and focal sheets of collagen, and a variable, often prominent reticulin network. There was no endothelial proliferation. Light microscopy with hematoxylin and eosin stain (Figure 1) revealed a varied tumour appearance predominantly composed of larger tumour cells, with randomly dispersed nests of small undifferentiated cells close to the interface of tumour and normal brain tissue. The larger cells had several growth patterns: some were loosely arranged bi- or multi-polar cells forming cords and acinar structures against a faintly basophilic mucinous background, some were elongated cells arranged in coarse follicles or other compact bundles, while others were arranged in sheets or nests with prominent, sometimes glassy eosinophilic and cytoplasmic inclusions and eccentric nuclei (rhabdoid cells). Larger cells had nuclei with vesicular chromatin and prominent nucleoli, and smaller cells had hyperchromatic nuclei. Mitoses were focally numerous with some atypical in form, and cytoplasmic glycogen was inconspicuous. Immunohistochemical studies revealed widespread staining for epithelial membrane antigen (EMA), vimentin (Figure 2), glial fibrillary acid protein (GFAP), groups of cells with membranous patterns of staining for CD99, regional staining for neuron-specific enolase (NSE), scattered staining for transthyretin, and rare staining for actin, cytokeratin, and synaptophysin. The tumour was positive for chromosome 22 monosomy. Electron microscopy showed polygonal cells arranged in ill-defined acini that formed inconspicuous, sometimes entwined short microvillus processes. Variable numbers of intermediate filaments were identified and some cells showed a rudimentary basal lamina. Desmosomes were not seen, and there was no evidence of neural differentiation.
Long Survival of Primary Pineal Melanoma with Radiation Treatment Only
Authors: Barron Jane , Morris-Larkin Carolyn , Finch Terry , Maroun Falah , Hache Nanette , Yousef George M.
Published: December 2014

Pineal gland tumors in adults account for about 0.4 to 1 % of all intracranial tumors. A wide variety of tumors can occur in the pineal gland. The most common are the pineal parenchymal tumors (pineocytomas, pineoblastomas, and pineal parenchymal tumors of intermediate differentiation), germ cell tumors, and glial tumors. Uncommonly in the pineal gland one may encounter a metastatic tumor, a meningioma or a melanoma.

We here report a case of primary pineal gland melanoma with prolonged survival of 56 weeks after radiotherapy alone. To our knowledge, this is the first reported case of prolonged survival after radiotherapy alone, without surgical intervention or chemotherapy. Contrary to previous reports, our case demonstrates that a pineal melanoma with leptomeningeal dissemination can still yield a better survival with radiation treatment only.
Group Education with Personal Rehabilitation for Idiopathic Parkinson's Disease
Authors: Guo Liping , Jiang Yuping , Yatsuya Hiroshi , Yoshida Yoshitoku , Sakamoto Junichi
Published: December 2014

Objective:

To evaluate the effect of a group education program with personal rehabilitation for idiopathic Parkinson's disease (IPD).

Methods:

A single-blind, randomized, controlled clinical trial, with a pre-test/post-test quasi-experimental design. Fortyfour stable, non-demented patients with IPD were randomly assigned to an intervention group (n=23) and control group (n=21). Three group lectures on health education specific to IPD were delivered to the intervention group. The theme of lectures was “Not a lonely journey, as your friend, we—experts specializing in IPD— would love to join with you.” Three domains of the management of IPD were mentioned: “How to manage your routine Meal, Moving and Mood after suffering from IPD” Each lecture was scheduled for 45 minutes (excluding breaks), and designed as an interactive dialogue rather than a traditional lecture. To strengthen the impact of the lectures, relevant information was published on a website: http://www.parkinsonism.cn/. Following the group lecture, individualized and tailored rehabilitation made up of 24 half-hour sessions over eight weeks were provided for the intervention group.

Results:

The program ran smoothly and IPD patients were satisfied with this program. After only eight weeks, the health-related quality of life (HR-QOL) in the intervention group improved significantly (p<0.001). Patients and their caregivers in this group also reported their mood elevations following the program.

Conclusion:

Group education combined with personal rehabilitation program appears to be a beneficial and practical intervention, not only complementing the medical treatment for IPD patients but also meeting the growing demand for long-term care.
Bulbar Signs in Normal Population
Authors: Lim K S , Hew Y C , Lau H K , Lim T S , Tan C T
Published: December 2014

Background and Objectives:

There is lack of published data on bulbar signs among the healthy population. This study aims to determine the range of normality of bulbar signs particularly among the elderly.

Methods:

Systemic examination of bulbar signs was carried out according to a predetermined protocol on a cohort of young and elderly healthy subjects.

Results:

A total of 206 subjects were recruited in the study, 104 young adults with mean age of 20 years, and 102 elderly with mean age of 73 years. Uvula deviation was seen in 28 (26.9%) young subjects and 22 (21.6%) elderly. Irregular tongue border was seen in 17 subjects, unilateral in 4 subjects. Fourteen (6.8%) subjects had deviation on tongue protrusion. Occasional tremor of tongue on protrusion is common in both young and old. Persistent (severe) tongue tremor on protrusion was seen in 18.6% of the elderly, and 4.8% of the young. None of the subjects had tremor of tongue at rest. In gag reflex, absence of gagging response was common in elderly, seen in two thirds of the subjects on stimulation of the posterior pharyngeal wall. However, all the subjects had uvular movement. Habituation or suppression of gagging response was seen in close to 90% of young males.

Conclusion:

There is wide range of normality in bulbar signs in normal population, particularly among the elderly.
Malformations of the Fetal Dural Sinuses
Authors: McInnes Matthew , Fong Katherine , Grin Andrea , ter Brugge Karel , Blaser Susan , Halliday William , Shannon Patrick
Published: December 2014

Background:

Dural sinus malformation (DSM) is a term used to describe congenital vascular malformations characterized by massive dilation of one or more dural sinuses: these dilatations are typically associated with arteriovenous shunts. Such malformations can present antenatally but their early natural history and anatomy is poorly defined.

Methods:

We reviewed five years of autopsy experience and retrieved three primary vascular malformations of the fetal dural sinuses with ultrasound, magnetic resonance imaging (MRI) and post-mortem correlation.

Results:

Fetal ultrasound and MRI obtained between 19 and 23 weeks gestational age demonstrated in all cases dilation of the dural sinuses. In two cases vascular thromboses were present in either the dilated dural sinus (one of three) or the associated arteriovenous fistula (one of three). All cases were autopsied at 22-23 weeks gestational age. In one there was imaging and autopsy evidence of remodeling of the dural sinuses associated with a pial arteriovenous fistula. In two cases, no arteriovenous malformation was identified on initial imaging, but only became evident at autopsy. One case showed morphological overlap with vein of Galen aneurysmal malformation, with a midline arteriovenous shunt and vein of Galen ectasia. The other demonstrated a perisylvian dural arteriovenous fistula.

Conclusion:

In utero thrombosis of feeding vascular malformations or of the dural sinus malformation may be prominent. The early in utero developmental trajectory of dural sinus malformation (DSM) is poorly defined and deserves further study.
Movements in Brain Death: A Systematic Review
Authors: Saposnik Gustavo , Basile Vincenzo S. , Young G. Bryan
Published: December 2014

Brain death is the irreversible lost of function of the brain including the brainstem. The presence of spontaneous or reflex movements constitutes a challenge for the neurological determination of death. We reviewed historical aspects and practical implications of the presence of spontaneous or reflex movements in individuals with brain death and postulated pathophysiological mechanisms. We identified and reviewed 131 articles on movements in individuals with confirmed diagnosis of brain death using Medline from January 1960 until December 2007, using ‘brain death’ or ‘cerebral death’ and ‘movements’ or ‘spinal reflex’ as search terms. There was no previous systematic review of the literature on this topic. Plantar withdrawal responses, muscle stretch reflexes, abdominal contractions, Lazarus's sign, respiratory-like movements, among others were described. For the most part, these movements have been considered to be spinal reflexes. These movements are present in as many as 40-50% of heart-beating cadavers. Although limited information is available on the determinants and pathophysiological mechanisms of spinal reflexes, clinicians and health care providers should be aware of them and that they do not preclude the diagnosis of brain death or organ transplantation.
Early Ischemic Stroke Presentation in Pakistan
Authors: Kamal Ayeesha Kamran , Khealani Bhojo Asumal , Ansari Sajjad Ahmed , Afridi Maria , Syed Nadir Ali
Published: December 2014

Introduction:

There are no studies from Pakistan that describe stroke presentation rates or factors associated with early or delayed presentation. This is important to know because current clinical protocols limit the use of recombinant tissue plasminogen activator (rtPA), the only available therapy for acute ischemic stroke, to a three-hour window from symptom onset.

Methods:

All patients aged 14 years or above with acute ischemic stroke of ≤ 48 hours duration were prospectively identified from the Aga Khan University Stroke Data Bank over a 22-month period ending May 2001.

Results:

269 ischemic stroke patients presented within 48 hours of stroke onset. 55 out of 269 (21%) presented within first three hours and 110 out of 269 (41%) within first six hours. Unawareness of treatment options (p <0.001) and inappropriate diagnosis and field triage (p=0.005) were associated with delayed presentation. Small vessel occlusion or lacunar stroke in the TOAST (Trial of ORG 10172 in Acute Stroke Treatment) ischemic stroke subtype was associated with delayed presentation (p=0.047) and cardioembolic stroke was associated with earlier presentation (p=0.048). Stroke severity assessed with the National Institutes of Health Stroke Scale at a cut off score of ≥ 15 was not associated with earlier time to presentation at three hours (p=0.114) but there was some tendency at six hours (p=0.097).

Conclusions:

The rate of early stroke presentation in a Pakistani tertiary care facility is comparable to certain developed countries. To increase the proportion of patients who can benefit from thrombolytic therapy, programs need to be instituted to increase public awareness of treatment options for stroke and expedited referral by the primary care provider.
Migraine: Prevalence and Associated Disability Among Nigerian Undergraduates
Authors: Wahab Kolawole Wasiu , Ugheoke Asuwemhe Johnson
Published: December 2014

Background:

Studies on migraine in Nigeria are scanty while the disability associated with the disease has not been determined. We aimed at determining the lifetime prevalence of migraine and its associated disability among the students of a Nigerian university.

Method:

Using a multi-stage sampling method, undergraduates of Ambrose Alli University, Nigeria were screened with the International Headache Society (IHS) criteria. Those who satisfied the criteria for migraine were then assessed with the Migraine Disability Assessment questionnaire to ascertain the level of disability resulting from migraine in the three months preceding the study. Information was also obtained on the use of preventive and abortive medications.

Results:

Out of the 1513 respondents screened, 145 satisfied the IHS criteria for the diagnosis of migraine giving an overall lifetime prevalence of 9.6% (females 10.3%, males 8.9%, p>0.05). Migraine associated disability was little or none in 53.1% (males 55.2%, females 51.3%, p>0.05), mild in 10.3% (males 11.9%, females 9.0%, p>0.05), moderate in 20.7% (males 20.9%, females 20.5%, p>0.05) and severe in 15.9% (males 11.9%, females 19.2%, p>0.05). In spite of the fact that 53 (36.6%) of the migraineurs had moderate-severe disability, only 19/53 (35.8%) reported being on preventive treatment while none had ever used a triptan.

Conclusion:

Prevalence of migraine among our respondents falls within the range reported from similar studies among university students outside Africa. In spite of the high disability associated with the disease, use of effective preventive and abortive therapies is very poor.
Neuroleptic-Induced Tardive Cervical Dystonia: Clinical Series of 20 Patients
Authors: Godeiro-Junior Clecio , Felício Andre C. , de Carvalho Aguiar Patrícia , Borges Vanderci , Silva Sonia M. A. , Ferraz Henrique B.
Published: December 2014

Background:

Cervical dystonia (CD) may be classified according to the underlying cause into primary or secondary CD. Previous exposure to neuroleptics is one of the main causes of adult-onset secondary dystonia. There are few reports that characterize the clinical features of primary CD and secondary neuroleptic-induced CD. Herein our aim was to investigate a series of patients with neuroleptic induced tardive CD and to describe their clinical and demographic features.

Patients and Methods:

We retrospectively evaluated 20 patients with neuroleptic-induced tardive CD and compared clinical, demographic and therapeutic characteristics to another 77 patients with primary CD. All patients underwent Botulinum toxin type-A therapy.

Results:

We did not identify any relevant clinical and demographic characteristics in our group of patients that could be used to distinguish tardive and primary CD.

Conclusion:

Patients with tardive CD presented demographic characteristics and disease course similar to those with primary CD.
Progress in Clinical Neurosciences: The ‘Antiplatelet’ Agents and the Role of the Endothelium
Authors: Mateen Farrah J. , Shuaib Ashfaq
Published: December 2014

The antiplatelet drugs, commonly used in the prevention and treatment of cerebrovascular disease, possess a number of effects that are independent of direct antiplatelet actions. Beneficial and detrimental effects both occur. The endothelium is an important mediator of these non-antiplatelet effects. We performed a literature search to locate articles related to acetylsalicylic acid (aspirin), clopidogrel, ticlopidine, and dipyridamole and the interactions of these medications with the endothelium. The role of each of the above medications is explored in relation to vasodilation, inflammation, oxidation, platelet-leukocyte interactions, and thrombogenic tendency via platelet-vessel wall interactions.
The Cardiac R-R Variation and Sympathetic Skin Response in the Intensive Care Unit
Authors: Bolton Charles , Thompson Jillian , Bernardi Linda , Voll Christopher , Young Bryan
Published: December 2014

Background and Purpose:

The central and peripheral nervous systems are often affected in intensive care unit (ICU) patients, especially those with prolonged assisted ventilation and sepsis or systemic inflammatory response syndrome (SIRS). The autonomic nervous system, however, has been under-investigated in such patients. We evaluated autonomic nervous system (ANS) function in 29 ICU patients with various neurological disorders.

Methods:

Testing involved cardiac R-R variation (CRRV) as an index of parasympathetic function and the sympathetic skin response (SSR) for sympathetic assessment.

Results:

Only those 8 patients with sepsis-related neuropathy or encephalopathy had abnormal CRRV, while the SSR was absent in all but 2 patients.

Conclusions:

Our preliminary study revealed a high incidence of autonomic dysfunction in ICU patients with various neurological disorders.
Parkinson's Disease, Multiple Sclerosis and Changes of Residence in Alberta
Authors: Yiannakoulias Nikolaos , Schopflocher Donald P. , Warren Sharon A. , Svenson Lawrence W.
Published: December 2014

Background:

Our objective is to examine how persons diagnosed with Multiple Sclerosis (MS) and Parkinson's disease (PD) change residence following disease onset. We hypothesize that persons choose to change residence (locally or regionally) in different ways depending on whether or not they have been diagnosed with MS/PD. We also estimate the effects of residence change on measures of disease prevalence made at several different levels of geography.

Methods:

Using fee-for service and hospitalization data, we identify cases of MS and PD between 1994 and 2004. Both of these case groups are matched to controls based on age, sex, socioeconomic status and municipality of residence. We tabulate and compare the changes of residence among persons in the case and control groups. We also use these data to estimate the effects that changes in residence have on disease prevalence at three different levels of geography.

Results:

Both MS and PD patients were more likely to change residence following disease onset compared to groups of matched controls (p<=0.001). Most changes of residence occur within the same municipality. The total magnitude of these changes is small, however, and is unlikely to affect estimates of disease prevalence; over our study period, the largest change in geographical prevalence estimates due to individual changes in residence was about 1%.

Conclusions:

Persons diagnosed with MS and PD both have mobility characteristics that differ from those of their respective control groups, and in general, are more likely to move to or between Edmonton and Calgary, and less likely to move out of province. However, the balance of mobility characteristics of persons with PD and MS appear unlikely to greatly affect the patterns observed on maps of disease prevalence.
Radiation-Induced Cavernous Hemangiomas: Case Report and Literature Review
Authors: Keezer Mark Robert , Del Maestro Rolando
Published: December 2014

The case of a 51-year-old man diagnosed with two acquired cavernous hemangiomas 17 years after cranial irradiation for a cerebellar astrocytoma is reported. A review of 84 cases of radiation-induced cavernous hemangiomas found in the literature is presented. In this series the mean age at the time of irradiation (±SD) was 10.4 ± 2.0 years (median = 8 years), while the mean time to cavernous hemangioma diagnosis (±SD) was 10.3 ± 1.9 years (median = 8 years). Time to cavernous hemangioma diagnosis was found to be inversely related to radiation dose. Hemorrhage from radiation-induced cavernous hemangiomas was found in 40.0% of patients, with an incidence of 3.9% per patient year. An inverse trend was identified between radiation dose and symptomatic presentation, cavernous hemangioma hemorrhage or surgical resection. This review of radiation-induced cavernous hemangiomas confirms that both younger patients and those who received a larger dose of radiation are at increased risk of radiation-induced cavernous hemangiomas. Our results suggest that, based on an assessment of CT or MR images, there may be an increased risk of hemorrhage when comparing radiation-induced to congenital cavernous hemangiomas. Increasing radiation doses appear to stabilize these lesions, decreasing the risk of a symptomatic presentation, cavernous hemangioma hemorrhage and surgical intervention.
Paul Yakovlev Remembered: An Interview with Maurice Victor
Authors: Laureno Robert
Published: December 2014

Maurice Victor, the great Canadian neurologist, was an expert on the neurology of alcoholism including the Wernicke-Korsakoff syndrome. In Boston, Massachusetts he encountered the leading neuroanatomist, Paul Yakovlev, an immigrant from Russia. Together Victor and Yakovlev collaborated on translating the work of S.S. Korsakoff from Russian to English. This interview of Victor about Yakovlev is informative about both of these great figures in North American Neurology.
National Human Resources Survey of Clinical Neurophysiologists in Canada
Authors: Chan K. Ming , Young G. Bryan , Warren Sharon
Published: December 2014

Background:

Although electromyography (EMG), electroencephalography (EEG) and evoked potential (EP) studies are common investigation tools for patients with neurologic illnesses, no formal data on the manpower supply in Canada exists. Because of the importance of these on training requirements and future planning, the purpose of this study was to establish a comprehensive profile of the human resources situation in clinical neurophysiological services across Canada.

Methods:

A questionnaire was sent to all clinical neurophysiologists in Canada. To capture the maximal number of respondents, a total of three rounds of mail out were done. In addition, to obtain accurate demographic data on supporting technologists, a separate survey was also carried out by the Association of Electrophysiological Technologists of Canada.

Results:

Of the 450 clinical neurophysiologists identified and surveyed, the provincial response rate was 59±14% (mean±SD). Of these, the vast majority practiced in urban centres. There was substantial regional disparity in different provinces. While the wait time for most EEG and EP laboratories was less than six weeks, the wait time for EMG was substantially longer. With the age of the largest number of practitioners in their sixth decade, projected retirement over the next 15 years was 58%. The demographic distribution of the supporting technologists showed a similar trend.

Conclusions:

In addition to considerable regional disparity and urban/rural divide, a large percentage of clinical neurophysiologists and supporting technologists planned to retire within the coming decade. To ensure secure and high standard services to Canadians, solutions to fill this void are urgently needed.
Severe Proximal Myopathy with Remarkable Recovery after Vitamin D Treatment
Authors: Al-Said Yousef A. , Al-Rached Hiyam S. , Al-Qahtani Hussien A. , Jan Mohammed M.S.
Published: December 2014

Background:

Osteomalacia is an uncommon cause of muscle weakness. Our objectives were to describe features of myopathy associated with Vitamin D deficiency and examine the contributing factors leading to osteomalacic myopathy in our region.

Methods:

Patients identified retrospectively for the six year period ending in December 2006 with the diagnosis of osteomalacia and/or Vitamin D deficiency associated proximal muscle weakness were included. They were followed in three major centers in western Saudi Arabia. Clinical, biochemical, radiological, and electrophysiological findings were collected before and after Vitamin D treatment by chart review.

Results:

Forty seven female patients aged 13-46 years (mean 23.5, SD 4.5) were included. All were veiled and covered heavily when outside the house for social and cultural reasons. Only eight (17%) had adequate varied diet with daily milk ingestion. All patients presented with progressive proximal muscle weakness lasting 6-24 months (mean 14) prior to our evaluation. The weakness was severe in six (13%) patients leading to wheel chair bound states. Associated musculoskeletal pain involving the back, hips, or lower limbs was common (66%). Osteomalcia was the referral diagnosis in only 11 patients and the remaining 36 (77%) patients were misdiagnosed. All patients had metabolic and radiological profiles suggestive of osteomalacia. Remarkable recovery was documented in all patients following oral cholecalciferol and calcium supplementation.

Conclusions:

Vitamin D deficiency is an important treatable cause of osteomalacic myopathy in Saudi Arabia. The diagnosis is frequently delayed or missed. Screening for Vitamin D deficiency in patients with acquired myopathy is needed to identify this treatable disorder.
Development of a Concise QOL Questionnaire for Brain Tumor Patients
Authors: Goffaux Philippe , Boudrias Marie , Mathieu David , Charpentier Catherine , Veilleux Nadia , Fortin David
Published: December 2014

Background:

The purpose of this study was to develop and validate a self-administered questionnaire to measure the health-related quality of life (QOL) of patients with brain cancer. We wanted to assess both core and disease-specific concerns in a single, easy-to-use instrument, thus promoting concision and clinical utility. The questionnaire departs from its predecessors in that it was designed for- and validated among French speaking Canadians.

Methods:

A focus group of health professionals was used to develop items for the questionnaire, which was later validated with 105 patients suffering from brain cancer. The underlying structure of the questionnaire was investigated using principal component analysis and confirmed using a principal factor analysis.

Results:

The final version of the questionnaire contains 30 items. Seven multi-item scales, tapping into distinct dimensions of QOL, were uncovered (i.e., functional well-being, symptom severity/fear of death, social support/acceptance of disease, autonomy in personal care, digestive symptomatology, neurocognitive function, and pain). Assessment of reliability revealed elevated internal consistency for each of the seven scales (Cronbach coefficient alpha ≥≥.65), whereas known-groups validity (anchor-based approach) revealed that the different dimensions uniquely discriminated between patients with different functional levels (Karnofsky Performance Scores) and clinical status (exposure to neurosurgery, radiotherapy, and use of chemotherapy and anticonvulsants).

Conclusion:

Our QOL questionnaire, the Sherbrooke Neuro-Oncology Assessment Scale, or SNAS, taps into both core and disease-specific issues relevant to neuro-oncology patients. It has good validity and reliability, and clearly reflects the multidimensional nature of QOL. Depending on the research focus, it may be used in clinical trials to track the impact of disease and/or treatment on satisfaction, functional status, and general well-being.
Adjuvant Chemotherapy for Adults with Malignant Glioma: A Systematic Review
Authors: Perry James , Laperriere Normand , Zuraw Lisa , Chambers Alexandra , Spithoff Karen , Cairncross J. Gregory
Published: December 2014

Objective:

This systematic review examines the role of chemotherapy following surgery and external beam radiotherapy for adults with newly diagnosed malignant glioma.

Methods:

MEDLINE, EMBASE, and the Cochrane Library databases were searched to August 2006 to identify relevant randomized controlled trials (RCTs) and meta-analyses. Proceedings from the 1997 to 2006 annual meetings of the American Society of Clinical Oncology were also searched.

Results:

Two RCTs reported a survival advantage in favour of radiotherapy with concomitant and adjuvant temozolomide compared with radiotherapy alone in patients with anaplastic astrocytoma or glioblastoma. Twenty-six RCTs and two meta-analyses detected either no advantage or a small survival advantage in favour of adjuvant chemotherapy.

Conclusion:

Concomitant temozolomide during radiotherapy and post-radiation adjuvant temozolomide is recommended for all patients ages 18-70 with newly diagnosed glioblastoma multiforme who are fit for radical therapy (ECOG 0-1). Temozolomide may be considered in other situations (i.e., ECOG 2, biopsy only, age >70, intermediate grade glioma), but there is no high-level evidence to support this decision. Moreover, there are few data on long-term toxicities or quality of life with temozolomide. Adjuvant chemotherapy may be an option for younger patients with anaplastic (grade 3) astrocytoma and patients with pure or mixed oligodendroglioma. However, there is no evidence of a survival advantage from adjuvant chemotherapy in these patients, and treatment-related adverse effects and their impact upon quality of life are poorly studied. The combination of procarbazine, lomustine, and vincristine (PCV) is not recommended for patients with anaplastic oligodendroglioma and oligoastrocytoma.
A Case-Control Study of Tissue Plasminogen Activator for Acute Ischemic Stroke
Authors: Reid J M , Phillips S J , Gubitz G J , Jarrett J , Christian C , Dai D
Published: December 2014

Background:

Randomized trials demonstrate that intravenous tissue plasminogen activator (tPA) improves outcome in acute ischemic stroke (AIS). To assess translation of this efficacy into effectiveness in routine clinical practice we performed a case-control study of tPA treatment for AIS in a single hospital.

Methods:

151 tPA-treated AIS patients (1996-2005) were matched 1:1 with blinding to outcome to controls from a prospective registry based on age, gender, pre-stroke Oxford handicap scale (OHS), stroke severity, and subtype. The outcomes were in-hospital death, symptomatic intracranial hemorrhage (SICH), length-of-stay (LOS), discharge OHS and long-term survival.

Results:

In-hospital mortality (23% vs. 24%) or long-term survival (median follow-up of 2 years) was not different between cases and controls (p=0.83). SICH occurred in 7.8% (95% CI 4.2-13.5%) of tPA-treated patients. Median LOS was non-significantly shorter for cases (13 [7-29] vs. 16 [8-32] days, p=0.14) but significantly shorter in tPA-treated vs. non-treated women (14 [7-28] vs. 20 [11-34] days, p=0.04). At discharge 6.6% (95% CI 1.1-12.0%) more tPA-treated patients than controls had no disability (OHS ≤1, p=0.02). However, there was no difference in discharge independence rates or proportion discharged home.

Conclusion:

We demonstrate minor improvements in early recovery after stroke with tPA but the impact is less dramatic than that reported in randomized trials. This may relate to timing of treatment and the type of patients treated.
PLEDS : corrélations cliniques
Authors: Fitzpatrick W. , Lowry N.
Published: December 2014

Objective:

We reviewed our experience in 96 consecutive patients exhibiting periodic lateralized epileptiform discharges (PLEDs) on EEG.

Methods:

EEG reports from January 1, 1999 to September 30, 2006 were screened for the term ‘PLEDs’ and its variants. A retrospective chart review, including examination of neuroimaging and other investigations, was conducted on each patient identified.

Results:

Acute stroke, tumor and central nervous system infection were the most common etiologies, accounting for 26%, 12% and 12% of cases respectively. Acute hemorrhage and traumatic brain injury combined accounted for another 12%. Previously unreported etiologies included posterior reversible encephalopathy syndrome (PRES), familial hemiplegic migraine and cerebral amyloidosis. There were 9 cases of chronic PLEDs attributable to underlying cortical dysplasia or severe remote cerebral injury, all with an accompanying partial seizure disorder. A prominent role for alcohol withdrawal was noted, and in 6 cases was the sole etiological factor. Fever was present as a potential contributing factor in 40% of cases, and significant metabolic abnormalities in 35%. Seizure activity occurred in 85% of patients overall, but in 100% of patients with PLEDs Plus and BiPLEDs Plus. The overall mortality rate was 27%. Mortality among patients with BiPLEDs however was almost twice that, at 52%.

Conclusions:

This case series demonstrates the wide variety of potential PLED etiologies. It also emphasizes that despite advances in neurocritical care, the morbidity and mortality associated with PLEDs has changed little since their recognition four decades ago.
Sporadic Creutzfeldt-Jakob Disease with Worsening Depression and Cognition
Authors: Hudon Mark , Farb Richard , Muayqil Taim , Siddiqi Zaeem A.
Published: December 2014

A 58-year-old male presented with a one-year history of low mood, early morning awakening from sleep, apathy, difficulty with memory, concentration and organization. This had been associated with intrusive concerns of a recent social stressor. He was no longer able to work and was on medical disability. Except for a 20kg weight loss there were no other constitutional or neurological symptoms. He had hypertension and hypercholesterolemia and was on atorvastatin and aspirin. He scored 28/30 on mini-mental status examination (MMSE) with errors on object recall; however he could recall forgotten items after cueing. He had difficulty with concentration, was apathic andhad a negative outlook to the future. His neurological examination and a detailed hematological work up including chemistry, cell counts, vitamin B12, folate, and renal, hepatic and thyroid function tests were normal. A brain magnetic resonance image (MRI) showed mild cerebral atrophy. Based on a formal neuropsychological assessment he was diagnosed with depression and started on Venlafaxine.
Remote Cerebellar Haemorrhage after Spinal Surgery
Authors: Calisaneller Tarkan , Yilmaz Cem , Ozger Ozkan , Caner Hakan , Altinors Nur
Published: December 2014

Remote cerebellar haemorrhage (RCH) is a rare but potentially lethal complication of spinal surgery resulting from inadvertent opening of the dura. The precise mechanism of this type of haemorrhage remains undetermined. Magnetic resonance imaging (MRI) may show a ‘Zebra sign’ representing the horizontal curvilinear configuration of haemorrhage between the
cerebellar folia.
Spontaneous Spinal Epidural Hematoma in a Child with Hemophilia B
Authors: Bisson Erica F. , Dumont Travis , Tranmer Bruce
Published: December 2014

Central nervous system hemorrhage is an uncommon and potentially devastating event in hemophiliacs, with an incidence of 2-8%. Intraspinal hematomas account for 8.5% of CNS hemorrhages in patients with factor VIII or IX deficiencies. Although most reported cases in this population are secondary to trauma, there are a few reports of spontaneous hemorrhage. We present a case of spontaneous spinal epidural hematoma (SSEH) in a child with Hemophilia B, factor IX deficiency, and review the relevant management strategies.
Acute Epidural Hematoma Secondary to Skull Metastasis from Esophageal Carcinoma
Authors: Ellis Michael J. , McDonald Patrick J.
Published: December 2014

The skull may be a site of metastasis in malignancies such as carcinoma of the breast, prostate, lung, and multiple myeloma. The majority of patients with skull metastases are asymptomatic or present with localized swelling which is often painless. While subdural hematoma has been commonly associated with metastatic disease involving the dura, hemorrhage from a skull
metastasis remains a rare occurrence. We present the case of acute epidural hematoma resulting from metastatic esophageal carcinoma to the skull.
Postural Sway and Effect of Levodopa in Early Parkinson's Disease
Authors: Beuter Anne , Hernández Roberto , Rigal Robert , Modolo Julien , Blanchet Pierre J.
Published: December 2014

Objective:

To characterize postural stability control and levodopa responsiveness in early Parkinson's disease (PD).

Methods:

Postural sway was studied during quiet stance in ten patients within six years of PD onset, both before (OFF) and after (ON) regular oral levodopa dosing. Postural sway was recorded using a force platform during 30 sec with eyes open, and six dependent variables were examined.

Results:

Mild baseline subclinical changes in postural sway were recorded in our patients. Clear benefit was observed in five out of six characteristics (mean sway, transversal sway, sagittal sway, sway intensity, and sway area) in the ON condition.

Conclusion:

Postural control mechanisms are affected early in PD and modulated by dopamine.
The Learning Curve of Minimally-Invasive Lumbar Microdiscectomy
Authors: McLoughlin Gregory S. , Fourney Daryl R.
Published: December 2014

Object:

The safe integration into practice of a new surgical technique requires an appreciation of the learning curve. The object of this study was to assess the learning curve for minimally invasive microdiscectomy (MIM) utilizing a tubular retractor system.

Methods:

A prospective evaluation of a single surgeon's first 52 consecutive MIM cases for radiculopathy secondary to single-level posterolateral lumbar disc herniation was performed. The learning curve was assessed using operative time, conversion to open rate, complications, and length of hospitalization.

Results:

The duration of operative time decreased over the course of the study (range, 49-151 min). By case 15, operative time was typically 60 min or less. There was only one conversion to an open procedure (Case 2). Complications occurred in three cases. All but nine patients were discharged home on the day of surgery.

Conclusion:

The learning curve for MIM was demonstrated. Further assessment of this curve for a large group of surgeons is necessary before a randomized controlled trial comparing standard microdiscectomy to MIM can be conducted.
ONF Trainee Awards Contribute to Capacity Building in Neurotrauma
Authors: Turner Jane , Turner Dan , Riopelle Richard J. , Bassett-Spiers Kent , Kagan Corinne
Published: December 2014

Objective:

Injury to the brain and spinal cord is one of the most catastrophic and costly occurrences in the Ontario health system. The objective of the present study was to evaluate the impact of past Ontario Neurotrauma Foundation (ONF) studentships and fellowships in terms of capacity building in the neurotrauma field in Ontario.

Method:

An online, cross sectional survey amongst past recipients of studentships and fellowships that terminated prior to July 2005. Explicit data were collected on various aspects of career development including current activity, awards and publications.

Results:

Thirty-six out of 42 (86%) eligible past trainees responded; 12 (33%) were Masters students, 12 (33%) were PhD students and 12 (33%) were Post-Doctoral students. A majority of the recipients (61%) are currently involved in neurotrauma-related activities (clinical, research and teaching) in more than 20% of their time, with no substantial differences between the degree groups. Half the recipients are currently involved in neurotrauma-related research in more than 20% of their time. The awardees published 1.5 peer-review manuscripts/person-year and received multiple awards. A high majority of our recipients (86%) feel that the ONF award had a substantial impact on their career.

Conclusions:

A high proportion of past award recipients remain involved in neurotrauma activities, especially in research. These results may lead to a cautious conclusion of the positive impact of the ONF studentships and fellowships on neurotrauma capacity building. These results should be considered in strategic planning of funding agencies similar to ONF.
Glioblastoma in a Patient with a Hereditary Cancer Syndrome
Authors: Steckley J L , Watling C J , Ng W.
Published: December 2014

A 68-year-old Caucasian male presented to emergency with a spontaneous sudden onset headache localized behind the left eye. Pain was severe and associated with vomiting, agitation, anxiety, memory loss, and word-finding difficulty. There was no preceding trauma and no meningeal symptoms. Further history was significant for mitral valve repair, atrial fibrillation treated with coumadin, colorectal cancer treated with right-sided hemicolectomy 17 years prior, and multiple skin tumors, which included squamous cell and basal cell carcinomas of his face and ears. Family history was significant for colon cancer in his father (at age 50), a sister (at age 40), and a niece (at age 38), as well as primary brain cancer in his paternal aunt.
Nodular Fasciitis Presenting in the Obturator Nerve and Gracilis Muscle
Authors: Fallah Aria , Grochmal Joey , Lu Jian-Qiang , DiFrancesco Lisa M. , Khalil Moosa , Clark Arthur W. , Midha Rajiv
Published: December 2014

Nodular fasciitis is a reactive proliferation of fibroblasts/myofibroblasts characterized by a rapidly growing non-tender subcutaneous mass and subsequently self-limited course. It is commonly found on the upper extremities, face, and shoulder, although it may occur in any superficial location. Its etiology is largely unknown, but associations have been made with local trauma, infection and inflammation. We report a case of an intraneural and intramuscular nodular fasciitis presenting in the obturator nerve and gracilis muscle, respectively. To our knowledge, this is the second reported case of this disease presenting in a nerve of the lower extremity, as well as the first reported case of it presenting within muscle and nerve simultaneously.
Ataxias with Autosomal, X-Chromosomal or Maternal Inheritance
Authors: Finsterer Josef
Published: December 2014

Heredoataxias are a group of genetic disorders with a cerebellar syndrome as the leading clinical manifestation. The current classification distinguishes heredoataxias according to the trait of inheritance into autosomal dominant, autosomal recessive, X-linked, and maternally inherited heredoataxias. The autosomal dominant heredoataxias are separated into spinocerebellar ataxias (SCA1-8, 10-15, 17-23, 25-30, and dentato-rubro-pallido-luysian atrophy), episodic ataxias (EA1-7), and autosomal dominant mitochondrial heredoataxias (Leigh syndrome, MIRAS, ADOAD, and AD-CPEO). The autosomal recessive ataxias are separated into Friedreich ataxia, ataxia due to vitamin E deficiency, ataxia due to Abeta-lipoproteinemia, Refsum disease, late-onset Tay-Sachs disease, cerebrotendineous xanthomatosis, spinocerebellar ataxia with axonal neuropathy, ataxia telangiectasia, ataxia telangiectasia-like disorder, ataxia with oculomotor apraxia 1 and 2, spastic ataxia of Charlevoix-Saguenay, Cayman ataxia, Marinesco-Sjögren syndrome, and autosomal recessive mitochondrial ataxias (AR-CPEO, SANDO, SCAE, AHS, IOSCA, MEMSA, LBSL CoQ-deficiency, PDC-deficiency). Only two of the heredoataxias, fragile X/tremor/ataxia syndrome, and XLSA/A are transmitted via an X-linked trait. Maternally inherited heredoataxias are due to point mutations in genes encoding for tRNAs, rRNAs, respiratory chain subunits or single large scale deletions/duplications of the mitochondrial DNA and include MELAS, MERRF, KSS, PS, MILS, NARP, and non-syndromic mitochondrial disorders. Treatment of heredoataxias is symptomatic and supportive and may have a beneficial effect in single patients.

**Please see page 424 for abbreviation list.
Seizure Attacks While Driving: Quality of Life in Persons with Epilepsy
Authors: Tiamkao Somsak , Sawanyawisuth Kittisak , Towanabut Somchai , Visudhipun Pongsak ,   Thai QOL Epilepsy Investigators
Published: December 2014

Objective:

To study the effect on quality of life (QOL) of a seizure attack while driving in persons with epilepsy (PWE).

Methods:

From four provincial and eight university hospitals in Thailand, we enrolled epileptic patients who drove a car or motorcycle or used to drive. The SF-36 questionnaire was used to evaluate QOL. The mean SF-36 score for all dimensions was calculated and compared with patients who either had or did not have a seizure attack while driving and in those who either had or had not been involved in a traffic accident while driving.

Results:

We had 245 adult PWE who drove a car or motorcycle or used to drive. Of these, 69 cases (28%) had a seizure attack whilst driving. Over half (36/69; 57%) had had seizure-related accidents, most of which were mild but about 20% needed hospitalization. PWE having a seizure attack while driving had a significantly lower QOL in four of the eight categories compared with patients who had not. PWE who had a seizure-related accident had a significantly lower mean value in the vitality category than those who did not.

Conclusions:

Seizure attacks while driving diminished QOL in PWE even though they only suffered minor injuries. Driving as a QOL issue should be discussed with patients. A good public transportation system would ease the need to drive.
Safety and Yield of Early Cessation of AEDs in Video-EEG Telemetry and Outcomes
Authors: Moien-Afshari Farzad , Griebel Robert , Sadanand Venkat , Vrbancic Mirna , Hernandez-Ronquillo Lizbeth , Lowry Noel , Téllez Zenteno José F.
Published: December 2014

Background:

Video-electroencephalography (VEEG) telemetry is the simultaneous recording of ictal and interictal EEG pattern and paroxysmal behavior to investigate the nature of paroxysmal events.

Methods:

This is a prospective study performed to asses the safety and yield of early discontinuation of antiepileptic drugs (AEDs) in the telemetry unit. Over a 2.5-year period, 50 patients that met the indications for VEEG monitoring were admitted by an epileptologist to neuro-observation units with continuous monitoring, nursing coverage and EEG technicians support during working hours and on-call thereafter. In most cases AEDs (except Phenobarbital) were discontinued in 24h. We prospectively assessed the yield and safety of the telemetry investigation as well as epilepsy surgery outcomes.

Results:

Our monitoring answered the study question in 88% of the patients. The question was not answered in 12% of cases due to the lack of recorded events. Our results changed the management in 74% of cases and potentially improved quality of life by decreasing the AEDs consumption and number of seizures per month. Over all, 22% received epilepsy surgery and became either seizure free or their seizures became non-disabling. Our method significantly decreased the duration of hospital admission for monitoring and minimal complications occurred only in 8% of patients.

Conclusions:

In conclusion, our method for short VEEG monitoring has a high yield for diagnosis, minimal complications and is cost effective. These qualities, together with good surgery results validate our method for the investigation and treatment of refractory seizure cases.
Underlying Venous Pathology Causing Perimesencephalic Subarachnoid Hemorrhage
Authors: Lee Jaejoon , Koh Eun-Mi , Chung Chin-Sang , Hong Seung-Chul , Kim Yong-Bum , Chung Pil-Wook , Suh Bum-Chun , Moon Heui-Soo
Published: December 2014

Background:

Perimesencephalic subarachnoid hemorrhage (PSH) is a relatively benign clinical entity with a low risk of recurrent bleeding. The precise etiology of PSH has not yet been determined. We report here three cases of PSH with clinical and radiological features that support a venous system as a cause.

Case Presentation:

The first patient, a 72-year-old woman, had PSH and venous hemorrhagic infarct in the left thalamus on non-contrast CT. Subsequent cerebral angiography revealed widespread thrombosis in the cerebral venous system, a potential cause for reflux overflow hemorrhage. The second patient, a 55-year-old man with an established diagnosis of neuro-Behçet's disease, a well-known cause for cerebral venulitis, presented with PSH one year later. The third patient, a 39-year-old female, with incomplete Behçet's disease was admitted with PSH.

Discussion:

Current concepts on the anatomic origin and the possible pathophysiologic mechanism leading to PSH are discussed. The underlying pathological conditions in the venous system in our cases provide theoretical clues to the anatomic origin of PSH in general.