bluuebunny/crossref_metadata_2025 huggingface dataset:
Vasculitic Basilar Artery Thrombosis in ChronicCandida AlbicansMeningitis
Authors: Grimes D.A. , Lach B. , Bourque P.R.
Published: February 2016

Background:

Cerebrovascular complications of meningitis have been most extensively documented in the setting of acute bacterial or chronic tuberculous meningitis. Involvement of major cerebral vessels is rare and basilar artery thrombosis has not been reported in fungal meningitis secondary to Candida infection.

Methods:

We describe the clinical course and neuropathological findings in a woman with chronic meningitis due to Candida albicans.

Results:

The diagnosis remained elusive antemortem despite analysis of 7 large volume CSF samples and examination of a meningeal and cortical biopsy. Death followed extensive brainstem and temporo-occipital infarction secondary to basilar artery thrombosis. The basilar artery occlusion was secondary to an intense, granulomatous and necrotizing basal meningitis focally extending to the media and intima.

Conclusions:

This paroxysmal and devastating complication of untreated chronic candida meningitis reinforces that a trial of empirical therapy with both antituberculous and antifungal agents should be considered in most cases of chronic culture-negative lymphocytic meningitis.
Migraine-like Symptoms Triggered by Occipital Lobe Seizures: Response to Sumatriptan
Authors: Ogunyemi Abayomi , Adams Dermot
Published: February 2016

Background:

The relationship of ictal and post-ictal cephalic pain to migraine remains debatable. We hereby report 2 patients whose post-ictal migraine-like symptoms ameliorated in response to oral sumatriptan.

Methods:

Two patients, a 27-year-old woman and 41-year-old man were referred for assessment of migraine of 10 years and 4 years duration, respectively. The man described a recent episode of “vacant stare” and the woman has sensitivity to bright colours and light. Neurological examination, visual field testing, CAT scan, MRI scan, and prolonged EEG recordings were accomplished for each patient.

Results:

The man had a spontaneous seizure with ictal discharge arising from the right occipital lobe. Bilateral occipital spikes, associated with palinopsia was triggered in the woman by photic stimulation.
Both patients developed post-ictal headaches with symptoms similar to their migraines. The symptoms subsided following treatment with oral sumatriptan.

Conclusion:

Migraine-like symptoms occurring following occipital lobe seizures may share similar pathophysiological substrates with idiopathic migraine. Serotonergic mechanisms may be implicated.
Hydrocephalus: Obliterated Perimesencephalic Cisterns and the Danger of Sudden Death
Authors: Garvey Marjorie A. , Laureno Robert
Published: February 2016

Objective:

We report a possible risk factor which could identify patients with chronic hydrocephalus who are at risk for sudden death.

Methods:

A retrospective review of medical records and computed tomographic (CT) scans was conducted on three patients with chronic hydrocephalus who suffered acute cardiorespiratory arrest without those signs which are normally associated with a progressive worsening of hydrocephalus.

Results:

All three of these patients were awake and communicative shortly before the life threatening or terminal event. All had experienced some recent worsening of neurologic signs or symptoms, but none had shown a progressive impairment of consciousness or major neurologic decline ordinarily associated with life threatening elevation of intracranial pressure. Absence of the perimesencephalic cisterns on head CT scans done prior to or just after the life threatening event was the only new radiologic finding common to all these patients.

Conclusions:

The absence of the perimesencephalic cisterns in an awake and alert patient with severe hydrocephalus indicates that the patient may be at risk for neurogenic cardiorespiratory failure. In such cases, (especially when there has been a recent, albeit mild, change in neurologic signs or symptoms), the neurologist should urge emergency ventriculostomy or shunting for the hydrocephalus.
Long-term Glioblastoma Multiforme Survivors: a Population-based Study
Authors: Scott J.N. , Rewcastle N.B. , Brasher P.M.A. , Fulton D. , Hagen N.A. , MacKinnon J.A. , Sutherland G. , Cairncross J.G. , Forsyth P.
Published: February 2016

Background:

Long-term glioblastoma multiforme survivors (LTGBMS) are uncommon. The frequency which these occur in an unselected population and factors which produce these unusually long survivors are unknown.

Objectives:

To determine in a population- based study 1) the frequency of LTGBMS in a population and 2) identify which patient, treatment or tumor characteristics would predict which glioblastoma (GBM) patient would become a LTGBMS.

Methods:

The Alberta Cancer Registry was used to identify all patients diagnosed with GBM in southern Alberta between 1/1/75 - 12/31/91. Patient charts were reviewed and histology re-examined by a blinded neuropathologist. LTGBMS were defined as GBM patients surviving ≥ 3 years after diagnosis. Each LTGBMS was compared to three age-, gender-, and year of diagnosis-matched controls to compare patient, treatment, and tumor factors to GBM patients without long-term survival.

Results:

There were 279 GBMs diagnosed in the study period. Five (1.8%) survived ≥ three years (range, 3.2-15.8 years). Seven additional long-term survivors, who carried a diagnosis of GBM, were excluded after neuropathologic review; the most common revised diagnosis was malignant oligodendroglioma. LTGBMS (avg. age = 45 years) were significantly younger when compared to all GBM patients (avg. age = 59 years, p - 0.0001) diagnosed in the study period. LTGBMS had a higher KPS at diagnosis (p = 0.001) compared to controls. Tumors from LTGBMS tended to have fewer mitoses and a lower Ki-67 cellular proliferative index compared to controls. Radiation-induced dementia was common and disabling in LTGBMS.

Conclusions:

These data highlight the dismal prognosis for GBM patients who have both a short median survival and very small chance (1.8%) of long-term survival. The LTGBMS were younger, had a higher performance status, and their tumors tended to proliferate less rapidly than control GBM patients. When long-term survival does occur it is often accompanied by severe treatment-induced dementia.
Unusual Aneurysms of the Distal Internal Carotid Artery
Authors: Redekop Gary J. , Woodhurst Barrie
Published: February 2016

Background:

Supraclinoid carotid aneurysms have traditionally been classified according to their relation to the major carotid branches, but considerable variation exists with respect to site of origin, projection, and relationship to the skull base. Distal internal carotid aneurysms with a superior or medial projection are uncommon vascular lesions, with an unusually high incidence of operative complications.

Methods:

Surgical experience with five patients suffering from subarachnoid hemorrhage due to ruptured aneurysms of the dorsomedial wall of the distal internal carotid artery is presented, with emphasis on their angiographic appearance, anatomical features, and operative management.

Results:

All five patients underwent surgical clipping. Intra-operative rupture occurred in two cases, with avulsion of the aneurysm from the internal carotid artery in both. A third patient experienced recurrent subarachnoid hemorrhage three days after uneventful surgery, due to the clip shearing off of the parent vessel.

Conclusions:

Distal internal carotid aneurysms do not conform to the usual principles of aneurysm formation and are unique in their dorsomedial location unrelated to an arterial bifurcation. Although their anatomy is straightforward and exposure is not restricted by bone or dural structures of the skull base, they possess extremely fragile necks which make surgical management particularly hazardous.
Canadian Collaborative Project on Genetic Susceptibility to MS, Phase 2: Rationale and Method
Authors: Sadovnick A.D. , Risch N.J. , Ebers G.C. ,   the Canadian Collaborative Study Group
Published: February 2016

Background:

Results from the Canadian Collaborative Project on Genetic Susceptibility to Multiple Sclerosis (MS)-Phase 1 (CCPGSMS-Phase 1) together with other family data published since 1982 have led to the following conclusions about the etiology of MS: (i) genetic and non-genetic (environmental) factors are involved in the etiology of MS on a population basis; (ii) the familial aggregation of MS is genetic; (iii) maternal factors do not influence the risk for siblings to develop MS; and (iv) MS appears to be oligogenic. The present paper describes the rationale and methodology for the CCPGSMS-Phase 2.

Methods:

The CCPGSMS-Phase 2 is a nation-wide collaborative effort involving all the 15 Canadian MS clinics. A series of structured questionnaires is administered to MS index cases, spouse controls and mothers of index cases and spouse controls (if available) by trained interviewers. Blood samples are taken for molecular genetic studies. This national effort is coordinated by the MS Clinics in Vancouver and London.

Results:

The CCPGSMS-Phase 2 is in progress so specific results are not available. The study is designed to (i) increase the database for genetic epidemiological/molecular genetic research and (ii) gather population-based data to further our understanding of the non-genetic factors in the etiology of MS.

Conclusions:

It is anticipated that the results from this study will impact on the eventual prevention, cure and treatment of MS.
Hospital-based Psychiatric Service Utilization and Morbidity in Multiple Sclerosis
Authors: Fisk John D. , Morehouse Susan A. , Brown Murray G. , Skedgel Chris , Jock Murray T.
Published: February 2016

Background:

Despite the common association of psychiatric morbidity and multiple sclerosis (MS), population-based prevalence estimates of these disorders are limited. Such estimates are of particular importance to those conducting trials of interventions for the treatment of MS. This study examined the prevalence of bipolar disorder, depression, and attempted suicide among hospital service utilizers in Nova Scotia and compared these measures for the MS and non-MS population.

Methods:

Data regarding diagnosis and utilization were extracted from two linked databases which included all hospital separation records for Nova Scotia over a 3 year period (1992/93-1994/95).

Results:

The prevalence of bipolar disorder in hospitalized MS patients was 1.97% and depression was 4.27%. These rates were significantly higher than the 0.92% and 2.04%, respectively, for the non-MS hospital utilizers. These diagnoses also accounted for more than half of the primary diagnostic codes for psychiatric service separations by MS patients. The proportion of total hospital utilization which was accounted for by psychiatric services did not differ between MS and non-MS utilizers. While suicide attempts were rare, the estimated frequency of suicide attempts in the total MS population was more than three times that of the general population.

Conclusions:

Bipolar disorder and depression were twice as prevalent in hospitalized MS patients as in the general population of hospital utilizers while the estimated frequency of suicide attempts was at least three times greater. These results illustrate that psychiatric morbidity and service utilization are important considerations in the care of MS patients.
Monitoring Severe Head Injury: a Comparison of EEG and Somatosensory Evoked Potentials
Authors: Moulton Richard J. , Brown Jennifer I.M. , Konasiewicz Stefan J.
Published: February 2016

We report on our experience with long-term monitoring of the EEG power spectrum and somatosensory evoked potentials (SSEPs) in 103 patients with severe closed head injury (Glasgow Coma Scale - GCS ≤ 8). Patients were monitored for an average of 5 days post injury and monitoring was terminated when they died, regained consciousness or their intracranial physiologic parameters (primarily intracranial pressure - ICP) were stable for 2-3 days. Patients were treated according to a standard protocol that included mechanical ventilation, sedation, and neuromuscular blockade. At 7 of 9 twelve hour time intervals post injury, SSEPs were significantly (p < .05) different between outcome groups using the Glasgow Outcome Score collapsed to 3 categories. The percent slow (delta) activity in the EEG was not significantly different between outcome groups at any time point, post injury. The total power in the EEG power spectrum differed only at the last time epoch post injury (108 hr.). Based on the superior prognostic capabilities of the SSEP, we routinely base critical management decisions on SSEP values. We have not been able to rely on EEG parameters for these same decisions due to the lack of clear distinction between good and poor prognosis groups based on common EEG parameters.
Metabolic and Inflammatory Cerebral Diseases: Electrophysiological Aspects
Authors: Young G.B.
Published: February 2016

The electroencephalogram (EEG) is an important diagnostic tool in coma. Although comatose patients may be similar on neurological examination, the EEG reveals a wide range of abnormalities. For metabolic and septic encephalopathies, the “anesthesia model” is a useful analogy. The EEG is very sensitive to the depth or severity of brain dysfunction in coma as well as the direction of the process if serial tracings or continuous recordings are used. While the EEG is rarely specific for the etiology of coma, it may help determine the class or general category of disease process. In conditions capable of causing neuronal death, e.g., anoxia-ischemia, the EEG can be of prognostic value.
Quantitation in EMG
Authors: Chan K. Ming , Brown William F.
Published: February 2016

The neuromuscular system may be affected by disorders of the central nervous system as well as other disorders affecting motoneurons, axons, neuromuscular transmission, the sarcolemmal membrane, the contractile elements and other components of the muscle fibers themselves. One or a combination of these possibilities can present in patients in the critical care unit. This paper reviews various qualitative and quantitative methods for assessing the various components of the peripheral contributions to the electrical and force output as well as the central motor drive to motoneurons. These methods all have their own strengths and weaknesses but many are complementary and together, can provide important diagnostic and prognostic information to guide management.
Electrophysiological Studies in the Critical Care Unit: Investigating Polyneuropathies
Authors: Chen Robert
Published: February 2016

Polyneuropathies frequently contribute to ventilator dependency and prolonged stay in the intensive care unit. As clinical examination is often limited in critically ill patients, electrophysiological studies are invaluable in establishing the diagnosis of neuropathy, determining its pathophysiology, severity and in following the patients' progression. Guillain-Barré syndrome (GBS) developing before intensive care unit admission and critical illness polyneuropathy (CIP) developing as a complication of sepsis and multiorgan failure are the commonest causes of neuropathy. Electrophysiological findings in CIP are that of an axonal neuropathy whereas the findings in GBS are usually consistent with a demyelinating neuropathy. Axonal GBS can be distinguished from CIP by the preceding illnesses, slow nerve conduction velocity in some cases, lack of spontaneous activity on the initial needle electromyographic study and cerebrospinal fluid findings.
Monotherapy versus Polytherapy in Epilepsy: a Framework for Patient Management
Authors: Schneiderman J.H.
Published: February 2016

The long-standing debate between proponents of monotherapy and those of polytherapy for treatment of epilepsy has been rekindled by the recent development of several new antiepileptic drugs. The likelihood of improved seizure control on polytherapy must be weighed against the risk of increased side effects, complex drug interactions and cost. Providing maximal seizure control while avoiding overtreatment is a challenge which requires an ongoing critical evaluation of each patient's management. This review provides a framework for decision-making by considering issues affecting the choice between monotherapy and polytherapy in five clinical situations: 1) newly diagnosed epilepsy; 2) seizures on monotherapy; 3) seizures controlled on polytherapy; 4) not controlled on polytherapy; 5) change in medical condition.
The Canadian Epilepsy Database and Registry
Authors: McLachlan R.S.
Published: February 2016

Epilepsy encompasses medical, psychological, social and demographic factors which are best studied in large populations. The Canadian Epilepsy Database and Registry (CEDAR) is a comprehensive English language, multicentre epilepsy database which has been developed to study the impact of these and other factors on epilepsy in Canada. In addition, it can be used locally in the clinic for office record keeping, automated printout of a referral letter or graphing seizures and other data over time. The data in CEDAR are similar to what is found in the patient's chart. There are 20 participating centres across Canada which have entered data on over 8000 adult and pediatric epilepsy patients. The information in CEDAR will be available for research purposes to centres entering data as well as to academic researchers, the pharmaceutical industry and government agencies.
Postirradiated and Nonirradiated Gliosarcoma: Immunophenotypical Profile
Authors: Ang Lee C. , Perry James R. , Bilbao Juan M. , Ozane Wayne , Peschke Eva , Young Beverley , Nelson Nahid
Published: February 2016

Background: Thirty-one gliosarcomas (25 nonirradiated and 6 postirradiated tumors) were selected based on the presence of two distinctive areas: a malignant gliomatous and a sarcomatous component. In all cases, the sarcomatous component appears like fibrosarcoma or malignant fibrous histiocytoma. Two tumors showed additional areas consisting of osteochondroid differentiation. Methods: All tumors were examined using antibodies against Ulex europaeus agglutinin I (UEA), glial fibrillary acidic protein (GFAP), vimentin (VM), epithelial membrane antigen (EMA), desmin, collagen IV, alpha-1-antitrypsin (a-l-AT) and smooth muscle actin (SMA). Results: While the VM highlighted the sarcomatous areas of all tumors there were only scattered spindle cells that were GFAP-positive in the same areas. The a-l-AT was diffusely reactive in the sarcomatous areas in 20 cases. Focal immunoreactivity was seen with SMA (20 tumors), UEA (8 tumors), EMA (5 tumors), collagen IV (5 tumors) and desmin (4 tumors) in the nonvascular sarcomatous cells. Conclusions: The range of immunophenotypical expression is likely to be a reflection of the capacity of a multipotential progenitor to undergo divergent differentiation. There is very little morphological difference between the postirradiated and nonirradiated tumors except that a higher proportion of postirradiated tumors are immunoreactive to SMA and desmin.
The Occurrence of Multiple Sclerosis in the Hutterites of North America
Authors: Hader Walter J. , Peter Seland T. , Hader Mary B. , Harris Colleen J. , Dietrich Dennis W.
Published: February 2016

Objective: To report the occurrence, clinical characteristics and genealogical analysis of multiple sclerosis in the Hutterites of North-Western United States and Western Canada.Background: The incidence of multiple sclerosis is reported to be lower or rare in certain ethnic groups and genetic isolates and was previously observed to be absent in the Hutterite population.Methods: After long-term surveillance, six patients were identified and clinical examinations and laboratory investigations including VER and MRI were completed.Results: The six cases included two brothers, two first cousins, male and female, another male and female, all representing two of the three endogamous groups of Hutterites, are linked to two common ancestors through lines of descent dating to 1723. The individual pedigrees were analyzed from extensive genealogical records covering eight generations.Conclusion: The incidence of multiple sclerosis in Hutterites is low in a high risk area of North America. A specific mode of inheritance pattern has not been established and a common founder effect may play a role in the development of multiple sclerosis. The genetic contribution of the Hutterites seems greater than previously recognized.
Abscess Within a Brain Metastasis
Authors: Pui Ng Wai , Lozano Andres
Published: February 2016

Background: Neoplastic metastases to the cerebellum are a frequent complication in patients with carcinoma. However, the co-existence of an abscess within a CNS metastasis is a rare event. Methods: We report the case of a 79-year-old woman with two cerebellar abscesses within metastatic lesions in the cerebellum. She presented with a rapidly progressing syndrome characterized by elevated intracranial pressure and cerebellar findings. Results: At surgery, a purulent exudate within discrete metastatic tumours was identified. Pathological and microbiological examinations confirmed the coexistence of an abscess within a metastatic carcinoma. Significance and Conclusion: The radiological diagnosis of intracranial abscesses and metastases can be non-specific and brain imaging may not reliably identify both processes when they coexist. Furthermore, brain metastases often contain liquefied material which can be mistaken for tissue necrosis rather than an infectious process. It is important to be aware that a brain metastasis can also be infected. Therefore, acquisition of lesionai tissue for both pathological and microbiological examinations is essential for accurate diagnosis and to direct optimal therapy in situations where the intracranial lesion could be either an abscess or a metastatic deposit.
Excitation and Inhibition in Epilepsy
Authors: Engel Jerome
Published: February 2016

The term epileptic seizures is used to describe a variety of paroxysmal signs and symptoms resulting from a large number of underlying pathological processes. Generalized tonic-clonic convulsions, therefore, reflect entirely different basic neuronal mechanisms than those of typical absences. Animal research suggests that the former result from disturbances that involve disinhibilion, whereas the abnormality giving rise to the latter requires intact, if not enhanced, inhibition in order to sustain hypersynchronous neuronal discharges. Investigations in human mesial temporal lobe epilepsy and chronic experimental animal models indicate that partial seizures can also reflect multiple underlying mechanisms, with some disinhibitory in type, while others appear to be hypersynchronous and associated with enhanced inhibition. Just as more than one epileptogenic disturbance can result in transition to ictus, it is likely that more than one mechanism can be engaged to terminate epileptic seizures, that these diverse processes could result in different postictal manifestations, and that they could conceivably contribute differently to the development of interictal behavioral disturbances. An appreciation for the fact that epilepsy is not merely an increase in excitation and/or a decrease in inhibition, but a variety of complicated neuronal interactions in different patients, or even at different times in the same patient or same seizure, is essential to the development of effective treatments to control epileptic seizures and prevent their consequences.
Epidemiological Study of Ruptured Intracranial Aneurysms in the Saguenay-Lac-Saint-Jean region (Quebec, Canada)
Authors: Mathieu Jean , Pérusse Louis , Allard Pierre , Prévost Claude , Cantin Léo , Bouchard Jean-Marie , DeBraekeleer Marc
Published: February 2016

Background: Using a population-based register of the Saguenay-Lac-Saint-Jean region (Quebec, Canada), the genealogical reconstruction of 533 individuals with intracranial aneurysm (IA) showed a familial aggregation (the presence of aneurysm in two or more first- to third-degree relatives) for 159 (29.8%) of them; this proportion is much higher than reported elsewhere. Objective: As part of an ongoing project to assess a genetic predisposition to intracranial aneurysms in the Saguenay-Lac-Saint-Jean population, the objective of the present study was to determine whether age-specific rates of ruptured cerebral aneurysms were higher than in other populations. Design: A retrospective study of cases of proven ruptured I As which were hospitalized during the 1973 to 1992 period was conducted. Age-adjusted rates were computed and compared to those reported in the Helsinki population. Results: We identified 412 cases of ruptured aneurysms. The age-adjusted incidence rate was 7.2/100,000/year (6.2 for men, 8.1 for women), which is similar to the incidence rates reported in other studies. Although the mean age at time of rupture was younger (46.6 years ± 13.8) than usually reported, no increase in age-specific incidence rates was detected. Conclusions: The results of this epidemiological study neither support nor reject the hypothesis of a genetic predisposition to intracranial aneurysms in the Saguenay-Lac-Saint-Jean population.
A Novel Approach to the Determination and Characterization of HIV Dementia
Authors: Brouwers P. , Mohr E. , Hildebrand K. , Hendricks M. , Claus J.J. , Baron I.S. , Young M. , Pierce P.
Published: February 2016

Background: Neuropsychological studies of the pattern and extent of cognitive impairment in HIV-infected patients have mostly used deviations from control values and/or cut-off scores as criteria for classification of dementia. There is, however, no agreement as to how to define impairment, and classification is imprecise. Method: The current study used a dementia classification matrix, developed with a step-wise linear discriminant analysis of neuropsychological data from patients with primary neurodegenerative dementias, to classify symptomatic HIV patients as demented or non-demented, and further to differentiate cortical and subcortical dementia patterns. Thirty-two male and 2 female patients (mean age 39 ± 2) with symptomatic HIV disease (mean absolute CD4 count 195 ±41) participated in the study. Results: Thirty-five per cent of patients were classified as demented. Of these, 83% showed a subcortical pattern and 17% a cortical profile of deficits. Significant differences between patients classified as subcortically demented and those categorized as normal on neuropsychological measures associated with subcortical integrity further validated the classification. Measures of psychiatric status between subgroups were similar. Conclusion: Since certain treatments may delay or reverse cognitive deficits, the use of an objective classification method based on discriminant analysis may help to identify patients who may benefit from therapy.
A Normal Bias Toward a Pictorially Defined Top in Line Bisection
Authors: Toth Cory , Kirk Andrew
Published: February 2016

Background: We set out to determine whether separable visual and representational components underlie normal subjects’ upward and distal biases in bisecting vertical and radial lines under visual guidance. Methods: Thirty-four normal subjects were asked to bisect lines oriented horizontally, vertically, and radially. Human silhouette figures were placed at either end of each line. These figures were presented upright or upside down in order to pictorially define a “top” to each line independent of the actual top of the visual field. Results: Although subjects erred toward the top of the visual field, they also demonstrated a significant bias toward the heads of the figures for lines in all spatial orientations. Conclusions: This result supports the existence of two biases: one toward the upper visual field, and another toward an internally represented “top” as suggested pictorially. These findings provide further support for the hypothesis that normal subjects’ upward and distal biases on bisection of vertical and radial lines under visual guidance have both representational and visual-based components.
Post-traumatic Frontal Lobe Epilepsy with Structural Changes: Excellent Results After Cortical Resection
Authors: Cukiert Arthur , Olivier André , Andermann Frederick
Published: February 2016

Background: The observation that resection of structural lesions of the frontal lobe provides an effective treatment of intractable epilepsy has been made by surgeons specializing in this field for over sixty years! However, the excellent results achieved when such resections are coupled with the use of modern diagnostic and operative technologies are not fully appreciated by the modern clinician. Methods: We review the results of resection of large post traumatic frontal lesions in six patients with intractable frontal seizures. No invasive EEG recording was required pre-operatively. Results: The imaging characteristics of the underlying lesions, usually due to depressed skull fractures are illustrated. Surgical treatment rendered five patients seizure free and the sixth had over 85% improvement in seizure frequency. Conclusions: Modern imaging permits ready visualization of the post traumatic lesions with cause intractable seizures. It is important to recognize that resection of these post traumatic scars usually leads to excellent seizure control.
Pre-radiation Chemotherapy for Malignant Glioma in Adults
Authors: Kirby Sarah , Macdonald David , Fisher Barbara , Gaspar Laurie , Cairncross Gregory
Published: February 2016

Purpose: To review our experience with pre-radiation chemotherapy for malignant glioma. Methods: Consecutive adults with newly diagnosed glioblastoma, anaplastic astrocytoma, anaplastic oligodendroglioma and anaplastic mixed glioma with a Karnofsky Performance Score of 60 or greater were treated with one cycle of procarbazine, lomustine and vincristine or lomustine alone, prior to radiation. Computed tomographic scans were obtained soon after surgery, eight weeks later, after radiation, and at regular intervals thereafter. The effects of chemotherapy and subsequent radiation and durations of tumor control and survival were assessed in this single arm, single center, prospective trial. Results: Thirty-seven patients started chemotherapy, 36 were rescanned eight weeks after diagnosis. Five patients (16%) responded to the first cycle of chemotherapy, three had glioblastoma and two anaplastic oligodendroglioma. Seven (19%) progressed during the first cycle, 6 had glioblastoma; with the addition of radiation one progressive case responded, three stabilized, and three continued to progress. Median times to progression and median durations of survival were 26 weeks and 60 weeks for the entire group, 24 weeks and 44 weeks for glioblastoma, and greater than 104 weeks for anaplastic astrocytoma. Conclusions: Most patients with glioblastoma do not respond to one cycle of nitrosourea-based chemotherapy given prior to radiation, but patients with anaplastic oligodendroglioma sometimes do. Patients with anaplastic astrocytoma may not respond to one cycle of chemotherapy, but often respond to subsequent radiation. Judging by survival results, radiation can be delayed eight weeks without appearing to compromise patient outcome. Implications: Pre-radiation chemotherapy with newer agents can be evaluated more fully in the future knowing that brief delays in radiation are unlikely to yield substantially inferior results.
Add-on Gabapentin for Refractory Seizures in Patients With Brain Tumours
Authors: Perry James R. , Sawka Carol
Published: February 2016

Background: Seizures associated with intracranial neoplasms are occasionally refractory to conventional anti-epileptic drugs. Gabapentin (GBP) is one of several novel anti-epileptic drugs effective as an add-on therapy for intractable seizures but has not been studied in patients with cerebral tumours. Patients and Methods: We used GBP in a open-label add-on fashion to treat 14 patients with intractable seizures associated with intracranial tumours including four glioblastomas, four metastases, three recurrent glioblastomas, and one each of anaplastic and low grade astrocytoma and meningioma. GBP was added if optimization of pre-existing therapy failed and was titrated until seizures were controlled. Results: One patient experienced adverse drowsiness. Follow-up ranged from 3–24 weeks during which time 7 patients died from disease progression. Concurrent therapy included dexamethasone in eight, cranial irradiation in four, and radiosurgery in one. Responder rate (number with at least 50% fewer seizures) was. 100% and persisted throughout follow-up. Complete resolution of seizures occurred in 8/14 patients. Conclusions: GBP was well tolerated in patients with brain tumours. Seizure frequency was reduced in all patients and efficacy persisted over time; however, the mechanism of this improvement is unclear. Concurrent therapy, regression of frequency to the mean, and the lack of controls may account for apparent benefit. In addition, because GBP may interact with a leucine-related neuronal binding site we also speculate that this novel mechanism of action may have been enhanced in our patients due to the abnormal blood-brain barrier associated with cerebral tumours. Further investigation and a controlled trial are warranted.
Intracranial Chondroma. Report of Two Cases and Review of the Literature
Authors: hacerte Daniel , Gagné Francois , Copty Michel
Published: February 2016

Introduction: Chondromas are rare intracranial tumors. The authors present two cases of intracranial intradural chondroma, one originating from the falx cerebri and the other from the dura mater of the convexity. Method and Results: Diagnostic procedures, including magnetic resonance imaging, and surgical findings are described. In both cases, pre-operative diagnosis could have been at least suspected, and the tumor was completely removed, without recurrence after a follow-up of many years. The pathogenesis and pathological findings are discussed, and cases from the literature are reviewed. Conclusion: Benign intradural chondroma has a good prognosis, with no recurrence after surgical excision in most cases.
Reciprocal Inhibition in Hemiplegia: Correlation with Clinical Features and Recovery
Authors: Okuma Yasuyuki , Lee Robert G.
Published: February 2016

Background:

Previous reports have described changes in reciprocal la inhibition in hémiplégie patients, but correlations between the amount of la inhibition and the clinical deficits have not been well established.

Methods:

We studied reciprocal inhibition between ankle flexors (tibialis anterior) and extensors (soleus) in 16 hemiplegic patients at various stages following a stroke and in 26 control subjects. The amount of disynaptic la inhibition was determined from the short latency suppression of the soleus or tibialis anterior H-reflexes by conditioning stimulation of the antagonistic muscle nerves.

Results:

Disynaptic la inhibition from peroneal nerve afférents to soleus motoneurones was increased in patients who showed good recovery of function with mild spasticity. However, it was not changed, or even sometimes diminished, in patients who made a poor recovery and had more marked extensor spasticity. In patients where serial recordings were obtained there was an increase in la inhibition during the recovery period following stroke. la inhibition to the tibialis anterior motoneurones tended to be greater in the poor recovery patients with marked spasticity than in the good recovery patients. The late (Dl) inhibition, presumably due to presynaptic inhibition, was decreased in the patients, although consistent correlations between the amount of this inhibition and the clinical features were not clearly demonstrated.

Conclusions:

Changes in excitability of la inhibitory pathways can be correlated with some of the clinical features seen in hemiplegia. Increased la inhibition of soleus motoneurones during recovery may be a mechanism to compensate for loss of descending motor commands.
Symptomatic Dystonias Associated with Structural Brain Lesions: Report of 16 Cases
Authors: Kostià Vladimir S. , Stojanovié-Svetel Marina , Kacar Aleksandra
Published: February 2016

Background:

Symptomatic (secondary) dystonias associated isolated lesions in the brain provide insight into etiopathogenesis of the idiopathic form of dystonia and are a basis for establishing the possible correlation between the anatomy of a lesion and the type of dystonia according to muscles affected.

Methods:

In 358 patients with differently distributed dystonias, a group of 16 patients (4.5%) was encountered in whom dystonia was associated with focal brain lesions.

Results:

Of the 16 patients, 3 patients had generalized, 3 segmental and 4 hemidystonia, while the remaining 6 patients had focal dystonia. The most frequent etiologies were infarction in 7, and tumor in 4 patients. These lesions were usually found in the lenticular and caudate nucleus, thalamus, and in the case of blepharospasm in the upper brainstem.

Conclusions:

Our results support the suggestion that dystonia is caused by a dysfunction of the basal ganglia.
Historical Neurology and Neurosurgery A History of Neurology in Toronto 1892–1960: Part II
Authors: Wherrett John R.
Published: February 2016

In Part 1 of this history of neurology in Toronto, the advent of neurology as a specialty in 1892 and its progress over the next 20 years was described (Can J Neurol Sci 1995; 22: 322–332). Donald Campbell Meyers, the first neurologist received his training from “founders” in Paris, Vienna and London and returned to Toronto to establish his own private neurological hospital and a special unit – “the Nervous Wards” – in the Toronto General, the major teaching hospital. These pioneering initiatives clashed with an alienist establishment, itself internally in conflict, and the “Nervous Wards” were lost in the ensuing competition for medical resources.
Historical Neurology and Neurosurgery On the Names of Babiński
Authors: Gasecki Andrew P. , Hachinski Vladimir
Published: February 2016

The 100th anniversary of the discovery of the extensor plantar response will be celebrated in 1996. It was Joseph François Félix Babinski who became known worldwide for the sign that bears his name. In order to help Joseph in establishing his career, brother Henri gave up his aspirations and abandoned engineering. Clovis Vincent, ‘father’ of French neurosurgery and pupil of Joseph, stated: “Joseph Babinski lived for science, and Henri lived for his brother; without Henri Babinski, Joseph would not have accomplished that much”. However, Henri’s name became famous in all Paris for a cookbook Gastronomie Pratique written under the pseudonym of ‘Ali-Bab.’ Throughout Joseph’s career his surname remained distorted despite his own efforts to spell and pronounce it correctly. Several people can claim the name Babinski, but in neurology and neurosurgery there is only one, Joseph.
Tibial Mononeuropathy from a Lower Limb Synovial Cyst
Authors: Sun J.C.L. , Wallace C. , Zochodne D.W.
Published: February 2016

Background

Tibial mononeuropathy due to compression by a synovial cyst is uncommon with fewer than 10 cases reported in the literature.

Method

Case Study.

Results

Tibial motor mononeuropathy with focal conduction block between the popliteal fossa and ankle was identified by electrophysiological testing. Magnetic resonance imaging and surgical exploration identified a synovial cyst compressing the nerve just distal to the popliteal fossa.

Conclusions

The combination of electrophysiological localization and imaging in cases of unexplained tibial mononeuropathy may identify an unexpected structural cause.
Supratentorial Ectopic Ependymoma
Authors: Vernet Olivier , Farmer Jean-Pierre , Meagher-Villemure Kathleen , Montes José L.
Published: February 2016

Background

Ependymomas usually arise from the ventricular surface.

Methods

We report an 11-year-old female who presented with a supratentorial ectopic ependymoma.

Results

The patient presented with a two-month-history of progressive headache, nausea and vomiting. Examination revealed papilledema, horizontal nystagmus, diplopia on upward gaze, and right pronator drift. CT scan showed an enhancing left precentral subcortical lesion measuring 3 cm in diameter with associated edema and mass effect. Its medial border was located 3 cm from the ependymal surface of the ventricle. A firm tumour was dissected from the centrum semiovale white matter, and removed in toto as confirmed on MRI. Pathological examination revealed histological, immuno-histochemical and electron microscopic features consistent with an ependymoma. Spine MRI and bone marrow aspirate, as well as lumbar puncture of cytology failed to show any dissemination.

Conclusion

From the literature review, this represents an exceptional ependymoma located at the distance from the ventricular system or cisterns. Different pathogenic alternatives are discussed.
Sulindac in Established Experimental Diabetes: a Follow-up Study
Authors: van der Sloot Paul , Mizisin Andrew , Zochodne Douglas
Published: February 2016

Background

In two previous studies we have demonstrated prevention of electrophysiological abnormalities of nerve in experimental STZ (streptozotocin)-induced diabetes (ED) of rats using nonsteroidal anti-inflammatory agents: indomethacin and sulindac. Sulindac might benefit ED because it inhibits both cyclo-oxygenase and aldose reductase.

Methods

In this work, we examined whether 1 month of sulindac treatment reversed or improved established biochemical and electrophysiological abnormalities in experimental diabetes of 3 months duration. Sulindac-treated diabetic rats (6.0 mg/kg 5/7 days weekly by gavage) were compared to untreated diabetics, nondiabetic controls and sulindac treated control rats.

Results

Diabetic rats developed slowing of conduction velocity in caudal sensory, sural sensory, caudal motor and sciatic tibial motor fibers. Sulindac improved caudal motor and, to a lesser extent sural sensory conduction but not caudal sensory or sciatic tibial motor conduction. Sulindac did not alter sciatic sugars or polyols.

Conclusions

Sulindac provided modest improvement in some indices of experimental neuropathy in this reversal study, but there was less efficacy than in the preventative study. Reversal paradigms should be examined in all experimental therapies for diabetic neuropathy.
Environmental Exposures in Elderly Canadians With Parkinson’s Disease
Authors: Chaturvedi S. , Østbye T. , Stoessl A.J. , Merskey H. , Hachinski V.
Published: February 2016

Background

Etiologic hypotheses for Parkinson’s disease have implicated environmental factors, genetic factors, or a combination of the two.

Methods

Data from a survey of elderly Canadians (n = 10,263) with regard to their history of Parkinson’s disease and previous environmental exposures were analyzed. Exposure to various environmental factors was compared between 87 patients with Parkinson’s disease and 2070 elderly controls without Parkinson’s disease.

Results

Exposure to plastic resins (OR (odd ratio) = 8.79), epoxy resins (OR = 6.94), glues (OR = 4.26), paints (OR = 3.84), and petroleum (OR = 2.30) products was significantly (p < 0.05) associated with Parkinson’s disease.

Conclusion

These substances deserve further exploration with respect to the possible development of parkinsonism.
Long Term Treatment of Intractable Reflex Sympathetic Dystrophy With Intrathecal Morphine
Authors: Becker W.J. , Ablett D.P. , Harris C.J. , Dold O.N.
Published: February 2016

Background:

Some patients with reflex sympathetic dystrophy (RD) develop intractable symptoms unresponsive to conventional therapy. Recently, intrathecal morphine therapy has been used with some success in such patients.

Methods:

The clinical course of two patients with intractable reflex sympathetic dystrophy (RSD) is described. Both patients developed intractable leg pain, swelling and autonomic changes after a leg injury. Numerous medical treatments and surgical sympathectomies failed to provide long term relief.

Results:

Relatively satisfactory symptom control was achieved only with the use of long term intrathecal morphine therapy delivered by subcutaneously implanted infusion pumps. Exacerbations of the RSD continued to occur, at times in association with further leg trauma, but these could be controlled by a temporary escalation of the intrathecal morphine dose. Complications of morphine therapy were relatively minor. A red rash appearing over the pump site was the first sign that a drug catheter break had occurred, necessitating surgical catheter revision.

Conclusion:

Long term intrathecal morphine therapy is a useful treatment option for patients with intractable severe RSD who have failed other therapies and remain markedly disabled.
Vertebral Ligament Amyloid Deposits in Spinal Stenosis
Authors: Gagné François
Published: February 2016

Objective:

We have searched for amyloid substance in 41 consecutive samples of tissue taken during laminectomies performed to relieve spinal canal stenoses. We also evaluated the relation between the occurrence of deposits and the age of patients.

Methods:

Tissue sections were stained with Congo red; positive areas had to show green dichroism under polarized light.

Results:

Small amyloid deposits were found in fibrous and fibro-elastic (ligamentum flavum) tissues in 14 subjects (34%). After age 60, the frequency was close to 50%. It reached 58% in those aged 70 or over. Our 3 patients aged 80 or more all had deposits. There was also a relation between age and importance of the infiltration. Both sexes appeared evenly involved. Systemic amyloidosis (multiple myeloma) was demonstrated in a single patient. In this case infiltration was slightly more abundant and exceeded ligamentary structures, also involving vascular walls.

Conclusions:

Small amyloid deposits were frequent in our material. Their presence and their abundance were closely related to the age of patients. Rarely, a more important infiltration, exceeding ligamentary structures will perhaps suggest a systemic form of amyloidosis. A few other rare amyloid conditions (amyloidosis associated with prolonged dialysis, amyloid tumour) have been reported to induce spinal canal stenosis. The occurrence of amyloid in vertebral ligaments of non-stenosed elderly persons is not documented; it may represent a new localization of senile amyloidosis.
Intrathecal Baclofen Therapy for Adults with Spinal Spasticity: Therapeutic Efficacy and Effect on Hospital Admissions
Authors: Nance Patricia , Schryvers Orpha , Schmidt Brian , Dubo Hy , Loveridge Brenda , Fewer Derek
Published: February 2016

A prospective trial to demonstrate the efficacy of intrathecal baclofen therapy by implanted pump for adults with spasticity due to spinal cord injury or multiple sclerosis was initiated in our hospital. Of the 140 patients assessed, 7 met the following criteria for inclusion in the study: a modified Ashworth score > 3, a spasm frequency score > 2, and an inadequate response to oral antispasticity drugs, (i.e., baclofen, clonidine and cyproheptadine). All patients responded to intrathecal bolus injection of baclofen in the double blind, placebo-controlled screening phase (mean bolus dose = 42.8 μg). Programmable Medtronic pumps were implanted in 4 patients while 3 patients received nonprogrammable Infusaid pumps. Post-implantation, a marked decrease in spasticity occurred with a significant reduction of the Ashworth score (mean = 1.8, p < .005), a reduced spasm score (mean = 0.8, p < .005), and an improved leg swing in the pendulum test. These effects were maintained during a follow-up of 24 – 41 months (average infusion dose = 218.7 μg/day). The gross cost-savings due to reduced hospitalizations related to spasticity was calculated by comparing the cost for the two year period before pump implantation to the same period after treatment for 6 of the 7 patients. The cost of in-hospital implantation as well as the cost of the pumps were deducted from the gross savings. There was a net cost-saving of $153,120. Our findings agree with the reported efficacy and safety of intrathecal baclofen treatment, and illustrate the cost-effectiveness of this treatment.