bluuebunny/crossref_metadata_2025_split huggingface dataset:
Neuropathological Changes in Chronic Adult Hydrocephalus: Cortical Biopsies and Autopsy Findings
Authors: Del Bigio Marc R. , Cardoso Erico R. , Halliday William C. 
Published: February 2016

ABSTRACT:Background:The cortical changes resulting from chronic hydrocephalus in adults are not well defined.Methods:Retrospective analysis of twenty-one patients (age 64-88 years) with a clinical diagnosis of “normal pressure hydrocephalus” who underwent cortical biopsy at the time of intracranial pressure monitoring or shunt insertion, and eight patients who were biopsied but not shunted. Eleven brains (age 26-92 years), seven from patients who could be considered to have “normal pressure hydrocephalus”, were also examined following autopsy. Age- and sex-matched control brains with small ventricles and no history of dementia were compared to the hydrocephalic brains. Senile plaques and neurofibrillary tangles were assessed semiquantitatively and a non-parametric statistical analysis was employed.Results:Five biopsies exhibited both senile plaques and rare neurofibrillary tangles, while two had only neurofibrillary tangles. Neurofibrillary tangles were more prevalent in hydrocephalic brains than in controls. There was no difference in the prevalence of senile plaques between the two groups. Grumose bodies in the substantia nigra were identified in five autopsy brains, a prevalence higher than in control brains.Conclusions:These pathological features are not specific for hydrocephalus; however, they suggest that long-standing ventriculomegaly is associated with degenerative brain changes in sites beyond the periventricular white matter. The presence of senile plaques in cortical biopsies from hydrocephalic patients does not appear to be a contraindication to shunting; however a prospective study in patients undergoing intracranial pressure monitoring would better address the issue.
Role of the Ipsilateral Motor Cortex in Voluntary Movement
Authors: Chen Robert , Cohen Leonardo G. , Hallett Mark 
Published: February 2016

ABSTRACT:The ipsilateral primary motor cortex (M1) plays a role in voluntary movement. In our studies, we used repetitive transcranial magnetic stimulation (rTMS) to study the effects of transient disruption of the ipsilateral M1 on the performance of finger sequences in right-handed normal subjects. Stimulation of the M1 ipsilateral to the movement induced timing errors in both simple and complex sequences performed with either hand, but with complex sequences, the effects were more pronounced with the left-sided stimulation. Recent studies in both animals and humans have confirmed the traditional view that ipsilateral projections from M1 to the upper limb are mainly directed to truncal and proximal muscles, with little evidence for direct connections to distal muscles. The ipsilateral motor pathway appears to be an important mechanism for functional recovery after focal brain injury during infancy, but its role in functional recovery for older children and adults has not yet been clearly demonstrated. There is increasing evidence from studies using different methodologies such as rTMS, functional imaging and movement-related cortical potentials, that M1 is involved in ipsilateral hand movements, with greater involvement in more complex tasks and the left hemisphere playing a greater role than the right.
Hormones, Radiosurgery and Virtual Reality: New Aspects of Meningioma Management
Authors: Black Peter McLaren 
Published: February 2016

ABSTRACT:The understanding and management of meningiomas is changing significantly today. One of the most striking features of their pathophysiology is their predominance in women. In a series of 517 patients with meningiomas seen by the Brain Tumor Group at Brigham and Women's Hospital, the female:male ratio was 2.4:1. The progesterone receptor appears to be the major candidate to explain this difference. Although meningioma cells variably express receptors for estrogen, androgen, platelet-derived growth factor, epidermal growth factor, and somatostatin, these molecules do not explain the differences because they are not differentially expressed or are not activated. Progesterone receptor can be shown to be expressed in 81% of women and 40% of men with meningiomas; it can also be shown to be activated by transfecting a construct with the progesterone responsive element and a reporter in it and using the cell's own receptors to activate this construct. Surgery remains the mainstay of meningioma management. At the Brigham and Women's Hospital three-dimensional reconstruction techniques have markedly improved the ability to visualize the tumor as well as its relation to vascular structures. With MRI reconstruction, it is possible to know the tumor's relation to the sagittal and other sinuses, to identify feeders and proximity to major arteries, and to establish its location and relation to cortex by frameless stereotaxis. These techniques can be used in a virtual reality format are some of the most powerful in neurosurgery both for teaching and for the surgical procedure itself. External beam radiation has been shown by others to be an effective adjunctive treatment to prevent meningioma recurrence. Recently, linear accelerator radiosurgery and stereotactic radiotherapy have changed the pattern of radiation at our institution. In a series of 56 skull base meningiomas, for example, 95% were controlled (i.e., showed no growth) over a four year period. Fractionated focal radiation potentially offers the same control rate with fewer complications. With increasing understanding and treatment possibilities, meningiomas remain one of the most intriguing and challenging tumors in the nervous system.
Familial Intracranial Aneurysms: Recurrence Risk and Accidental Aggregation Study
Authors: Mathieu Jean , Hébert Gilles , Pérusse Louis , Prévost Claude , Cantin Léo , Bouchard Jean-Marie , DeBraekeleer Marc 
Published: February 2016

ABSTRACT:Background:The Saguenay-Lac-Saint-Jean (SLSJ) region is a geographically isolated area (population 285,955) located in the Northeastern part of the Province of Quebec, Canada. Using a population-based register, the genealogical reconstruction of 502 individuals with ruptured intracranial aneurysm (RIA) showed a familial aggregation (the presence of aneurysm in two or more first- to third-degree relatives) for 144 (28.7%) of them; this proportion is much higher than reported elsewhere.Objective:In order to assess the genetic predisposition to RIA in the SLSJ population, the objective of the present study is to compare familial and non-familial cases and to provide an estimate of the recurrence risk ratio for siblings.Results:The age at the time of rupture, the number of intracranial aneurysms for each patient and the location of RIAs were not statistically different in the familial versus the non-familial group. Of the 3449 siblings, 20 (0.58%) had suffered a RIA. The recurrence risk ratio calculated for siblings (defined as the risk of disease among siblings divided by the estimated population prevalence) is 1.6 (CI 95% 1.0 - 2.4). In other respects, we observed very large kinships in the SLSJ population, with an average number of siblings of 7.2 (SD ± 3.4), ranging from 0 to 17 individuals. With such large families and on the basis of chance alone, we expected 31.3% of the patients to have at least one first- to third-degree relative with RIA.Conclusion:These data show that siblings of patients with RIA in the SLSJ population have a greater risk of RIA than the general population. Nevertheless, the largest part of the familial occurrence observed in the SLSJ region can be explained by accidental aggregation, due to large kinships. We propose that, in this population, an underlying genetic predisposition must be suspected only when three or more cases of RIA are identified among first- to third-degree relatives.
Epilepsy and Driving: A Survey of Canadian Neurologists
Authors: McLachlan Richard S. , Jones Michael W. 
Published: February 2016

ABSTRACT:Background:A seizure is the most common cause of loss of driving privileges for medical reasons but there is variability in how physicians and the authorities who regulate driving approach this issue.Methods:A questionnaire regarding epilepsy and driving was sent to all adult neurologists in Canada (n = 494).Results:Of 289 (59%) neurologists responding, 50% usually report patients with seizures to the department of motor vehicles compared to only 4% for stroke/TIA, 26% for dementia and 8% for other neurologic disorders (p &lt; 0.0001). In the five provinces with mandatory reporting laws, seizures were reported most of the time by 84% compared to only 19% in the five provinces with discretionary reporting (p &lt; 0.0001). Nationwide, 44% agreed with mandatory reporting but this also differed in provinces with and without mandatory reporting legislation (63% vs. 37%, p &lt; 0.0001). Only 49% agreed with the current recommendation of at least one year seizure free interval before resuming driving.Conclusions:Seizures are disproportionately reported compared to other neurological conditions. Many neurologists disagree with the recommended Canadian standards for duration of driving restriction after seizures. Variability in the attitude and practice of neurologists in regard to reporting of seizures is confirmed.
Episodic Cluster Headache in a Community: Clinical Features and Treatment
Authors: Riess C.M. , Becker W.J. , Robertson M. 
Published: February 2016

ABSTRACT:Objective:To study the clinical features and treatment given to episodic cluster headache patients in the Calgary region.Patients:Fifty-one (51) patients who responded to a media campaign, had previously been diagnosed by their family physicians, and who met International Headache Society (IHS) criteria for episodic cluster headache, formed the population for this study.Methods:The media campaign consisted of newspaper advertisements and radio publicity including physician interviews and talk shows. Patients were required to complete a 200-item questionnaire detailing clinical features and treatment of their cluster headache syndrome. Each patient was also interviewed by our research nurse for clarification and proper completion of questionnaire.Results:Fifty-one percent (51%) of our patients had short headache attacks lasting one hour or less. Almost one-half (45%) had three or four attacks per 24 hour period. Eighty-six percent (86%) had been referred to a neurologist. Sixty-nine percent (69%) had never used oxygen, but of those who had, onehalf were still using it. Sumatriptan by injection had been tried by 26% of patients and of these, 93% considered it effective. Subcutaneous dihydroergotamine had been tried by 8%. For prophylaxis, 41% had tried methysergide, 31% prednisone, and 4% verapamil. Many patients had been prescribed migraine prophylactic drugs which are ineffective for cluster headache, and some had also undergone dental procedures or nasal and sinus surgeries.Conclusion:Many cluster headache patients had not, to their knowledge, been prescribed or used the best symptomatic and prophylactic treatments for cluster headache. This should be addressed through educational programs and through making up-to-date information on the treatment of cluster headache readily available to physicians and patients.
Ras Activation in Astrocytomas and Neurofibromas
Authors: Guha Abhijit 
Published: February 2016

ABSTRACT:Oncogenic mutations resulting in activated Ras Guanosine Triphosphate (GTP) are prevalent in 30% of all human cancers, but not primary nervous system tumors. Several growth factors/receptors are implicated in the pathogenesis of malignant astrocytomas including epidermal growth factor (EGFR) and platelet derived growth factor (PDGF-R) receptors, plus the highly potent and specific angiogenic vascular endothelial growth factor (VEGF). A significant proportion of these tumors also express a truncated EGFR, which is constitutively activated. Our work demonstrates that the mitogenic signals from both the normal PDGF-R and EGFR and the truncated EGFR activate Ras. Inhibition of Ras by genetic or pharmacological strategies leads to decreased astrocytoma tumorgenic growth in vitro and decreased expression of VEGF. This suggests that these agents may be potentially important as novel anti-proliferative and anti-angiogenic therapies for human malignant astrocytomas.In contrast to astrocytomas, where increased levels of activated Ras GTP results from transmitted signals from activated growth factor receptors, the loss of neurofibromin is postulated to lead to functional up-regulation of the Ras pathway in neurofibromatosis-1(NF-1). We have demonstrated that NF-1 neurofibromas and neurogenic sarcomas, compared to non-NF-1 Schwannomas, have markedly elevated levels of activated Ras GTP. Increased Ras GTP was associated with increased tumor vascularity in the NF-1 neurogenic sarcomas, perhaps related to increased VEGF secretion. The role of Ras inhibitors as potential therapy in this tumor is also under study.
Mild Non-lesional Temporal Lobe Epilepsy: A Common, Unrecognized Disorder with Onset in Adulthood
Authors: Aguglia Umberto , Gambardella Antonio , Plane Emilio Le , Messina Demetrio , Oliveri Rosario L. , Russo Concetta , Zappia Mario , Quattrone Aldo 
Published: February 2016

ABSTRACT:Objective:To compare mild vs. severe non-lesional temporal lobe epilepsy (TLE).Methods:Data from 104 consecutive patients with non-lesional TLE were reviewed. Seventy-three of the 104 fulfilled the criteria for inclusion in this study of a follow-up period longer than three years at our Institute. Patients were considered to have a mild TLE if they were seizure free for at least three years after appropriate antiepileptic medication, or had rare (≤ 2/year) complex partial or secondarily generalized seizures for at least three years with or without appropriate antiepileptic therapy. Clinical, EEG and MRI data of mild vs. severe non-lesional TLE patients were compared on the basis of a cross-sectional study design.Results:Of the 73 patients with non-lesional TLE included in the study, 43 (59%) had mild TLE, and 30 (41%) had severe TLE. Duration of epilepsy was significantly shorter (mean 15.2 ± 10.5 years vs. 26.4 ± 13.2 years) and age at onset was significantly higher (mean 34.3 ± 15.3 years vs. 7.8 ± 6.8 years) in mild than in severe TLE group. Patients with mild TLE had also a significantly higher prevalence of positive family history of epilepsy (37.2% vs. 10%), and a significantly lower occurrence rate of febrile convulsions (FC) (4.7% vs. 33.3%), mesial temporal sclerosis (MTS) (6.9% vs. 36.7%), and intelligence deficiency (0% vs. 20%). In mild TLE there was also a significantly high rate (58.1% vs. 0%) of delayed diagnosis (from 1 to 28 years), because of misdiagnosis (39.5%) or no medical counseling (18.6%).Conclusions:Mild non-lesional TLE is a common, unrecognized disorder mainly characterized by both onset in adulthood and high prevalence of familial history of epilepsy. The present findings suggest that mild non-lesional TLE may represent a clinical entity different from severe non-lesional TLE.
Safety and Tolerability of Subcutaneous Cladribine Therapy in Progressive Multiple Sclerosis
Authors: Selby R. , Brandwein J. , O'Connor P. 
Published: February 2016

ABSTRACT:Objective:To evaluate the safety and tolerability of subcutaneous (s.c.) cladribine therapy in patients with chronic progressive multiple sclerosis (CPMS), and to evaluate the effects on lymphocyte subsets.Background:Cladribine, a synthetic antineoplastic agent with immunosuppressive effects, may favourably affect the course of CPMS. However results of a previous reported clinical trial showed significant myelosuppression in some patients.Design/Methods:19 patients with severe (mean extended disability status score [EDSS] = 6.7) CPMS were treated on a compassionate basis with cladribine 0.07 mg/kg/ day s.c. for 5 days per cycle, repeated every 4 weeks for a total of 6 cycles. Patients underwent clinical evaluation, EDSS, and hematologic analysis before, during, and following therapy.Results:The treatment was very well tolerated with no clinically significant side effects observed. Between baseline and the end of cycle 6, mean decreases were noted in absolute lymphocyte count from 1697 to 463 (p = 0.000012), CD4 count from 865 to 187 (p = 0.0000008), CD8 from 418 to 165 (p = 0.005) and CD19 from 197 to 26 (p = 0.000002). Platelet, granulocyte and RBC counts were unaffected. Approximately one year after completion of therapy, some recovery of CD4 and CD8 counts had occurred although both counts remained suppressed compared to baseline (302 and 227 respectively); the CD19 count had recovered essentially to normal by one year. EDSS scores post-therapy revealed some deterioration in 8 patients and stable scores in the remaining 11. Global patient evaluations of the treatment were mixed.Conclusions:Cladribine therapy, at lower doses than previously reported, was remarkably well tolerated in CPMS, with no significant myelosuppression. Profound effects occurred in total lymphocyte count and CD4, CD8 and CD19 subsets.
Segmental Hyperhidrosis as a Manifestation of Spinal and Paraspinal Disease
Authors: Schulz Valerie , Ward Donna , Moulin Dwight E. 
Published: February 2016

ABSTRACT:Background:Segmental hyperhidrosis is an uncommon finding which is usually associated with irritation or infiltration of pre-ganglionic sympathetic fibres or the sympathetic chain.Methods:We report two cases of segmental hyperhidrosis with striking clinical features.Results:In one case, a mesothelioma produced ipsilateral simultaneous underactivity and overactivity of sympathetic outflow and in the other case a thoracic central disc herniation was probably responsible for a band of sweating which clearly extended beyond the segmental level of injury.Conclusion:Segmental hyperhidrosis should trigger a search for structural disease in the spinal and paraspinal region.
A Measure of Peripheral Nerve Stimulation Efficacy Applicable to H-Reflex Studies
Authors: Boorman G.I. , Hoffer J.A. , Kallesoe K. , Viberg D. , Mah C. 
Published: February 2016

AbstractBackground: When H-reflexes are recorded during movement in human subjects, the stimulator current output is not a good indicator of sensory stimulation efficacy because of unavoidable nerve movement relative to the stimulus electrodes. Therefore, the M-wave amplitude has been used by researchers as an indicator of the efficacy of the stimulus. In this study we have examined the general validity of the hypothesis that the M-wave amplitude is directly proportional to the group I sensory afferent volley evoked by the stimulus. Methods: A nerve recording cuff, stimulating electrodes, and EMG recording electrodes were implanted in cats. Nerve cuff recordings of centrally propagating volleys evoked by electrical stimuli were directly compared to M-waves produced by the same stimuli. Compound action potentials (CAPs) recorded in the sciatic nerve were compared with soleus M-waves during either tibial nerve or soleus muscle nerve stimulation. CAPs in the ulnar nerve were correlated with flexor carpi ulnaris M-waves during ulnar nerve stimulation. Results and Conclusions: Our findings indicate that for mixed nerve stimulation (e.g., tibial or ulnar nerve) the M-wave can be a reliable indicator of the centrally propagating sensory volley. Due to the high correlation between CAP and M-wave amplitude in these nerves, a small number of M-waves can give a good estimate of the size of the group I sensory volley. On the other hand, when nerves with only partially overlaping fibre diameter populations are stimulated (e.g., the soleus muscle nerve), the M-wave is not well correlated with the group I sensory volley and thus may not be used as a measure of the size of the input volley for H-reflex studies.
Measurement of Health-Related Quality of Life in Multiple Sclerosis Patients
Authors: Brunei Donald G. , Hopman Wilma M. , Singer Michael A. , Edgar Catherine M. , MacKenzie Thomas A. 
Published: February 2016

ABSTRACT:Background: Patient outcomes in multiple sclerosis (MS) have generally been measured by their neurological impairment using specific scales such as the Kurtzke Expanded Disability Status Scale (EDSS). However, this scale does not measure the multiple dimensions of health-related quality of life (HRQOL) such as functional status and general well-being, which are also important outcomes along with disease-specific measurements. Methods: HRQOL was measured in a group of 97 MS patients using the RAND 36-item Health Survey 1.0. The EDSS score was assigned by the clinic neurologist. Additional data were collected from the clinical record for each patient. Results: MS patients scored poorly in a number of HRQOL domains such as physical and role functioning and energy or vitality. Disability as quantified by the EDSS correlated only with the physical functioning domain. Regression models were developed to measure the relationship between patient characteristics (independent variables) and HRQOL domains (dependent variables). Discussion: A number of patient characteristics were associated with higher or lower scores on the HRQOL domains. Of particular interest is the finding that a family history of MS was associated with poorer physical and social functioning as well as more pain and less vitality. The occurrence of seizures had a negative impact on role functioning, social functioning and general health perceptions. HRQOL gives caregivers a broader measure of disease burden than the EDSS alone, and should be useful in planning and monitoring interventions.
Familial Parkinson’s Disease: A Clinical Genetic Analysis
Authors: Bonifati Vincenzo , Fabrizio Edito , Vanacore Nicola , Mari Michele De , Meco Giuseppe 
Published: February 2016

AbstractObjectiveTo study the frequency, clinical features and clinical genetics of familial Parkinson’s disease (PD).MethodsFamily history for PD and tremors was studied in 100 consecutive PD cases. Spouses served as controls. Clinical features were compared between personally verified familial and sporadic PD cases, from the same consecutive clinical series. Clinical genetic analysis was performed in a larger group of non-consecutive multicase PD families.ResultsFamily history for PD was positive in 24% of consecutive PD cases and in 6% of spouse controls (p < 0.001). When family history for isolated tremor is also considered, the number of positive cases rises to 43% compared with 9% in controls (p < 0.001). Nine of the consecutive cases had at least one living affected relative, for a total of 20 familial PD cases. These familial cases showed an earlier onset age when compared with sporadic ones from the same consecutive series. Within 22 non-consecutive PD families with at least two living and personally examined PD cases (total 52 PD cases), the crude segregation ratios were similar for parents and siblings and the lifetime cumulative risks approached 0.4 in siblings and tended to be comparable, but at later ages, in parents. Ancestral relatives were all unilaterally distributed. In some families, anticipation of onset age in new generations was observed.ConclusionsThe frequency of positive family history for PD and for PD and tremor is higher among PD cases than controls. Familial and sporadic PD only differ in onset age. The clinical genetic analyses support autosomal dominant inheritance with strongly age-related penetrance as most likely in familial PD.