bluuebunny/crossref_metadata_2025_split huggingface dataset:
Dissociated Loss of Vibration, Joint Position and Discriminatory Tactile Senses in Disease of Spinal Cord and Brain
Authors: Ross R.T. 
Published: July 2016

ABSTRACT:The clinical functions of the posterior columns of the spinal cord and the signs of disease of these structures have been debated for years. Todd in 1847 and Schiff in 1858 knew the functions of the posterior columns and 10 years later Brown-Sequard knew as well. Reynolds, Romberg, and Duchenne, each described a posterior column syndrome based on a disease in which the primary lesion was not in the posterior columns. In the last 150 years almost every white matter structure of the cord has been credited with serving the sensations that we now know are a function of the posterior columns. Vibration, joint position and movement as well as discriminatory touch each seem to be served by separate fibres of the posterior columns and medial lemniscus. There is evidence of this in cat and man. These sensations may be lost individually, totally, or in certain stereotyped combinations. Vibration or joint sense is commonly lost alone. When a discriminatory touch sensation is lost with one other sense, it is almost inevitably joint position sense. Absent discriminatory touch and vibration sense with normal joint position sense appears to be unknown. This functional separation continues into the thalamus. At the highest level there is no evidence that vibration sense has any conscious somatosensory cortical affiliation, while joint position and discriminatory touch senses definitely do.
Entrapment of an Accessory Superficial Peroneal Sensory Nerve
Authors: Rubin Michael , Menche David , Pitman Mark 
Published: July 2016

ABSTRACT:A 29 year old man had an accessory branch of the superficial peroneal nerve which entered the foot by rostro-caudally traversing the lateral malleolus laterally. The nerve was entrapped by a fascial band, resulting in pain over the lateral malleolus and dorsum of foot. Symptoms resolved when the nerve was surgically released.
Inter-Observer Agreement in Assessing Comatose Children
Authors: Seshia Shashi S. , Yager Jerome Y. , Johnston Bruce , Haese Philip 
Published: July 2016

ABSTRACT:Inter-observer agreement was evaluated for twelve items used in the neurological assessment of comatose children. Data were obtained prospectively on fifteen patients examined independently by two observers in a double-blind fashion. Observer variability was measured by using the Disagreement Rate and Kappa statistic. The Disagreement Rate ranged from 0.01 to 0.12 for all items. Values for Kappa statistic were generally in accordance with those for Disagreement Rate. The data suggest fair to almost perfect inter-observer agreement for the items used to assess comatose children in this study.
Clinical Correlates and Electroencephalographic Characteristics of Two Additional Patterns Related to 14 and 6 per Second Positive Spikes
Authors: Reiher J. , Carmant L. 
Published: July 2016

ABSTRACT:Two additional patterns, minuscule 28 per second positive spikes and huge N-shape potentials, have been identified exclusively in the EEGs of patients with 14 and 6 per second positive spikes. They occur predominantly during drowsiness and light sleep, usually in children, seldom in young adults. Their presence adds little to the clinical relevance of positive spikes. Familiarization with the N-shape potentials — the commoner of the two patterns — is important, lest they are mistaken for interictal abnormalities of significance such as atypical spike-wave complexes.
Clival Chordoma Presenting with Acute Brain Stem Hemorrhage
Authors: Levi Allan D.O. , Kucharczyk Walter , Lang Arnold P. , Schutz Hart 
Published: July 2016

ABSTRACT:The authors present a case of a 29-year-old man who developed rapidly progressive cranial nerve palsies and a right hemiparesis secondary to a pontine hemorrhage. The rare but correct diagnosis of a clival chordoma which had invaded the brain stem and subsequently hemorrhaged was based on computed tomography and magnetic resonance imaging. The diagnosis was confirmed at surgery when the patient underwent a successful operative decompression of tumor and clot from the pons via a sub-occipital craniotomy. This represents the first case of a clival chordoma to hemorrhage into the brain stem, which was diagnosed preoperatively and the patient survived.
Amyotrophic Lateral Sclerosis/Parkinsonism/Dementia: Clinico-Pathological Correlations Relevant to Guamanian ALS/PD
Authors: Hudson Arthur J. 
Published: July 2016

ABSTRACT:In a recent report on the clinical and pathological features of Guamanian ALS/PD and post-encephalitic parkinsonism/ALS a number of similarities were described, notably in the distribution of neurofibrillary tangles throughout the nervous system. In this account additional pathological features which these disorders share (and which differ from classical ALS, Parkinson's and Alzheimer's diseases) are described. These include atrophy of the globus pallidus and the entire substantia nigra, viz. pars compacta and pars reticulata. Moreover, neither Lewy bodies nor senile plaques are features of the Guamanian and post-encephalitic disorders. The significance of these observations and their relationship, more generally, to parkinsonism, ALS and dementia are discussed.
Biochemistry and Genetics of Tay-Sachs Disease
Authors: Gravel Roy A. , Triggs-Raine Barbara L. , Mahuran Don J. 
Published: July 2016

ABSTRACT:Tay-Sachs disease is one of the few neurodegenerative diseases of known cause. It results from mutations of theHEXAgene encoding the a subunit of β-hexosaminidase, producing a destructive ganglioside accumulation in lysosomes, principally in neurons. With the determination of the protein sequence of the α and β subunits, deduced from cDNA sequences, the complex pathway of subcellular and lysosomal processing of the enzyme has been determined. More recently, detailed knowledge of the gene structure has allowed the determination of specific mutations causing Tay-Sachs disease. The high incidence of the disease in Ashkenazi Jews is attributed predominantly to three mutations present in high frequency, while in non-Jews some two dozen mutations have been identified thus far. The cataloguing of mutations has important implications for carrier screening and prenatal diagnosis for Tay-Sachs disease.
β-Amyloid Protein in Alzheimer's Disease
Authors: Nalbantoglu Josephine 
Published: July 2016

ABSTRACT:β-amyloid protein, a 42-43 amino acid polypeptide, accumulates abnormally in senile plaques and the cerebral vasculature in Alzheimer's disease. This polypeptide is derived from a membrane-associated precursor which has several isoforms expressed in many tissues. The precursor protein is processed constitutively within the P-amyloid domain, leading to the release of the large β-terminal portion into the extracellular medium, β-amyloid protein may be toxic to certain neuronal cell types and its early deposition may be an important event in the pathogenesis of Alzheimer's disease.
Hereditary Canine Spinal Muscular Atrophy: An Animal Model of Motor Neuron Disease
Authors: Cork Linda C. 
Published: July 2016

ABSTRACT:Motor neuron diseases selectively produce degeneration and death of motor neurons; the pathogenesis of these disorders and the specificity for this population of neurons are unknown. Hereditary Canine Spinal Muscular Atrophy produces a lower motor neuron disease which is clinically and pathologically similar to human motor neuron disease: motor neurons dysfunction and degenerate. The canine model provides an opportunity to investigate early stages of disease when there are viable motor neurons still present and might be responsive to a variety of therapeutic interventions. The canine disease, like the human disease, is inherited as an autosomal dominant. The extensive canine pedigree of more than 200 characterized individuals permits genetic analysis using syntenic linkage techniques which may identify a marker for the canine trait and provide insights into homologous regions for study in human kindreds.
The Role of Axonal Transport in Neurodegenerative Disease Spread: A Meta-Analysis of Experimental and Clinical Poliomyelitis Compares with Amyotrophic Lateral Sclerosis
Authors: Brooks Benjamin Rix 
Published: July 2016

ABSTRACT:ALS symptom spread results from local spread of the neuronal degeneration because contiguous areas are more quickly involved than non-contiguous areas. Local spread to contiguous areas of motor neuron dysfunction is faster at the brainstem, cervical and lumbar regions than spread to non-continguous areas. The time for caudal-rostral symptomatic spread of ALS to involve a distant region is a function of the distance of that region from the site of onset. The time for spread to the bulbar region is shorter following arm onset than leg onset. Spread to non-contiguous areas is faster within the spinal cord than from the spinal cord to the bulbar region. These kinetics are consistent with axonal transport of the etiological agent in a manner similar to spread of poliovirus in poliomyelitis patients. Spread from the bulbar region to the spinal cord, on the other hand, occurs faster than symptom spread from the limb region to the bulbar region in limb onset patients. This rapid limb involvement following bulbar onset is more dramatic in males compared with females. Females with leg onset, on the other hand, show more rapid involvement of the opposite leg, either arm or bulbar structures than males. Gender effects may determine the course of ALS depending on the original site of onset.
Development of Surgical Therapy of Epilepsy at the Montreal Neurological Institute
Authors: Feindel William 
Published: July 2016

ABSTRACT:Wilder Penfield's development of surgical methods for treating focal cerebral seizures, beginning with his early work in Montreal in 1928, is reviewed. The reliance on seizure pattern and focal brain pathology was enormously enhanced by the advent of EEG and direct electrical recording from the cortex during surgery. The recognition in the early 1950's of mesial temporal structures in the pathogenesis of temporal lobe epilepsy with automatism lead to more rational and successful surgical treatment. Positron emission tomography and especially magnetic resonance imaging have recently added to the essential diagnostic information of focal epilepsy provided by EEG.
Preoperative Electroencephalographic Localization of Large Epileptogenic Zones in the Frontal and Temporal Lobes
Authors: Ajmone-Marsan Cosimo 
Published: July 2016

ABSTRACT:The concept of epileptogenic zone is defined as a large area of cortical neurons arranged in concentric circles of variable degrees of epileptogenicity. This is particularly so in frontal lobe epilepsy since the interictal and ictal epileptic abnormalities are poorly localized, often absent and at times misleading in terms of localization. In temporal lobe epilepsy, the epileptogenic zones may be more restricted.
PET Scanning in Partial Epilepsy
Authors: Engel Jerome 
Published: July 2016

ABSTRACT:Many biologically active tracers are available for positron emission tomography (PET) investigations, but most studies of epilepsy have utilized l8F-fluorodeoxyglucose (FDG) to measure local cerebral metabolic rate for glucose. Over 70% of patients with medically refractory partial seizures demonstrate an interictal zone of hypometabolism corresponding to the epileptogenic region. This metabolic defect commonly involves the temporal lobe in patients with complex partial seizures of mesial temporal origin, and is encountered less consistently with seizures of extratemporal neocortical origin. Although false localization is less likely with FDG-PET than with EEG, the hypometabolic zone merely reflects a focal functional deficit and its epileptogenicity must still be demonstrated electrophysiologically. When hemispherectomy or large multilobar resections are planned in small children, FDG-PET also provides useful supporting evidence that the contralateral hemisphere is functioning normally. It is difficult to obtain FDG-PET scans and to interpret results during spontaneous partial seizures. Ictal scans can be more easily obtained with single photon emission computed tomography (SPECT), which may provide information for planning surgical resections.
Surgery for Central, Parietal and Occipital Epilepsy
Authors: Rasmussen Theodore 
Published: July 2016

ABSTRACT:Patients with epileptogenic lesions that are more or less restricted to the central, parietal or occipital regions represent 14% of our total epilepsy surgery series and consisted of 322 patients at the end of 1980. Tumoral or major vascular lesions were present in 117 patients (37%), but this report is limited to the remaining 203 patients (63%) with non-tumoral epileptogenic lesions. The presumed etiology was birth trauma or anoxia in 25%, postnatal brain trauma in 25%, and unknown in 24%, miscellaneous lesions were present in 16%, post-inflammatory gliosis or multiple factors were present in the remaining 10%. Follow-up data of 2-51 years' duration (median 18 years) are available for 186 of these 203 patients. Sixty-three patients (34%) remain seizure-free. Forty-two patients (23%) have a marked reduction of seizures. The remaining 81 patients (44%) have a lesser reduction. The variation in clinical seizure patterns is considerably greater than in temporal lobe epilepsy. Focal sensorimotor phenomena are common at the onset, whether the seizures begin in the central region or further posterior. The success following cortical resection is equally gratifying and the failures just as distressing as in surgery for epilepsy involving other brain regions.
A Clinical Classification of Hereditary Ataxias
Authors: Barbeau A. , Sadibelouiz M. , Sadibelouiz A. , Roy M. 
Published: July 2016

AbstractWe present a working and flexible classification of inherited ataxic syndromes based on the use of simple tools available to every clinician: a good history (particularly pinpointing the age of onset, the rate of progression and the mode of inheritance) and a neurological examination (identifying the presence of ataxia, deep tendon reflexes in the knee, optic nerve, retinal and/or 8th nerve signs). This classification is easily coded for computer translation on any personal computer. The place occupied by a given disorder may, by contiguity, give a clue to its pathophysiology.
Visual evoked potentials and brain stem auditory potentials in Friedreich's ataxia — A longitudinal study
Authors: Bogacz J. , Chouza C. , Romero S. , Bogacz A. , Correa H. , Barbeau A. 
Published: July 2016

AbstractSix patients with Friedreich's ataxia, 4 males and 2 females, their ages ranging from 13 to 33 years, were studied. The early manifestations started between age 7 and 13 with an evolution time between 6 and 20 years. Serial visual and brain stem auditory evoked potential recordings were made. A progressive increase in latency, reduction in amplitude and in latency inter-ocular difference of PlOO were observed. The pattern of the reversal checker-board visual evoked potential was preserved. A disorganized BAEP pattern, a well defined potential I, a very small potential V and a delay in the interpeak latency were constant findings. The assumption is made of a progressive involvement of both visual and central auditory pathways. Pathophysiological mechanisms are discussed.
The cardiomyopathy in friedreich's ataxia: isotopic ventriculography and myocardial imaging with thallium-201
Authors: Therriault L. , Lamoureux G. , Côté M. , Plourde G. , Lemieux B. 
Published: July 2016

AbstractMyocardial scanning after the intravenous administration of Thallium 201 was used to evaluate regional myocardial perfusion in 14 patients with Friedreich's ataxia. Isotopic ventriculography was also used to assess left ventricular contractility. Myocardial images in patients with Friedreich's ataxia were found to be precociously abnormal irrespective of the degree of neurological impairment or of the severity of myocardial hypertrophy.
Hair trace elements in Friedreich's disease
Authors: Barbeau A. , Roy M. , Paris S. 
Published: July 2016

AbstractConcentrations of zinc, copper, manganese, chromium, cobalt and selenium were measured in the hair obtained from subjects with Friedreich’s disease, other inherited ataxias and neurological control patients. Althought zinc and copper concentrations were significantly higher in Friedreich than in the two control groups, the mean values for all groups were well within the normal range. No major deficiency in zinc or selenium was demonstrated in Friedreich’s disease using the approach. This does not, however, indicate that there is no defect in zinc and selenium metabolism, availability or transport in this disorder.
Zinc and taurine in Freidreich's ataxia
Authors: Shapcott D. , Giguère R. , Lemieux B. 
Published: July 2016

AbstractZinc and taurine were measured in urine in the fasting state and following a 4mg/kg load of taurine in subjects with Friedreich's Ataxia (FA), and healthy controls (C), and subjects with Duchenne type muscular dystrophy (MD). Of the FA, 25% had increased fasting excretion of zinc, and 50% had increased excretion of zinc following the taurine load. The MD subjects all had increased zinc excretion at all times. The increased zinc excretion did not correlate with increased excretion of taurine. As an index of zinc deficiency, uptake of zinc by erythrocytes was measured in all subjects and in heterozygotes for FA. The pattern of uptake was abnormal for FA and heterozygotes. Hair analysis for zinc showed that 10 of the 12 FA subjects had low values.We conclude that significant abnormalities in zinc metabolism exist in some, but not all cases of FA. The evidence available does not permit definition of the cause of these abnormalities, whether zinc deficiency or abnormal zinc transport is the primary factor.
Friedreich's ataxia: malic enzyme activity in cellular fractions of cultured skin fibroblasts
Authors: Melançon S.B. , Cloutier R. , Potier M. , Dallaire L. , Vanasse M. , Geoffroy G. , Barbeau A. 
Published: July 2016

AbstractWe have measured the activity of malic enzyme NADP+ dependent in the nuclear, mitochondrial, lysosomal and cytosolic fractions of cultured skin fibroblasts from twelve patients with Friedreich's ataxia and nine control subjects. Hexosaminidase, cytochrome-C-oxidase, lactate dehydrogenase and malic enzyme NAD+ dependent were used as marker enzymes. The activity of malic enzyme NADP+ dependent was not significantly reduced in the mitochondrial fraction of patients with Friedreich's ataxia as compared with controls. When corrected for possible contamination between mitochondrial and cytosolic fractions, malic enzyme NADP+ dependent activity was still not significantly reduced in patients with Friedreich's ataxia. Unless critical methodological differences were overlooked in this or previously published studies, we conclude that mitochondrial malic enzyme deficiency is not the primary genetic defect underlying Friedreich's ataxia.
Nuclear Magnetic Resonance Imaging of Skull Base Lesions
Authors: Potts D.G. , Zimmerman R.D. 
Published: July 2016

ABSTRACT:The basic principles of nuclear magnetic resonance (NMR) are reviewed and the appearances of some lesions of the skull base are discussed. NMR is a valuable diagnostic study for skull base lesions and it provides information that is not shown on the CT scan. The modern investigation of unusual skull base lesions should include both CT and NMR.
Neurologic Disorders Associated with Mitral Valve Prolapse
Authors: Jackson Alan C. 
Published: July 2016

ABSTRACT:Mitral valve prolapse has been reported to be associated with a variety of neurologic disorders, including cerebral ischemia, transient global amnesia, migraine, autonomic dysfunction, and psychiatric disease. The evidence supporting these associations and possible pathogenetic mechanisms are discussed. Some neurologic disorders may be direct complications of mitral valve prolapse, while others may occur as part of an underlying genetic defect or common link.
Primary Lymphoma of the Central Nervous System: Manitoba Experience and Literature Review
Authors: Hobson Douglas E. , Anderson Brian A. , Carr Ian , West Michael 
Published: July 2016

ABSTRACT:We describe eight cases of primary cerebral lymphoma seen in Manitoba from 1980 to 1985.The clinical presentation is similar to other primary brain tumors. The diagnosis should be considered when single or multiple, often deep lesions, show dense enhancement on computerized tomographic (CT) scan, but are avascular at angiography. These tumors are histologically indistinguishable from non-Hodgkins lymphomas arising outside the CNS.The prognosis is poor. However, radiotherapy with or without surgery may offer significant palliation. Although there is no consensus on the value of chemotherapy, corticosteroids alone or multiagent chemotherapy have shown promise in a few cases. For these reasons, histologic diagnosis should be sought in all cases and surgery, radiotherapy, and chemotherapy should be considered, as prolonged survival is possible.
Dorsal Midbrain Syndrome in Multiple Sclerosis with Magnetic Resonance Imaging Correlation
Authors: Costantino A. , Black S.E. , Carr T. , Nicholson R.L. , Noseworthy J.H. 
Published: July 2016

ABSTRACT:We describe the clinical characteristics and a series of magnetic resonance imaging (MRI) studies in a patient with the features of dorsal midbrain syndrome occurring in the setting of multiple sclerosis. A T2-weighted MRI study revealed a discrete abnormality in the tectum of the midbrain whereas a high volume delayed computed tomography (CT) scan was uninformative. In parallel with remission of the clinical findings, the MRI abnormality diminished over time and was no longer visible at one year suggesting that some MRI detected MS lesions can completely disappear with time. This report demonstrates the use of MRI to detect and to follow sequentially sites of known disease activity in MS.
Seizures Associated with Spontaneous Subarachnoid Hemorrhage
Authors: Sundaram M.B.M. , Chow F. 
Published: July 2016

ABSTRACT:We analysed the charts of 131 consecutive cases of spontaneous subarachnoid hemorrhage — without arteriovenous malformations — for seizures. Convulsions occurred in 31 patients (24%) and most often within 24 hours of bleeding. Motor manifestations of partial seizures were of no lateralizing value to aneurysm site. Early mortality, rebleeding and intracerebral hematoma were similar in both seizure and non-seizure groups. Late seizures were infrequent in survivors who had suffered seizures in the acute stage — thus questioning the necessity for routine, long term prophylactic anticonvulsants in these patients.
Electroencephalograms of Children with Permanent Cortical Visual Impairment
Authors: Robertson Robyn , Jan James E. , Wong Peter K.H. 
Published: July 2016

ABSTRACT:The electroencephalograms of 40 children with permanent cortical visual impairment (CVI) were studied. CVI was diagnosed in the presence of severe visual loss, normal or minimal ocular findings and clinical, electrodiagnostic and CT evidence of postgeniculate lesions involving the visual cortex. All patients had a multidisciplinary evaluation, including extensive neurological and ophthalmological investigations. The records did not contain any specific features which would be diagnostic of CVI. Isolated occipital spikes were rare and more commonly multifocal EEG disturbance was seen because of diffuse cerebral involvement. Photic stimulation was of little use in the diagnosis. Multihandicapped children with profound CVI tended to show multifocal abnormalities, no alpha rhythm and a suppressed posterior waking background in their EEGs, whereas those with more residual vision usually had an alpha rhythm. The presence or absence of alpha rhythm seemed to reflect the intactness of the striate cortex. However, the lack of alpha rhythm was not always associated with total blindness and not all children with useful residual vision had alpha rhythm.