bluuebunny/crossref_metadata_2025 huggingface dataset:
Recent Progress: Progress in Understanding Huntington’s Chorea
Authors: Barbeau André
Published: July 2016

The present paper analyses the new data accumulated since 1972 concerning the etiology and pathogenesis of Huntington’s chorea. Particular attention is paid to the respective roles of the dopaminergic and GABA-ergic systems.
Cellular Hypersensitivity to Basic Myelin (P2) Protein in the Guillain-Barré Syndrome
Authors: Sheremata William , Colby Susan , Karkhanis Y. , Eylar Edwin H.
Published: July 2016

Lymphocytes of 29 subjects were assayed for MIF production in response to P2 peripheral nerve protein, crude human peripheral nerve and human central nervous system Al basic myelin protein. Seven were performed in normal control subjects, 12 in Guillain-Barré patients (GB), 5 with other polyneuropathies and 5 in patients with multiple sclerosis (MS). Only GB patients with acute illness produced MIF in response to neuritogenic P2 protein and crude human nerve. Two MS patients in the acute phase of an exacerbation and one GB patient produced MIF in Response to Al protein. The results of this study demonstrate cellular hypersensitivity to a neuritogenic constituent in peripheral nervous tissue and support the concept that this may be important in the pathogenesis of GB.
Neurophysiological Changes Following Spinal Cord Lesions in Man
Authors: Ashby P. , Verrier M.
Published: July 2016

A study has been made of the neurophysiological changes that follow spinal cord lesions in man. The Achilles tendon reflex (ATR) is used to estimate transmission in the la monosynaptic pathway, and the tonic vibration reflex (TVR) to estimate transmission in the la polysynaptic pathway to motoneurons. The inhibition of the H reflex by vibration is used as an estimate of presynaptic inhibition of the la monosynaptic pathway. Immediately following a complete lesion of the spinal cord presynaptic inhibition of the la monosynaptic pathway appears to be greatly increased. This enhanced inhibition may last several months but it eventually declines and in some instances becomes less than normal. Transmission in the la polysynaptic pathway is permanently abolished by a complete spinal lesion. A hypothesis is developed from these findings to explain the evolution of some of the clinical features that follow complete spinal lesions in man. Distinct differences are observed when the spinal lesion is incomplete. Transmission in the la polysynaptic pathway may be preserved and there may be no increase in presynaptic inhibition. These differences may depend upon the integrity of certain spinal long tracts which cannot be tested clinically.
The J. C. Richardson Lecture: Prospects for Canadian Medical Neurology
Authors: Baxter D.W.
Published: July 2016

In 1975, medical neurology is a well established, viable, growing specialty in Canada. There are now almost 250 neurologists scattered across the country, congregated largely in the hospitals associated with our 16 medical schools. Neurology is a major component of the undergraduate curriculum in each of these schools, and the majority of schools are approved for postgraduate training in our specialty. The Royal College has certified an average of 18 new neurologists each year for the past five years. In our hospitals we work closely with our internist and neurosurgical colleagues and we receive the support of highly competent teams of neuropathologists, clinical electrophysiologists and neuroradiologists. We believe that we provide a generally high standard of consultation service to our medical and surgical colleagues. We accept direct responsibility for patients suffering from a host of acute neurological problems, but we accept long term responsibility for very few. We do a great deal of teaching at both the undergraduate and graduate levels. In fact, the major role which neurology plays in undergraduate curricula, and the stimulus of postgraduate education, probably have been the two most influential factors shaping the number, work patterns and distribution of Canadian neurologists. Many of us are engaged in clinical investigation, but relatively few medical neurologists in Canada make fundamental research contributions.
Meningo-Encephalomyelitis Due to the Saprophagous Nematode, Micronema Deletrix
Authors: Hoogstraten Jan , Young W. Gerard
Published: July 2016

A five-year-old boy succumbed 24 days following an unusual farm accident in which considerable manure was deposited in multiple lacerations.Death was due to an extensive meningo-encephalomyelitis caused by a nematode that is ordinarily saprophagous.
Fetal Cerebellar Tissue Associated with a Primitive Neuro-epithelial Tumor in an Ovarian Teratoma
Authors: Boor Paul J. , Schoene William C.
Published: July 2016

This is a single case report of an ovarian teratoma. It is a unique case of a primitive neuroepithelial tumor with many similarities to a medulloblastoma arising in an ovarian teratoma, and the second report of fetal cerebellum occurring in a teratoma of the ovary.
Recent Progress: Supersensitivity of Central Neurons — A Brief Review of an Emerging Concept
Authors: Yarbrough G.G. , Phillis J.W.
Published: July 2016

The concept that “denervation” or “pharmacological disuse” supersensitivity develops in central neuronal systems subsequent to sustained attenuation of normal neurohumoral mechanisms is reviewed. Particular emphasis is placed on biochemical and electrophysiological parameters of supersensitivity in dopaminergic (striatal) neuronal systems. The possible applicability of theories invoking changes in receptor sensitivity to the phenomenon of narcotic tolerance and physical dependence and to psychoactive drug therapy is discussed.
The Effects of Feedback on Focal Epileptic Discharges in Man
Authors: Upton A.R.M. , Longmire D.
Published: July 2016

The history of the control of epileptic disturbances by conditioning techniques is reviewed. The preliminary results of a three year trial of feedback techniques in 13 epileptic patients are presented.

Thirteen epileptic patients (age 2.5 → 39 mean, 15.1 years) with lateralized focal discharges in the EEG were given repeated trials of feedback, the focal discharges being used to trigger auditory and somatosensory stimuli. Dosages and serum levels of medication were unchanged throughout the experimental period. The number of epileptic spikes per 15 seconds was assessed by automatic trend analysis during 20 to 30 minute control, biofeedback and post-feedback epochs. Ongoing EEG activity was quantified by 8 channel frequency analysis over 10 second epochs. The patients made efforts to increase and decrease the number of spike discharges with and without feedback and the results of both triggered and random auditory, somatosensory, photic and combined stimulation were compared at various intervals over a period of up to three years. A marked reduction in the number of focal discharges was noted in eight (61.5%) patients during and immediately following the sessions.

Intermittent biofeedback sessions were not associated with a serial reduction in the number of focal EEG discharges. There was a reduction in the number of clinical epileptic disturbances in six patients (46%) and possible reasons for this improvement are discussed.

One patient suffered an increase in focal temporal lobe discharges during triggered and random auditory stimulation whereas there was a marked reduction in the number of discharges during minimal electrical stimulation of the contralateral arm. The need for careful assessment of each patient to determine appropriate feedback stimulation is stressed.

One aim of this research has been to assess the feasibility of using miniature units for continuous feedback of focal discharges in epileptic patients.
Secondary Epileptogenesis in Frog Forebrain: Effect of Inhibition of Protein Synthesis
Authors: Morrell F. , Tsuru N. , Hoeppner T.J. , Morgan D. , Harrison W.H.
Published: July 2016

Secondary epileptogenesis was induced in the hippocampal cortex of the paralyzed bullfrog by means of localized, unilateral, intermittent electrical stimulation (kindling). Stimuli were designed to yield a brief but definite after-discharge. In control animals a progressive increase in after-discharge duration occurred at the 1° (stimulated) site and then at the 2° site (contralateral hippocampus). Spontaneous epileptiform potentials (SEP's) occurred between stimuli, eventually independently on both sides.

Cycloheximide (50 mg/kg) caused 88-99% reduction in protein synthesis, measured by14C-leucine incorporation into brain protein. Cycloheximide-treated animals revealed no evidence of progressive prolongation of after-discharge duration when subjected to the kindling procedure (p = 0.1205xl0-7). SEP's were reduced in the cycloheximide-treated animals, and confined to 1° hemisphere (p=0.6xl0-10).

Since cycloheximide did not disturb normal electrogenesis or disrupt the after-discharges, this experiment distinguishes processes dependent upon electrical events from those requiring macromolecular synthesis. Protein synthesis seems critical to the emergence of spontaneous and autonomous epileptiform behavior of neural aggregates.
Split-Brain Rat: Transfer and Interference of Kindled Amygdala Convulsions
Authors: McIntyre Dan C.
Published: July 2016

Rats were subjected to varying degrees of commissurotomy, followed by implantation of a bipolar electrode into each amygdala. After the kindling of six convulsions at one electrode (primary site), the procedure was applied to the contralateral amygdala (secondary site). Convulsions were observed to develop more rapidly, independent of the degree or kind of transection. After 6 secondary site convulsions, the primary site was re-tested and convulsion-triggering was blocked, except in animals with transection of the rostral portion of the corpus callosum (CC).

Collectively, the data indicate: (i) amygdala kindling develops a lasting trace which operates through the midbrain or brainstem; (ii) kindling from a second site utilizes this trace; (iii) a series of 6 convulsions produces negative after-effect which manifests itself at the convulsion level via the anterior CC; (iv) the anterior CC is important in determining the laterality of the forelimb clonus; and (v) the inter-amygdala propagation of after-discharge is blocked by the combined sectioning of the anterior CC and the anterior commissure.
Kindling: Secondary Epileptogenesis, Sleep and Catecholamines
Authors: Sato Mitsumoto , Nakashima Toyoji
Published: July 2016

Seizure development and transference phenomenon were investigated in hippocampal and amygdaloid kindled cats. The behavioral and electrographic findings during the kindling procedures showed that motor seizure development in hippocampal seizures occurred with the emergence of independent discharging in the amygdala, globus pallidus and contralateral hippocampus. Furthermore, secondary site convulsions developed upon the first stimulation of these structures in the hippocampal group but only after over a month of hippocampal stimulation in the amygdaloid group. It was, therefore, concluded that role of the amygdala and globus pallidus in hippocampal seizure development was more essential than that of hippocampal stimulation in amygdaloid seizure development. The common findings between the hippocampal and amygdaloid kindled animals were the systematic progression to seizures, the all-or-nothing nature of the electrical response and the relative permanency of the seizure susceptibility. Seizure susceptibility increased during slow wave sleep and decreased during REM sleep. These latter findings were examined with preliminary data of brain bioassays of catecholamines.
Frontal Cortical Kindling in Cats
Authors: Wake Akira , Wada Juhn A.
Published: July 2016

Our observation of amygdaloid seizure development in cats indicated early afterdischarge propagation into basal cortical areas prior to the development of bifrontal sharp theta discharge. In view of the implied participation of the frontal lobe in amygdaloid kindling, both fractional lesioning and kindling of selective areas were performed. This paper summarizes our stimulation study involving premotor, prefrontal, mesial frontal and orbital cortices. Except for the orbital series which showed a rather strikingly similar pattern of seizure development to that of amygdaloid kindling, all areas showed significantly different features in terms of the speed of seizure development, afterdischarge propagation, fragility of developing seizure, final stage 5 seizure and post-ictal behavior pattern, interictal discharge morphology and propagation, and generalized seizure triggering threshold intensity. All these findings suggest that the frontal lobe participates in, but is not essential for, the amygdaloid seizure development. The results of ongoing fractional lesion series support such a conclusion.
Effects of Venesection on Cerebral Function in Chronic Lung Disease
Authors: Bornstein R. , Menon D. , York E. , Sproule B. , Zak C.
Published: July 2016

Regional cerebral blood flow measurements and neuropsychological testing were conducted before and after venesection on 6 patients with polycythemia secondary to chronic obstructive pulmonary disease. Venesection resulted in lowered viscosity and hematocrit, and an accompanying improvement in cerebral perfusion and mental function. Blood flow was significantly increased in the left cerebral hemisphere following phlebotomy, and there was significant improvement in sensory I mental function. Cerebral function would appear to be related to blood flow alterations influenced by the viscosity of the blood.
EMG Biofeedback In Patients With Motor Disorders: An Aid For Co-Ordinating Activity In Antagonistic Muscle Groups
Authors: Davis Alan E. , Lee Robert G.
Published: July 2016

A computer program was developed to analyse the relative amount of EMG activity in an agonist-antagonist pair of muscles while subjects performed voluntary flexion-extension movements at the wrist to track a visual target. The data were presented to the subjects in the form of a vector display, the angle and length of which was determined from calculation of EMG power in the two muscles.

This new approach to EMG biofeedback was evaluated in two hemiplegic patients and three patients with cerebellar incoordination. Over a training period of several weeks, all the subjects were able to modify
the pattern of EMG activity in the muscles to reduce the amount of inappropriate coactivation of flexors and extensors and to produce more sustained and regular activation of individual muscle groups.
Motor Radiculopathy — An Unusual Presentation of Multiple Sclerosis
Authors: Noseworthy J.H. , Heffernan L.P.M.
Published: July 2016

A 28 year old female presented with clinical and electrical evidence indicative of L5 motor radiculopathy. Subsequently she was found to have symptoms and signs of multiple sclerosis. When clinical assessment and investigation fail to disclose a cause then demyelinating disease should he considered in the differential diagnosis of motor radiculopath.
Organization of GABA-Containing Neurons in Some Extrapyramidal Nuclei
Authors: Fibiger H.C.
Published: July 2016

Nuclei of the extrapyramidal system contain among the highest levels of GABA and its synthesizing enzyme glutamic acid decarboxylase (GA D) in the central nervous system. In recent years the anatomical organization of GABAergic neurons in the extrapyramidal system has been the subject of considerable experimental enquiry. In this note, current knowledge concerning the origin and projections of GABAergic neurons in certain extrapyramidal nuclei is briefly reviewed.
Pharmacokinetic Aspects of Anxiolytic Drug Therapy
Authors: Greenblatt David J. , Shader Richard I.
Published: July 2016

Benzodiazepine derivatives are neuropharmacologically similar but may appear clinically distinct due to pharmacokinetic differences. The clinical effects of single doses depend mainly on rates of drug absorption and distribution, whereas drug accumulation is a major determinant of clinical action during
multiple dosage. This paper outlines potential clinical implications of benzodiazepine pharmacokinetics.
Immunohistochemical Study of Foci of Recent Cell Death in Huntington's Disease
Authors: Averback P.
Published: July 2016

Foci of recent cell death were identified in 10 consecutive cases of Huntington's disease in the caudate nucleus and putamen. Indirect immunohistochemical staining procedures were performed on these areas and controls using fluorescein conjugated anti-globulin and peroxidase-conjugated anti-globulin. Positive results were found when human antiserum to Cytomegalovirus was used. The possibility of abnormal antigen presence within the dying neurons in this disease is discussed.
Cerebellar Infarction — Review of Recent Experiences
Authors: Woodhurst W.B.
Published: July 2016

Six cases of acute cerebellar infarction seen on a neurosurgery service in a general hospital during a twenty-six month period are reviewed. The clinical presentation, course, and treatment are presented and discussed. This is an important clinical syndrome which requires a high level of clinical suspicion for detection. The diagnosis rests primarily upon the clinical signs and symptoms. The C.T. Scan may provide useful confirmatory evidence and clarifies the differential diagnosis. Surgial treatment by resection of the infarcted tissue — mass lesion is urgently required for those patients who deteriorate progressively.
Clinical and Experimental Aspects of Injection Injuries of Peripheral Nerves
Authors: Gentili Fred , Hudson Alan R. , Hunter Daniel
Published: July 2016

Injury to peripheral nerves complicating deep intramuscular injections of antibiotic and other agents is well recognized and can result in significant permanent neurological deficit. The purpose of this paper is to review the subject of nerve injection injuries, and report on a series of recent experimental studies carried out in this laboratory designed to improve our understanding of the pathophysiology of this condition and help provide a rational basis for its treatment. A wide variety of chemotherapeutic, prophylactic, and local anaesthetic agents in common use were injected into the sciatic nerve of the adult Wistar rat. Both intrafascicular and extrafascicular injections were examined. Results revealed that the site of injection was the most crucial factor in determining the degree of nerve fiber injury. Following intrafascicular injection, the degree of injury varied significantly, depending upon the specific agent injected. The most severe injuries were associated with wide-spread axonal and myelin degeneration. Pathological alterations in the nerve were evident as early as 30 minutes following injection injury. Regeneration was a constant finding in nerve damage by injection of the various agents. The mechanism of injury appeared to be a direct toxic effect of the injected compound on neural tissue, with an associated break down of the bloodnerve barrier.
Meningo-Ependymitis in Whipple's Disease
Authors: Lapointe L.R. , Lamarche J. , Salloum A. , Beaudry R.
Published: July 2016

Six years after apparent complete recovery from intestinal Whipple's disease, a 56 year old man developed insidious progressive somnolence and gait ataxia. Studies showed hydrocephalus with obstruction of the aqueduct and CSF leukocytosis and elevated protein. Arachnoid biopsy during craniotomy revealed chronic inflammatory infiltration with PAS-positive macrophages. The patient died 5 years later despite two courses of antibiotic therapy. This is the first report of histologically confirmed cerebral Whippie's disease during life.

Whipple's disease is a systemic infectious disorder. Cerebral involvement even in neurologically asymptomatic patients should be sought with periodic CSF cytologic studies and a search for hydrocephalus. The possibility of cerebral Whipple's disease should be considered in the presence of unexplained hydrocephalus and/or chronic inflammatory changes in the spinal fluid, especially in those with past or active intestinal disease.
Medical Aspects of the Preoperative Management of Aneurysms: A Review
Authors: Weir Bryce
Published: July 2016

The aim of medical management of a patient with a recently ruptured intracranial aneurysm is to preserve residual brain function and prevent systemic complications. Surgery should be performed as soon as the patient is in good neurological condition. Most fatalities result from the destructive effects of the initial hemorrhage, but delayed ischemic neurologic deficit can result from vasospasm and rebleeding. Systemic complications of the brain damaged state result in a smaller proportion of deaths.

Common medical problems are reviewed as well as their medical management. In particular, some special problems related to subarachnoid hemorrhage such as cerebral edema and herniations, rebleeding, and vasospasm are also considered. Major recent advances have been the introduction of antifibrinolytic therapy, the realization of the importance of maintaining blood volume and pressure, as well as general advances in respiratory care.
Spinal Epidural Infection
Authors: Russell N.A. , Vaughan Richard , Morley T.P.
Published: July 2016

Thirty cases of spinal epidural abscess are reviewed with particular emphasis on mode of presentation and
results of treatment. In spite of repeated exhortations in the literature for earlier diagnosis, the results remain unsatisfactory because many patients have progressed to severe or complete paralysis before the correct diagnosis is made and surgical treatment carried out.
Automatic Quantitative Analysis of the Electromyogram in Partially Denervated Distal Muscles: Comparison with
Motor Unit Counting
Authors: McComas A.J. , Sica R.E.P.
Published: July 2016

Using theANOPS-101 minicomputer, an automatic analysis of muscle action potentials (MAP) was performed on a total of 53 extensor digitorum brevis (EDB) and abductor pollicis brevis (APB) muscles in patients with other evidence of denervation. The results were compared with observations made on 27 control subjects and they were also matched against estimates of functioning motor units made by the incremental stimulating technique of McComas et al (1971). Although the incidence of abnormal MAP parameters was relatively low in both muscles, it was possible to improve the diagnostic yield by combining the results for individual muscles. The relative merits and disadvantages of motor unit counting and automatic MAP analysis are discussed.
Clinical Efficacy of Valproic Acid in Relation to Plasma Levels
Authors: Bruni J. , Wilder B.J. , Willmore L.J. , Villarreal H.J. , Thomas M. , Crawford L.E.M.
Published: July 2016

Valproic acid is a new antiepileptic drug recently introduced in the United States for the treatment of absence seizures. In this study on patients with absence and other seizure types, the majority of patients achieved optimal control within four weeks of therapy. No patient responded to valproic acid who did not show an initial clinical response by four weeks of active therapy. Optimal response was generally achieved when plasma levels were greater than 55 μg/ml. Excellent clinical response was observed in the treatment of absence and myoclonic seizures. Twenty-two patients continued in a long term study have maintained the same degree of seizure control as observed at the time of optimal control.
Accumulation and Removal of Hg203 in Different Regions of the Rat Brain
Authors: Butterworth R.F. , Gonce M. , Barbeau A.
Published: July 2016

We have studied the brain regional distribution of methyl mercury following intravenous administration of CH3203HgCl in rat. Early peak levels were obtained in cerebellum, medulla oblongata and midbrain. The efficacy of removal of 203Hg by different chelators is also region dependent. The most efficient chelator for brain mercury proved to be mesodimercaptosuccinic acid.
Virology and Histopathology of the Trigeminal Ganglia of Americans and Japanese
Authors: Warren K.G. , Wroblewska Z. , Okabe H. , Brown S.M. , Gilden D.H. , Koprowski H. , Rorke L.B. , Subak-Sharpe J. , Yonezawa T.
Published: July 2016

Herpes simplex virus in the trigeminal ganglia of humans was studied in Philadelphia, Pennsylvania, United States of A merica, and in Kyoto, Japan. The prevalence of recurrent herpes labialis and of clinically latent herpes simplex virus within trigeminal ganglia was determined in inhabitants of the two cities. In addition, a comparison was made of the prevalence of mononuclear cell infiltration in the trigeminal ganglia of Americans and Japanese. Recurrent herpes labialis was found to be significantly less common in the Japanese city than in the American city. Herpes simplex virus was rescued less commonly from the trigeminal ganglia of cadavers in Japan than in America. The difference was significant. The frequency of mononuclear cell infiltration in the trigeminal ganglia of Americans and Japanese is not significantly different. These observations, as well as previously reported serological studies, suggest that despite the ubiquitous nature of herpes simplex virus in A merica and Japan, the Japanese have less clinically overt desease caused by this virus.
Cerebral Tissue Response to Electrode Implantation
Authors: Dodson Ronald F. , Chu L.W-F , Ishihara N.
Published: July 2016

Tissue response to platinum electrodes was assessed after an eight-day implantation period. The regions of study included the cortical areas at the opercular gyri and at the sulcus parielo-occipitalis externus, as well as the sub-cortical white matter in these areas.

Perivascular and intraparenchymal hemorrhagic lesions as well as edematous changes characterized both by extensive intra and extracellular swelling were noted. Numerous phagocytic elements and degenerative structures were present at the electrode I parenchymal interface.

Tissue alterations were asymmetrical as observed both around and at the lip of the implanted electrode. The parenchymal alterations extended from 0.2mm to 3.5mm distance from the electrode path/ parenchymal interface.

Greater tissue involvement was found in the subcortical white matter as compared to the adjacent cortical gray matter.
The Neural Hypothesis of Muscular Dystrophy — A Review of Recent Experimental Evidence with particular reference to the Duchenne form
Authors: Sica R.E.P. , McComas A.J.
Published: July 2016

Recent observations are considered to provide further evidence for an abnormality involving motoneurones in DMD. The dystrophic process appears to take place in two stages of which the first occurs during early embryonic life. This stage is thought to involve faulty inductive actions of the neural tube upon mesoderm and upon itself. The neural consequences vary among individuals and are manifested as mental retardation, EEG abnormalities and losses of functioning motor units. While the first two abnormalities are non-progressive, a further loss of motor units, associated with striking reductions in the numbers of excitable muscle fibers, takes place in trunk and large limb muscles at 9-12 years. The latter process, the cause of which is uncertain, constitutes the second stage of DMD.
Regulation of Brain Pyruvate Dehydrogenase Multienzyme Complex
Authors: Ngo T.T. , Barbeau A.
Published: July 2016

A number of excellent and comprehensive reviews on various aspects of pyruvate dehydrogenase multienzyme complex have been written recently. The purpose of the present review is to summarize briefly the reaction mechanism and the regulation of this enzyme. Emphasis is put on the most recent literature not covered by previous reviews. Particular attention is also paid to the regulation of brain pyruvate dehydrogenase multienzyme complex, since a number of patients with neuromuscular diseases, such as Friedreich's ataxia, show a decreased rate of pyruvate oxidation.
Paraganglioma of the Filum Terminale
Authors: Lagacé Réal , Delage Claude , Gagné François
Published: July 2016

An unusual tumor arising in the filum terminale is described. The clinical data revealed an extensive and slowly growing lesion. The histologic picture was characterized by a proliferation of lobules and sheets of regular cells within a rich vascular network. Electron microscopic studies showed light and dark cells with sustentacular extensions. Typical neurosecretory granules were obsen'ed in both cell types, establishing the diagnosis of para-ganglioma. The glomus coccygeum could be the site of this tumor.
A Non-Permanent Tonic Pupil in Rheumatoid Arteritis
Authors: Victor David I. , Green W. Richard , Stark Walter J. , Walsh Frank B.
Published: July 2016

A 76-year-old male with a severely deforming rheumatoid arthritis, eosinophilic!, polymyositis, and episcleritis developed a transient tonic pupil. The episcleritis, and a muscle biopsy revealing an occlusive arteritis with eosinophilia, suggest that a widespread rheumatoid arteritis caused a reversible ischemic insult to the ciliary ganglion and thus created a transient denervation of the pupil.
Design of the Investigation
Authors: Barbeau A.
Published: July 2016

The general outline of the complete prospective study of 50 cases of spino-cerebellar degeneration is given. The general protocol followed, the criteria for inclusion and the mode of analysis are described. The aim of this study was to establish a base of clinical, physiological and biochemical facts upon which a logical and systematic approach to pathogenesis and treatment of Friedreich's ataxia could be attempted.
Intellectual Performance in Multi-Infarct Dementia and Alzheimer's Disease A Replication Study
Authors: Perez Francisco I. , Stump David A. , Gay Joe R.A. , Hart Vicki R.
Published: July 2016

A consistent feature in dementia is an overall intellectual deterioration. The present study investigated the intellectual performance of patients with Alzeheimer's disease (AD) and multi-infarct dementia (MID) using the Wechsler Adult Intelligence Scale (WAIS). For reliability and generality purposes two independent samples of patients were collected. Significant differences in Education Level (EDU) and Performance IQ (PIQ) were obtained for the first sample, with the AD group having a significantly higher EDU level. The MID group obtained a higher PIQ. No significant differences were found in the second sample, but EDU level approached significance with the AD group again having a higher EDU level. A discriminant function analysis classified 81% of the patients in the first sample and 100% diagnostic accuracy was obtained for the second sample using the II predictor variables. A maximum R stepwise regression was performed in order to detect the “best” model of variables discriminating between the diagnostic groups. For the first sample the “best” model was the two variable model, including EDU and Full Scale IQ, accounting for 40% of the variance. The simplest model for the second sample was the one variable model including EDU, accounting for 20% of the variance. Quantitative, differences were found between the AD samples. Time since onset of the disease was offered as a possible influence in the quantitative differences in the AD samples. Sampling biases in the behavioral study of dementia are discussed. Successful replication was obtained.
Congenital Intraspinal Extradural Cysts (Intraspinal Meningocele)
Authors: Parkinson Dwight , Chaudhuri A. , Shwartz I.
Published: July 2016

A case of congenital, intraspinal, extradural cyst is reported with pain as the only presenting feature. In the past, the lack of pain has been considered one of the characteristics of these rare lesions. In other reported cases, when pain has been present, it was minimal and never the presenting feature. The clinical and radiographic features and the surgical treatment are described. The pathogenesis is discussed and the literature reviewed. An addition to the surgical technique is given whereby one of the post operative complications may be obviated.
Computer Tomography in Disseminated Sclerosis
Authors: Warren K.G. , Ball M.J. , Paty D.W. , Banna M.
Published: July 2016

A case is reported where the appearance of acute, diffuse, disseminated sclerosis on computer tomography (Ct scan) is described and the literature is reviewed. This disease may give rise to multiple, small areas of diminished X-ray absorption which may decrease in size during the course of the disease. The histological features of one of the lesions was correlated with the radiological findings. It is suggested that, perhaps, only during the active stage of demyelination can the lesions be detected on the Ct scan.
Dual Dorsal Columns: A Review
Authors: Beck Charles H. M.
Published: July 2016

Recent evidence indicates that Wall (1970) may have been premature in concluding that dorsal column lesions produce no discernable sensory defects. Much of the negative evidence Wall presented to support this view is inconclusive. In addition several studies have reported significant sensory deficits in animals with severed dorsal columns. On the other hand, the literature strongly supports Wall's view that dorsal column lesions cause motor disturbances. A review of the anatomical and electrophysiological literature reveals growing evidence for the dissociation of two major subsystems relaying in the dorsal column nuclei. The possible functions of these two systems are discussed.
Von Hippel-Lindau's Disease
Authors: Miller Robert G. , Porter Roger J. , Nielsen Surl L. , Hosobuchi Hoshio
Published: July 2016

A patient with Von Hippel-Lindau's disease was followed from the time of diagnosis to the time of his death 13 years later. He was asymptomatic when the diagnosis was made, although a hemangioblastoma of the medulla was found on angiography. The patient's course and autopsy are described and the features of this disease are reviewed.
Computerized Transaxial Tomography: Its Role in the Post-Operative Tumor Case
Authors: Wortzman G.
Published: July 2016

The role of computerized transaxial tomography (CTT) of the brain in 100 post-operative tumor cases is assessed. CTT proves an accurate method of determining tumor recurrence and in evaluating such complications as intracerebral hemorrhage, subdural or extradural hemorrhage, cerebral infarction, hydrocephalus and shunt patency. Its accuracy decreases the need for interventional procedures. Its usefulness may be limited by the presence of metal clips or plates, by radio-opaque contrast media or intracranial air.
Transient Responses of Rabbit Retinal Ganglion Cells to Photic and Electrical Stimuli
Authors: Molotchnikoff S.
Published: July 2016

The relationships between the center and the surround of the receptive field of the rabbit retinal ganglion cell were investigated. This was done by coupling localized light spots and electrical activation of the retina and by analyzing the time of the excitatory and inhibitory periods. The responsiveness to the electrical transretinal pulse revealed a) that ON stimulation in OFF-center cells and OFF stimulation in ON-center cells, elicited a primary period of inhibition with a short latency; b) the long latency response of surround stimulation was not preceded by an inhibitory period unless the center was simultaneously stimulated in the same direction; c) a transient response to a stationary spot of light is followed by a period of inhibition. These results are discussed in relation to the known cellular retinal networks.
Indomethacin is Effective Against Neurogenic Hyperthermia Following Cranial Trauma or Brain Surgery
Authors: Benedek G. , Tóth-Daru P. , Janáky J. , Hortobágyi A. , Obál F. , Colner-Sasi K.
Published: July 2016

The effectiveness of indomethacin treatment (1 mg/kg) as an antipyretic was tested in patients after cranial trauma or brain surgery involving the centromedial forebrain. Indomethacin was effective in reducing temperature in 10 of 11 cases which showed a dipyrone-resistant hyperthermia developing in the first 24 hours after brain damage, while no significant antipyretic effect was seen in hyperthermic cases developing more than 72 hours after cranial trauma or brain surgery. Biochemical tests estimating the effect of indomethacin, and pyrazolone derivatives on the arachidonic acid metabolism showed significant effects of indomethacin only in influencing cyclooxygenase activity and no effect of any drugs on lipoxy — genase actions. In view of these observations, the use of indomethacin is recommended as a treatment for neurogenic hyperthermia.
Treatment of Paramyotonia Congenita with Acetazolamide
Authors: Benstead Timothy J. , Camfield Peter R. , King David B.
Published: July 2016

Treatment of paramyotonia congenita with acetazolamide has been shown to reduce myotonic symptoms but severe weakness has developed in some patients leading to a recommendation not to use the drug in this disorder. We studied a patient with the characteristic clinical and electrophysiological profile of paramyotonia congenita. Myotonia was effectively treated with a very low dose of acetazolamide and no weakness developed. We conclude that acetazolamide can be a safe and effective medication in paramyotonia congenita.
Pseudoatrophy of the Brain with Valproic Acid Monotherapy
Authors: McLachlan Richard S.
Published: July 2016

A patient is described who, while on valproic acid monotherapy, developed reversible shrinkage of the brain, documented by computerized tomography with associated cognitive deficit. Although pseudoatrophy has been reported with steroid therapy, this is the first implication of an anticonvulsant drug in the etiology of that condition.
Herpes Zoster Ophthalmicus with Delayed Cerebral Infarction and Meningoencephalitis
Authors: Freedman Mark S. , Macdonald Ronald D.
Published: July 2016

Herpes zoster ophthalmicus can be complicated by a delayed ipsilateral cerebral angiitis which may cause infarction and a smoldering meningoencephalitis. We describe such a case treated successfully with steroids and acyclovir. It is important to consider the diagnosis of this disorder early since therapeutic intervention may prevent an otherwise high morbidity and mortality. Steroids may have to be continued for some time after clinical resolution, using the ESR as a guideline for decreasing dosages.
Surgical Neuroangiography of Intracranial Vascular Malformations
Authors: Ter Brugge K.G. , Lasjaunias P. , Chiu M.C.
Published: July 2016

In the three year period of 1982 to 1985, 152 patients with intracranial vascular malformations were referred to the Bicetre Hospital in Paris, France and the Toronto Western Hospital in Canada. Sixty-six patients with cerebral vascular malformations and 48 patients with dural vascular malformations underwent endovascular treatment. In 8 patients surgery was performed in combination with the embolization procedure. Detailed analysis of the angio-architecture of the vascular malformation reveals certain characteristics which may be of value in predicting the clinical outcome. Comprehensive data analysis and long term follow-up is necessary to assess the role of embolization in the treatment of intracranial vascular malformations.
Anterior Versus Posterior Decompression for Symptomatic Spinal Metastasis
Authors: Perrin R.G. , McBroom R.J.
Published: July 2016

Management of patients with symptomatic spinal metastasis is designed to (1) relieve pain, and (2) restore or preserve neurologic function. The surgical strategy must include provisions for decompression of the dural sac and nerve roots, and stabilization of the spinal column. The optimal surgical approach, whether from in front or from behind is determined by a number of factors including (1) tumour location, (2) spinal level, (3) fixation factors, (4) patient debility.
Management of Chronic Ventilatory Insufficiency with Electrical Diaphragm Pacing
Authors: Vanderlinden R. Graham , Epstein Stanley W. , Hyland Robert H. , Smythe Harley S. , Vanderlinden Loren D.
Published: July 2016

We have had experience with diaphragm pacing in 24 patients at the Toronto Western Hospital. Fourteen patients have undergone bilateral implants to treat chronic ventilatory insufficiency (CVI) caused by traumatic tetraplegia at the C1/2 level (eight patients), neurogenic apnea (five) and one case of neonatal apnea. Unilateral stimulators for nocturnal pacing have been implanted in five patients with central alveolar hypoventilation (sleep apnea) and five patients who suffered CVI resulting from various etiologies. Of the patients who were ventilatory dependent, 80% were successfully weaned and in the entire series, 58% of the patients are living. Diaphragm pacing was successful in 67%, partially successful in 8% and ineffective in 25%. The major complications were: death by pneumonia, failure of the radio receivers, and infection. Diaphragm pacing is the treatment of choice for patients who are ventilator dependent and tetraplegic from upper cervical trauma or in some cases of neurogenic apnea; it may be life saving for patients who suffer central alveolar hypoventilation.
Novel Uses of EMG to Study Normal and Disordered Motor Control
Authors: Stein Richard B.
Published: July 2016

The electromyogram (EMG) is unique in that it gives the output of a single class of neurons, o-motoneurons, in a way that can be readily recorded and studied in normal subjects as well as in patients with a variety of disorders. Although the EMG has been used for many years, new applications continue to be found. This paper reviews in succession four recent approaches to EMG recording that have provided insight into the function of the normal and the disordered nervous system.
Late Progression of Post-Encephalitic Parkinson's Syndrome
Authors: Calne Donald B. , Lees Andrew J.
Published: July 2016

We have studied 11 patients with neurological deficits due to the pandemic of encephalitis lethargica, all of whom had remained in hospital for more than forty years. Retrospective information obtained from physicians and nurses dating back as far as 1931, our own Webster and Northwestern University Disability Rating Scales available from 1976, photographic records, published narrative and hospital charts all indicate that neurological disabilities attributable to basal ganglia damage frequently increase in late life. Deterioration was most marked in motor function and largely spared the intellect, special senses and somatosensory system. The findings are discussed in relation to current hypotheses concerning the aetiology of Parkinson's disease.
The High Cost of Not Doing Neurological Research
Authors: Gibson William C.
Published: July 2016

It is easy to imagine a new bumper sticker with the following message:

“If you think medical research is expensive, try disease.” Specifically in today's context, it might well read:

“If you think neurological research is expensive, try the diseases of the nervous system.”

That is precisely what we are doing as a country - “trying disease”! Canada spends $36 billion per year on health care, or $700 million per month. Every hour Canada spends $4 million on "health care" and only $16,000 on medical research.
Abnormal Visual Adaptation to Flicker in Multiple Sclerosis
Authors: Raymond J.E.
Published: July 2016

A visual psychophysical adaptation procedure was used on patients with Multiple Sclerosis (MS) in an attempt to induce a temporary and local exacerbation of subclinical visual impairment. Using a flicker detection task, sensitivity before and after adaptation to a flickering stimulus was measured in 9 MS patients and 9 control subjects. Although only 22% of patient eyes had abnormal flicker sensitivity prior to adaptation, visual deficit was observed in 83% of eyes studied after adaptation. Of the 7 MS eyes studied for which no other sign or symptom of visual involvement was present, 5 were found to have visual deficits after flicker adaptation. In addition, 10 of the 11 eyes affected by MS showed an abnormal response to flicker adaptation. Recovery from the effects of adaptation was complete in all patients within 2 minutes. The results suggest that partial demyelination of visual pathway neurons may exist in patients without signs or symptoms of visual involvement. The prolonged stimulation provided during adaptation may produce a temporary fatiguing or conduction blockade of such neurons which may lead to reductions in sensory sensitivity.
Que savez-vous de Joseph Babinski?
Authors: Bélanger Claude
Published: July 2016

C'est avec une appréhension certaine que j'ai accepté le redoutable honneur d'être le conférencier cette année de la Société Canadienne de Neurologie dont je fais partie depuis trente ans.

C'est avec la même appréhension que j'ai choisi d'évoquer certains aspects de la carrière d'un grand de I'histoire du 20e siècle des sciences neurologiques, Joseph Babinski.

Je souhaite que vous soyiez indulgents à l'endroit de l'apprenti historien que je voudrais être.
Vascular Headache: A Presenting Symptom of Multiple Sclerosis
Authors: Freedman Mark S. , Gray Trevor A.
Published: July 2016

Vascular headache of migraine-type may be a presenting symptom of multiple sclerosis (MS), a condition usually not considered in the differential diagnosis of a severe headache accompanied by neurological signs. We reviewed records of 1,113 patients with MS seen from 1967-1987 and found 44 cases whose initial attack or subsequent exacerbations were heralded by a migraine-type headache. Twenty-seven patients had no prior history of migraine, and of these, 12 presented simultaneously with their first headache and MS attack. Twenty-three patients had symptoms of a posterior fossa mass lesion. The significance of these results and possible pathogenesis is discussed.
Sturge-Weber-Dimitri Disease: Role of Hemispherectomy in Prognosis
Authors: Oluwole Ogunmekan A. , Hwang Paul A. , Hoffman Harold J.
Published: July 2016

We report 12 patients with Sturge-Weber-Dimitri disease treated surgically between January 1975 and December 1987. Hemispherectomy was performed on ten, two others underwent occipital lobectomy for intractable seizures. All operations were performed between the ages of 3 months and 20 months, except in two at age 8 and 9 years. The onset of seizures in all was between 2 and 8 months of age, except for two at 15 months. There were no postoperative deaths. Postoperative shunt procedures were required in 3 out of 12 (25%). Postoperative seizure control for one year or more was achieved in 11 out of 12 patients. The remaining patient is on medication with no seizures, but follow-up is less than a year. Intellectual deterioration was not seen after surgery except in 2 patients who had late operations. We conclude that patients with Sturge-Weber-Dimitri syndrome who have intractable seizures in the first 6 months of life and unilateral hemispheric involvement should be considered for early resection of the involved hemisphere.
Isolated Basilar Artery Occlusion Associated with a Clivus Fracture
Authors: Guha Abhijit , Fazl Mahmood , Cooper Perry W.
Published: July 2016

Most vascular injuries to the brain secondary to blunt head trauma involve the internal carotid circulation. A case of isolated basilar occlusion secondary to a clival fracture is described and compared to three other cases in the literature.
Partial Syndrome of Myotonic Dystrophy: Clinical Presentation and Follow-up
Authors: Mathieu Jean , Simard Marcel , De Braekeleer Marc , Boily Camil , Deschênes Aurèle
Published: July 2016

The neurological and ophthalmological investigation of 602 members of 88 Saguenay kindreds affected by myotonic dystrophy (MyD) revealed 130 persons with a partial syndrome. These patients, whose average age was 34.1 years, showed different abnormalities such as particular ophthalmic and/or neuro-muscular signs, suggesting MyD in the absence of myotonia or typical lens abnormalities. After an average period of 2,4 years, 44 of these 130 patients were reassessed by the same neurologists and ophthalmologists. Thirty still had a partial syndrome, 8 showed a typical form of MyD and 6 no longer presented any identifiable anomaly. This preliminary follow-up study of the partial MyD syndrome did not allow us to identify any clinical anomaly from which the presence of the MyD gene could be predicted in a significant way. It furthermore suggested that the identification of equivocal or unspecific signs among these patients can sometimes lead to misdiagnosis. This must be taken into account when providing genetic counselling. It furthermore indicates that the use of DNA probes is essential for a reliable identification of asymptomatic MyD gene carriers.