bluuebunny/crossref_metadata_2025 huggingface dataset:
Effect of Methylprednisolone on CSF IgG Parameters, Myelin Basic Protein and Anti-Myelin Basic Protein in Multiple Sclerosis Exacerbations
Authors: Warren Kenneth G. , Catz Ingrid , Jeffrey Verona M. , Carroll Dorothy J.
Published: July 2016

Clinical exacerbations of multiple sclerosis (MS) are characterized by elevated levels of cerebrospinal fluid (CSF) myelin basic protein (MBP). The purposes of this study were to determine whether anti-MBP antibodies are present in increased titer in CSF of MS patients with exacerbations, and whether they can be suppressed by the administration of immunosuppressive dosages of methylprednisolone (MP). A solid phase radio-immunoassay (RIA) was used to detect free and total anti-MBP antibodies before and after acid hydrolysis of CSF. In MS exacerbations, the majority of elevated anti-MBP is in the free form. With the exception of subacute sclerosing panencephalitis (SSPE) and some cases of post infectious encephalomyelitis, anti-MBP antibodies are not present in either MS patients in remission or in non-MS controls. Anti-MBP levels remained elevated over a 10 day period when patients are managed by bed rest only or when treated with intravenous (IV) ACTH. IV administration of MP in “high” (160 mg/day) or “mega” (2 g/day) dosages produces a highly significant reduction of both MBP (p<0.01) and anti-MBP (p<0.001) levels. Total intrathecal IgG synthesis is also significantly suppressed by IV-MP but not by ACTH.
Spinal Cord Blood Flow Measured with Microspheres Following Spinal Cord Injury in the Rat
Authors: Wallace M. Christopher , Tator Charles H.
Published: July 2016

A decrease in spinal cord blood flow (SCBF) is a known sequela of spinal cord injury. The radioactive microsphere technique permits repeated measurement of spinal cord blood flow (SCBF) and cardiac output (CO) in the same experimental animal. The purpose of this study was to adapt the radioactive microsphere technique for use in the rat extradural clip compression injury model used in our laboratory.

Thirteen adult Wistar rats were anaesthetized and ventilated. Mean systemic arterial pressure (MSAP) was recorded continuously. Control animals (n = 8) did not have a surgical procedure whereas the injured animals (n = 5) underwent a C7-T1 laminectomy followed by a one minute, 50 gram extradural clip compression injury at Tl. Radioactive microspheres were used for two blood flow and CO determinations in both groups.

MSAPfell 59% in the injured animals (p<0.01), but this was not accompanied by significant changes in heart rate or CO. There was a 50% reduction in SCBF in the injured cord (p<0.02), and there were significant reductions in cerebral blood flow (p<0.05) and cerebellar blood flow (p<0.02) following spinal cord injury.
An Unusual Form of Motor Neuron Disease Following a Cat Bite
Authors: Hudson Arthur J. , Vinters Harry V. , Povey R. Charles , Hatch Leslie A. , Percy Dean H. , Noseworthy John H. , Kaufmann John C.E.
Published: July 2016

A case of motor neuron disease with clinical and pathological resemblance to amyotrophic lateral sclerosis (ALS) in a woman who was severely bitten on the ankle by a cat is described. Weakness first appeared at the ankles and relentlessly advanced proximally, terminating in death from pulmonary failure in a year. A number of unusual features that are uncharacteristic of ALS were found that included a markedly elevated antinuclear antibody titre in the serum and the presence of prominent oligoclonal bands in the cerebrospinal fluid. The spinal cord showed loss of anterior horn cells and pyramidal degeneration that are characteristic of ALS, but an extraordinary finding was the presence of transmural granulomatous inflammation of numerous small and medium sized vessels, especially veins, in the subarachnoid space around the cord. There were also inflammatory changes in the brainstem and spinal cord consisting of microglial and astrocytic nodules and perivenous cuffing by mononuclear cells. Ill-defined hyaline eosinophilic cytoplasmic inclusions were seen in some remaining anterior horn cells. No viral particles were found by electron microscopy despite an extensive search. Virological studies were inconclusive but there is reason to believe that this patient's illness was caused by an as yet unidentified virus.
Parkinson's Disease: A Genetic Study
Authors: Alonso Ma. Elisa , Otero Enrique , D'Regules Rosalinda , Figueroa Hector Hugo
Published: July 2016

A sample of 122 patients with Parkinson's Disease was studied for the purpose of investigating if the frequency of relatives affected with Parkinson in this group was higher than in a control group and to see if the genetic load was more important in some of the subtypes of Parkinson described by Barbeau and Pourcher (1982).7In our 122 patients, we found that 1.7% were post-encephalic parkinsonian, 12.3% were symptomatic cases and 86% of the idiopathic variety. There were 16.1% early onset patients in the idiopathic group and among these we found 23.5% with a positive family history of Parkinson in the first-degree relatives. In 6 cases with the tremor onset form of the disease, the family history was positive and 5 patients, 4.7% had familial essential tremor-related Parkinsonism. Our results support Barbeau's hypothesis7.19that Parkinson is a heterogeneous disease in which some subtypes (such as early onset Parkinson) have an important genetic subceptibility component.
Differential Diagnosis of the Dementias of Unknown Origin: A Clinician's View
Authors: Kral V.A.
Published: July 2016

The close cooperation of clinical and laboratory research has helped to clarify the etiology of some of the dementing processes of the senium. However, the necessary investigations are complicated, laborious, expensive and can be carried out only in well equipped centres in larger cities. This restricts the number of patients who eventually may benefit from these investigations to a small number. What is needed for the psychogeriatric practice particularly in rural areas and smaller cities are simple diagnostic guidelines for the psychiatrist to answer the question whether the patient suffers from a dementia and if so whether the dementia is in all probability due to a primary degenerative process of the brain parenchyma or of the cerebral vasculature or is it due to another cause.

If degeneration of the brain parenchyma seems the prevalent pathogenetic mechanism one would like to establish in a given case which of the known degenerative processes is most probably present in order to avoid mistakes in clinical judgement with their often life threatening consequences.
Cell Loss from the Nucleus Basalis of Meynert in Alzheimer's Disease
Authors: Doucette R. , Fisman M. , Hachinski V.C. , Mersky H.
Published: July 2016

We examined the degree of neuronal loss from the nucleus basalis of Meynert (nbM) in two groups of Alzheimer patients differing in the degree of intellectual impairment. Significant cell loss from the nbM was found only in the more severely demented group of patients. Mean cell counts (per lOu, paraffin section) were compiled separately for the anterior, intermediate and posterior subdivisions of the human nbM in three groups of subjects: Group 1 (N = 4) was severely demented and was untestable on the Extended Scale for Dementia (ESD) for at least the last two years of life; Group 2 (N = 4) was less demented and had completed at least one ESD test within 12 months of death; Group 3 (five controls) had died of non-neurological causes. In Group 2 there was a small (but insignificant) trend toward cell loss in the anterior subdivision, and a normal complement of neurons in both the intermediate and posterior subdivisions. There was, however, significant cell loss from all subdivisions of Group 1. How these cell counts may relate to the severity of the dementia is discussed.
Failed Compensatory Dendritic Growth as a Pathophysiological Process in Alzheimer's Disease
Authors: Flood Dorothy G. , Coleman Paul D.
Published: July 2016

In normal human aging the remaining neurons of two areas of the hippocampal region have been found to compensate for age-related neuronal loss by proliferating new dendrites. In Alzheimer's disease (AD) the layer II pyramidal neurons of the parahippocampal gyrus fail to show this compensatory response, in spite of a probable, exaggerated disease-related loss of neurons. In AD the dentate gyrus granule cells of the hippocampus also show a reduced amount of the compensatory response. This failure of the AD brain to show the normal compensatory plastic response, seen in normal aging as dendritic growth, may be viewed as one of the pathophysiological processes of the disease.
Clinicopathological Studies of Some Non-Alzheimer Dementing Diseases
Authors: Munoz-Garcia David , Ludwin Samuel K.
Published: July 2016

The brains of most demented patients show at autopsy the lesions of Alzheimer's disease (AD). However, the brains of other demented patients show either no morphological changes or lesions distinct from those of AD. We report clinicopathological studies on two diseases in this latter group. The study of these diseases can improve our understanding of AD.

Pick's disease is characterized by dementia, lobar cerebral atrophy, and neuronal cytoplasmic inclusions. Most cases, which we have called “classical”, show inclusions made up of straight fibrils that are immunologically cross-reactive with the paired helical filaments of AD. In other “generalized” cases, similar fibrils are coated by granular material and are less reactive with anticytoskeletal antibodies.

In contrast to the cytoplasmic localization of the lesions in Pick's disease, it is the cell nucleus that shows abnormalities in neuronal intranuclear hyaline inclusion disease. This disease can present clinically as dementia of adult onset. Thus, either nuclear or cytoplasmic lesions can produce a pattern of neuronal dysfunction resulting in dementia.
Neurotransmitter and Receptor Deficits in Senile Dementia of the Alzheimer Type
Authors: Quirion R. , Martel J.C. , Robitaille Y. , Etienne P. , Wood P. , Nair N.P.V. , Gauthier S.
Published: July 2016

Multiple neurotransmitter systems are affected in senile dementia of the Alzheimer's type (SDAT). Among them, acetylcholine has been most studied. It is now well accepted that the activity of the enzyme, choline acetyltransferase (ChAT) is much decreased in various brain regions including the frontal and temporal cortices, hippocampus and nucleus basalis of Meynert (nbm) in SDAT. Cortical M2-muscarinic and nicotinic cholinergic receptors are also decreased but only in a certain proportion (30-40%) of SDAT patients. For other systems, it appears that cortical serotonin (5-HT)-type 2 receptor binding sites are decreased in SDAT. This diminution in 5-HT2 receptors correlates well with the decreased levels of somatostatin-like immunoreactive materials found in the cortex of SDAT patients. Cortical somatostatin receptor binding sites are decreased in about one third of SDAT patients. Finally, neuropeptide Y and neuropeptide Y receptor binding sites are distributed in areas enriched in cholinergic cell bodies and nerve fiber terminals and it would be of interest to determine possible involvement of this peptide in SDAT. Thus, it appears that multi-drug clinical trials should be considered for the treatment of SDAT.
Alzheimer's Disease: Metabolic Uncoupling of Associative Brain Regions
Authors: Rapoport Stanley I. , Horwitz Barry , Haxby James V. , Grady Cheryl L.
Published: July 2016

Evidence indicates that Alzheimer's disease (AD) causes functional disconnection of neocortical association areas. In mildly demented AD patients without measurable neocortically-mediated cognitive abnormalities, positron emission tomography demonstrates reduced parietal lobe glucose metabolism and left/right metabolic asymmetries in neocortical association areas. Similar metabolic abnormalities occur in moderately demented patients, but are accompanied by appropriate language and visuospatial discrepancies. Left/right metabolic asymmetries correspond with reduced numbers of partial correlations between metabolic rates in homologous right and left regions, and in the frontal and parietal cortices, indicating metabolic uncoupling among these regions. The affected association regions are those which demonstrate Alzheimer-type neuropathology post-mortem.
Magnetic Resonance Imaging in Down's Syndrome
Authors: Pelz David M. , Karlik Stephen J. , Fox Allan J. , Viñuela Fernando
Published: July 2016

100% of brains of Down's adults over age 40 will show Alzheimer-type neuropathologic changes in the frontal and temporal lobes. In an attempt to image these lesions, magnetic resonance imaging (MRI) was performed in seven patients with Down's syndrome, ranging in age from 17 to 45 years, using a resistive unit operating at 0.15 Tesla. All scans were within normal limits except for one 45 year-old patient with severe left temporal lobe atrophy. No areas of abnormal signal were seen in the frontal or temporal lobes and the white matter lesions commonly seen in elderly demented subjects were not visualized in this group. We conclude that these white matter lesions are likely coincidental and not causally related to Alzheimer's changes. The pathologic process leading to the formation and development of Alzheimer's changes in the brains of Down's adults may not be visible on magnetic resonance images.
Observations About Amyotrophic Lateral Sclerosis and the Parkinsonism-Dementia Complex of Guam with Regard to Epidemiology and Etiology
Authors: Steele John C. , Guzman Tomasa
Published: July 2016

For more than 150 years, Chamorro natives of the Mariana Islands in the Western Pacific Ocean, have developed fatal paralysis in middle and later life, which we term amyotrophic lateral sclerosis/parkinsonism-dementia (ALS/PD). The cause of the disease might be exposure to seeds of the indigenous cycad. Motor system disease is induced in cynomolgus monkeys by feeding them beta-N-methylamino-L-alanine (BMAA), an amino acid present in cycad seeds. We believe that the cycad seeds which usually cause no immediate adverse symptoms when prepared and eaten as flour, or applied topically as medicine, can give rise to widespread and severe nerve cell degeneration after a latency of many decades. Furthermore, it may be that only a single exposure to this potent but silent toxin(s) can result in fatal neurological disease years later.
Cognition and the Basal Ganglia: A Possible Substrate for Procedural Knowledge
Authors: Phillips Anthony G. , Carr Geoffrey D.
Published: July 2016

Disruption of neural activity within the basal ganglia of experimental animals causes selective learning deficits in tasks requiring switching between response strategies. These data along with reports of both general and specific intellectual impairment in patients with neurodegenerative disorders such as Parkinson's disease, appear to support the theory of cognitive functions of the basal ganglia. Recent studies have failed to confirm general cognitive or memory deficits in parkinsonian patients, but have identified deficiencies in devising and executing certain cognitive strategies. Following the lead of theorists such as Squire and Mishkin, this brief review emphasizes the distinction between procedural and declarative knowledge and examines the possible role of the basal ganglia in the acquisition and retention of procedural knowledge.
Neurotransmitters in the Mammalian Striatum: Neuronal Circuits and Heterogeneity
Authors: Semba K. , Fibiger H.C. , Vincent S.R.
Published: July 2016

The major input and output pathways of the mammalian striatum have been well established. Recent studies have identified a number of neurotransmitters used by these pathways as well as by striatal interneurons, and have begun to unravel their synaptic connections. The major output neurons have been identified as medium spiny neurons which contain ɣ-aminobutyric acid (GABA), endogeneous opioids, and substance P. These neurons project to the pallidum and substantia nigra in a topographic and probably chemically organized manner. The major striatal afferents from the cerebral cortex, thalamus, and substantia nigra terminate, at least in part, on these striatal projection neurons. Striatal interneurons contain acetylcholine, GABA, and somatostatin plus neuropeptide Y, and appear to synapse on striatal projection neurons. In recent years, much activity has been directed to the neurochemical and hodological heterogeneities which occur at a macroscopic level in the striatum. This has led to the concept of a patch-matrix organization in the striatum.
Receptors in the Basal Ganglia
Authors: Guttman Mark
Published: July 2016

The study of neurotransmitter receptors aids in the understanding of the normal anatomy, pharmacology, therapeutics and pathophysiology of disease processes involving the basal ganglia. Receptors may be studied in vitro by homogenate binding experiments, enzyme analysis or quantitative autoradiography and in vivo with positron emission tomography. In the substantia nigra (SN), receptors have been identified for somatostatin, neurotensin, substance P, glycine, benzodiazepine and GABA, opiates, dopamine, angiotensin converting enzyme (ACE) and serotonin. The striatum has receptors for dopamine, GABA and benzodiazepines, acetylcholine, opiates, substance P, glutamate and cholecystokinin. GABA and benzodiazepine receptors are also located in the globus pallidus. In Parkinson's disease, striatal dopamine D-2 receptors are elevated in patients that have not received L-DOPA therapy. This supersensitivity is reversed with agonist therapy. Muscarinic binding to cholinergic receptors seems to correlate with dopamine receptors. Delta opiate receptors are increased in the caudate and mu binding is reduced in the striatum. In the SN of patients with Parkinson's disease, there is reduced binding of somatostatin, neurotensin, mu and kappa opiates, benzodiazepine and GABA and glycine. In Huntington's disease, there is reduced binding of GABA and benzodiazepines, dopamine, acetylcholine, glutamate and CCK. There is increased binding of GABA in both the SN and globus pallidus. Glycine binding is increased in the substantia nigra and ACE is reduced.
MPTP-Induced Parkinsonism in the Monkey: Neurochemical Pathology, Complications of Treatment and Pathophysiological Mechanisms
Authors: Crossman A.R. , Clarke C.E. , Boyce S. , Robertson R.G. , Sambrook M.A.
Published: July 2016

MPTP induces parkinsonism in monkeys by destruction of the substantia nigra, pars compacta. It can also damage ventral tegmental dopamine neurones and the noradrenergic locus coeruleus, both of which may be affected in Parkinson's disease. Motor symptoms in MPTP-treated monkeys respond readily to levodopa or dopamine agonist therapy. Administration of levodopa over 4-8 weeks leads to the emergence of “peak- dose” dyskinesia. Such abnormal movements are not seen following challenge doses of levodopa in animals not on long-term therapy. Radioligand studies reveal a 40-180% increase in D2 receptor binding in the striatum of parkinsonian monkeys. 2-deoxyglucose studies of regional brain metabolism indicate that MPTP-induced parkinsonism is characterised by abnormally increased activity of medial pallidal neurones which project to the thalamus and pedunculopontine nucleus and reduced activity of subthalamic nucleus neurones.
A Rostrocaudal Gradient for Aromatic Acid Decarboxylase in the Human Striatum
Authors: Garnett E.S. , Lang A.E. , Chirakal R. , Firnau G. , Nahmias C.
Published: July 2016

The local concentration of 6-[18F]fluoro-L-dopa (18F) reflects the activity of aromatic acid decarboxylase (AADC), the enzyme that generates dopamine from its precursor amino acid, L-dopa. In young healthy adults, the local concentration of 18F, and hence AADC activity, is constant in coronal slices taken in a rostrocaudal direction. With increasing age a gradient representing decreasing activity in the putamen develops. This decrease is less marked than was expected from the literature. In five children with primary dystonia, the striatal distribution of 18F resembled that seen in the normal older adults. In established clinical Parkinson's disease the rostrocaudal gradient becomes steep; the putamen is more damaged.
The Evaluation of “Spasticity”
Authors: Ashby P. , Mailis A. , Hunter J.
Published: July 2016

Lesions of the upper motor neuron cause: 1. Alterations in segmental reflex activity. For example increased tendon jerks and velocity dependent stretch reflexes ("spasticity"), clonus, the clasp knife response, release of flexion reflexes and extensor plantar reflexes. 2. Impaired ability to activate motoneurons rapidly and selectively. Voluntary movements may also be restrained by co-contraction of antagonists muscles, by segmental reflexes (enhanced during voluntary effort) or by contractures. A combination of these factors may impair overall functional ability. Segmental reflexes, voluntary power and overall functional abilities can be assessed using clinical scoring systems. Recordings of muscle length, tension andEMG offer more objective measures of reflex and voluntary activity and of overall functions such as locomotion, and can separate weakness from co-contraction, spasticity from contracture. Methods are now available for exploring individual (transmitter specific) segmental reflex pathways and descending pathways in man. Lesions of the upper motor neuron are complicated by secondary changes in segmental neurons. Segmental reflex activity and muscle mechanics depend on the immediate past history of events. These factors must be taken into account.
Myoclonies du voile associées à une diminution de l’acide 5-hydroxy-indole-acétique céphalo-rachidien et répondant au clonazepam
Authors: Gauthier S. , Young S.N. , Baxter D.W.
Published: July 2016

A patient presenting with palatal myoclonus was diagnosed as suffering from possible multiple sclerosis. A reduction of cerebrospinal 5-hydroxy-indole-acetic acid suggested a decreased central serotonergic turnover. A therapeutic trial with clonazepam, a serotonergic agonist, improved the myoclonus symptomatically. The possible involvement of serotonergic inhibitory systems in the expression of palatal myoclonus is discussed.
The Effect of Several Intertrial Intervals on the 1 Hz Interference Effect
Authors: Gaito John , Gaito Stephen T.
Published: July 2016

Experiments were conducted to evaluate the effect of two intertrial intervals of 1-Hz brain stimulation on kindling behavior induced by 60-Hz sine wave stimulation. In two experiments, the effective threshold intensity (ETI) to elicit a convulsion was determined on four separate occasions with 5 days of daily trials between determinations. On each day experimental rats were stimulated with 1-Hz current on the first and third trials for 120 seconds duration and with 60-Hz current for 30 seconds on the second trial (1-60-1 group). A second group was stimulated with 60-Hz current on each trial (60-60-60 group). A third group received no stimulation on Trials 1 and 3 and 60-Hz current on Trial 2 (X-60-X group). In Experiment I, the intertrial interval was 3 hours; a 24 hour interval was used in Experiment 2. The results were similar in both experiments. For the 1-60-1 group, there was a steady increase in the intensity required to elicit a convulsion with 60-Hz current from ETI to ETI. However, the 24 hour interval produced a lesser effect than did the 3 hour interval (or the 1 hour interval used in previous experiments). Rats in the other groups maintained relatively stable values from ETI to ETI, with a slight decline occurring. Suppression of convulsive behaviour on daily trials was present with the 1-60-1 groups, and nonexistent with the other groups.
Multiple Regression Analysis of Diagnostic Predictors In Optic Nerve Disease
Authors: Kirkham T.H. , Coupland S.G.
Published: July 2016

Demyelination is assumed to be the cause of the majority of cases of isolated optic neuritis. Because of the importance of establishing the presence of optic nerve dysfunction in patients suspected of having multiple sclerosis several new indices of optic nerve conduction have been reported including the visual evoked potential, the edge-light pupil cycle time, and the Pulfrich test. These measures purport to detect optic nerve dysfunction but with varying degrees of success. This study of 93 patients with clearly documented previous optic neuritis was conducted to determine the statistical relationship between these three measures and other clinical diagnostic indices for detection of previous optic nerve disease and the utility of the diagnostic predictors taken individually, and in combination. The other indices used were the presence of detectable optic atrophy, color vision defect and the presence of a relative afferent pupillary defect. The variables were submitted to linear stepwise multiple regression analysis which indicated that the presence of optic atrophy, defective color vision and prolonged pupil cycle time when used in combination provided the most useful diagnostic prediction of previous optic neuritis in this group of patients. The addition of visual evoked potentials, the Pulfrich test or presence of a relative afferent pupillary defect did not significantly increase predictive reliability.
Neurenteric Cysts of the Spinal Cord Mimicking Multiple Sclerosis
Authors: Vinters H.V. , Gilbert J.J.
Published: July 2016

A 54-year-old man died following a 20-year course of intermittent neurologic symptoms. He carried a diagnosis of multiple sclerosis, with most severe involvement thought to be in the spinal cord. At autopsy, two neurenteric (enterogenous) cysts were found to indent the anterior spinal cord — at the T4 and Tl I cord levels. Partial diplomyelia was present in the lumbosacral cord. These findings represent a unique combination of congenital abnormalities. Their embryogenesis and pathogenesis are discussed.
Abnormal Electroretinograms and Visual Evoked Potentials in Chronic Papilledema Using Time-Difference Analysis
Authors: Kirkham T.H. , Coupland S.G.
Published: July 2016

Visual function is usually considered to be normal in papilledema. We report previously undescribed abnormalities in the electroretinograms (ERG) and visual evoked potentials (VEP) of two patients with chronic papilledema. The steady-state latencies of the ERG and VEP were calculated using time-difference analysis. The ERG was abnormal under both scotopic (slow) and photopic (fast) flicker conditions, but the VEP was abnormal only to fast flicker photopic stimulation. This may indicate a relative vulnerability of a high frequency signal system under conditions of raised intracranial pressure. The abnormalities resolved rapidly once the intracranial pressure had been normalised. The cause of the V EP and particularly the ERG abnormalities remains unknown.
Heterotopic Growth of Dysplastic Cerebellum in Frontal Encephalocele in an Infant of a Diabetic Mother
Authors: Sarnat Harvey B. , deMello Daphne E. , Blair John D. , Siddiqui Saeeda Y.
Published: July 2016

An infant of a diabetic mother lived 13 days after birth. She had a small dysplastic 12.6 gm. brain, partly forming a frontal encephalocele. The uncleaved forebrain contained a mass of poorly organized heterotopic cerebellar cortex. The cerebellum itself had normal lamination, but was small and continuous with the dysplastic tissue. The ventricular system was absent except for a few midline ependymal rosettes, and the cerebral cortex was not developed. The cerebellar dysplasia resembled a proliferative and invasive lesion by its rostral extension.
A Case of Charcot-Marie-Tooth Disease Mimicking Friedreich’s Ataxia: is there any association between Friedreich’s ataxia and Charcot-Marie-Tooth disease?
Authors: Salisachs P. , Findley L.J. , Codina M. , Torre P. La , Martinez-Lage J.M.
Published: July 2016

The authors report a case of Charcot-Marie-Tooth disease that mimicked Friedreich’s ataxia and featured impaired tendon reflexes in the limbs, incoordination mimicking cerebellar disease in the extremities, extensor plantar responses on both sides, bilateral foot deformity, impaired position sense in the toes, absent vibratory sense in the distal parts of the legs and minimal distal weakness with wasting. Motor conduction velocity in the upper limbs was substantially reduced.

Other cases similar in nature reported in the literature resemble spino-cerebellar degeneration in general, and Friedreich’s ataxia, in particular. It is emphasized that the natural history, EMG, motor conduction velocity studies and examination of other affected members of the family permit the correct diagnosis to be made in such cases. It is also emphasized that patients similar to the one reported here may also resemble, and should be differentiated from, cases of familial dorsal column ataxia (Biemond type). Stress is put upon the fact that when Charcot-Marie-Tooth disease mimicks spino-cerebellar degeneration, substantial slowing of motor conduction in the upper limbs is generally sufficient to establish the diagnosis.

The relation between Friedreich’s ataxia and Charcot-Marie-Tooth disease is reviewed and it is concluded that these two disorders are distinct clinical and pathological entities.
Kinematics of the Foot
Authors: Allard P. , Thiry P.S. , Duhaime M. , Geoffroy G.
Published: July 2016

Orthogonal stereoradiographs are frequently utilized in determining three-dimensional geometrical parameters of human body segments. They have been applied here in the estimation of the length and elongation of the ligaments of the normal foot. Three small spherical metallic markers were respectively encrusted into the tibia and fibula, the seven bones of the tarsus and into the five metatarsals of an amputed lower limb to identify uniquely their spatial location. The foot was then positioned on a rotating platform. Standardized antero-posterior and lateral radiographs were taken. Afterwards the foot was dissected and the proximal and distal insertions of most of its ligaments were located by means of spherical markers. A second series of orthogonal radiographs were taken of each of the fourteen bones. The radiographs were digitized. The length of each ligament and elongation for a simple and complex movements were calculated by means of a computer program. The results of a simple movement of rotation representing a normal 20° dorsiflexion at the talocrural joint and of complex movements of rotation simulating an abnormal high arch such as encountered in Friedreich’s ataxia are presented and discussed.
Oral Lecithin and Linoleic Acid in Friedreich’s Ataxia: III. Biochemical Results
Authors: Melancon S.B. , Dallaire L. , Potier M. , Cousineau J. , Vanasse M. , Geoffroy G. , Fontaine G. , Grignon B.
Published: July 2016

Lecithin and sqfflower oil brought about the same changes in serum LAD activity and kinetics in patients with Friedreich’s Ataxia as in controls when results of this double-blind crossover study were analyzed according to group assignation. According to functional stages, pretrial LAD activity decreased with advancing severity while Km for lipoamide increased. Lecithin and sqfflower oil supplements corrected the elevated Km for lipoamide but produced a further reduction in LAD activity. These changes may have been due to the increased intake of linoleic acid, a precursor of lipoic acid, which is present in high percentage in both lecithin and safflower oil. Results of the biochemical study thus agreed with the clinical data gathered during the course of the one-year trial in suggesting that linoleic acid may well have been the active factor through which biochemical and clinical improvement was previously observed in patients with Friedreich’s Ataxia supplemented with lecithin.
Amino Acid Changes in the Mouse Mutant Dystonia Musculorum Similar to Those in Friedreich’s Ataxia
Authors: Messer A.
Published: July 2016

An autosomal recessive mutant strain of mouse with a progressive neurological disorder is described. Histopathology is dramatic in the sensory afferents and in the red nucleus. In the cerebellar vermis the concentrations of glutamate, aspartate, glycine and GABA are significantly reduced, and in the cerebellar hemispheres the taurine/glutamate ratio is elevated. These mice may provide a useful experimental model of Friedreich’s ataxia.
Increased Plasma Catecholamines in Patients with Friedreich’s Ataxia
Authors: Pasternac A. , Wagniart P. , Olivenstein R. , Petitclerc R. , Krol R. , Andermann E. , Melancon S. , Geoffroy G. , Champlain J. de , Barbeau A.
Published: July 2016

We studied free plasma catecholamines in 23 patients with Friedreich’s ataxia, having a mean age of 22 ± 9.6 (SD) years. Conjugated catecholamines were also studied in 10 patients. Mean plasma norepinephrine and epinephrine were significantly higher than controls both in the supine and standing positions. In total 15 out of 23 patients (65%) had increased free and/or conjugated plasma catecholamines. The increase in plasma catecholamines was more marked in patients with severe neuromotor impairment. Among the patients with left ventricular concentric hypertrophy (wall thickness >12 mm), only 3 had no demonstrable sympathetic hyperfunction.

Since the high local concentrations of norepinephrine at the site of release from sympathetic nerve terminals may serve as a trigger for the hypertrophic response of the myocardial cell, it is suggested that early pharmacological intervention could prevent or limit the cardiomyopathic process or its clinical consequences.
Effect of a Valine Load Test on Plasma Alpha-Keto Acids in Friedreich Ataxia
Authors: Barbeau A. , Bertrand M. , Bouchard R. , Gauthier G.L. , Bouchard J.P.
Published: July 2016

To test the physiological significance in vivo of our previous in vitro finding of reduced valine dehydrogenase (VDH) activity inpatients with Friedreich’s Ataxia, we subjected ataxic patients and controls to an oral valine load test (1.0g) and measured the levels of branched chain α-keto acids in the plasma for 24 hours. We demonstrated a significantly higher peak for α-keto isovaleric acid in Friedreich’s Ataxia and a general trend towards higher than control values in all other α-keto acids measured, and at all times in the experiment. These changes are compatible with the postulated defect in regulation of the activity of VDH in this illness, but because of their small amplitude, they also indicate that a VDH – deficiency is not the genetic defect in Friedreich’s A taxia.
Respiratory Arrest and Cervical Spinal Cord Infarction Following Lumbar Puncture in Meningitis
Authors: Norman Margaret G.
Published: July 2016

A 6-year-old child with meningitis had a respiratory arrest 20 minutes after a lumbar puncture. Thereafter she required maintenance on a ventilator, had a flaccid quadriplegia, and died 12 days later. Necropsy showed infarction of the central portion of the cord at the level of the decussation of the pyramids. The suggested mechanism of damage is compression of the arterial supply to the cord at the level of the foramen magnum by herniated cerebellar tonsils; concomitant hypotension may have contributed to production of the damage. Four similar cases, who survived with residual deficit, have also been reported. Other separate mechanisms by which the cord can be damaged in meningitis are vasculitis, thrombosis and arachnoiditis.
Results of Burr Hole and Open or Closed Suction Drainage for Chronic Subdural Hematomas in Adults
Authors: Weir B.K.A.
Published: July 2016

Chronic subdural hematomas in adults are not technically difficult to treat. They probably constitute one of the most common potentially curable lesions with which the average neurosurgeon deals. Despite this (or perhaps because of it) numerous treatments, radically differing from one another, have been advocated. Fine judgement is called for in deciding whether or not to operate in the first instance, make a flap or one or more burr or twist drill holes, use a drain, employ suction, inflate the brain, leave the bone flap out. In a personal series of 71 chronic subdural hematomas, 46 were treated with a red rubber catheter drain through a burr hole and 23 with a closed system suction drain. The results of this series suggest that closed system suction drainage through a single burr hole is the better of the two techniques. It has important theoretical advantages and the procedure is simple and fast. The complication rate is low and patients have a shortened postoperative hospital stay.
Endartériectomie carotidienne: histoire médicale préopératoire et devenir à long terme de 82 patients
Authors: Trudel L. , Fabia J. , Bouchard J.P.
Published: July 2016

Using medical files, death certificates and an interview at home for the survivors, a long term retrospective follow-up study was done for 82 subjects operated for carotid stenosis. Causes of death, survival rates and medical history of survivors were studied. Subjects were classified in two preoperative risk categories according to their medical history prior to angiography: low risk (36 subjects) and medium risk (46 subjects). None of the subjects were lost during the follow-up period which varies from 81 to 105 months.

At seven years, observed survival rate is significantly lower for the medium risk patients than for the low risk patients (43.0 ±8.6 versus 80.6 ± 6.6; X2 M-H, p = 0.006). The difference between the survival rates is markedly noticeable after the fifth year of follow-up. Besides, the percentage of survivors who have developed cerebrovascular problems after the operation is significantly higher in the medium risk group than the low risk group (54.5% versus 25.0%, X2 p ≃ 0,04).

Does this mean that the preoperative risk classification separates two different stages or two different forms of atherosclerosis? Can. J. Neurol. Sci. 1983; 10:27–31
Facilitation of Kindled Seizures in Rats Fed Choline-Supplemented Diets
Authors: McCann Kevin , Cain Donald P. , Philbrick Diana J.
Published: July 2016

Different groups of rats were maintained on choline-deprived (CD), choline-supplemented (CS) or standard rat chow diets beginning at a body weight of approximately 100 g, and electrically kindled in the amygdala beginning not less than 40 days later. The CS group kindled significantly faster than the CD group, whereas the CD and chow-fed groups did not differ in their rate of kindling. This result is consistent with both the known effects of the dietary manipulation of choline on brain acetylcholine level, and the idea that acetylcholine has a role in amygdaloid kindling.
Acute Hemorrhagic Leukoencephalopathy
Authors: Suchowersky O. , Sweeney V.P. , Berry K. , Bratty P.J.A.
Published: July 2016

Two patients with acute hemorrhagic leukoencephalopathy, one of which was pathologically proven, were serially studied with CT scanning. Both patients showed marked distinctive low density white matter changes throughout both hemispheres, which correlated with clinically involved areas. One patient recovered from the disease, perhaps due to steroid treatment, and showed slow but complete resolution of CT scan changes. We feel that CT scan findings significantly help in the diagnosis of this disease, which may be amenable to early treatment with steroids.
Hemispherectomy for Seizures Revisited
Authors: Rasmussen Theodore
Published: July 2016

The serious, late complication of superficial cerebral hemosiderosis, which appears after several years in 1/4–1/3 of patients who have undergone hemispherectomy, has resulted in recent years in a considerable reluctance to carry out this operation despite the fact it has proved to be highly effective in patients with medically refractory seizures associated with hemiplegia. Preservation of a small portion of the hemisphere, usually the frontal or occipital pole, has proved to be effective in preventing this late complication, but at the cost of a significant reduction in the effectiveness of the operation in reducing the patients’ seizure tendency. Preserving the frontal and occipital poles but disconnecting them from the rest of the brain, resulting in a functional complete but anatomical subtotal hemispherectomy, retains the therapeutic effectiveness of a complete hemispherectomy while still protecting adequately against the serious late postoperative complication of superficial cerebral hemosiderosis and its associated neurologic deterioration, hydrocephalus and sometimes death.
Febrile Seizures in Epileptic Chicks: The effects of Phenobarbital, Phenytoin and Valproate
Authors: Johnson D.D. , Crawford K.D.A. , Crawford R.D.
Published: July 2016

Epileptic seizures can be evoked in chicks homozygous for the epileptic seizure gene (epi, epi) by elevating their body temperature using microwave diathermy. These seizures precede and differ in motor seizure pattern from a second clonic-tonic seizure produced by hyperthermia in both epileptic and carrier (heterozygote, Epi, epi) chicks. Hyperthermia did not evoke seizures in adult epileptic chickens. Phenobarbital delayed the onset of epileptiform seizures whereas phenytoin and valproate had no effect. These data suggest that epileptic chicks may provide a suitable model for studies on febrile convulsions.
Neurology and Neurosurgery in Nepal
Authors: Lee Robert G. , Gongal Dinesh N.
Published: July 2016

During early 1982 one of the authors (RGL) was privileged to spend two months in Nepal as part of an exchange program between the University of Calgary, Faculty of Medicine and the new medical school at Tribhuvan University in Kathmandu. A short visit of this type does not qualify a Canadian neurologist to comment on the neurological-neurosurgical problems of a country such as Nepal with any degree of expertise. Nevertheless, as a result of a number of discussions between the two authors and interviews with many other physicians, both Nepalese and Western, it was possible to form some general impressions and draw some comparisons between neurologic illnesses in Nepal and Canada.

Disorders affecting the nervous system are common in Nepal. Some of the problems which are seen regularly would be considered rare and exotic in North America. However, despite the vast differences in culture, geography, dietary habits, and socio-economic conditions, there are some surprising similarities between the patterns of neurologic illness in Nepal and Canada. Disorders such as migraine, stroke, epilepsy and meningitis are well known to Nepali physicians. To appreciate the magnitude of health care problems in Nepal and to view neurologic illness in its proper perspective, it is helpful to have a few basic facts and figures concerning the geography and population of the country.
Evaluation of Radiculopathies by Segmental Stimulation and Somatosensory Evoked Potentials
Authors: Eisen Andrew , Hoirch Maureen , Moll Alex
Published: July 2016

Thirty-six patients with suspected or myelographically proven radiculopathies were investigated with motor and sensory conductions, F-waves, needle electromyography, and somatosensory evoked potentials (SEPs). SEPs were elicited by cutaneous nerve stimulation representative of input from individual cervical and lumbosacral dorsal roots. A myelographic defect was present in 83% of 30 patients who had myelograms. Overall 78% of patients had one or more abnormal electrophysiologic tests, the needle EMG giving the best diagnostic yield (75%). F-waves and SEPs were abnormal in 43% and 57% of cases respectively. Motor deficit correlated best with abnormal EMGs, whilst abnormal SEPs occurred most frequently when sensory deficit predominated. Prolonged latency of the SEP occurred rarely, reduced amplitude or abnormal morphology being the most useful characteristics. SEPs evoked by cutaneous nerve stimulation are a useful addition to conventionally available electrophysiological methods of evaluating radiculopathies, especially in the absence of motor deficit.
Intradural Rupture of Lumbar Intervertebral Disc
Authors: Lee Shih-Tseng , Fairholm David
Published: July 2016

Two cases of intradural disc rupture with cauda equina compression are reported. Myelography demonstrated complete block and at surgery intradural fragments of sequestrated disc material were found. The pathogenesis of this condition is reviewed and our recommendation for management is presented.
Paraneoplastic Subacute Necrotic Myelopathy
Authors: Handforth Adrian , Nag Sukriti , Sharp Daniel , Robertson David M.
Published: July 2016

A 79 year old male acutely developed a Brown-Sequard syndrome two weeks following resection of renal cell carcinoma. The subsequent clinical course was marked by subacute progression of spinal cord signs. Pathologic studies showed extensive multifocal spinal cord necrosis that could not be attributed to vascular occlusion or to vasculitis. This case appears to be a rare example of paraneoplastic subacute necrotic myelopathy.
Problems in the Diagnosis and Treatment of Pseudotumor Cerebri
Authors: Corbett James J.
Published: July 2016

It is an honor to be asked to deliver the Silversides Lecture. Dr. Silversides’ clear understanding of the need for the collaboration of the basic and clinical sciences is a measure of the good sense for which he is admired. For a clinician, the greatest challenge to his clinical judgment lies in formulation of strategies to deal with vexing and serious conditions. Often we are called upon to treat conditions for which no satisfactory pathophysiologic mechanism has been elucidated. It is said that the clinician must frequently “get on top of the problem before he gets to the bottom of it.” In the spirit of all clinicians who seek better clinical approaches to poorly understood disease, I will devote this discussion to the diagnosis and treatment of pseudotumor cerebri.
CSF Myelin Basic Protein Levels in Acute Optic Neuritis and Multiple Sclerosis
Authors: Warren K.G. , Catz Ingrid , McPherson T.A.
Published: July 2016

Normal CSF-MBP levels as determined by a RIA were <6.2 ng/ml CSF (mean 3.9). Eighty percent of patients with acute optic neuritis have CSF-MBP levels greater than 6.2 ng/ml (mean 7.6 ng/ml CSF). Five of 7 patients with acute internuclear ophthalmoplegia due to an initial exacerbation of demyelination have CSF-MBP levels above 6.2 ng/ml (mean 6.8 ng/ml). Fifty percent of MS patients with chronic progressive disease have CSF-MBP levels above 6.2 ng/ml (mean 6.7 ng/ml). MS patients experiencing monosymptomatic exacerbations show elevated CSF-MBP levels in 75% of cases (mean 8.2 ng/ml). MS patients experiencing polysymptomatic exacerbations show significantly higher levels of CSF-MBP (mean 22.3 ng/ml) than the patients with monosymptomatic exacerbations. Ninety-five percent of MS patients experiencing polysymptomatic exacerbations have elevated levels of CSF-MBP.
Spinal Injuries Due To Hockey
Authors: Tator Charles H. , Ekong Chris E.U. , Rowed David W. , Schwartz Michael L. , Edmonds Virginia E. , Cooper Perry W.
Published: July 2016

Although many types of sports and recreational activities have been identified as common causes of acute spinal cord injury, hockey has been a rare cause of acute cord injury in Canada or elsewhere. For example, from 1948 to 1973 there were no patients with cord injuries due to hockey in a series of 55 patients with acute cord injuries due to sports or other recreational activities admitted to two Toronto hospitals. In contrast, between 1974 and 1981, the Acute Spinal Cord Injury Unit, Sunnybrook Medical Centre treated six patients with cervical spinal injury due to hockey, five of whom were seen during a 13 month period from September, 1980 to October, 1981. Five of the six sustained a severe acute cervical spinal cord injury, and one a cervical root injury. The cord injury was complete in two cases, while three had complete motor loss but incomplete sensory loss below the level of the lesion. All were males aged 15 to 26 years. Of the players with cord injury, four struck the boards with the neck flexed, and one struck another player with the neck flexed. The one player without cord injury struck the boards with his neck extended. The commonest bony injury was a burst fracture of C5 or C6. One of the patients with a complete cord injury died three months later of a pulmonary embolus, and the other patients with cord injury showed some recovery of root function, but little or no cord recovery. The reasons for the increase in spinal injuries in hockey are unknown.