bluuebunny/crossref_metadata_2025 huggingface dataset:
Physiopathology of Experimental Parkinsonism in the Monkey
Authors: Poirier Louis J. , Filion Michel , Larochelle Louis , Péchadre Jean-Claude
Published: July 2016

Postural or Parkinson-like tremor, which results from the impairment of mechanisms which are predominantly lateralized in the brain, is most likely related to the combined impairment of the dopaminergic nigrostriatal pathway and the corresponding rubro-olivo-cerebello-rubral loop (without excluding the possibility that other nervous mechanisms interconnected with these structures may represent an alternative disturbance). The integrity of the internal division of the pallidum and the ventrolateral area of the thalamus and their efferent fibers as well as the motor cortex and certain of its cortico-subcortico-spinal pathways (Figures 1 and 2) is apparently an essential feature for the elaboration of the rhythmic bursts associated with the appearance of postural tremor. The integrity of the spinal sensory roots and the rubro-tegmentospinal tract is not a prerequisite for the expression of postural tremor, a condition which seems essential for the production of rigidity. The latter facts suggest that the disturbances which subserve these two types of motor impairment, often concomitantly present in Parkinsonism, partially involve the impairment of different mechanisms although the loss of the DA fibers originating in the substantia nigra and ending in the neostriatum (Figure 1) appears to represent a disturbance common to both types of disorders.

Bradykinesia which may be associated with an impairment of catecholamine metabolism (and more especially the neostriatal DA mechanisms) on both sides of the brain may also result from bilateral lesions of the pallidum or of its outflow corresponding, in the main, to the pallidothalamic fibers ending in the ventrolateral thalamus. The latter types of lesion most likely exclude the influence of the monoaminergic, cholinergic and gabaminergic activities normally originating in the striopallidal system and influencing the activity transmitted to other CNS mechanisms. Severe akinesia, however, apparently depends on more profound and generalized disturbances of brain monoamine metabolism with or without the involvement of other ill-defined mechanisms. At any rate the impairment of the brain DA mechanisms (and especially those of the neostriatum) seems to represent a major feature in the production of the Parkinsonian type of akinesia. Further work is needed to establish the relative importance of the loss of catecholaminergic mechanisms other than those of the neostriatum in the production of akinesia.
Congenital Displacement of Temporal Cortex into the Central Spinal Canal
Authors: Silver David A. , Robertson David M.
Published: July 2016

This is a report of the first recorded observation of displacement of temporal cortex into the central spinal canal in an infant with the Arnold Chiari malformation, platybasia, aqueductal atresia, hydrocephalus and meningomyelocele. The combination of an absent tentorium, absent right cerebellar hemisphere and malformed fourth ventricular roof provided the anatomical background for this unique event.
Recurrent Spontaneous Seizure State Induced by Prefrontal Kindling in Senegalese Baboons, Papio Papio
Authors: Wada J.A. , Osawa T. , Mizoguchi T.
Published: July 2016

In our earlier study of amygdaloid kindling in Papio papio (Pp), the development of partial complex seizure and of focal motor seizure was correlated with bifrontal theta discharge and increasing Rolandic spike discharge respectively and the final stage was characterized by primary generalized convulsive seizure. Since the latter seizure pattern is known to originate from the frontal focus in man, the frontal cortex became suspect in the development of the final stage seizure pattern. Daily prefrontal stimulation showed that Pp can be kindled from this site, culminating in a recurrent spontaneous seizure state identical to that induced by amygdaloid kindling in this species. However, our observation did not support our original assumption regarding the genesis of primary generalized convulsive seizure. Prefrontal and amygdaloid kindling are significantly different with respect to morphology, distribution and propagation of afterdischarge and interictal spike discharge, and speed and pattern of clinical seizure development. Most intriguingly, inter-ictal behavioral aberration associated with depth EEG changes was observed only in the prefrontal animals and not in the amygdaloid animals.
Frontal Lobe and Kindling in the Rat
Authors: Corcoran Michael E. , Urstad Harald , McCaughran James A. , Wada Juhn A.
Published: July 2016

To test the hypothesis that the cortex participates in amygdaloid kindling in rats, bilateral aspiration lesions were made in various cortical areas in rats prior to kindling. Lesions in orbital cortex (on the dorsal lip of the rhinal sulcus) or prefrontal cortex (area 10) significantly retarded the rate of amygdaloid kindling; lesions in motor cortex, anterior cingulate cortex, or visual cortex were without effect. Detailed analysis indicated that the orbital lesioned and frontal-lesioned rats kindled relatively normally up to the second-last stage of amygdaloid kindling, in which stage they perseverated significantly longer than the controls and the other lesioned rats. These results suggest that areas of the frontal lobe participate in the elaboration and generalization of amygdaloid seizures in rats. Although retarded in rate, kindling nonetheless occurred in the lesioned rats, indicating that these cortical areas are not essential for the development of amygdaloid seizures.
Anesthetic Barbiturates in Refractory Status Epilepticus
Authors: Young G.B. , Blume W.T. , Bolton C.F. , Warren K.G.
Published: July 2016

Two patients with previous cerebral damage and seizures and three patients with acute inflammatory cerebral lesions developed status epilepticus. They were unresponsive to standard anticonvulsants, but anesthetic barbiturates (thiopental and pentobarbital) stopped the seizures promptly.
Bilateral Symmetrical Brain Lesions in Tricyclic Antidepressant Overdosage
Authors: Sabet Laila M.
Published: July 2016

The cerebral pathology in a case of tricyclic antidepressant overdose by amitriptyline is described. A review of the literature of similar cases is summarized. The relation of the cerebral lesions to the development of a hypertensive episode possibly induced by amitriptyline is discussed.
Glycosylated Hemoglobins In Friedreich's Ataxia
Authors: Draper P. , Shapcott D. , Langlois M. , Lemieux B.
Published: July 2016

In a study of glycosylated hemoglobins in Friedreich's Ataxia patients and in family members, the level was found to be higher in insulin dependent diabetics than in healthy non-diabetic control subjects (p<0.01), but was similar to non-ataxic diabetic control subjects. Results for non-diabetic Friedreich's Ataxia patients and siblings were similar to those for non-diabetic control subjects while levels were slightly increased in the heterozygotes. It is concluded that in Friedreich's Ataxia patients and family members (apart from the insulin dependent diabetics), there was no hyperglycemia sufficient to be detected as an increase in glycosylated hemoglobins.
Lecithin: Cholesterol Acyltransferase Activity and Fatty Acid Composition of Erythrocyte Phospholipids in Friedreich's Ataxia
Authors: Huang Y.S. , Marcel Y.L. , Vezina C. , Barbeau A. , Davignon J.
Published: July 2016

In a study of the fatty acid composition of erythrocyte membrane phospholipids in Friedreich's ataxia, a lower percentage of linoleic acid in phosphatidylcholine was demonstrated. An enzyme involving the exchange of lipids between plasma and erythrocyte membrane, lecithin: cholesteryl acyltransferase (LCAT) was also studied. It was found that the LCA Tactivity had a trend towards low values. However, crossing-over studies indicated that when the LCAT enzyme of patients was exposed to its own substrate it gave low activity values but that the result reverted to normal when control substrate was used
Effects of Glutamate and Aspartate on Ataxic Gait Induced by 3-Acetyl Pyridine in Rats
Authors: De Michele G. , Jolicoeur F.B. , Rondeau D.B. , Butterworth R.F. , Barbeau A.
Published: July 2016

The main purpose of this study was to examine the effects of intraventricular injections of glutamate and aspartate on the walking gait of rendered ataxic by the administration of 3-acetyl pyridine. Both amino acids significantly improved the walking gait of these animals. The effects of other substances known to have a stimulatory influence on locomotor activity in rats were also investigated. Amphetamine, apomorphine and thyrotropin releasing hormone (TRH) had no effect on the ataxic gait of 3-AP treated animals. Substance P significantly improved the gait of ataxic animals, but to a lesser extent than that seen with glutamate and aspartate.
Friedreich's Ataxia 1980 An Overview of the Physiopathology
Authors: Barbeau André
Published: July 2016

Phase three of the Quebec Cooperative Study of Friedreich's Ataxia was devoted to an understanding of the physiopathology of individual symptoms on the basis of previously discovered biochemical leads. The present paper attempts to pull these results together by presenting, as a hypothesis, a unifying scheme of possible interactions and relationships. The central core of this hypothesis is the demonstration in Friedreich's ataxia of a state of mitochondrial energy deprivation. This is indirectly responsible for such associated and important symptoms as muscle weakness, dying-back neuropathy, scoliosis and hypertrophic cardiomyopathy. Secondarily, and possibly as an independent but linkedevent, the entry of glucose into cells and pyruvate oxidation, are slowed down, favoring the development of diabetes. As a consequence, tissue concentrations of glutamic acid and aspartic acid are decreased, particularly in more vulnerable areas such as the cerebellum, brain stem and dorsal root ganglia. This tissue deficiency in putative excitatory neurotransmitters is directly responsible for the symptom of ataxia. This conclusion is reinforced by the correction of the ataxia in glutaexperimental animals, by the intraventricular injection of the same amino acids, and not by the injection of other stimulants of motricity. The observed mitochondrial energy deprivation could be the metabolic consequence of major changes in the linoleic acid (18:2) composition of inner mitochondrial membrane phospholipids, such as cardiolipin. Such decreases in membrane 18:2 could be the result of interference with the normal incorporation of this fatty acid to lipoproteins and/or cell membranes. It is at this level that the search for the specific enzyme defect in Friedreich's ataxia is continuing.
Migraines Inhabituelles
Authors: Roy Louis E.
Published: July 2016

From 123 hospital charts with a diagnosis of migraine, seen between the years 1974 and 1978, ten cases have been selected of complicated migraine and three cases of migraine with special features. A review, and a discussion of the clinical characteristics, and of the treatment of these unusual migraines has been made. The three cases with special features include migraine in association with hyperlipidemia, with Raynaud's disease and with meningioma.
The Relationship Between Cerebral Blood Flow and the EEG in Normals
Authors: Menon Devidas , Koles Zoltan , Dobbs Allen
Published: July 2016

Using the Xel33 inhalation technique, measurements of the blood flow to the left and right parietal and temporal regions of the cerebrum were obtained in 5 healthy individuals while simultaneously recording their EEGs. Up to 3 measurements were obtained from each of the subjects the first while they were mentally at rest and the others while they were engaged in prescribed forms of mental activity. Relationships between the measured blood flow through grey matter, initial slope index, relative grey weight, percent grey flow and power in the delta, delta-theta, alpha, beta and gamma rhythms of the EEG were examined. The results showed that for the subject group as a whole there was a strong correlation between the power present in the low frequency components of EEG and the grey flow and relative grey weight parameters of blood flow. On an individual basis, the observed relationships were highly variable particularly at high flow rates and at low relative grey weights, but became much more definitive at low flows and high weights. The results as they relate to previous work of this kind are discussed.
Oculodermal Melanosis (Naevus of Ota) Complicated by Multiple Intracranial Tumors
Authors: Horsey W.J. , Bilbao J.M. , Nethercott J. , Myers R. , Hoffman H.J.
Published: July 2016

A thirty-seven year old, East Indian female was admitted to St. Michael's Hospital on July 26, 1979 complaining of headache, diplopia, nausea and vomiting.

In April of 1978 she was referred to a neurologist for investigation of headache. Neurological examination was normal. An X-ray of her skull revealed a lytic lesion, 2 cms. in diameter, in the right frontal area. A bone scan showed no abnormality. Further study was refused. A left facial “birth mark” was noted.
Effect of Dietary Phenylalanine Restriction On Visual Attention Span In Mentally Retarded Subjects With Phenylketonuria
Authors: Giffin F.D. , Clarke J.T.R. , d'Entremont D.M.
Published: July 2016

An ABA within-subject study design was used to assess the effect of dietary phenylalanine restriction on the visual attention span of three mentally retarded males (9, 15, and 21 years old)with phenylketonuria. Visual attention span was measured by recording the amount of time the subjects visually fixated pictures projected on a screen according to a standardized test protocol. After 4-6 weeks of baseline testing (A-phase), each subject was placed on a phenylalaninerestricted diet, designed to maintain plasma phenylalanine levels at 0.3 - 0.9 mM, for 8-14 weeks (B-phase). A return to baseline phenylalanine intake (A-phase) was achieved by surreptitiously adding sufficient L-phenylalanine to the therapeutic diets to increase plasma concentrations of the amino acid to pretreatment levels. Diet treatment (B-phase) was associated with highly significant improvementsin visual attention span in two subjects (multivariate analysis of variance, P <<0.001); the third subject, the most retarded, showed no effect. No objectivelydemonstrable change in behavior was documented in any subject. However, the results support the view that phenylalanine toxicity extends beyond early childhood and that some component of the toxicity is reversible, even in severely retarded patients with phenylketonuria.
Tissue Albumin and Water Content in the Early Stages of Vasogenic Brain Edema Formation
Authors: Pash Michael P. , Tweed W. Arnold
Published: July 2016

Brain edema (BE) research lacks quantitative regional methods. We modified the method of Pappius and McCann (1969), who used radioactive iodinated I125 serum albumin (RISA) as a label for vasogenic BE fluid. To correct for intravascular plasma volume we used Cr51 labelled red blood cells and calculated equivalent extravascular plasma volume (EVPV). The modified RISA method was compared with a standard method for measuring increased tissue water, i.e. the change in wef.dry weights. Anesthetized rabbits were subjected to unilateral cortical freeze injury and sacrificed three hours later. The lesion corresponding to the area of blood brain barrier (BBB) breakdown was delineated by Evan's blue staining. That area and the two adjacent poles were sectioned. The contralateral hemisphere was used as the control. Good agreement was found between the two methods. By the modified RISA method, 4.08% of the wet weight of the injured hemisphere was EVPV while the decrease in dry weight corresponded to 5.56% edema. In the freeze lesion, however, where BBB breakdown occurred extravascular (EV)protein exceeded EV water, while adjacent to the lesion in the area with intact BBB, EV water exceeded EV protein. This suggests that the diffusion rate for water through brain tissue exceeds that of albumin in the early stages of vasogenic BE formation. We conclude that the modified RISA method is a satisfactory measure of regional vasogenic BE in acute animal experiments.
A Comparative Study of b- and d-Waves of the Electroretinogram, in Frogs (Rana Catesbeiana)
Authors: Molotchnikoff S. , Guertin-Laurin F.
Published: July 2016

The aim of this study was to compare the b- and d- waves of the ERG, evoked respectively by ON and OFF stimuli. The experiements were carried out on frogs. The d-wave is facilitated by light adaptation and relatively high stimulus intensity. The depth profiles of b- and dwaves are different. Aspartic acid abolished only the b-wave whereas nembutal affected the d-wave. These results suggested that b- and d-waves were produced by separate neuronal mechanisms, even though both appeared as positive deflection in the corneal ERG and were sensitive to K+ ion.
Pseudotumor Cerebri
Authors: Moffat F.L.
Published: July 2016

Pseudotumor cerebri is a clinical syndrome in which signs and, sometimes, symptoms of raised intracranial pressure are present hut in which mental and neurological function are unaffected. Therefore, the diagnosis is reached after mass and other structural causes of raised pressure have been excluded.

Many causes of pseudotumour have been suggested, not all of them well documented. Pathogenesis, however, includes cerebral edema, increased cerebral blood volume, and decreased cerebro spinal fluid (CSF) absorption.

Except for the risk of vision in a minority of cases, the prognosis is excellent.
Regional Cerebral Blood Flow in Patients with Aneurysms: Estimation by Xenon 133 Inhalation
Authors: Weir Bryce , Menon Devidas , Overton Thomas
Published: July 2016

Seventy six regional cerebral blood flow (rCBF) studies were conducted on 32 patients who had a total of 39 aneurysms. Twenty three of these patients were studied pre- and post-operatively. Normal values were obtained from a control group of 33 subjects, each of whom underwent one rCBF study. Flow was reduced following subarachnoid hemorrhage (SAH); it increased significantly postoperatively. Lower flows were associated with poorer clinical grades. There was a greater variation in regional distribution of flow immediately following SAH than in normals or in patients who had recovered from the acute phase. rCBF studies correlated with CT scans demonstrated that a progressive increase in ventricular size was accompanied by a progressive reduction in flow. In addition, intraventricular hemorrhage (IVH) was associated with a significant reduction in cerebral blood flow (CBF). No significant correlation between CBF and spasm was demonstrable.
Internal Carotid Artery Embolism by Shotgun Pellet
Authors: Sethi Jitendar M. , Rozdilsky Bohdan
Published: July 2016

Various examples of foreign body embolization of cerebral arteries, usually followed by serious consequences, have been reported (Lindberg et al, 1961; Chason et al, 1963; Steele et al, 1972; Wetli et al, 1972). However, a shotgun pellet entering the left atrium of the heart through a gunshot wound of the chest with subsequent embolic occlusion of one of the carotid arteries appears to be unique. It is the subject of this short communication.
Problems in the Diagnosis of Progressive Supranuclear Palsy (Steele — Richardson — Olszewski Syndrome)
Authors: Perkin George David , Lees Andrew John , Stern Gerald Malcolm , Kocen Roman Stefan
Published: July 2016

Five patients with progressive supranuclear palsy are described, in whom the ophthalmoplegia developed late in the course of the disease. In two, an internuclear component was identified in the ophthalmoplegia, and one patient had an alternating nystagmus of a type not previously described in this condition.

The late appearance of the ophthalmoplegia, with a corresponding delay in establishing the diagnosis, is compared to the similar experience of Pfaffenbach et al (1972) in six patients.

Other clinical features, previously seldom described, have been encountered. Dysphasia was seen in two cases, both of whom had evidence of cortical atrophy on neuro-radiological investigation. The evidence that cortical changes, in particular the presence of neurofibrillary tangles, may be a specific morphological characteristic of the disease rather than a chance association is discussed. Disorders of respiratory rhythm in four patients were similar to those described by Mastaglia et al. (1973), and indistinguishable from those occurring after encephalitis lethargica.

A review of cases resembling progressive supranuclear palsy in the early part of the century fails to show any with post-encephalitic features, nor does a search of reviews of eye movement disorders in encephalitis lethargica and post-encephalitic Parkinsonism provide comparable cases. None of the forty patients with post encephalitic Parkinsonism examined at the Highlands Hospital had a clinical picture resembling progressive supranuclear palsy.

It is suggested that neither on clinical nor pathological grounds is it justifiable to equate this disorder with known postencephalitic syndromes.
Long Term Induction of Kindled Seizures in Rats: Interhemispheric Factors
Authors: Nobrega José N. , Gaito John
Published: July 2016

Previous research indicated that sequential alternation of stimulation of certain homologous brain areas via chronically implanted electrodes resulted in oscillation of high and low latencies for convulsions. This phenomenon suggested the establishment of interhemispheric facilitatory-inhibitory effects as a result of repeated stimulation of the two brain sites. In the present study, the latency oscillation pattern was observed in split-brain rats as well as in bilaterally stimulated controls, but not in rats stimulated on one side only. Significant differences
were observed between split-brain and control rats in terms of initial kindling rates, duration of convulsions and type of oscillation. Results are discussed in the context of possible interhemispheric mechanisms involved in long term kindling.
Cerebral Hemodynamics in Migraine
Authors: Hachinski V. C. , Olesen J. , Norris J. W. , Larsen B. , Enevoldsen E. , Lassen N. A.
Published: July 2016

Clinical and angiographic findings in migraine are briefly reviewed in relation to cerebral hemodynamic changes shown by regional cerebral blood flow (rCBF) studies. Three cases of migraine studied by the intracarotid xenon 133 method during attacks are reported.

In classic migraine, with typical prodromal symptoms, a decrease in cerebral blood flow has been demonstrated during the aura. Occasionally, this flow decrease persists during the headache phase. In common migraine, where such prodr.omata are not seen, a flow decrease has not been demonstrated.

During the headache phase of both types of migraine, rCBF has usually been found to be normal or in the high range of normal values. The high values may represent postischemic hyperemia, but are probably more frequently secondary to arousal caused by pain.

Thus, during the headache phase rCBF may be subnormal, normal or high. These findings do not exclude the possibility of distension of the larger intracranial arteries during migraine headache, but the angiographic evidence, however limited, does not support this speculation.
A Community Neurologist's Personal Viewpoint on Neurological Training
Authors: Simpson Charles A.
Published: July 2016

A study of 200 patients referred to a community neurologist showed that 87.5% of the patients were seen in the office and only 12.5% in hospital. Neurological signs were present in 52% and 28.5% had neurological signs which materially affected the diagnosis. A questionnaire sent to several teaching centers showed that only one center sent students and residents to community neurologists' offices at all and in most centers the resident spent just 10 to 20% of his time seeing out-patients. It was felt that the balance of in-patient/out-patient teaching for students and residents was wrong, and that more emphasis should be placed on the neurological history than on the examination. Proposals are made to involve the community neurologist as well as the academic neurologist in the training of students and residents which would benefit all four groups.
Afterdischarge Thresholds and Kindling Rates in Dorsal and Ventral Hippocampus and Dentate Gyrus
Authors: Racine Ronald , Rose Patty A. , Burnham W. M.
Published: July 2016

Electrodes were implanted into dorsal hippocampus (CAI), ventral CAI, dorsal dentate gyrus or ventral dentate gyrus. Epileptiform afterdisvharge (AD) thresholds were lower in dorsal areas than in ventral areas. Dorsal areas, however, required a greater number of stimulations to develop (“kindle”) a fully generalized convulsion than did ventral areas. Thresholds and kindling rates in the dentate gyrus were intermediate between dorsal and ventral CAI, except for the ventral dentate which had higher AD thresholds than ventral CAI.

Secondary sites within the hippocampus subsequently kindled within a few stimulations following completion of kindling in the primary site, regardless of which hippocampal area served as the primary site.
Seven Cases of Gilles de la Tourette's Syndrome: Partial Relief with Clonazepam: A Pilot Study
Authors: Gonce M. , Barbeau A.
Published: July 2016

The histories of seven consecutive cases of Gilles de la Tourette's syndrome are presented to exemplify the range of clinical manifestations in this disease and to collate preliminary results with the new benzodiazepine, clonazepam, as a possible adjuvant therapy of this disorder. Controlled trials with clonazepam alone and in association with haloperidol are now justified. Five of our 7 patients had a positive family history of tics, and 2 a confirmed family history of gout. Because clonazepam improves myoclonia and tics and because its mechanism of action possibly involves serotonin, we thought it worthwhile to study simultaneously the relative roles of serotonin and dopamine metabolism in the production of tics, and their relationship to possible defects in purine metabolism in Gilles de la Tourette's syndrome.
Giant Cell Tumor of the Sphenoid Bone
Authors: Doshi Rasikbala , Chaudhari Abdul Basit , Thomson Gordon
Published: July 2016

The clinical and histological features of two cases of giant cell tumor of the sphenoid bone are described. Both presented with similar symptoms and signs, comparable to previously described cases. The problems in histological differential diagnosis are discussed and radiotherapy as the treatment of choice is suggested.
A Theory of the Mechanism of Cerebral Vasospasm and Its Reversal, the Role of Calcium and Cyclic AMP
Authors: Peterson Eric W. , Leblanc Richard
Published: July 2016

It is proposed that the basic mechanism of vasospasm which sometimes follows subarachnoid hemorrhage is dependent on increased free intracellular calcium ion produced by spasmogens from closely applied extravasated blood. Relaxation of this spasm occurs when the intracellular cyclic AMP levels are raised, resulting in sequestration of calcium ion by the vascular smooth muscle cell sarcoplasmic reticulum.
The Reactivity of Canine Cerebral Arteries to O2 and CO2 in Vitro
Authors: Steinbok P. , Kendall M.J. , Clarke R.J. , Peerless S.J.
Published: July 2016

The responses of canine middle cerebral arteries to changes in pCO2 and pO2 were tested in vitro. It was found that there was no response to changes in pCO2 from 38.1 mm. Hg to 26.6 mm. Hg. but there was some constriction of the vessels with lowering of the pCO2 below 26.6 mm. Hg and there was minimal dilatation of the vessels when the pCO2 was increased from 38.1 nan. Hg to 87.2 mm. Hg. There was no reresponse to changes in pO2 from more than 500 mm. Hg to 59.6 mm. Hg, but when pO2 was lowered below 50 mm. Hg there was a sudden, massive constriction of the arteries tested. It is postulated that this constriction is due to build-up of a substance (substances) during a period of hypoxia (pO2 < 50 mm. Hg). The significance of the results obtained are discussed.
Hemodynamic Findings in Friedreich's Ataxia
Authors: Cote M. , Davignon A. , Elias G. , Solignac A. , Geoffroy G. , Lemieux B. , Barbeau A.
Published: July 2016

Thirteen patients with classical Friedreich's ataxia underwent cardiac catheterization with recordings of retrograde cardiac pressures, measurements of cardiac output and calculation of the left ventricular volumes and mass. The cardiomyopathy in Friedreich's ataxia falls into the hypertrophic group of cardiomyopathies with decreased compliance of ventricular myocardium, varying degrees of concentric and asymmetric hypertrophy and outflow tract obstruction. Although there is no clear parallel between the degree of abnormal hemodynamic findings and the degree of neurological impairment, severely handicapped patients may present a diffusely hypertrophied and hypokinetic left ventricular myocardium.
Clinical Laboratory Findings in Friedreich's Ataxia
Authors: Butterworth R.F. , Shapcott D. , Melançon S. , Breton G. , Geoffroy G. , Lemieux B. , Barbeau A.
Published: July 2016

All clinical laboratory tests carried out in 4 groups of patients with the diagnosis of typical or atypical Friedreich's ataxia have been found to be within the normal range. In this prospective study of 50 patients, a number of findings previously reported to be abnormal in the literature, have not been confirmed.
The Placing Reaction in Adult Neurology
Authors: Botez M.I.
Published: July 2016

The first description of the placing reaction (PR) in 12 human adult cases is given. The optimum position for eliciting the placing reaction (PR) is the dangling leg posture, i.e. the same as for the forward groping of the foot. There are three forms of PR quite similar to those noticed in animal physiology, i.e. the visual-PR, the dorsum-PR and the sole-PR. The term contact placing is preferred for the last two forms. The PR is encountered only in patients displaying a forward and medial groping of the foot as well as a groping of the hand on the same side. The PR is usually ipsilateral to the main cerebral lesion but there was no single case with a well limited unilateral lesion. An involvement of retrorolandic areas seems to be necessary for the occurrence of PR. It is concluded that both groping phenomena and the PR are highly coordinated reflexes subserving self-preservation and belonging to antigravity mechanisms, i.e. the standing posture.
The Location of Conduction Abnormalities in Human Entrapment Neuropathies
Authors: Brown William F. , Ferguson Gary G. , Jones Michael W. , Yates Stephen K.
Published: July 2016

Direct stimulation of 23 median, 13 ulnar and 2 peroneal nerves at the time of surgical exploration has been used to locate, and characterize the conduction abnormalities in the
nerves. The most frequent location of the major conduction abnormalities in the median nerve was in the first 1-2 cm distal to the origin of the carpal tunnel. In the ulnar nerve the important conduction abnormalities were located most frequently in the segments 1 cm proximal and distal to the medial epicondyle. In the peroneal nerve the major conduction abnormalities occurred proximal or distal to the entry point of the common peroneal nerve into the peroneus longus
muscle.
Classification of Aphasic Phenomena
Authors: Kertesz Andrew
Published: July 2016

A brief but comprehensive survey of classifying aphasia reveals that most investigators describe at least four major groups, conveniently labelled Broca's, Wernicke's, anomic and global. Conduction and transcortical aphasias are less generally described and modality specific syndromes rarely, if ever, exist purely. The controversy between unifiers and splitters continues but objective numerical taxonomy may solve some of the problems of classification.
The Relative Significance of Factors Affecting Postoperative Survival in Astrocytomas, Grades One and Two
Authors: Weir Bryce , Grace Michael
Published: July 2016

The records of 107 patients with supratentorial astrocytomas, Grades 1 and 2, treated surgically between I960 and 1970 were analyzed. Abstracted clinical data was retrospectively analyzed with respect to the relative significance of different factors affecting survival. Twenty-eight patients were alive at the time of follow-up in 1974. Age, clinical grade at surgery and radiation therapy were the most important determinants of prolonged postoperative survival.

The more malignant astrocytomas occurring in the Province of Alberta had previously been reviewed. The present study was carried out to demonstrate the relative influence of histologic grade of astrocytomas and to analyze the factors affecting postoperative survival in the low grade, supratentorial astrocytomas.
Treatment of Experimental Allergic Neuritis With Prednisolone
Authors: Feasby T.E. , Gilbert J.J. , Hahn A.F. , Lovgren D.S.
Published: July 2016

Experimental allergic neuritis (EAN) in Lewis rats was treated with prednisolone given prophylactically or therapeutically. Rats treated from the time of immunization with myelin or after the establishment of clinical disease improved more rapidly than controls. Treatment at the onset of clinical signs resulted in less severe disease and more rapid recovery. Rats treated just prior to the onset of clinical signs (day 10) did not develop significant clinical disease and appeared to have less inflammation in their nerves and nerve roots on microscopic examination.
Massive Necrosis of the Brain in Rabies
Authors: Dolman C.L. , Charlton K.M.
Published: July 2016

A young man developed virologically proved hydrophobic rabies three months after being scratched on the cheek by a bat in Northern Alberta. He became comatose after 8 days and died 5 weeks after vigorous therapy with immune globulin, interferon and Vidarabine, and excellent maintenance of oxygenation. Electroencephalographic deterioration was gradual. At postmortem examination, the brain showed widespread loss of nerve cells, mild diffuse inflammatory changes and no Negri bodies. The lesions of massive laminar necrosis of the cerebral cortex, total loss of Purkinje cells with preservation of the granule cell layer, and severe softening of the amygdala are attributed to direct viral action rather than to anoxia or brain swelling with respirator brain.
Intracranial Endodermal Sinus Tumour — Role for Chemotherapy
Authors: Anderson Brian A.
Published: July 2016

Pineal tumours are commonly of germ cell origin. The endodermal sinus tumour is a rare histologic subtype that is highly malignant and radioresistant. A single case is described and compared to six reported intracranial cases in which chemotherapy was used in addition to surgery and/or radiation. Although a beneficial effect of chemotherapy cannot be proven, the probable benefit in non-CNS cases requires that it be strongly considered. A uniform approach to diagnosis, investigation and treatment for intracranial tumours of this type is suggested.
A Randomized Controlled Trial of Amantadine in Fatigue Associated With Multiple Sclerosis
Authors:   The Canadian MS Research Group
Published: July 2016

One hundred and fifteen patients with definite multiple sclerosis (M.S.) and chronic persistent fatigue were studied. This ten-week cross-over study consisted of a 2-week baseline period and two 3-week treatment periods separated by a 2-week washout. Patients received either amantadine 100 mg bid or matching placebo capsules. Fatigue, the effect of fatigue on an individually pre-selected activity and its effect on activities of daily living, were evaluated. Amantadine produced a small but statistically significant decrease in fatigue. An important placebo effect was noted. Mean fatigue during the washout period was lower than during the placebo run-in period, independently of which treatment had been given first. Side effects were numerous both on amantadine and on placebo. Only insomnia was significantly more common with amantadine.
Tizanidine Versus Baclofen in the Treatment of Spasticity in Patients with Multiple Sclerosis
Authors: Bass B. , Weinshenker B. , Rice G.P.A. , Noseworthy J.H. , Cameron M.G.P. , Hader W. , Bouchard S. , Ebers G.C.
Published: July 2016

Tizanidine (Sirdalud) was compared to baclofen (Lioresal) in a randomized, double-blind, cross-over trial. Each medication was introduced over a three week titration period and then maintained at the highest tolerated dose for five weeks. The two treatment phases were separated by a one week drug withdrawal and a two week washout period. Sixty-six patients entered the trial and forty-eight completed both treatment phases. At the end of the trial, neurologists and physiotherapists thought that baclofen was superior on the basis of perceived efficacy and tolerance (p≤0.05). Although the efficacy of tizanidine or baclofen was judged as good to excellent by 24 and 39% of patients respectively, this difference was not statistically significant. Muscle weakness was the most common adverse effect. This was significantly more troublesome in patients treated with baclofen. Somnolence and xerostomia were more common in patients treated with tizanidine. Both baclofen and tizanidine appear to be useful adjuncts in the treatment of spasticity in patients with multiple sclerosis. Preference of either drug is tempered principally by side-effects.
Multimodal Evoked Potential Studies in Leukodystrophies of Children
Authors: De Meirleir L.J. , Taylor M.J. , Logan W.J.
Published: July 2016

Evoked potentials were studied in 22 children with leukodystrophy [10 metachromatic leukodystrophy (MLD), 4 Pelizaeus-Merzbacher (PM), 3 Krabbes, 2 adrenoleukodystrophy (ALD), and one each of Alexander's, Canavan's and multiple sulphatase deficiency (MSD) diseases]. The ABRs were abnormal in all patients (except for the younger ALD), but varied with the type of leukodystrophy. The PM and Krabbes patients had abnormal ABRs with a loss of the rostral waves, accompanied in Krabbes with delayed I-III interpeak latencies; in MLD, ALD and MSD prolonged interpeak latencies were found. Three patients who had no clinical signs, but were positively diagnosed as MLD on the basis of absent arylsulphatase A, also had abnormal ABRs. The SEPs were abnormal in all patients. Cortical SEPs were absent in 16 and abnormal in 5 who were in the earlier stages of their disease. Cervical SEPs were within normal limits except for the Krabbes and MLD patients studied, who showed peripheral slowing. The VEPs were normal in only 6 and, unlike the ABRs and SEPs, did not seem to covary with clinical severity across the various leukodystrophies but did correlate with disease progression. Thus, multimodal EPs are useful in the diagnostic differentiation of the leukodystrophies.
Harrington Rod Instrumentation: A Cause of Brown-Sequard Syndrome
Authors: Van Orman C.B. , Darwish H.Z.
Published: July 2016

We describe a child with Brown-Sequard syndrome beginning at C8-T1, immediately following Harrington rod instrumentation for idiopathic scoliosis. We believe this is most likely related to either a common variation of the branches of the anterior spinal artery to one side or due to rupture of the ipsilateral spinal vein. This possible complication should be considered in patients undergoing Harrington rod instrumentation.
Electrophysiological Monitoring During Acoustic Neuroma and Other Posterior Fossa Surgery
Authors: Linden R.D. , Tator C.H. , Benedict C. , Charles D. , Mraz V. , Bell I.
Published: July 2016

Techniques used to monitor the function of the seventh and eighth cranial nerves during acoustic neuroma and other posterior fossa surgery are reviewed. The auditory brainstem response (ABR), electrocochleogram (ECochG) and direct recording from the auditory nerve (CNAP) were compared. The best technique is the ECochG, although in many cases, the CNAP should be used as a back-up technique. The CNAP is especially useful for the identification of the auditory nerve. Both can provide real-time feedback on the physiological integrity of the auditory nerve. The ABR may be helpful in monitoring brainstem function. For some procedures, optimal monitoring requires the combined recording of all three techniques.

Monopolar constant-voltage intracranial stimulation of the facial nerve is helpful for the identification and preservation of the facial nerve. Audio monitoring of spontaneous electromyographic activity provides real-time feedback on the effect of surgical manipulation of the nerve. Monitoring of ephaptic transmission in the facial nerve during microvascular decompression for hemifacial spasm aids in the identification of the offending vessel.
Immunocytochemical Analysis of Intermediate Filaments in Human Ependymal Tumors
Authors: Izukawa D. , Lach B.
Published: July 2016

The peroxidase anti-peroxidase technique was used for localization of glial fibrillary acidic protein (GFAP) and vimentin (VM) in 19 ependymal tumors in order to determine if a unique pattern of intermediate filament (IF) expression could be demonstrated. Cytokeratin (CK) immunoreactivity was examined in a subgroup of 7 tumors with papillary pattern. Nineteen non-ependymal neuroectodermal tumors were used as controls. Ependymomas, subependymomas and astrocytomas were positive for both IF. Oligodendrogliomas, oligodendroglial portions of mixed gliomas and the majority of medulloblastomas were negative for GFAP and VM. Areas of poor differentiation in all tumors demonstrated little expression of any IF. A composite ependymoma/choroid plexus papilloma showed the presence of GFAP, VM and CK in the papillomatous portion only. Four papillary ependymomas were negative for CK. This study emphasizes the parallel distribution of GFAP and VM in well differentiated ependymomas and other glial tumors and casts doubt upon the concept of VM as a marker for de-differentiation in neuroectodermal neoplasia.
Magnetic Resonance Imaging in the Assessment of Disease Activity in Multiple Sclerosis
Authors: Paty Donald W.
Published: July 2016

Magnetic resonance imaging (MRI) has been shown to be a good method of visualizing the lesions in MS. We have studied several applications of MRI to the evaluation of patients and experimental models. In diagnosis, MRI is the most sensitive test for the demonstration of dissemination of lesions in space. Pathological correlation studies show that MRI reliably measures the extent of chronic demyelination. Experimental studies show that MRI detects acute inflammatory lesions and measures their evolution. MRI also is a reliable measure of the extent of the MS process, serial MRI scans detect evidence of disease activity in MS not always disclosed by clinical evaluation. MRI will have an enormous future impact on the evaluation of patients in clinical studies and in understanding the evolution of pathological processes.
Botulinum Toxin Injections in the Treatment of Blepharospasm, Hemifacial Spasm, and Eyelid Fasciculations
Authors: Kraft Stephen P. , Lang Anthony E.
Published: July 2016

Seventy-six patients with blepharospasm (mean age 56.9 years) received 248 injection treatments with botulinum A exotoxin (mean 3.1 treatments per patient): 87.0% of treatments led to total relief of spasms for a mean interval of 14.1 weeks. The average duration of response remained fairly constant over the first six injection series, although patients with the most severe spasms had shorter intervals than patients with less severe symptoms. Twenty patients with hemifacial spasm (mean age 56.9 years) received 44 treatments (mean 1.9 treatments per patient): In 93.1% of cases there was total relief of periocular and perioral spasms, with a mean interval of 17.4 weeks. The average duration of response for the third series of treatments was much shorter than the mean durations for the first two treatments. Side effects were always transient and included ptosis (23.3%), dry eyes (18.1%), tearing (5.5%), and strabismus (1.4%). No patient had a systemic reaction to the drug. Chronic benign eyelid fasciculations were also successfully treated in 3 patients with single treatments.
Changes in Serum Anticonvulsant Levels with Febrile Illness in Children with Epilepsy
Authors: Goulden Keith J. , Camfield Peter R. , Camfield Carol S. , Tibbles John A.R. , Dooley Joseph M. , Fraser Albert D. , Renton Kenneth W.
Published: July 2016

Changes in anticonvulsant serum levels during intercurrent illness may cause toxicity or decreased seizure control in children with epilepsy. We studied prospectively the effect of intercurrent illness and its treatment in 111 children being treated with AC monotherapy. Free fraction and total serum AC levels were determined when the child was well, on the fifth day of any illness with fever and one month after recovery. There were 55 episodes of febrile illness in 39 children during the study period. Twelve illnesses were associated with significant increases or decreases in serum AC levels; 7 children became clinically toxic; 1 child had increased seizures during illness. The mechanisms of AC level changes appeared to include interaction with antibiotics, with antipyretics or with viral illness. Amoxycillin and acetaminophen did not appear to interact with the AC's used. Physicians caring for children with epilepsy should be aware of the frequency and complexity of potential interactions between intercurrent febrile illness and anticonvulsant medication.
Un aperçu «échologique» des sciences neurologiques ultramontaines
Authors: Poirier Louis J.
Published: July 2016

En premier lieu, je tiens à féliciter les principaux responsables de l'organisation de cette première journée des sciences neurologiques de l'Université de Montréal, soit les docteurs Guy Bouvier, Pierre Duquette, Yves Lamarre, Gérard Mohr, Pedro Molina-Negro et Serge Rossignol. Grâce à eux, à Madame Hélène Auzat, secrétaire, et à bien d'autres, cette journée s'est avérée un franc succès.
Interactions entre recherches fondamentale et clinique. Deux exemples tirés d'une expérience personnelle
Authors: Albe-Fessard D.
Published: July 2016

Basic research helps to understand clinical knowledge which in turn leads to new fundamental research. Two examples are taken from the studies of the anomalies existing in the brain of patients suffering from Parkinson disease or deafferentation Pain. The researches performed to solve these two problems for the past 40 years (in particular by the author's working group) are replaced in the international context. In Man the precision of stereotaxic technique was improved by using the organisation of the somato-sensory thalamic projection described in animals by the physiologists. In the thalamus of Parkinsonian patients, the presence of cells bursting at tremor frequency led to search for the relations existing between the striato-nigral system and the motor thalamus. In the patients suffering from deafferentation Pain, a special role is attributed to the medial thalamus, whose characteristics were formerly studied in animals. The origin of the deafferentation Pain is nowadays studied in an animal model of this disease.