bluuebunny/crossref_metadata_2025 huggingface dataset:
10.—Norm Inequalities for C*-algebras
Authors: Duncan J. , Taylor P. J.
Published: July 2016

SYNOPSIS

Some characterisations of commutatitivity for C*-algebras are given in terms of inequalities involving sums and products of self-adjoint elements, and optimal constants are obtained for the corresponding inequalities for non-commutative C*-algebras.
The exceptional condition and unboundedness of solutions of hyperbolic systems of conservation type
Authors: Jeffrey A.
Published: July 2016

Synopsis

This paper is concerned with quasilinear hyperbolic systems of conservation type and establishes two main results. The first is that when a general system is considered in one space dimension and time, then the exceptional nature of a characteristic field implies the coincidence of a shock with one of the characteristics of that field. The second result involves the demonstration by example that quasilinear hyperbolic systems of conservation type may possess solutions that become unbounded after only a finite time, even though they are exceptional.
Inverse semigroups generated by a pair of subgroups
Authors: McAlister D. B.
Published: July 2016

Synopsis

The aim of this paper is to describe the free product of a pair G, H of groups in the category of inverse semigroups. Since any inverse semigroup generated by G and H is a homomorphic image of this semigroup, this paper can be regarded as asking how large a subcategory, of the category of inverse semigroups, is the category of groups? In this light, we show that every countable inverse semigroup is a homomorphic image of an inverse subsemigroup of the free product of two copies of the infinite cyclic group. A similar result can be obtained for arbitrary cardinalities. Hence, the category of inverse semigroups is generated, using algebraic constructions by the subcategory of groups.

The main part of the paper is concerned with obtaining the structure of the free product G inv H, of two groups G, H in the category of inverse semigroups. It is shown in section 1 that G inv H is E-unitary; thus G inv H can be described in terms of its maximum group homomorphic image G gp H, the free product of G and H in the category of groups, and its semilattice of idempotents. The second section considers some properties of the semilattice of idempotents while the third applies these to obtain a representation of G inv H which is faithful except when one group is a non-trivial finite group and the other is trivial. This representation is used in section 4 to give a structure theorem for G inv H. In this section, too, the result described in the first paragraph is proved. The last section, section 5, consists of examples.
Some unsolved problems of unknown depth about operators on Hilbert space
Authors: Halmos P. R.
Published: July 2016

Synopsis

The paper presents a list of unsolved problems about operators on Hilbert space, accompanied by just enough definitions and general discussion to set the problems in a reasonable context. The subjects are: quasitriangular matrices, the resemblances between normal and Toeplitz operators, dilation theory, the algebra of shifts, some special invariant subspaces, the category (in the sense of Baire) of the set of non-cyclicoperators, non-commutative(i.e. operator) approximation theory, infinitary operators, and the possibility of attacking invariance problems by compactness or convexity arguments.
Formation theory and groups of automorphisms of -groups
Authors: Tomkinson M. J.
Published: July 2016

Synopsis

Further results from the theory of finite soluble groups are extended to the class of locally finite groups with a satisfactory Sylow structure. Let be a saturated U-formation and A a -group of automorphisms of the -group G. A is said to act -centrally on G if G has an A-composition series (Λσ/Vσ; σ ∈ ∑) such that A induces an f(p)-group of automorphisms in each p-factor Λσ/Vσ. We show that in this situation A is an -group, thus generalising the result of Schmid [8]. Associated results of Schmid and of Baer are also extended to the infinite case.
Hilbert boundary value problems—a distributional approach
Authors: Orton Marion
Published: July 2016

Synopsis

Hilbert boundary value problems for a half-space are considered for analytic representations of Schwartz distributions: given data g ∈D'(ℛ) and a coefficient x we seek functions F(z) analytic for Jmz≠0 whose limits exist in D'(ℛ) and satisfy F+—XF– = g on an open subset U of the real line R. U is the complement of a finite set which contains the singular support and the zeros of X·X and its reciprocal satisfy certain growth conditions near the boundary points of U. Solutions F(z) are shown to exist, and their general form is determined by obtaining a suitable factorisation of x.
A note on the exterior problem for linear viscoelasticity
Authors: Beevers C. E.
Published: July 2016

Synopsis

Some continuous dependence theorems are presented for classical solutions of initial-boundary value problems in viscoelastic materials which occupy the exterior of a bounded domain in Euclidean three space. Two broad classes of anisotropic viscoelastic materials are considered. The theorems are proved by a combination of the Protter and the Graffi methods.
An elliptic boundary value problem for strongly non-linear equations in unbounded domains
Authors: Mustonen Vesa
Published: July 2016

Synopsis

The existence of a variational solution is shown for the strongly non-linear elliptic boundary value problem in unbounded domains. The proof is a generalisation to Orlicz-Sobolev space setting of the idea introduced in [15] for the equations involving polynomial non-linearities only.
An oscillation theorem for characteristic initial value problems in linear hyperbolic equations
Authors: Pagan Gordon
Published: July 2016

Synopsis

It is established that under certain restrictions the solution u of the characteristic initial value problem uxy+g(x, y)u = 0, u(x, 0) = p(x) and u(0, y) = q(y), where p(x) > 0 and q(y) > 0, in [0, ∞) x [0, ∞) changes sign along a monotonic decreasing curve which is asymptotic to the axes.
A Review of Acrylamide Neurotoxicity Part I. Properties, Uses and Human Exposure
Authors: Spencer Peter S. , Schaumburg Herbert H.
Published: July 2016

Two factors have stimulated the present intense investigation of acrylamide neurotoxicity. These are the health hazard accompanying the vast and increasing industrial production of acrylamide, and the promise of illuminating the mechanism of dying-back disease in the human nervous system by employing acrylamide as an experimental tool. The present paper discusses the industrial uses of acrylamide, its regulation, and the prevention, detection and clinico-pathologic features of human intoxication. Bearing on the cumulative nature of acrylamide neurotoxicity, separate sections review the chemistry, biochemistry, toxicology and metabolic fate of acrylamide. Clinical, electrophysiological and morphologic data on acute and chronic acrylamide intoxication of experimental animals, and possible etiologies of nervous system damage, are considered in detail in a companion paper (P. S. Spencer and H. H. Schaumburg, Canadian Journal of Neurological Sciences, 1:151, 1974).
Patterns of Motoneuron Dysfunction and Recovery
Authors: McComas Alan , Upton Adrian , Jorgensen Per
Published: July 2016

Electrophysiological studies have been carried out on five patients with neuropathies of different etiologies. In each patient serial estimates were made of the numbers of functioning motor units in various muscles. It was found that the intensity of the neuropathic process and the rate of recovery differed in a consistent way among the motoneuron pools investigated. The lesion was more severe in extensor digitorum brevis neurons than in thenar neurons, while the hypothenar ones were least affected. A stage of partial synaptic failure has been recognized in which a motoneuron appears to be no longer able to excite a muscle fiber, but still capable of maintaining certain trophic activities. By comparing the number of functioning motor units with the size of the maximum evoked muscle response it has been possible to detect the adoption of denervated muscle fibers by axonal sprouts from ‘healthy’ surviving neurons (collateral reinnervation). Lastly, in some muscles it appears that the adopted muscle fibers may subsequently be recaptured by the original motoneurons following recovery of the latter from the neurotoxic insult.
Respiratory Inhibitory Apraxia
Authors: Atack E.A. , Suranyi Leslie
Published: July 2016

Ten patients have been described showing inability to stop breathing on command, spontaneous respiration and voluntary respiratory stimulation being unaffected. This abnormality not previously described in the literature, we feel should be named respiratory inhibitory apraxia (R.I.A.). The anatomical organization of respiration is briefly reviewed. R.I.A. is often associated with other forms of apraxia or motor impersistence. It is thought that the urinary and bowel incontinence present in some of these cases might also represent a form of inhibitory apraxia. Information is presented which supports the view that respiratory inhibitory apraxia is due to a minor hemisphere lesion, usually deepseated. Our one autopsied case showed a lesion in the descending motor pathways in the internal capsule, in middle cerebral artery branch territory, disconnecting the voluntary respiratory inhibitory center in the cortex in anterior and middle cerebral cortical branch territories.
Morphological Responses of Cerebral Tissues to Temporary Ischemia
Authors: Dodson Ronald F. , Tagashira Yukio , Kawamura Yasuo , Chu Lena Wai-Fong
Published: July 2016

The ultrastructural responses of cerebral tissue following temporary periods (½, 1, 2, 3, or 4 hour) of right, middle cerebral artery, (MCA) occlusion were studied acutely after a 3 day or 7 day interval following the removal of the MCA clip. Cortical and basal ganglia tissues for each ischemic duration were compared at 3 post-occlusive periods (acute, 3 days, 7 days).

With the short periods of ischemic insult (½, 1, 2, 3, and 4 hour), the temporal and insular cortex contained no greater changes in the 7 day group than in the 3 day group.

The basal ganglia were more susceptible to MCA occlusion as indicated by more marked cytological changes and/or necrosis in all intervals of ischemia.
Central Mechanisms of Tremor in Some Feline and Primate Models
Authors: Lamarre Y. , Joffroy A.J. , Dumont M. , De Montigny C. , Grou F. , Lund J.P.
Published: July 2016

For several years our interest has been in a postural Parkinson-like tremor at 4-6/sec. which can be produced in the monkey by lesions of the central nervous system. We have also studied the effects of harmaline, a drug which evokes or intensifies the Parkinson-like tremor in lesioned animals and which also induces a fine, generalized tremor at 7-12/sec. in normal animals. The results obtained so far indicate that these two types of tremor are generated by two independent central mechanisms which do not require the integrity of peripheral feedback loops. The experimental Parkinson-like tremor is generated by a thalamo-cortical mechanism while the olivo-cerebellar system is responsible for the faster ”physiological“ tremor. Similar tremor mechanisms may be involved in some movement disorders in man.
Why Transcortical Reflexes?
Authors: Wiesendanger Mario , Rüegg Dieter G. , Lucier Gregory E.
Published: July 2016

Experiments in humans and in monkeys have indicated that load perturbations, occurring during voluntary movements and postural activity, may be automatically compensated for. Overall muscle stiffness opposing load changes is determined by the viscoelastic properties of the muscle, by segmental reflex actions and finally by long-loop reflexes. Under certain circumstances, for instance when the subject or the experimental monkey is “prepared” to counteract perturbations which are unpredictable in time, the long-loop “reflexes” appear to be responsible for most of the corrective muscle tension. Experiments in anaesthetized monkeys revealed that signals from stretch afferents reach neurons of the motor cortex, possibly via a relay in the cortical area 3a. The latencies of these responses to well controlled muscle stretches were in the same range as motor cortical cell discharges recorded in alert monkeys subjected to load perturbations. Furthermore, these responses of cells in the motor cortex also had the appropriate timing to indicate a causal relationship with the long-latency electromyographic responses to load changes referred to above. These experimental results therefore strongly support the hypothesis, first proposed by Phillips (1969), of a transcortical servoloop adjusting motor cortical output according to the load conditions in which movements are performed.

The major advantage of transcortical regulations as opposed to segmental regulations, seems to be a powerful gain control acting at the cortical level; it was repeatedly shown that the long-loop reflexes are strongly modifiable and under voluntary control. It is suggested that an adaptive gain control at the cortical level is a prerequisite to preserve the complex capabilities of the motor cortex as the chief "executive" for skilled, preprogrammed movements. A loss of this adaptive gain control may be, at least partly, the cause of motor disorders such as rigidity in Parkinsonian patients, as reported by Tatton and Lee (1975). It is suggested that further investigations of the control of transcortical reflexes may aid in the understanding of the pathophysiology of motor disabilities.
Patterns of Memory Performance in the Neurologically Impaired Aged
Authors: Perez Francisco I. , Gay Joe R.A. , Taylor Ronald L. , Rivera Victor M.
Published: July 2016

The specific behavioral manifestation associated with the different disorders producing the syndrome of dementia have remained poorly investigated. We examined the memory performance of three distinct groups of patients with dementia secondary to Alzheimer's disease (AD) multiple-infarctions (MID) and vertebrobasilar insufficiency (VBI) on the ten subtests of the Wechsler Memory Scale (WMS). Statistical methods of analysis were used to maximize the differences between the groups. Univariate statistical procedures revealed that the AD group performed significantly and consistently lower than the two cerebrovascular groups. There were no significant differences between the two cerebrovascular disease groups, even though the MID group tended to perform consistently more poorly than the VBI group. For heuristic and conceptual purposes as well as to determine which combination of the ten WMS variables produced the “best” statistical model differentiating the groups the data was analyzed by multivariate techniques. A discriminate function analysis obtained a 100% valid positive hit rate in discriminating among the three groups. One hundred percent diagnostic accuracy was also obtained in discriminating between MID and AD as well as AD and VBI. The two cerebrovascular groups tended to overlap in their probability distributions with an 81% hit rate. Different predicitive statistical models were identified to differentiate the various diagnostic groups. It was possible to discriminate the three diagnostic groups by different patterns of memory performance.
Technical Report: Method of Fixation of Subluxed or Dislocated Cervical Spine Below C1-C2
Authors: Tucker H.H.
Published: July 2016

A method of internal fixation of adjacent vertebrae that have been dislocated or subluxed has been presented. In the author's hands and in the hands of associates this has proven to be a satisfactory method of fixation in this type of injury. It has the advantage over many other posterior techniques that only two vertebrae are fixed together.
Kindling, Unit Discharge Patterns and Neural Plasticity
Authors: Racine Ronald , Tuff Larry , Zaide Josef
Published: July 2016

Two approaches to the study of the kindling phenomenon were discussed: 1) an attempt to identify the pattern of neural activity required to produce the changes underlying kindling and 2) an investigation into the nature of those changes. Three experiments were reported that used the neocortical transcallosal system as a monosynaptic model system in which to study possible synaptic mechanisms of the kindling effect. Experiment I showed an increase in the transcallosal evoked potential following neocortical kindling. Experiment II showed an increase in the strength of the transcallosal evoked cell discharge following neocortical kindling. Experiment III reported the results of an histological examination of neocortical tissue in kindled and non-kindled animals using the Golgi-Cox technique. Spine density, spine dimension and branching were measured for pyramidal cell apical dendrites. No differences were found between primary and secondary (contralateral) foci or between kindled and non-kindled animals.
Seizures Following Intracranial Surgery: Incidence in the First Post-Operative Week
Authors: Matthew E. , Sherwin A.L. , Welner S.A. , Odusote K. , Stratford J.G.
Published: July 2016

Consecutive craniotomies (118) drawn from major hospitals, and performed for disorders other than epilepsy or acute trauma were reviewed. The final diagnosis included tumor (70), subdural hematoma (13), aneurysm (10), arteriovenous malformation (7), and miscellaneous lesions (18). Eighty-seven (73.7%) patients had not experienced seizures prior to neurosurgery, II of these (12.6%) had a seizure within the first week, in six the attack occurred within 24 hours, and of these three had further attacks. In contrast, of the 31 patients (26.3%) that had one or more seizures prior to operation, II patients (35.5%) had seizures within the first week. In ten patients seizures occurred within the first 24 hours and of these seven had one or more recurrences later in the week. Anticonvulsant drugs were administered to 72 patients before operation, including all those with a history of seizures, hut loading doses were not utilized to ensure therapeutic levels. In patients with predisposing factors to postoperative seizures, anticonvulsant drugs should be administered before or immediately following craniotomy in adequate dosage to rapidly achieve and maintain effective plasma levels. Phenytoin, owing to its minimal sedative effects is the drug of choice. A loading dose of18 milligrams per kilogram can be safely administered as an admixture to an intravenous infusion of 0.9% saline with careful monitoring of cardiopulmonary function.
Dysplastic Vertebral Artery With Paradoxical Infarction
Authors: Curry Bernadette , Sima Anders A.F.
Published: July 2016

A rare abnormality of the vertebral artery is described in a 63-yearold male. The right vertebral artery consisted of several minute endothelial lined channels which failed to perfuse at autopsy. The anterior spinal artery showed areas of muscular media dysplasia. These anomalies were associated with large posterior communicating arteries. The terminal event was precipitated by thrombotic occlusion of the left vertebral artery. The embryological aspect of these anomalies are discussed.
Friedreich's Ataxia in the South of Italy : A Clinical and Biochemical Survey of 23 Patients
Authors: Campanella G. , Filla A. , De Falco F. , Mansi D. , Durivage A. , Barbeau A.
Published: July 2016

We report a clinical and biochemical survey of 23 patients with Friedreich's ataxia from southern Italy. They were studied clinically and by means of a clinical rating scale devised by us (Inherited Ataxias Clinical Rating Scale). Laboratory tests, based on the Quebec Cooperative Study, were also performed on our patients. No major clinical or biochemical differences were found between Italian and Canadian patients. Investigation of CSF monoamine metabolites showed that HVA decreased after probenecid and metoclopramide loading
Active Pyruvate Dehydrogenase In Platelets From Friedreich's Ataxia Patients
Authors: Filla A. , Butterworth R.F. , Barbeau A.
Published: July 2016

Pyruvate dehydrogenase (PDH) activity was measured in platelets from 10 patients with Friedreich's ataxia, and 10 age-matched healthy control subjects. Both total PDH and active PDH activity were measured. There were no significant differences between the two groups.
Effect of Asparagine, Glutamine and Insulin on Cerebral Amino Acid Neurotransmitters
Authors: Butterworth Roger F. , Landreville France , Hamel Edith , Merkel Andrea , Giguere François , Barbeau André
Published: July 2016

Treatment of rats with asparagine or glutamine caused substantial increases in glutamine concentrations in cerebellum and medulla oblongata. Insulin treatment caused a diminution of glutamate and GA BA in these regions of brain. Since it is now well-established that glutamine is a very efficient precursor of the neurotransmitter pool of glutamate in mammalian brain, treatment with asparagine or glutamine could be of therapeutic (replacement) value in the treatment of neurological disorders such as Friedreich's ataxia, in which cerebral glutamate concentrations have been found to be diminished.
Spongiform Encephalopathy of Long Duration: A Family Study
Authors: Rice G.P.A. , Paty D.W. , Ball M.J. , Tatham R. , Kertesz A.
Published: July 2016

Seven members of a family succumbed to prolonged dementing illnesses. In two, whose dementia lasted 10 years, spongiform encephalopathy was found at autopsy. This duration of illness has not been a feature in other instances of familial Creutzfeldt-Jakob disease.
Normal Pressure Hydrocephalus — High Pressure Normocephalus
Authors: Schutz Hart , Fleming J.F.R. , Humphreys R.P. , Deck J.H.N. , Keith William S.
Published: July 2016

The case history to be presented spans most of the lifetime of a young lady who is now twenty-two years old, and it reflects the stages of development of our knowledge of several important neurosurgical concepts.
Physiologic Basis for Focal Motor Seizures and the Jacksonian “March” Phenomena
Authors: Murphy John T. , Kwan Hon C. , MacKay W.A. , Wong Yiu C.
Published: July 2016

Mechanisms underlying focal motor seizures and the Jacksonian “march” have been explored on the basis of recent physiologic data concerning the “nested ring” spatial and functional organization of motor cortex. Focal motor seizures can be understood in terms of the focal representation at motor cortex of elements controlling movement of a limb part about a single joint. The preponderance of three foci of origin of motor seizures, first reported by Hughlings Jackson, are related to lower thresholds of excitability at these loci, as demonstrated for forelimbs in the case of subhuman primates, and to the resultant bias of peripheral and central input to activate these loci. The “march” phenomenon which typically involves specific patterns of spread in the forearm, is explained in terms of the recently discovered nested ring organization. Thus the centrifugal spread of excitation from a particular locus in motor cortex would demand the pattern of spread of convulsive movements generally observed in clinical situations. The latter involves spread from face or leg to shoulder, forearm, then to hand, or conversely.

The physiological basis of activation and/or inhibition of focal motor epilepsy by peripheral stimuli is also now available on the basis of current experimental evidence. Thus the point to point coupling of periphery and motor cortex as defined in terms of control of limb parts about single forelimb joints, provides a substantial understanding of many of the clinical events sometimes termed “reflex” epilepsy.
Evaluation of Cerebral Blood Flow in Arteriovenous Malformations by the Xenon 133 Inhalation Method
Authors: Menon Devidas , Weir Bryce
Published: July 2016

Fifteen regional cerebral blood flow studies (rCBF) were conducted on 14 patients with arteriovenous malformations (AVM). Only one patient was studied at the time of a hemorrhage. None of the patients were operated upon. All patients had angiographically demonstrated lesions. All the CT scans performed demonstrated the lesions. rCBF was increased in the involved hemisphere compared to the non-involved hemisphere and the difference was greater when the malformations were superficial. There was a higher incidence of high flow regions in the involved hemisphere of patients with AVMs compared to our normal control group and patients with subarachnoid hemorrhage (SAH) from aneurysms. Flow rales within the involved hemisphere demonstrated abnormal distributions and greater variability than normals. Some peaks previously demonstrated in the intraarterial flow studies are not apparent using the inhalation method. This noninvasive technique has a potential to provide additional useful information on the natural history of this disorder.
Pathophysiology and Pharmacotherapy of Spasmodic Torticollis: A Review
Authors: Lal S.
Published: July 2016

The few existing neuropathological, neurochemical, and neuropharacological studies have shed little light on the pathophysiology of spasmodic torticollis (ST). The relevance of experimental ST in animals and drug-induced ST in man to idiopathic ST is unclear.

Most pharmacotherapeutic endeavors have focused on drugs affecting basal ganglia function. Unfortunately, problems of sample size, clinical heterogeneity of patient population, research design, objective evaluation of response, documentation of key data, and adequacy of duration of follow-up make interpretation of published results difficult. Because of the heterogeneity of ST, investigations aimed at establishing a neurotransmitter profile for each patient by observing the acute response to a test dose of drugs affecting cholinergic, dopaminergic, serotonergic, and gammaaminobutyric acid systems may provide a more rational basis to the selection of treatment.
The Use of Baclofen in Treatment of Spasticity in Multiple Sclerosis
Authors: Sawa G.M. , Paty D.W.
Published: July 2016

Baclofen was used in a double-blind crossover placebo-controlled trial to treat spasticity in patients with multiple sclerosis (MS). While on Baclofen, patients obtained a significant (p<0.001) reduction in spasticity compared to controls. The drug was particularly effective in alleviating flexor and extensors spasms, as well as their associated pain. Side effects were common in this study, but were usually well tolerated by the patients. The commonest side effects were sedation, nausea and vomiting. There were no changes in hepatic, renal, or hematological function in any patients. Increased weakness due to loss of spasticity for support was also a fairly common complaint. The drug seems best indicated in patients in whom spasticity is not required for support or other activities of daily living. Careful monitoring of the patient is essential for effective use of this drug.
Cold Induced Rhabdomyolysis in Carnitine Palmyityl Transferase Deficiency
Authors: Brownell A.K.W. , Severson D.L. , Thompson C.D. , Fletcher T.
Published: July 2016

A case of carnitine palmityl transferase deficiency in skeletal muscle is described. The usual symptoms associated with this disease (recurrent muscle cramps or pain and pigmenturia) were not observed but sudden exposure to cold precipitate rhabdomyolysis in this patient.
Strokes and Head Injury
Authors: Blau A. , Richardson J.C.
Published: July 2016

This is a report of a series of patients who suffered major cerebral hemorrhage or infarction complicating industrial head injuries. The variable importance of trauma and of pre-existing vascular disease is exemplified. Accuracy of assessment of such complex problems is facilitated by modern applied neuroscientific techniques.
Effects of Stimulus Shape on Visual Evoked Potentials
Authors: Purves Sherrill J. , Low Morton D.
Published: July 2016

Visual evoked responses (VER) to four geometric shapes (a square, circle, el and omega) were recorded from multiple scalp locations in twelve subjects. Significant differences were found between the occipital VERs to the square and el and between the VERs to the circle and omega. Consistent differences could not be demonstrated between the responses to the square and circle or to the el and omega.

The differences between the responses were quantified by three different methods, including measurement of peak latencies and amplitudes, computation of a ratio index called λ, and the performance of discriminant functions derived by Stepwise Discriminant Analysis Program (SWDA) in classifying other single trial responses. The amplitudes (but not the latencies) of a negative peak at approximately 150 msec, and a positive one at 220 msec, latency were found to be different in the pairs of shapes described. SWDA was able to separate these same pairs and classify from 63 to 68% of new trials correctly. The latency points chosen to make up the discriminant functions correlated partly with the and components that were found to be significantly different in amplitude.

It is suggested that the VER differences in the earlier part of the wave-form are related to the physical properties of the stimuli, including the contrast densities in the central 1.5° of the visual field and/'or the spatial frequencies contained in the stimuli. Differences demonstrated in later parts of the VERs may reflect differences in symbolic meaning of the stimuli.
The Syndrome of Carnitine Deficiency: Morphological and Metabolic Correlations in Two Cases
Authors: Scarlato G. , Pellegrini G. , Cerri C. , Meola G. , Veicsteinas A.
Published: July 2016

Two cases of systemic carnitine deficiency are described. In both patients, carnitine concentration was lower than normal in serum and muscle tissue. In the first case, the illness began at age 35; the clinical manifestations were only muscular. In the second case, the illness began in childhood; there were intermittent episodes of hepatic enlargement and coma. An excessive lipid content was present in muscle tissue, especially in type I fibers, of both cases, and in the liver of the second patient. Ultrastructural studies of muscle tissue revealed important changes of mitochondria.

During muscular exercise, aerobic and anaerobic metabolism were in vestigated. For a given relative work intensity, these patients showed abnormally high blood lactic acid concentration and lactic acid/pyruvic acid ratios. These data, together with the morphological alterations observed in mitochondria, suggest an impaired function of the respiratory chain, leading to a shift of the red/ox potential of the tissue towards a non reduced state.
Central Nervous System Vasculitis in Rheumatoid Arthritis
Authors: Watson Peter , Fekete John , Deck John
Published: July 2016

The history of a patient with rheumatoid arthritis and necrotizing vasculitis affecting only the central nervous system is reported. Clinical and pathological involvement by this process was present in both cerebral hemispheres, the pons and spinal cord. Review of the literature revealed that cerebral vasculitis in rheumatoid arthritis has been reported rarely and spinal cord vasculitis not at all.
Electroencephalographic Findings in Friedreich's Ataxia
Authors: Remillard G. , Andermann F. , Blitzer L. , Andermann E.
Published: July 2016

Electroencephalographic tracings of 50 patients who presented the classical features of Friedreich’s ataxia were reviewed. Mild nonspecific abnormalities were found in 33% and consisted of:

a) Abnormal slow or irregular background rhythms in 15 patients (30%).

b) Intermittent paroxysmal rhythms, considered to be projected from diencephalic or upper midbrain structures, in 4 patients (8%).

c) Unilaterally absent driving responses in 2 affected siblings (4%).

There was no response to intermittent photic stimulation in 60% of the patients. This finding is not considered a definite abnormality, and its significance remains unclear.

Four patients (8%) had epileptic seizures, but of these only two had interictal epileptic abnormalities.

There was no correlation between the duration and severity of the disease and the presence of electroencephalographic abnormalities.

Friedreich’s ataxia is mainly a spinal disorder. Involvement of supraspinal and in particular brain stem or diencephalic structures may be more extensive in those patients who show electrographic abnormalities. This would require confirmation with comparative data based on pathological observations.

Impaired function of brain stem inhibitory mechanism may be responsible for the slightly raised incidence of seizures in patients with Friedreich’s ataxia and other cerebellar degenerations.
Myophosphorylase Deficiency (McArdle's Disease): Report of a Family
Authors: Mahmud M. Zafar , Howell R. Rodney , Stevenson Roger E. , Gilroy John
Published: July 2016

The clinical and biochemical findings are presented of two brothers suffering from McArdle's Disease (Myophosphorylase Deficiency). Tissue enzyme estimations and lactate levels were done in affected and non-affected members of the family. Affected members showed absence of phosphorylase enzyme by histochemical and quantitative estimation. No quantitative abnormalities were found in other enzyme systems of glycolytic pathways in the family investigated. Various other aspects of clinical features, biochemical abnormalities and inheritance are discussed.
Direct Leukocyte Migration Inhibition In Multiple Sclerosis — A Possible Assessment of Activity
Authors: Day M.P. , Day J.H. , Mann P.L.
Published: July 2016

Twenty-four patients with multiple sclerosis were evaluated and classified according to their clinical state. Specific migration inhibition studies were carried out on blood samples
from each using myelin basic protein, an acid soluble protein fraction isolated from normal human CNS white matter, and multiple sclerosis myelin basic protein isolated from patients who
had the disease, as the antigenic material. This test system employed selected media. Results were compared with those of normal controls and patients with other neurological disease states.
Antigen concentration of 500 μ glml in serum free medium combined to produce the greatest inhibiting effect on leukocytes in patients with apparent multiple sclerosis and differentiated these patients from those in the other groups tested.

Leukocytes in patients who had probable multiple sclerosis with partial impairment, signifying possible current activity of the disease, were especially inhibited as compared to the leukocytes from other groups tested against myelin antigen. Cerebrospinal fluid from affected patients when used as a media enhanced the test.

This study suggests that migration inhibition of peripheral leukocytes using myelin protein may be useful in the diagnosis of patients with multiple sclerosis. There is additional evidence that the degree of leukocyte migration inhibition may reflect activity of the disease with
its consequent implications on treatment and prognosis.
Studies of Human Papovavirus Tumor Antigen in Experimental and Human Cerebral Neoplasms
Authors: Becker L.E. , Narayan O. , Johnson R.T.
Published: July 2016

Three types of papovaviruses (JC, BK, and SV40) have been isolated from man. All three are oncogenic in hamsters, cause frequent infection of man, and share a common T antigen. Augmentation of the expression of T antigen by in vitro cultivation of SV40-induced tumors of hamsters suggested that growing human brain tumors in vitro might provide an effective screening technique for the SV40 virus. In a series of human brain tumors examined in cryostat sections and in tissue culture, T antigen could not be demonstrated, suggesting that by this immunofluorescent technique SV40 was not implicated in the etiology of these tumors.
Ecogenetics of Parkinson's Disease: Prevalence and Environmental Aspects in Rural Areas
Authors: Barbeau André , Roy Madeleine , Bernier Gilles , Campanella Giuseppe , Paris Suzanne
Published: July 2016

We make use of the unique combination of a homogeneous genetic and racial origin in the rural population of Quebec and the facilities of free and universal access to medical care, to study the distribution of the prevalence of Parkinson's disease in the 9 rural hydrographic regions of the Province. Through 3 different methods of ascertainment, confirmed by two control probes, we demonstrate that the prevalence of Parkinson's disease is of uneven distribution within rural areas. We further investigated the characteristics of the regions of high prevalence. These regions which are predominantly agricultural and areas of intensive market gardening were also the areas with the highest use of pesticides.
Adult Onset Systemic Carnitine Deficiency: Favorable Response to L-Carnitine Supplementation
Authors: Levitan M.D. , Murphy J.T. , Sherwood W.G. , Deck J. , Sawa G.M.J.
Published: July 2016

We report the case of a patient who at age 39 first developed an episode of muscle weakness and transient ketoacidosis with biopsy proven fatty infiltration of the liver. Over the next several years, myopathy ensued; biopsy revealed extensive deposition of lipid globules in type 1 muscle fibres. Further investigations established the diagnosis of systemic carnitine deficiency (SCD) with skeletal muscle, hepatic, and cardiac involvement. The patient has benefited from L-carnitine supplementation. Our case represents an unusually late onset of SCD and highlights the necessity, when appropriate, of a high index of suspicion of this rare but treatable disorder.
Carotid Endarterectomy Without Indwelling Shunts and Intraoperative Electrophysiologic Monitoring
Authors: Reddy Kesava , West Michael , Anderson Brian
Published: July 2016

Although carotid endarterectomy is a common surgical procedure in North America, controversies exist regarding the type of anesthesia, the use of indwelling shunts and the need for intraoperative cerebral monitoring. We present a prospective study of 100 carotid endarterectomies performed over a three year period by a single surgeon without the use of indwelling shunts, patch grafts, or EEG monitoring. The combined stroke and mortality rate was 1%. Our results confirm those of other authors; that indwelling shunts and EEG monitoring are not absolutely essential for a satisfactory outcome in carotid endarterectomies.
Bilateral Homonymous Hemianopia with Sparing of Central Vision After Subdural Hematoma
Authors: Soza Marco , Tagle Patricio , Kirkham Trevor , Court Jaime
Published: July 2016

A patient with bilateral homonymous hemianopia with preservation of the central vision, spatial disorientation and memory deficits secondary to bilateral occipital and mesial temporal infarctions, is presented. The cerebral damage followed a subdural hematoma with tentorial herniation and compression of both posterior cerebral arteries.
Interhemispheric Subdural Hematoma
Authors: Russell Neville A. , Carpio-O'Donovan Raquel del , Mallya K.B. , Benoit Brien G. , Belanger Gary
Published: July 2016

Although relatively uncommon, interhemispheric subdural hematoma (ISDH) occurs more frequently than was suspected before the advent of computerized tomographic (CT) scanning. When its mass is sufficiently large to compress the medial cerebral hemisphere, specific focal neurological abnormalities may occur. These include weakness of the contralateral leg, or contralateral hemiparesis with the leg being weaker than the arm. On the unenhanced CT scan ISDH is seen as a crescent shaped, midline hyperdensity. Treatment is dictated by the clinical course. Evacuation of the hematoma by parasagittal craniotomy is recommended if the symptoms are pronounced.
Involuntary Movements with Cerebellar Tumour
Authors: Jayakar P.B. , Seshia S.S.
Published: July 2016

We describe a child with a cerebellar astrocytoma who presented with paroxysmal segmental rhythmic myoclonus. The movement disorder was characterized by focal onset in the left eyelid followed by a sequential march of clinical events. There were no clinical or laboratory findings to suggest brainstem infiltration or cerebral involvement. Marked clinical improvement followed tumour resection. We suggest that the cerebellar lesion was primarily responsible for the movement disorder.
Normality and Disease
Authors: Calne Donald B. , Calne Joanna S.
Published: July 2016

There have been trends to equate normal with an ideal state of health, and disease with disturbances that are determined solely by subclinical abnormalities. While in any living language there is a conflict between established definition and the need for change, modification in the use of words that are of such central importance to medical writing requires cogent justification that has not been forthcoming in these instances. To avoid further obscuration of the literature, the term normal should be limited to its traditional connotation of average, and the term disease should be reserved for disturbances of health that are clinically manifest.
Primary Hypothyroidism Presenting as Growth Delay and Pituitary Enlargement
Authors: Farley John D. , Toth Ellen L. , Ryan Edmond A.
Published: July 2016

We report the case of a young boy being considered for pituitary surgery because of pituitary enlargement found during assessment of growth delay. There was no goitre but he was hypothyroid clinically and biochemically. The finding of an elevated TSH suggested primary thyroid disease with thyrotroph hyperplasia. Treatment with L-thyroxine resulted in prompt resolution of his pituitary enlargement and improvement in his visual fields.
Parental Age and Birth Order in Alzheimer's disease: A Case-Control Study in the Saguenay-Lac-St-Jean Area (Quebec, Canada)
Authors: De Braekeleer M. , Froda S. , Gautrin D. , Tetreault H. , Gauvreau D.
Published: July 2016

Parental age at the time of birth of 133 clinically diagnosed Alzheimer patients from the Saguenay-Lac-St-Jean area (Quebec, Canada) were compared with those of 6 control groups formed out a population registry. The birth order of the Alzheimer patients was also analyzed. The results did not show any parental age or birth order effect, which is in agreement with previous reports. The importance of control selection in such studies is stressed.
Platelet Aggregability in Sleep-Related Stroke
Authors: Voll C.L. , Chetty N. , Atkinson P.
Published: July 2016

We examined platelet aggregability during nocturnal sleep and daytime wakefulness in patients with a history of sleep-related stroke onset (SOS) and compared it to that of matched awake-onset stroke (AOS) patients and controls without evidence of vascular disease. Aggregability was evaluated in-vitro at least seven weeks following stroke onset. Platelets were more aggregable to ADP, collagen and arachidonic acid (AA) during both sleep and wakefulness in patients with AOS (p<0.01). No significant difference in the mean aggregation thresholds during sleeping or waking periods were found between SOS and control groups. However, platelets were significantly more responsive to AA during sleep than during wakefulness in the SOS patients (p<0.01). This difference was confined to the subgroup of SOS patients who had experienced nocturnal as opposed to daytime sleep-related stroke onset, suggesting that the observed difference in platelet responsiveness to AA may be related to a circadian fluctuation in platelet aggregability rather than to a sleep-related fluctuation. Significant sleep-related changes in platelet aggregability were not identified in the other two groups.
Hypertriglyceridemia and Lower LDL Cholesterol Concentration in Relation to Apolipoprotein E Phenotypes in Myotonic Dystrophy
Authors: Moorjani Sital , Gaudet Daniel , Laberge Claude , Thibault Marie Christine , Mathieu Jean , Morissette Jean , Lupien Paul J. , Brun Daniel , Gagné Claude
Published: July 2016

Plasma lipid, lipoprotein levels and apolipoprotein apo E phenotypes were determined in 70 patients with myotonic dystrophy (MyD) and 81 controls. Marked differences were noticed in the apo E phenotype frequencies between the two groups. Plasma triglycerides and VLDL cholesterol were higher in MyD than controls, but only the latter was related to differences in the apo E phenotypes between two groups. Accordingly, the ratio of VLDL cholesterol/plasma triglycerides was increased significantly in MyD, suggesting accumulation of intermediary density particles due to lower affinity of E2 containing lipoproteins for lipoprotein cell receptors. The LDL cholesterol concentration was lower in MyD than controls and was related to differences in the apo E phenotype frequencies between the two groups. These results indicate increased removal of LDL particles in the apo E2 phenotypes, perhaps due to upregulation of LDL (B, E) receptor activity. Plasma cholesterol and HDL cholesterol concentrations were similar in both groups. Another feature of the study was lower levels of plasma cholesterol, triglycerides, VLDL and LDL cholesterol in the homozygous E4:E4 phenotype. These results suggest increased clearance rate of both VLDL and LDL particles and support the concept that apo E4-containing lipoproteins have higher in vivo affinity for ape E and/or B, E receptors.
Myotonic Dystrophy: Linkage with Apolipoprotein E and Estimation of the Gene Carrier Status with Genetic Markers
Authors: Thibault Marie-Christine , Mathieu Jean , Moorjani Sital , Lescault André , Prévost Claude , Gaudet Daniel , Morissette Jean , Laberge Claude
Published: July 2016

The genes for myotonic dystrophy (MD) and for apolipoprotein E (ApoE) belong to a chromosome 19 synthenic group of markers. A familial linkage analysis between MD and ApoE was performed using the J Ott LIPED program (IBM PC/XT, April 1984) to estimate the genetic distance between these 2 genes. Of a total of 136 individuals in 11 MD families, 81 were confirmed to be affected by the disease and 41 were asymptomatic. ApoE phenotypes were determined in 115 of these 122 individuals. No recombinant was observed out of 74 meioses which were informatives for both MD and the ApoE isoproteins. A global maximal lod score Z of 19.00 was obtained at the recombination fraction Θ = Θ. The upper 0 value at the confidence interval corresponding to the peak lod score (Z max) - 1 was 0.03. This suggests that the loci for MD and ApoE are at a distance of 0 to 0.03 Morgan. Since ApoE and apolipoprotein C2 (ApoC2) have been shown by others to be about 40 kb apart, our data are therefore consistent with the distance estimate of 0.02 Morgan reported between MD and ApoC2. The D19S19 (LDR152) polymorphic DNA sequence is also tightly linked to MD on chromosome 19. The segregation of ApoE isoproteins and of ApoC2 and D19S19 DNA polymorphism was utilized for evaluating the probability for individuals at risk of inheriting the disease gene in MD families. Data are presented on 3 families to emphasize the usefulness of genetic markers to estimate the MD gene carrier status of asymptomatic individuals and also for those presenting a partial syndrome. The limitations of such approach are also discussed.
Neuropathy with Onion Bulb Formations and Pure Motor Manifestations
Authors: Auer Roland N. , Bell Robert B. , Lee Mary Anne
Published: July 2016

A thirty-eight-year-old man presented with a six year history of symptoms resembling an anterior horn cell disorder. There was progressive upper extremity wasting and weakness in the absence of sensory complaints. Electrophysiologic abnormalities were confined to motor nerve conduction and indicated a demyelinating process involving the brachial plexus and major proximal upper extremity nerve trucks bilaterally. Biopsy of the proximal right ulnar nerve revealed changes suggesting a chronic demyelinating process, and onion-bulb formations were present. Immunohistochemical staining for S-100 protein was positive in the cells comprising the onion-bulbs, indicating a Schwann cell, not a perineurial origin of these cells. After 8 years, symptoms have failed to appear in the lower limbs. Recent reports in the literature have begun to delineate the syndrome, which appears to represent an unusual, localized or multifocal, sometimes inflammatory, clinically benign neuropathy that can mimic motor neuron disease in its earlier stages. We report the first such case with underlying pathology.