bluuebunny/crossref_metadata_2025 huggingface dataset:
The Neurological Complications of Brucellosis
Authors: Larbrisseau A. , Maravi E. , Aguilera F. , Martinez-Lage J.M.
Published: July 2016

Neurological complications of brucellosis have seldom been reported. Typical cases of meningoencephalitis, myelitis, and peripheral nerve involvement due to brucellosis, which have been collected over the past ten years, are described. Only four cases of extradural brucellar abcesses have been published previously. We now add four new observations. A CSF electrophoresis was done in the cases of meningoencephalitis and meningomyelitis and showed a marked increased of gamma globulin. This has never been reported previously.
Functional Implications of Changes in the Senescent Brain: A Review
Authors: Beck Charles H.M.
Published: July 2016

The morphological, chemical, and physiological changes in the brain accompanying old age are reviewed. The deterioration of the striatal and hypothalamic dopaminergic systems were implicated in the onset of age related Parkinsonian-like slowing of performance and altered affect. Cholinergic hippocampal and neocortical systems were chemically and physiologically abnormal in the aged. The implications for slowed cognitive processing and persistance of the memory trace are presented.
Alterations of Membrane Phosphorylation in Erythrocyte Membranes from Patients with Duchenne Muscular Dystrophy
Authors: Vickers J.D. , McComas A.J. , Rathbone M.P.
Published: July 2016

The phosphorylation of spectrin, hand 3 protein, and the phospholipids of erythrocyte membranes (ghosts) was examined in 10 patients with Duchenne muscular dystrophy (DMD) and in healthy age- and sex-matched controls. The rales of phosphorylation of spectrin and band 3 protein were significantly higher in ghosts prepared from patient blood than from control blood at both 30° C and 37° C. However, the mean increases in the rate of phosphorylation of both spectrin and band 3 protein in response to a temperature change from 30° C to 37° C were identical in gliosis from patient and controls. Phosphorylation of phospholipid and its temperature response did not differ between patients and controls. These results complement previous observations of differences in erythrocytes from patients with DMD. The similarity of the changes in phosphorylation of both spectrin and band 3 protein indicates a common cause, possibly their lipid environment.
Case Report and Ultrastructural Study of Intracranial Embryonal Carcinoma
Authors: Ejeckam Gerson , Norman Margaret G. , Ivan Leslie P.
Published: July 2016

A case of a primary intracranial embryonal carcinoma, the first with ultrastructural study, is reported. The tumor was associated with precocious puberty in a 6½-year-old female. Characteristic embryoid bodies were present. At the ultrastructural level three cell types were noted: undifferentiated, differentiated, and intermediate types. The undifferentiated showed scanty cytoplasmic organelles and numerous free polysomes, while the differentiated cells contained well-developed mitochondria, Golgi apparatus, rough endoplasmic reticulum, and some contained secretory granules. The intermediate cells possessed dilated and irregularly-shaped mitochondria but still retained large numbers of free polysomes. The authors suggest that intracranial germ cell tumors be named in conformity with germ cell tumors in other sites, and that terms such as “ectopic pinealoma” and “atypical teratoma of the pineal” be used no longer.
F-wave and Cervical Somatosensory Response Conduction from the Seventh Cervical Spinous Process to Cortex in Multiple Sclerosis
Authors: Eisen Andrew , Nudleman Kenneth
Published: July 2016

Transit (conduction) times from the wrist to the seventh cervical spinous process (C7) were measured by using the F-wave response (TA) and the cervical somatosensory response (SEPC). The mean values obtained in 25 controls were 10.1 ± 0.9 s-3 and 10.9 ± 1.0 s-3 respectively. The 0.8 ± 1.0 s-3 difference between the two methods represents central delay of SEPC, possibly at the level of the dorsal root ganglion. The mean onset latency of the cortical somatosensory response (SEPA) evoked by median nerve stimulation in the same subjects was 15.5 ± 1.0 s-3. Transit time from C7 to the cortex (TB) given by either SEPA - TA or SEPA - SEPC - 0.8, where 0.8 is the necessary corrective factor for central delay of SEPC, was 5.3 ± 1.0 s-3. These techniques were applied to 10 patients suspected of having multiple sclerosis, but without clinical evidence of involvement above C7. Six of the 10 cases showed prolongation of TB. In 4 of these, this could only be calculated by the F-wave method since SEPC was absent. It is concluded that transit times derived from either the F-wave or SEPC are equally valid and interchangeable. The absence of one response allows for its replacement by the other.
CSF Electrophoresis: An Adaptation Using Cellulose Acetate for the Identification of Oligoclonal Banding
Authors: Paty D.W. , Donnelly M. , Bernardo M.E.
Published: July 2016

An adaptation of cellulose acetate electrophoresis for studying concentrated cerebrospinal fluid is described. Two hundred and twenty-one patients have been studied, and the specificity for multiple sclerosis and sub-acute sclerosing panencephalitis is discussed. This has been positive for oligoclonal banding (OB) in 79% of patients with clinically definite multiple sclerosis.
Serial Radionuclide Scans in Multiple Sclerosis
Authors: Murray Shirley , Veidlinger Otto F.
Published: July 2016

A 28 year old woman with multiple sclerosis has been followed for 10 months with serial brain scans on 5 admissions. The correlation between the clinical picture and the brain scans was clearly demonstrated.
Timing of the Electroretinogram Response and Dark Adaptation
Authors: Brunette Jean Real , Lafond Gilles
Published: July 2016

The time response of the Electroretinogram (ERG) is an essential part of the examination. Peak time of
response increases with dark adaptation and decreases with the intensity of stimulation. When working with constant amplitude during dark adaptation sensitivity increases but the intensity of stimulation decreases. It is essential to clarify the relationship of these two factors for valid interpretation of results. The present work suggests that the state of adaptation or retinal sensitivity affects amplitude, while peak time of the response is modified by the absolute value of the stimulating source whatever the state of adaptation. This observation has been done on rod responses and is valid as long as pigments remain unbleached and able to respond.
Dominantly Inherited Hypertrophic Neuropathy
Authors: Mongia S.K. , Ghanem Q. , Preston D. , Lewis A.J. , Atack E.A.
Published: July 2016

Clinical, electrophysiological, and histopathological studies of some members of a family with dominantly
inherited hypertrophic neuropathy are presented. Twenty-five members were studied. Seventeen were abnormal on clinical examination. Their ages varied from 2½ to 78 years. Age at onset in 14 of the 17 varied between 2½ and 56 years. Pes cavus and palpable nerve thickening were present in more than half of the affected individuals. All patients had areflexia. Fifteen of the 17 had distal motor weakness as well as mild to moderate sensory impairment. Motor weakness affecting the proximal hip and shoulder girdle muscles was seen in 13 patients. Four patients gave a history of trigeminal neuralgia. Motor nerve conduction velocities were markedly impaired in all the clinically affected members. These studies were normal in the 8 unaffected members. Motor conduction velocities of the proximal segment of the ulnar nerve were slower compared to the distal segment in almost all the affected members. There was no significant correlation between the degree of clinical disability and the extent of impairment in the motor nerve conduction velocities. Sural nerve biopsies were studied. These observations are discussed.
Hydranencephaly in Association with Roberts Syndrome
Authors: Ekong Chris E.U. , Rozdilsky Bohdan
Published: July 2016

A clinicopathological study in a case of Roberts syndrome (tetraphocomelia, cleft lip and palate, and phallic hypertrophy) is reported. This patient had hydranencephaly and imperforate anus, two additional congenital abnormalities so far not reported in this syndrome.
Platelet Dopamine Uptake in Huntington's Chorea and Gilles de la Tourette's Syndrome: Effect of Haloperidol
Authors: Butterworth Roger F. , Gonce Michel , Barbeau André
Published: July 2016

Uptake of 14C-dopamine by human platelets has been studied in two diseases, namely Gilles de la Tourette's syndrome and Huntington's chorea, in which abnormal metabolism of dopamine has been implicated. Platelets from untreated Huntington's chorea patients showed a small increase in Km and Vmax.; platelets from patients in all other groups showed an uptake identical with the controls. Haloperidol (10 -5M) was also shown to be a strong non-competitive inhibitor of 14C-DA uptake by platelets. This property is probably unrelated to the drugs' action in ameliorating the symptoms of Huntington's chorea which is likely related to the increase in cholinergic neuronal activity produced by neuroleptic blockade of dopamine receptors.
The Role of Cyclic Nucleotides in the CNS
Authors: Phillis John W.
Published: July 2016

On the basis of the information presented in this review, it is difficult to reach any firm decision regarding the role of cyclic AMP (or cyclic GMP) in synaptic transmission in the brain. While it is clear that cyclic nucleotide levels can be altered by the exposure of neural tissues to various neurotransmitters, it would be premature to claim that these nucleotides are, or are not, essential to the transmission process in the pre- or postsynaptic components of the synapse. In future experiments with cyclic AMP it will be necessary to consider more critically whether the extracellularly applied nucleotide merely provides a source of adenosine and is thus activating an extracellularly located adenosine receptor, or whether it is actually reaching the hypothetical sites at which it might act as a second messenger. The application of cyclic AMP by intracellular injection techniques should minimize this particular problem, although possibly at the expense of new difficulties. Prior blockade of the adenosine receptor with agents such as theophylline or adenine xylofuranoside may also assist in the categorization of responses to extracellularly applied cyclic AMP as being a result either of activation of the adenosine receptor or of some other mechanism. Ultimately, the development of highly specific inhibitors for adenylate cyclase should provide a firm basis from which to draw conclusions about the role of cyclic AMP in synaptic transmission. Similar considerations apply to the actions of cyclic GMP and the role of its synthesizing enzyme, guanylale cyclase.

The use of phosphodiesterase inhibitors in studies on cyclic nucleotides must also be approached with caution. The diverse actions of many of these compounds, which include calcium mobilization and block of adenosine uptake, could account for many of the results that have been reported in the literature.
Haloperidol-Induced Dyskinesias in the Monkey
Authors: Bédard P. , Delean J. , Lafleur J. , Larochelle L.
Published: July 2016

Haloperidol (0.25 mglkg i.m.) was injected daily for 6 months in six normal monkeys. Over a 24 hour period, the following symptoms could he observed: akathisia, circling, akinesia, choreoathetoid and dystonic movements, oro-facial dyskinesias and postural tremor with or without harmaline. Six months after cessation of haloperidol, harmaline-induced postural tremor could still be observed in all animals and oro-facial abnormal movements, in one monkey. The neuropathologic study of the experimental material did not disclose any alteration of the central nervous system.
The Possible Local Synthesis of Antibodies to Herpes Simplex Virus in Normal Cerebrospinal Fluid
Authors: Russell Anthony Science
Published: July 2016

We have used the technique of antibody mediated cell dependent immune lysis to examine paired samples of serum and CSF for antibody to herpes simplex virus. The 40 patients studied had no inflammatory disease of the nervous system, yet 20 of the CSF specimens did have antiviral antibody. This is an extremely sensitive technique for the detection of at least one type of antiviral antibody and “in vitro” is a very effective way of killing virus infected cells. There is no correlation between the level of antiviral antibody in the CSF with the total protein content, but the high CSF: serum antibody ratio in some subjects who are particularly susceptible to recurrent herpes infection raises the possibility that local stimulation and production of this antibody may occur.
Clinical Description and Roentgenologic Evaluation of Patients with Friedreich's Ataxia
Authors: Geoffroy G. , Barbeau A. , Breton G. , Lemieux B. , Aube M. , Leger C. , Bouchard J.P.
Published: July 2016

The 50 patients in this survey were classified by a panel of neurologists into 4 clinical sub-groups: Group la (“typical” Friedreich's ataxia, complete picture), Group lb (“typical” Friedreich's ataxia, incomplete picture), Group Ila (“atypical” Friedreich's ataxia, possible recessive Roussy-Levy syndrome), Group lib (heterogeneous ataxias). The clinical symptoms and signs were analyzed for each of these groups. A constellation of signs constantly present in Friedreich's ataxia and obligatory for diagnosis was described. Other important symptoms, such as the Babinski sign, kyphoscoliosis and pes cavus were found to be progressive, but not essential for the diagnosis at any given time. Finally, a host of other symptoms can only be called accessory. The progression of scoliosis was found to be an important tool in the differential diagnosis of ataxias. Our study also indicates, in contrast to the opinion of some authors, that absent deep tendon reflexes in the lower limbs and early dysarthria are essential in “typical” Friedreich's ataxia.
Electrocardiographic and Vectocardiographic Findings in Friedreich's Ataxia
Authors: Malo S. , Latour Y. , Cote M. , Geoffroy G. , Lemieux B. , Barbeau A.
Published: July 2016

Electrocardiographic and vectocardiographic changes are frequent in Friedreich's ataxia. In one of 35 patients both tests were normal. The vectocardiogram is more explicit in demonstrating the severity of the QRS changes with a right ventricular hypertrophy pattern present in 60% of cases. Serial examination and ECG tracings are recommended to monitor the cardiomyopathy in this progressive neurological disorder, in order to detect the onset of congestive heart failure, significant tachyarrythmias, or obstructive cardiomypathy.
Echocardiographic Findings in Friedreich's Ataxia
Authors: Gattiker H.F. , Davignon A. , Bozio A. , Batlle-Diaz J. , Geoffroy G. , Lemieux B. , Barbeau A.
Published: July 2016

Echocardiographic examination of 21 patients with Friedreich's ataxia (age 7 to 28 years) showed cardiac abnormalities in 90% of the cases. They were characterized by varying degrees of septal hypertrophy in 81%, left ventricular free wall hypertrophy in 61%, and a slight reduction of left ventricular internal dimension in 57% of the cases. Asymmetric septal hypertrophy (ASH) with a septal/left ventricular free wall ratio of over 1.3 was found in 29% of the cases, and systolic anterior motion (SAM) of the mitral valve in three patients. Two other patients showed evidence of a different type of cardiomyopathy with marked symmetric left ventricular hypertrophy and marked left ventricular enlargement.
Pathology of the Heart in Friedreich's Ataxia: Review of the Literature and Report of One Case
Authors: Sanchez-Casis G. , Cote M. , Barbeau A.
Published: July 2016

A single case of typical Friedreich's ataxia was analyzed for cardiac changes and compared to the findings from the literature. Macroscopically, there was a cardiomegaly with some degree of ventricular hypertrophy and probable mild dilatation of the auricles. The more important and constant histologic changes were myocardial fibrosis and degeneration of the cardiac muscle cells. Granular deposits of calcium salts and iron were found in the muscle cells. A cardiomyopathy hypertrophic in type and occasionally obstructive appears to be an integral part of Friedreich's ataxia.
An Unusual Occurrence of Multiple Sclerosis in a Small Rural Community
Authors: Murray Thomas John
Published: July 2016

Ten patients with multiple sclerosis were found to have lived in close proximity in a Nova Scotia farming community of 150 people. All had drunk unpasteurized milk as children, were teetotallers, ate a high animal fat diet, and were well educated. Of greater interest was the observation that six of the ten cases were related in two family groups.

The only time all patients lived in the community at the same time was in 1951 and 1952 during a polio outbreak. The relationship of polio to multiple sclerosis bears further study.

The average age of the patients when they had measles was 11.8 years. Evidence suggests a link between risk of multiple sclerosis and both late onset of measles and pubertal age. Late onset of measles may be important in this cluster. Further epidemiological studies are needed to examine the age of onset of measles in M.S. cases.
Shy-Drager Syndrome. Neuropathological Correlation and Response to Levodopa Therapy
Authors: De Lean Jacques , Deck John H.N.
Published: July 2016

The post-mortem examination of the nervous system of a patient with Shy-Drager syndrome successfully treated with levodopa (Sharpe et al, 1972) revealed features of striato-nigral degeneration and amyotrophic lateral sclerosis, a cerebellar system degeneration and a loss of approximately 75% of sympathetic preganglionic neurons. Lewy bodies were not present and no detectable changes were observed in the sympathetic prevertebral ganglia.

While the limited and transient beneficial effect of levodopa on the bradykinesia in our case is possibly due to the progressive loss of striatal dopaminergic receptors seen in striatonigral degeneration, we propose that in Shy-Drager syndrome, levodopa therapy benefits orthostatic hypotension because of a suppression of the central depressor action of this drug. This suppression is attributable to functional disconnection of sympathetic ganglia secondary to the loss of preganglionic neurons or to degeneration of central autonomic catechoiaminergic systems.
Intracranial Dysgerminomas
Authors: Leblanc G. , Francoeur J. , Copty M. , Contreras C. , Gagne F.
Published: July 2016

We have reported three cases of intracranial dysgerminoma. The origin of these tumors has been controversial for a long time. It is now accepted that they develop from germ cells. The diagnosis is often difficult to confirm because of the variety of signs. If their origin is now accepted, the best way to deal with these lesions is still controversial. They can be macroscopically removed if they are in the pineal region. Such treatment is not possible for suprasellar tumors. Since these tumors can metastasize, radiation should be administered as soon as a pathological diagnosis is obtained. It is our belief that it should be administered to the whole spinal axis.
Progress in Understanding and Treating Parkinson's Disease
Authors: Barbeau André
Published: July 2016

This review evaluates the long-term results of Levodopa therapy in Parkinson's disease upon quality of life, prolongation of survival and excess mortality. It also focuses on recent and new therapeutic approaches: Levodopa in comhindation with a Dopa-decarboxylase inhibitor or MAO-B inhibitor, dopamine agonists and an active tripeptide: L-prolyl-L-leucylglycine amide (M1F-I). It ends by looking at new avenues of etiological research in Parkinson's disease which may indicate specific accelerated ageing of catecholaminergic (pigmented) neuronal systems.
Integrative Versus Delay Line Characteristics of Cerebellar Cortex
Authors: MacKay W.A. , Murphy J.T.
Published: July 2016

In order to determine which of two general models (“tapped delay line” or “integrator”) provides a more accurate description of mammalian Purkinje cell (P-cell) activation by natural stimulation, the spatial and temporal characteristics of a population of neurons in cerebellar cortex responsive to small controlled stretches of forelimb muscles were examined in awake, locally anesthetized cats. Stretch of a single wrist muscle excited P-cells over a distance of about 1 mm in the long axis of a folium, a span which is at most half the length of parallel fibers. Both granule cells and molecular layer interneurons were excited over a wider zone than P-cells.

Furthermore, P-cells across a response zone all fired on the average at the same time, as determined by computing peristimulus cross-interval histograms from pairs of simultaneously recorded neurons. Consistent delays could only be demonstrated in the minimal response latencies as measured from peristimulus time histograms. These delays, however, were longer than could be ascribed to parallel fiber conduction velocity.

No evidence, therefore, was found in cat cerebellum to support the “tapped delay line” model, which postulates the successive activation of P-cells as an excitatory volley travels along a parallel fiber beam. Instead, an integrative mode of operation seems to predominate: a relatively wide substratum of activated granule cells simultaneously activates a narrower focus of P-cells centrally situated with respect to the granule cell population. The role of inhibitory interneurons in promoting the “integrator” model is discussed.
Interpretation of the Computerized Tomographic Scan
Authors: Banna M.
Published: July 2016

This paper is based on the experience gained from over three thousand examinations by computerized tomography (CT). The appearances of various intracranial lesions are presented.
The reliability and limitations of this new technique are also described and some pitfalls in scan interpretation are mentioned.
Ultrastructural Classification of Pituitary Adenomas
Authors: Horvath E. , Kovacs K.
Published: July 2016

Forty-eight surgically removed pituitary adenomas have been investigated hy electron microscopy. Distinct differences in fine structural appearances permitted their separation into 5 classes: 1) Growth hormone, (GH)-cell tumors; 2) Prolactin cell tumors; 3) Mixed adenomas composed of GH and prolactin cell's; 4) Adrenocorticotrophic-Melanocyte stimulating hormone (ACTH-MSH) cell tumors; 5) Undifferentiated cell adenomas. Densely and sparsely granulated tumors were distinguished within forms appeared to represent well defined entities, they may be variants of the same tumor differing only in pace of hormone production and/or release. Number and size of secretory granules varied considerably among tumors composed of the same cell type indicating that pituitary tumor classification cannot be based solely on granule morphology. This classification takes into account morphogenesis with emphasis on clinical features and structure-function relationship.
Cerebral Blood Flow in Patients with Intracranial Pressure Elevation due to Traumatic Brain Edema
Authors: Tweed W. A. , Overgaard Jørn
Published: July 2016

The object of this study was to determine if traumatic brain edema (BE) and increased intracranial pressure (ICP) reduce cerebral blood flow (CBF). Two groups of patients were studied, one with slight BE and ICP less than 20 mm Hg., the other with pronounced BE and ICP over 20 mm Hg. Although ICP was higher and cerebral perfusion pressure lower in pro-nounced edema there was only a small and non-significant reduction in CBF and no difference in cerebro-vascular resistance. Since traumatic BE does not increase resistance to blood flow through the brain, cerebral perfusion can be maintained if an adequate perfusion pressure is established. This in turn, demands the monitoring and control of ICP.
Neurophysiology of a Central Baroreceptor Pathway Projecting to Hypothalamic Vasopressin Neurons
Authors: Jhamandas Jack H. , Renaud Leo P.
Published: July 2016

Controversy exists as to the neural network whereby peripheral arterial baroreceptor information is transmitted to vasopressin (VP)-secreting neurons of the hypothalamic supraoptic nucleus (s.o.n.). In vivo electrophysiological studies in the rat were undertaken to characterize the selective depression of VP cell activity consequent to activation of peripheral baroreceptors. Electrical stimulation of the diagonal band of Broca (DB) in the rat evoked a similar selective inhibition of vasopressinergic neurons of the s.o.n. Local application of bicuculline, a GABA antagonist, abolished both the DB-evoked and baroreceptor-induced inhibition of VP-secreting neurons. In addition, recordings from DB neurons antidromically activated from the s.o.n. displayed an increase in firing consequent to baroreceptor activation, coinciding with the suppression of firing in s.o.n. VP neurons. These observations collectively indicate that an intrinsic GABA projection arising in the DB cell group selectively inhibits vasopressinergic neurons of the s.o.n. and that this pathway mediates peripheral arterial baroreceptor activity that influences the release of VP in the neurohypophysis. These data may be of critical importance in our understanding the etiology of those forms of experimental hypertension where abnormalities in central baroreceptor pathways have been implicated but not proven.
Speculations on Disease States Induced by Excitatory Amino Acids
Authors: Turnbull John
Published: July 2016

There is much current interest in excitatory amino acids and their receptors because of their postulated involvement in several disorders of the nervous system. They function as neurotransmitters, but can act as neurotoxins in some situations. They have been implicated in the pathogenesis of cerebral hypoxic/ischemic and hypoglycemic damage, in epilepsy, in some degenerative diseases, and in some forms of neurotoxin-induced cerebral dysfunction. These diseases may reflect abnormality in a system which has evolved to provide synaptic plasticity essential for learning and memory. The purpose of this paper is to explore the ramifications of such a hypothesis.
Peripheral Neurological Complications of Aortoiliac Vascular Disease
Authors: D'Amour Monique L. , Lebrun Louise Hélène , Rabbat Antoine , Trudel Jules , Daneault Nicole
Published: July 2016

Six patients with an aortoiliac vascular disease and a peripheral neurological deficit are presented. Clinical and electromyographic findings revealed lumbosacral plexus, sciatic and femoral nerve lesions. A correlation is made between the level of the vascular lesion (aortic, aortoiliac or distally) and the type of peripheral nerve deficit observed. In a patient complaining of pain, weakness, or numbness in a leg, the differential diagnosis should include aortoiliac vascular disease. The peripheral neurological symptoms may be the initial manifestation of the vascular disease or may appear in the early post-operative period.
Metrizamide CT Scanning in Spinal Nerve Root Cysts
Authors: Goyal R.N. , Russell N.A. , Belanger J.M.E.G. , Benoit B.G. , Rawa M.
Published: July 2016

Two cases of lumbosacral root cysts of different etiology are reported. Their specific radiographic features are described using the combined technique of metrizamide myelography followed by computerized tomography of the spine. The terminology of intraspinal cysts is reviewed and their distinguishing features discussed.
Spontaneous Temporary Remission in Primary CNS Lymphoma
Authors: Rubin Michael , Libman Israel , Brisson Marie-Laure , Goldenberg Marvin , Brem Steven
Published: July 2016

We report a case of primary CNS lymphoma in which complete, though temporary, spontaneous clinical and radiologic remission occurred. This is the first such case report to our knowledge.
Acetylcholine Receptor Antibodies in Myasthenia Gravis: Use of a Qualitative Assay for Diagnostic Purposes
Authors: Oger Joel , Kaufman Robin , Berry Kenneth
Published: July 2016

We have modified the techniques of Lindstrom and of Tindall to measure serum acetylcholine receptor antibody using human antigen bound to 125I-alpha Bungarotoxin. By using 10 ä1 of serum and precipitating antigen-antibody complexes with an excess of staph A, we found that only one out of 43 patients with clinically diagnosed active generalized Myasthenia Gravis had no antibodies. In pooling these results with the results of tests done for diagnostic purposes we found positive results in 54/55 generalized active MG, 8/21 MG in remission, 16/37 ocular MG and 0/55 healthy controls. Two out of 38 non MG were also positive and their clinical diagnosis of botulism and penicillamine treated rheumatoid arthritis have been confirmed by a one year follow-up. Most of these sera were also tested for reactivity with fetal calf AchR. Six out of 49 samples positive with the human receptor were negative with calf receptor. We conclude that our technique is extremely useful for the diagnosis of Myasthenia Gravis and that fetal calf antigen cannot replace human antigen in the assay.
Familial Parkinson's Disease: Possible Role of Environmental Factors
Authors: Calne Susan , Schoenberg Bruce , Martin Wayne , Uitti Ryan J. , Spencer Peter , Calne D.B.
Published: July 2016

We report here six families with Parkinson's disease in whom the onset of symptoms tended to occur at approximately the same time irrespective of the age of the patient. The mean difference in the time of onset in different generations was 4.6 years while the mean difference in age of onset in children and parents was 25.2 years. We construe this pattern of age separation within families as suggestive of an environmental rather than genetic cause. Support for this view derives from the lack of correlation between occurrence of the disease and the degree of consanguinity. We conclude that our findings are in accord with the hypothesis which attributes the cause of some cases of Parkinson's disease to early, subclinical environmental damage followed by age-related attrition of neurons within the central nervous system.
Interpretation of Single Band Patterns in CSF Protein Electrophoresis
Authors: Bass B.H. , Armstrong H. , Weinshenker B. , Ebers G.C. , Noseworthy J.H. , Rice G.P.A.
Published: July 2016

We have examined the diagnostic significance of finding one band in the immunoglobulin (IgG) region in spinal fluid protein electrophoresis. From January 1983 to January 1986, 855 consecutive CSF electrophoreses were performed on as many patients. A blinded observer identified a single band in the IgG region in 53 cases (6.2%). In only 14 patients (26%), were the clinical features ultimately felt to be due to clinically definite or possible multiple sclerosis (MS). The majority of patients with a single band had another neurological diagnosis (55%) or were neurologically normal (6%). Many of the neurological disorders in which a single band was found were not disorders in which an increased intrathecal synthesis of immunoglobulin or electrophoretic restriction would have been expected. A variety of conditions can produce a single band pattern. The significance of these patterns and the means by which they might be identified are described.
Gamma-Aminobutyric Acid (GABA) and Sepsis-Related Encephalopathy
Authors: Winder T.R. , Minuk G.Y. , Sargeant E.J. , Seland T.P.
Published: July 2016

In order to determine whether disturbances in GABA homeostasis might play a role in the pathogenesis of sepsis-related encephalopathy, serum and brain tissue GABA concentrations from six areas of the brain (cortex, diencephalon, striatum, hippocampus, midbrain, and pons-medulla) were determined in a rat model of bacterial sepsis (cecal ligation and perforation). The results were compared to those obtained from sham operated control animals. All septic animals demonstrated clinical signs of encephalopathy and had elevated serum GABA levels (0.92 ± 0.3 uM versus 0.48 ± 0.15 in controls, p < 0.01). GABA content in the specific subcompartments of the brain, however, were similar in the two groups. These results indicate that although serum GABA levels are elevated during sepsis, GABA is unlikely to play an important role in the pathogenesis of sepsis-related encephalopathy.
Lymphocytic Adenohypophysitis
Authors: McDermott Michael W. , Griesdale Donald E. , Berry Kenneth , Wilkins G. Edward
Published: July 2016

Lymphocytic adenohypophysitis (LAH) is an uncommon disorder in the spectrum of pituitary disease. Twenty-three cases proven by biopsy or at autopsy have been reported since 1962. We report 2 further cases and review the etiology, immunology and pathology of the disease. The diagnosis should be considered in afemale patient who presents during the post-partum period with the clinical picture of a non-functional or prolactin cell pituitary adenoma and evidence of hypopituitarism.
Treatment of Syringomyelia with a Syringosubarachnoid Shunt
Authors: Tator Charles H. , Briceno Carlos
Published: July 2016

The surgical results in 40 patients with syringomyelia, treated with a syringosubarachnoid shunt or other procedures are reviewed. The principal indication for surgery was that of significant neurological deterioration. There were 12 patients with idiopathic syringomyelia without tonsillar ectopia, 12 with an associated Chiari malformation, 11 with post-traumatic syringomyelia and five patients with spinal arachnoiditis. There were 38 syringosubarachnoid shunts performed in 35 patients, and an excellent or good result was achieved in 26 patients (74.3%). In terms of the type of syringomyelia, the best results were obtained in the idiopathic group without tonsillar ectopia and in the post-traumatic group. A short duration of pre-operative symptoms favoured a better outcome, and in our opinion, early surgical treatment is indicated for all patients with neurological deterioration. All eight patients in whom a posterior fossa decompression was performed as the initial surgical procedure required a second operation, either a syringosubarachnoid or syringoperitoneal shunt to achieve neurological improvement or stabilization. Thus, the syringosubarachnoid shunt is an effective therapeutic modality for patients with syringomyelia, particularly for the idiopathic and post-traumatic groups. More than one surgical procedure may be required to achieve cessation of deterioration. Overall, excellent or good results were achieved in 29 (72.5%) of the 40 patients.
Spectral Analysis of the EEG in Craniocerebral Trauma
Authors: Moulton Richard J. , Marmarou Anthony , Ronen Jacob , Ward John D. , Choi Sung , Lutz Harry A. , Byrd Steven , Desalles Antonio , Maset Angelo , Muizelarr J. Paul , Young Harold F.
Published: July 2016

The objectives of the present study were to evaluate the relationship between the fractional amplitudes of the EEG derived from power spectral analysis (PSA) of the electroencephalogram (EEG) and depth of coma measured clinically with the Glasgow Coma Score, and to assess the accuracy of PSA in predicting long-term outcome. Thirty-two patients rendered unconscious by blunt head injury (mean (GCS = 7) had intermittent EEG recordings daily from 1-10 days post injury. There was a significant correlation between fractional amplitude of the EEG and the GCS. The rate and magnitude of change in the EEG and GCS were also correlated. There were significant differences in PSA parameters between improved and deteriorated patient groups at the termination of monitoring (p = .02) and in the change of PSA parameters over time (p = .02). Using linear discriminant analysis of PSA parameters, the accuracy of outcome prognostication based on the six month outcome was approximately 75%. Accurate classification of outcome was possible in a number of patients in whom there was little or no change in the GCS during the period of monitoring.
Mechanisms of Action of Neural Grafts in the Limbic System
Authors: Buzsáki György , Gage Fred H.
Published: July 2016

This review summarizes the range of possible mechanisms of action of neuronal grafts in the central nervous system. It aims to illustrate the capacity and limitations of the transplanted tissue in the promotion of neurological recovery after experimental surgical insults.
Idiopathic Parkinson's disease: Revised Concepts of Cognitive and Affective Status
Authors: Taylor Ann E. , Saint-Cyr J.A. , Lang A.E.
Published: July 2016

Assumptions regarding increased risk of dementia in Parkinson's disease and of depression mimicking the endogenous form are reviewed and challenged from the perspectives of recent findings in both the neuropsychological and anatomical domains. Evidence suggests that depression, while frequent, behaviourally resembles the reactive variety and that selective impairment of cognitive functions considered to depend upon the integrity of the frontal lobes accompanies this disorder. In this regard, it is speculated that the cognitive alterations seen in non-demented parkinson patients are the consequences of dysfunction of the caudate nucleus which contributes significantly to the normal activities processed through the frontostriate “complex loop”.
Familial Alzheimer's Disease
Authors: Sadovnick A.D. , Tuokko H. , Horton A. , Baird P.A. , Beattie B.L. , Spiegel Sanford M.
Published: July 2016

A family with familial Alzheimer's disease (FAD) inherited as an apparent autosomal dominant trait is presented. Twelve individuals (6 females; 6 males) in 4 generations were affected. The disease had its onset in the late 30's, early 40's with death by age 50. Although FAD which appears to be transmitted as an autosomal dominant trait is relatively rare, such families must be identified and carefully counselled with respect to recurrence risks for subsequent generations. In this family, there are currently 20 members of Generation IV, aged 15-37, and 9 members of Generation V, aged 1-11. The majority of these individuals appear to have a 50% risk for developing this disease.
The Effects of Myoinositol on the Autonomic Neuropathy in the Streptozotocin Diabetic Rat - A Freeze Fracture Study
Authors: Monckton G. , Marusyk H.
Published: July 2016

This report describes a freeze fracture study of rat dorsal sympathetic chain in normal, streptozotocin diabetic on normal diet, and a group fed a 1% myoinositol normal diet. In nonmyelinated fibres, the diabetic group had significant loss of particles on the P face ofjuxta-axonal Schwann cell membranes, whilst myelinated nerves showed a profound loss of particles on P and E faces. In the group with adjuvant myoinositol the particle numbers were normal in both types of nerve. Axonal plasma membranes showed increased numbers of particles in the P face of the nonmyelinated membranes in the diabetic, but were normal in the myoinositol group. Myelinated axonal membranes showed no significant difference between the diabetic and the normal. Rats on a myoinositol diet showed a significant decrease in the E face particles. These results are consistent with those described in the sciatic myelinated nerves by earlier authors and reinforce the view that myoinositol is an important membrane constituent as phosphatidyhnositol and is essential for the expression of normal protein particle numbers.
Magnetic Resonance Imaging of Meningio-Angiomatosis
Authors: Kuzniecky Ruben , Melanson Denis , Robitaille Yves , Olivier André
Published: July 2016

We report the case of a patient with meningio-angiomatosis, a rare disorder of the cerebral cortex often associated with Von Recklinghausen's neurofibromatosis. Previous reports have described a variety of radiological findings but in general they have been of little value in the diagnosis of this disorder. We describe the magnetic resonance image, which identified a well-defined lesion. The imaging techniques are reviewed.
Stroke Assessment Scales: Guidelines for Development, Validation, and Reliability Assessment
Authors: Côté Robert , Battista Renaldo N. , Wolfson Christina M. , Hachinski Vladimir
Published: July 2016

The validity and reliability of clinical instruments, including clinical scales, need to be determined. This paper presents guidelines for development, validation, and reliability assessment of stroke assessment scales.
An Unusual Subacute Progressive Motor Neuronopathy with Myasthenia-like Features
Authors: Noseworthy J.H. , Rae-Grant A.D. , Brown W.F.
Published: July 2016

The initial presentation and clinical course of this 60-year old woman suggested a diagnosis of myasthenia gravis. The subsequent development of tongue fasciculations and the lack of response to treatment made a diagnosis of amyotrophic lateral sclerosis (ALS) more likely despite the presence of conjugate gaze paresis and the absence of many of the typical clinical and electromyographic (EMG) findings seen in this condition. The pathological findings were consistent with either a motor neuronopathy or an unusual variant of ALS. We review the clinical and pathological features of this unusual case in this report.
Sphenoethmoidal Sinusitis Complicated by Cavernous Sinus Thrombosis and Pontocerebellar Infarction
Authors: Macdonald Robert L. , Findlay J. Max , Tator Charles H.
Published: July 2016

An unusual case of sphenoethmoidal sinusitis complicated by cavernous sinus thrombosis, meningitis and pontine and cerebellar infarction is described. The patient presented with advanced intracranial complications which in retrospect caused delay in recognition and treatment of the underlying sphenoethmoidal sinusitis. Surgical drainage of the sinusitis was ultimately required. The pathogenesis of these complications is discussed, and the topic of sphenoid sinusitis reviewed in order to emphasize the numerous neurological manifestations of this disease.
Epilepsy: Relationships Between Electrophysiology and Intracellular Mechanisms Involving Second Messengers and Gene Expression
Authors: Gloor P.
Published: July 2016

It is well known that pure absence epilepsy is a benign form of seizure disorder, while most others, particularly partial and convulsive seizures may have transient or permanent deleterious consequences and are more difficult to bring under therapeutic control by anticonvulsants. The hypothesis is proposed that the preservation of GABA-ergic inhibition in absence attacks and its breakdown in most other seizures may explain these differences. Breakdown of GABA-ergic inhibition allows NMDA receptors to become active. This opens the way for Ca2+ to enter the cell. Such Ca2+ entry is a long-lasting phenomenon. It is likely to be massive during most seizures except during absence attacks, and may therefore damage the neuron transiently or permanently. It may even destroy it. Ca2+ entry is also a crucial factor in the activation of the second messenger cascade which involves cytosolic as well as nuclear (genomic) components. Activation of this cascade converts short-lived electrophysiological processes occurring at the membrane into much longer-lasting intracellular processes. These may include plastic changes at the synaptic and receptor level and may account for kindling and the increasing therapy-resistance of long-standing seizure disorders. Changes resulting from massive Ca2+ entry into the neuron may explain why most seizures, except absence attacks, may have deleterious consequences of various kinds, some short-lived, some of longer duration, and some even permanent.
An Update on the Cellular and Molecular Biology of Brain Tumors
Authors: Cusimano Michael D.
Published: July 2016

Patients with tumors of the central nervous system (CNS) remain difficult to treat despite recent advances in surgical, chemotherapeutic and radiotherapeutic techniques. A better understanding of the molecular and cellular biology of neoplasia is providing neuroscientists with a framework on which to devise novel therapies for these patients. It thus becomes imperative that neurologists and neurosurgeons be aware of these advances in basic science that may eventually have a positive impact on patient management. This paper reviews our present knowledge of the process of CNS oncogenesis and the roles that chemicals, viruses, oncogenes, growth inhibitor genes, and growth factors play in the process.
Partially Sequestered Fourth Ventricle: CT and MR Diagnosis of an Unusual Entity
Authors: Wagle V.G. , Melanson D. , Villemure J.G.
Published: July 2016

Encystment of the fourth ventricle, due to occlusion of the aqueduct as well as the foramina of Magendie and Luschka has been described previously. Partial sequestration such as that encountered in two cases described is a less common entity. In these two cases, the aqueduct of Sylvius was occluded, but the basal foramina (Magendie and Luschka) were patent. We discuss this partial sequestration or communicating hydrocephalus of the fourth ventricle on the basis of MR and CT scan findings. Although computed tomography following water soluble cisternography did diagnose the entity indirectly MRI proved to be a superior tool. It permitted direct visualization of the basal foramina noninvasively.
La mère et l'enfant atteints de dystrophie myotonique de Steinert
Authors: Paris G. , Laframboise R. , Bouchard J.-P.
Published: July 2016

The Mother and Infant with Myotonic Dystrophy Pregnancy and delivery present a number of risks for the mother suffering from myotonic dystrophy, and for her infant. Most of the time, she does not even know that she is affected by the disease and a carrier of the gene. We review the complications of pregnancy and delivery in myotonic patients, and propose a simple management with specific items for each gestational period. The child of a dystrophic mother has a 50% risk of inheriting the abnormal gene. He may also exhibit a developmental and malformation syndrome called "congenital myotonic dystrophy". From the beginning, he may show respiratory distress, thereafter inability to swallow and severely hypotonia. Later, he may demonstrate mental retardation. Some of the most obvious signs found in neonates in our practice are illustrated. We also add a few tests to the list of those already recommended for these children.
La dystrophie myotonique: II nuptialité, fécondité et transmission du gène
Authors: Veillette Suzanne , Perron Michel , Mathieu Jean
Published: July 2016

Myotonic Dystrophy: II Marriage, Fertility and Gene Transmission. A sociological study, using a representative sample (N = 218) of the myotonic dystrophy population, made possible an analysis of the relationship between certain demographic characteristics (marriage, fertility) and the mechanisms involved in the transmission of the deleterious gene. The results show a clear differentiation between the marriage rate of women and men affected by myotonic dystrophy. Whereas men show a considerable decline in marriage eligibility, women continue to marry at a young age and in a proportion almost equal to that of the unaffected population. The study also indicates that the average fertility rate among married patients in the Saguenay-Lac-Saint-Jean exceeds the fertility rate reported from affected populations found in other countries. Our study shows an above-normal male fertility rate. This demographic fact explains the overrepresentation of male transmitters noted in the affected population. However, if present trends remain unchanged, women will be more likely to transmit myotonic dystrophy to future generations. These conclusions illustrate how the transmission and spread of a dominant gene follow a pattern that cannot be dissociated from the socio-cultural characteristics taken as a whole, particularly demographic characteristics. They also provide us with useful avenues for setting up future prevention programmes.