bluuebunny/crossref_metadata_2025 huggingface dataset:
Progressive Multifocal Leukoencephalopathy with Gray Matter Involvement
Authors: Ledoux S. , Libman I. , Robert F. , Just N.
Published: July 2016

An unusual case of Progressive Multifocal Leukoencephalopathy (PML) in a Haitian man with AIDS is reported. The lesions involving both white and gray matter are described radiologically and at post-mortem. The implications regarding neuroradiological differential diagnosis in AIDS as well as PML virulence in this type of patient are discussed.
Regional Metal Concentrations in Parkinson's Disease, Other Chronic Neurological Diseases, and Control Brains
Authors: Uitti Ryan J. , Rajput A.H. , Rozdilsky B. , Bickis M. , Wollin T. , Yuen W.K.
Published: July 2016

Metal deficiency or toxicity states have been recognized as a cause of several neurological disorders and are suspected in others. We analyzed four brain regions (frontal cortex, caudate nucleus, substantia nigra, and cerebellum) in 36 human brains for concentrations of 24 metals (Ag, Al, As, B, Be, Ca, Cd, Co, Cr, Cu, Fe, K, Pb, Mg, Mn, Mo, Na, Ni, P, Se, Ti, V, W, Zn). Regional metal concentrations, measured using atomic absorption and atomic emission spectroscopy, were compared between 9 Parkinson's disease (PD) brains, 15 brains from patients with other chronic neurological diseases, and 12 control brains. No significant metal concentration differences were noted between brains from PD and other chronic neurologic disease. However, parkinsonian brains (PD and parkinsonism secondary to neurofibrillary tangle disease) showed lower concentrations of magnesium in the caudate nucleus and copper in the substantia nigra than control brains. These findings may represent an etiologically important clue to parkinsonism.
Distal Sciatic Nerve Compression by a Popliteal Artery Aneurysm
Authors: Beaudry Yves , Stewart John D. , Errett Lee
Published: July 2016

A 77-year-old man developed progressive sensory and motor symptoms in the right lower leg. Examination showed neurological deficits in the distribution of the right tibial and common peroneal nerves. Swellings in both popliteal fossae were palpated. Investigation showed these to be large thrombosed aneurysms. On surgical examination on the right, the aneurysm was found to be compressing the distal sciatic nerve.
Methanol Poisoning: Factors Associated with Neurologic Complications
Authors: Anderson Todd J. , Shuaib Ashfaq , Becker Werner J.
Published: July 2016

Hospital records of thirty patients with methanol poisoning were studied. Neurologic manifestations at presentation including coma, seizures and decreased visual acuity were seen in nineteen patients. The mean blood pH at presentation was significantly lower in the patients with these neurologic signs and symptoms than in the eleven patients without them (p < 0.05). Methanol levels at presentation tended to be higher in patients with neurologic manifestations at presentation and these patients tended to present later after methanol ingestion than those patients without neurologic manifestations. Fifteen patients with methanol poisoning developed serious neurologic sequelae or died. The mean blood pH was significantly lower in this patient group than in those who survived without neurologic sequelae (p < 0.05). Methanol levels at presentation were not different in the patients who developed neurologic sequelae or died as compared to those who did not. The time from ingestion of methanol to presentation at the hospital was however significantly longer in those patients who developed neurologic sequelae or died (p < 0.05). Initiation of treatment within eight hours of ingestion of methanol was associated with a better clinical outcome.
New Evidence for an Active Role of Aluminum in Alzheimer's Disease
Authors: Crapper McLachlan D.R. , Lukiw W.J. , Kruck T.P.A.
Published: July 2016

Application of molecular biological techniques and sensitive elemental analysis have produced new evidence implicating aluminum as an important factor in down regulation of neuronal protein metabolism. Aluminum in Alzheimer's disease may act by electrostatically crosslinking proteins, particularly the methionine containing histone Hl°, and DNA. The consequence of such crosslinking is reduced transcription of at least one neuron specific gene, the low molecular weight component of neurofilaments. In the superior temporal gyrus in Alzheimer's disease, down regulation of this gene occurs in approximately 86% of surviving neurons and, therefore, aluminum must be considered as having an active role in the pathogenesis. Epidemiological studies are reviewed that independently support the hypothesis that environmental aluminum is a significant risk factor. Preliminary evidence also suggests that a disorder in phosphorylation may be an important initiating factor.
Fukuyama Type Congenital Muscular Dystrophy in a Turkish Child
Authors: Topaloğlu Haluk , Renda Yavuz , Gögüs Safiye , Benli Sibel , Nurlu Gülay , Yildirim Mustafa
Published: July 2016

Congenital muscular dystrophy (CMD) is a heterogeneous group of disorders which is associated with more or less degrees of cerebral involvement. There are four separate entities within CMD nosology. Among these Fukuyama's CMD (FCMD) is highly prevalent in Japan, whereas the classic form with normal or subnormal intelligence, also known as the occidental type, covers the vast majority of cases in the West. We report a case of FCMD seen in a Turkish child.
Isolated Angiitis of the Central Nervous System in Childhood
Authors: Matsell D.G. , Keene D.L. , Jimenez C. , Humphreys P.
Published: July 2016

Isolated angiitis of the central nervous system, a rare inflammatory condition of the nervous system, characterized by vasculitis of the small vessels, has not, to the best of our knowledge, been described in childhood. It usually presents with a diversity of neurological symptoms in the fifth to eighth decades of life. We reviewed the case of an 8 year old male with autopsy proven isolated cerebral angiitis, to encourage the consideration of this disorder in children; and to emphasize the lack of sensitivity of present modalities of neurological investigation in the diagnosis.
Correlation of CT and MR with Impedance Monitoring and Histopathology in Stereotactic Biopsies
Authors: Gorecki J. , Dolan E.J. , Tasker R.R. , Kucharczyk W.
Published: July 2016

Magnetic Resonance Imaging (MR) and Computer Assisted Tomography (CT) guided stereotaxis combined with intraoperative impedance monitoring and multiple sequential biopsies provides the opportunity to correlate the results of radiologic imaging with impedance and histopathology. The authors present the methods used and preliminary results obtained from 30 stereotactic biopsies with complete correlation in 12 cases. Impedance changes correlate accurately with lesion margins as defined by histology. CT images of enhancing lesions correlate quite closely to the histopathologic lesion margins whereas the appearance of the lesion on MR images is often larger than subsequently identified by either impedance or at pathologic examination. Impedance monitoring is a useful adjunct to stereotactic biopsy as it helps to accurately define lesion margins and can help direct the choice of biopsy sites.
Lack of Association Between Two Restriction Fragment Length Polymorphisms in the Genes for the Light and Heavy Neurofilament Proteins and Alzheimer's Disease
Authors: Lacoste-Royal Ginette , Mathieu Martine , Nalbantoglu Josephine , Julien Jean-Pierre , Gauthier Serge , Gauvreau Denis
Published: July 2016

The etiology of Alzheimer disease (AD) remains unknown. The hypothesis of genetic factors playing a role in the causation of the disease, at least in its familial form, has been borne out by results showing linkage in several early-onset AD families to a locus on the proximal part of the long arm of chromosome 21. Linkage was not detected in several other families using the same markers. The metabolism of neurofilaments is perturbed in AD, as indicated by the presence of neurofilament epitopes in neurofibrillary tangles, as well as by the severe reduction of the expression of the gene for the light neurofilament subunit in AD brain. To detect a possible anomaly that might relate to the disease, we have searched for an association between the genes for the light subunit and the heavy subunit of the neurofilament triplet, and AD. Genotypes for restriction fragment length polymorphisms (RFLP) at each of the two loci were determined for an AD group and a control group. Allelic frequencies at a Taql-defined RFLP for the gene for the light neurofilament subunit were 0.70 for the 3.7 kb allele and 0.30 for the 2.9 kb allele. Hindi detected an RFLP for the heavy neurofilament subunit gene with frequencies of 0.31 for the 18.0 kb allele and 0.69 for the 6.8 kb allele. Frequencies were found to be similar in the two groups for both light and heavy neurofilament subunit loci. Although it cannot be excluded that mutations at other sites of the neurofilament genes are relevant to AD, the data reported here do not support an association between these genes and the disease.
Add-on Trial of Clobazam in Intractable Adult Epilepsy with Plasma Level Correlations
Authors: Guberman A. , Couture M. , Blaschuk K. , Sherwin A.
Published: July 2016

Clobazam, a novel benzodiazepine, was used as an add-on agent in 47 adult patients with intractable epilepsy and a variety of seizure types. A >75% reduction in seizure frequency was observed in 42% (18) of the patients and a >50% reduction in 65% (29) of the patients which was sustained over a mean follow-up period of 13.3 (±5.6) months. Nine patients had to discontinue the drug due to minor adverse effects or increased seizures. Possible tolerance developed in 6 patients. Plasma levels of the active metabolite N-desmethylclobazam were linearly related to dosage and appeared to correlate with both therapeutic and toxic responses. Clobazam appears to be a safe and effective add-on antiepileptic for a wide variety of seizure types in intractable epilepsy.
Legal Issues in the Practice of Neurology and Neurosurgery
Authors: Benoit Brien G. , Marshall T. David , Ivan Leslie P. , Forcier Pierre , Evans Kenneth G.
Published: July 2016

Canadians are suing their doctors for malpractice, at three times the rate they were 15 years ago, and during that period awards to injured patients have quadrupled in size. Since the middle 1970's when the first medical malpractice “crisis” occurred in the United States, Canadian clinical neuroscientists have felt increasingly threatened by the prospect of a lawsuit for negligence. Since medicine is intrinsically a risk taking business, adverse outcomes are inevitable. Nevertheless, accusations of negligence and carelessness set out in a Statement of Claim causes considerable stress for the defendant physician, who frequently reacts with self-doubt, depression and aggressive behaviour. A “tort” is defined as a wrongful act, and the 4 elements comprising the tort of medical malpractice are: a breech of standard care, the breech was the proximate cause of the injury, the injury produced measurable damage, and it was foreseeable that the injury would have been less injurious to the patient if the caregiver had conducted a different course. In order to put these issues into current perspective, this symposium was convened as part of the XXIV Canadian Congress of Neurological Sciences, held in Ottawa June 17, 1989.
Transfer Constants for Blood-Brain Barrier Permeation of the Neuroexcitatory Shellfish Toxin, Domoic Acid
Authors: Preston Edward , Hynie Ivo
Published: July 2016

The cause of the toxic mussel poisoning episode in 1987 was traced to a plankton-produced excitotoxin, domoic acid. Experiments were undertaken to quantitate the degree to which blood-borne domoic acid can permeate the microvasculature to enter the brain. Pentobarbital-anesthetized, adult rats received an i.v. injection of 3H-domoic acid which was permitted to circulate for 3-60 min. Transfer constants (Ki) describing blood-to-brain diffusion of tracer were calculated from analysis of the relationship between brain vs plasma radioactivity with time. Mean values (mL.g-1.s-1 x 106) for permeation into 7 brain regions (n = 10 rats) ranged from 1.60 ± 0.13 (SE) to 1.86 ± 0.33 (cortex, ponsmedulla respectively), and carrier transport or regional selectivity in uptake were not evident. Nephrectomy prior to domoic acid injection resulted in the elevation of circulating plasma tracer level and brain uptake. The Ki values are comparable to those for other polar compounds such as sucrose, and indicate that the blood-brain barrier greatly limits the amount of toxin that enters the brain. Together with absorbed dosage, integrity of the cerebrovascular barrier and normal kidney function are important to the outcome of accidentally ingesting domoic acid.
Analgesic Effects of Vibration and Transcutaneous Electrical Nerve Stimulation Applied Separately and Simultaneously to Patients with Chronic Pain
Authors: Guieu Régis , Tardy-Gervet Marie-Françoise , Roll Jean-Pierre
Published: July 2016

The analgesic effects of transcutaneous electrical nerve stimulation (TENS) and vibratory stimulation (VS), used both separately and simultaneously, were compared in 24 patients suffering from chronic pain. We tested the hypothesis that these combined procedures might improve the pain reducing effects obtained with a single type of stimulation, since they make it possible to recruit a larger number of large diameter afferents and/or to increase the discharge frequencies. Four 35-minute treatment sessions (VS, TENS, VS+TENS, Sham stimulation) were run with each patient. The vibrations (100 Hz) and TENS (100 Hz) were applied to the surface of the painful region. The sham stimulation treatment consisted of positioning the TENS electrodes without actually delivering any current. The short form of the McGill pain questionnaire was used to assess the subjects' pain levels. The assessments took place immediately after any treatment (Oh.), and again 4 hours and 24 hours later. The results showed that dual stimulation not only alleviated pain in more cases than either VS or TENS alone, but also had stronger and more long-lasting analgesic effects. On the other hand, all three types of stimulation used produced stronger analgesic effects than those obtained with the sham stimulation.
Graves' Thyrotoxicosis and Moyamoya Disease
Authors: Kushima Kenji , Satoh Yutaka , Ban Yoshio , Taniyama Matsuo , Ito Kunihiko , Sugita Koujiro
Published: July 2016

Cerebrovascular accidents due to Moyamoya disease, a disorder characterized by arterial stenosis at the base of the brain accompanied by typical net-like collateral vessels, occurred in two young Japanese women with Graves' disease when they were in thyrotoxicosis. In one patient, a second attack of cerebral infarction occurred with the recurrence of thyrotoxicosis. Association of Moyamoya disease and Graves' thyrotoxicosis is rare and the pathegenetic relationship is discussed.
Spasmodic Torticollis — Review of 220 Patients
Authors: Rondot P. , Marchand M.P. , Dellatolas G.
Published: July 2016

220 patients with isolated and idiopathic spasmodic torticollis were followed and treated over a 14 year period. Each patient was given a short questionnaire leading to the present retrospective data analysis. In most areas, including female preponderance and frequency of postural tremor, the findings confirmed previous studies and highlighted particular points: importance of psychopathological antecedents and association with stressful life-events. The discussion deals with some of the conflicting debates surrounding this unusual disorder. What is the role of psychopathological factors? What is the therapeutic prognosis? The best therapeutic results were obtained by combining anticholinergic drugs, local injections and rehabilitation.
Correlation between Cerebral Blood Flow, Somatosensory Evoked Potentials, CT Scan Grade and Neurological Grade in Patients with Subarachnoid Hemorrhage
Authors: Fazl M. , Houlden D.A. , Weaver K.
Published: July 2016

Cerebral blood flow (CBF) and central conduction time (CCT) were recorded from 58 subarachnoid hemorrhage patients and from 49 age-matched controls. CBF was calculated following Xenon inhalation and CCT was determined from somatosensory evoked potentials (SSEP's) following median nerve stimulation. Each patient had a CT scan on the day of admission which was graded from I-IV. CBF, CCT and neurological grade (Hunt and Hess classification) were concomitantly recorded 1, 4, 7 and 14 days after subarachnoid hemorrhage. Mean CBF was highest in patients with neurological grades I and II (48.6 ± 12.3 and 48.1 ± 10.3 ml/lOOgm/min respectively) and lowest in patients with neurological grade IV (37.3 ± 9.6 ml/lOOgm/min). Patients in neurological grade I or II had mean CBF and CCT measurements that were significantly different from those obtained from patients in neurological grade IV (P < 0.05). Neurological grade and CT scan grade correlated with CBF (P < 0.0001) better than CCT (P = 0.015). Unexpectedly low CBF's from patients in neurological grades II and III (< 37 and < 31 ml/lOOgm/min respectively) failed to significantly prolong CCT suggesting CCT is unable to detect marginal ischemia. A significant correlation between CBF and CCT occurred only when CBF was < 30 ml/lOOgm/min (R = 0.75, P = 0.05). It appears that prolonged CCT is associated with a drop in CBF only when CBF drops below a certain threshold.
3-Nitropropionic Acid - Exogenous Animal Neurotoxin and Possible Human Striatal Toxin
Authors: Ludolph A.C. , He F. , Spencer P.S. , Hammerstad J. , Sabri M.
Published: July 2016

3-Nitropropionic acid (3-NPA) — a suicide inhibitor of succinate dehydrogenase — is a widely distributed plant and fungal neurotoxin known to induce damage to basal ganglia, hippocampus, spinal tracts and peripheral nerves in animals. Recent reports from Northern China indicate that 3-NPA is also likely to be responsible for the development of putaminal necrosis with delayed dystonia in children after ingestion of mildewed sugar cane. This article discusses the role of 3-NPA in the causation of the disease in China, its neurotoxic effects in animals and the potential role for this compound as a probe of selective neuronal vulnerability.
Chronic Aluminum-Induced Motor Neuron Degeneration: Clinical, Neuropathological and Molecular Biological Aspects
Authors: Strong Michael J. , Garruto Ralph M.
Published: July 2016

The monthly intracisternal inoculation of young adult New Zealand white rabbits with low-dose (100 Μg) aluminum chloride induces aggregates of phosphorylated neurofilament that mimics the intraneuronal inclusions of amyotrophic lateral sclerosis. The chronic progressive myelopathy and topographically-specific motor neuron degeneration that occurs in the absence of suppressions of neurofilament messenger RNA levels in this model contrasts with the acute fulminant encephalomyelopathy and nonspecific gene suppressions that occur subsequent to high-dose (1000 Μg) aluminum chloride inoculations. Further analysis of this unique model of chronic motor system degeneration can be expected to provide additional insights into the pathogenesis of amyotrophic lateral sclerosis.
Preoperative Electroencephalographic Investigation in Temporal Lobe Epilepsy: Extracranial and Intracranial Recordings
Authors: Gloor Pierre
Published: July 2016

Preoperative EEG investigations of patients with temporal lobe seizures include extracranial interictal and ictal recordings during wakefulness and sleep, including long-term EEG and video-monitoring. Interictal epileptiform discharges when evaluated conservatively and in conjunction with other EEG and non-EEG localizing information, provide valuable guidance for the identification of the area to be resected, as do ictal recordings. When extracranial EEG features in conjunction with non-EEG data provide conflicting localizing information, intracranial recordings with stereotaxically implanted depth and epidural electrodes are used. Intracranial recordings must be designed to avoid biasing the exploration strategy in favor of one's preferred localizing hypothesis. Patients with evidence for bitemporal epileptogenic dysfunction in extracranial EEG recordings are suitable candidates for intracranial recordings. The majority of the patients explored in this manner show that all or more than 80% of their seizures arise from one temporal lobe. Excision of that lobe yields satisfactory results in a fair proportion of these patients. The number of satisfactory outcomes is, however, still somewhat less than in patients with unilateral temporal foci in extracranial EEG recordings.
Tailoring of Cortical Excisions for Frontal Lobe Epilepsy
Authors: Rasmussen Theodore
Published: July 2016

Follow-up data of 2 to 49 years' duration are presented on 257 patients with cortical excisions for non-tumoral frontal lobe epilepsy. Twenty-six percent have become and remained seizure free. Another 30% have had a marked reduction of seizure tendency. Thus a total of 56% have had a long term complete or marked reduction of seizure tendency after the cortical resection. The cortical resections were classified into 5 regional categories. Forty-seven percent of patients with anterior frontal resections were in the seizure free category, as were 35% of patients with excisions limited to the convexity cortex and 32% of patients with maximal or near maximal frontal lobectomy. Only 18% of patients with frontal parasaggital excisions were in the seizure free category, and 10% of the patients who had excision of frontal cortex plus limited excision of adjacent central and/or temporal cortex.
Origin of Friedreich's Disease in Quebec
Authors: Barbeau A. , Sadibelouiz M. , Roy M. , Lemieux B. , Bouchard J.P. , Geoffroy G.
Published: July 2016

We have been able to trace 40 cases of classical Friedreich's disease from 14 previously unrelated French Canadian kindreds to one common ancestral couple arriving in New France in 1634: Jean Guyon and Mathurine Robin.

One member of this couple presumably introduced one gene for Friedreich's disease into the French Canadian population. This gene has now been traced over 12 generations to both parents of the present cases. We plan to use this knowledge to study the spectrum of clinical manifestations of this gene and to carry out gene chromosomal localization studies, using the techniques of linkage and of molecular biology. Such studies in rare autosomal recessive disorders have previously been judged to be almost impossible.
A simple device to obtain reliable foot radiographs of ambulatory and non-ambulatory friedreich's ataxia patients
Authors: Billimoria M. , Allard P. , Sibille J. , Sirois J.P. , Duhaime M. , Geoffroy G.
Published: July 2016

A simple radiographic apparatus standardizing foot position and simulating weight-bearing effects in the feet is described in this paper. It provides an effective means of obtaining reliable radiographic data for both ambulatory and nonambulatory patients. This device is presently used in an on-going study on pes cavus in Friedreich's ataxia.
Lysosomal enzymes in ataxia: discovery of two new cases of late onset hexosaminidase A and B deficiency (adult sandhoff disease) in French Canadians
Authors: Barbeau A. , Plasse L. , Cloutier T. , Paris S. , Roy M.
Published: July 2016

We have measured in leukocytes the following lysosomal enzymes in II Friedreich disease cases, 11 “atypical” recessive ataxias, 13 neurological controls and 16 normal controls: hexosaminidase A and B; (3-galactosidase and neuraminidase (labile and cold stable, or A and B). The lysosomal enzyme deficiencies known to produce certain forms of spinocerebellar degeneration were not present in Friedreich's disease or the Charievoix-Saguenay syndrome. The very small scale survey of “atypical” recessive ataxias revealed 3 cases of severe deficiencies in hexosaminidase activity. Two adult brothers presenting with the clinical phenotype of Kugelberg-Welander disease (one also with ataxia), were shown to have a severe deficiency of both HEX A and HEX B activity (Sandhoff biochemical pattern). This is the first such report. A further adult female patient, unrelated to the others, had a severe isolated deficiency of HEX B and presented with a very slowly progressive and mild ataxia with severe internal strabismus. These patients and their families are being studied clinically and biochemically in greater detail and will be reported elsewhere. However these preliminary findings justify screening for such lysosomal defects in all cases of “atypical” recessive ataxia.
Plasma Cholesteryl Sulfate in Friedreich's ataxia
Authors: Nestruck A.C. , Huang Y.S. , Eid K. , Dufour R. , Boulet L. , Barbeau A. , Davignon J.
Published: July 2016

Alteration of membrane fluidity and anomalies of membrane structural proteins have been suspected in Friedreich's ataxia. Plasma lecithinxholesterol acyltransferase (LCAT) activity is also lowered in this disease, presumably because of a substrate effect. The membrane-stabilizing effect of cholesteryl sulfate (CS) and its inhibitory effect on LCAT activity prompted us to measure this substance in the plasma of Friedreich's ataxia patients as well as in normal subjects and in patients with Charlevoix-Saguenay disease. Plasma cholesteryl sulfate concentrations were significantly higher in Friedreich's ataxia, with levels above the upper limit of normal in nearly half of the cases. This increase was unrelated to age, sex or plasma cholesterol levels, but closely associated with the severity of the disease and thus considered to be secondary. A similar phenomenon (except the association with severity) was observed in Charlevoix-Saguenay ataxia. Levels also tended to be higher in first-degree relatives of Friedreich cases. The significance of these findings is discussed in the light of recent knowledge and experimental data obtained in this laboratory on rats made deficient in essential fatty acids. The highest concentrations of CS observed in Friedreich's ataxia (1097 µ-g/dL, 6 times the normal mean) was only 25% as high as the concentrations reported to inhibit LCAT activity.
The Brain of the Planarian as the Ancestor of the Human Brain
Authors: Sarnat Harvey B. , Netsky Martin G.
Published: July 2016

The planarian is the simplest living animal having a body plan of bilateral symmetry and cephalization. The brain of these free-living flatworms is a biiobed structure with a cortex of nerve cells and a core of nerve fibres including some that decussate to form commissures. Special sensory input from chemoreceptors, photoreceptor cells of primitive eyes, and tactile receptors are integrated to provide motor responses of the entire body, and local reflexes. Many morphological, electrophysiological, and pharmacological features of planarian neurons, as well as synaptic organization, are reminiscent of the vertebrate brain. Multipolar neurons and dendritic spines are rare in higher invertebrates, but are found in the planarian. Several neurotransmitter substances identified in the human brain also occur in the planarian nervous system. The planarian evolved before the divergence of the phylogenetic line leading to vertebrates. This simple worm therefore is suggested as a living example of the early evolution of the vertebrate brain. An extraordinary plasticity and regenerative capacity, and sensitivity to neurotoxins, provide unique opportunities for studying the reorganization of the nervous system after injury. Study of this simple organism may also contribute to a better understanding of the evolution of the human nervous system.
The Nucleus Basalis of Meynert
Authors: Ezrin-Waters Cheryl , Resch L.
Published: July 2016

The nucleus basalis of Meynert has been studied extensively in the recent literature. Interest in this nucleus has resulted from the discovery that it is a major source of cortical cholinergic input and that there is neuronal loss in the nucleus basalis in some dementing illnesses. Consistent and severe involvement of the nucleus basalis of Meynert has been found in Alzheimer's disease and in the dementia accompanying Parkinson's disease. Occasional involvement is present in other dementing illnesses, such as progressive supranuclear palsy, Parkinsonism-Dementia complex of Guam, dementia pugilistica, Pick's disease, Korsakoff's syndrome, Down's Syndrome and Creutzfeldt- Jakob disease. Huntington's disease spares this nucleus. However, the role of the nucleus in cognitive function is as yet undetermined. Even its alteration with normal aging remains controversial. This review details the pathological studies of this region to date, with particular emphasis on the dementias. Its role in the dementias of Alzheimer's disease and Parkinson's disease is specifically addressed.
Cytoplasmic RNA in Nervous System Tumours in Children: A Fluorochromic Histochemical Study using Acridine Orange
Authors: Sarnat Harvey B. , Curry Bernadette , Rewcastle N.B. , Trevenen Cynthia L.
Published: July 2016

Acridine orange was used as a fluorochromic histochemical stain of nucleic acids, applied to 78 neoplasms of the central and peripheral nervous systems of 60 children. Some cases were compared with 5 adults and 4 other cases of chronic reactive gemistocytic gliosis. Opposite concentration gradients of cytoplasmic ribonucleic acid (RNA) was demonstrated in tumours of the neuronal/neuroectodermal series, and those of the glial/neuroepithelial series. Minimal AO-RNA fluorescence was seen in 8 cerebellar medulloblastomas and in a retinoblastoma; strong AO-RNA fluorescence occurred in one cerebellar medulloblastoma and in 3 primitive neuroectodemal tumours of the cerebral cortex. Intermediate intensity of fluorescence was found in neuroblastomas, and strong fluorescence was shown in well differentiated ganglioneuroma cells and in cells of chromaffin tumours. Among glial tumours, by contrast, the most anaplastic cells displayed the most RNA fluorescence, while better differentiated astrocytoma cells showed much less. Gradients also were found within some astrocytomas, corresponding to zones of relative anaplasia. Minimal or no fluorescence was detected in reactive gemistocytes or in oligodendroglioma cells. Ependymomas were weakly fluorescent and choroid plexus papillomas showed more fluorescence, similar to the findings in normal ependyma and choroid plexus. Several non-neuroepithelial tumours of the nervous system and Schwannomas also were studied. The acridine orange technique applied to either frozen or paraffin sections of nervous system tumours, has value as an adjunct in the diagnosis and grading of these neoplasms and perhaps in distinguishing reactive gliosis from benign astrocytoma.
Prevalence of Multiple Sclerosis in British Columbia
Authors: Sweeney Vincent P. , Sadovnick Adele D. , Brandejs Vilma
Published: July 2016

A province wide prevalence study on multiple sclerosis (MS) was conducted in British Columbia (B.C.). The prevalence date was July 1, 1982. The major portion of this study was a review of all the files of neurologists practicing in B.C. as this was judged to be the most accurate source for identifying MS patients. 239,412 neurologists' files were hand searched by one researcher using modified Schumacher criteria for classification. Other sources used during the study for identifying MS patients were the MS Clinic, general practitioners, ophthalmologists, urologists, specialized facilities such as long term care facilities and rehabilitation centres, and patient self-referrals.

A total of 4,620 non-duplicated cases were identified and classified. 4,112 of these (89%) were classified according to information contained in neurologists' records.

The prevalence estimate for definite/probable MS in B.C. was 93.3/100,000 population. This increased to 130.5/100,000 population if possible MS and optic neuritis were also included. These rates are among the highest reported in Canada or elsewhere. The cooperation of B.C. neurologists made this study unique in its scope and accuracy of diagnosis.
Nitrazepam for Periodic Movements in Sleep (Sleep-related Myoclonus)
Authors: Moldofsky Harvey , Tullis Carole , Quance Grace , Lue Franklin A.
Published: July 2016

In a follow-up study (mean, approximately six months), nitrazepam was helpful in suppressing periodic movements in sleep (sleep-related myoclonus) and improving disturbed sleep physiology and daytime symptoms of 13 patients (mean age - 53 yr).
Rapid Reversal of Ergotamine-induced Vasospasm
Authors: Lewis P. Jeffrey , Noseworthy Thomas W. , Fitzgerald Avril A. , Andrews George C. , Geeraert Albert J.
Published: July 2016

Peripheral ischemia, secondary to ergotamine tartrate and caffeine suppositories is reported in a 48-year-old female. Lower extremity pre-gangrenous changes were unresponsive to surgical sympathectomy, calcium channel blockade, intra-arterial vasodilators and systemic anticoagulation. A dramatic clinical and radiological reversal of the vasospasm was obtained with intravenous sodium nitroprusside when surgical amputation appeared inevitable.
Tourette-like Syndrome Following Low Dose Short-term Neuroleptic Treatment
Authors: Lal Samarthji , AlAnsari Esam
Published: July 2016

A Tourette-like syndrome (TLS) may occur after long-term neuroleptic treatment. A review of 11 cases reported in the literature is given. We describe the onset of a TLS in a 13-year old boy with childhood schizophrenia after short term, low-dose treatment with thioridazine. The syndrome resolved 5 months after neuroleptic withdrawal. Subsequent exposure to neuroleptics (mainly perphenazine) induced a recurrence of motor tics and involuntary vocalizations which resolved on drug discontinuation. Awareness that neuroleptics may induce a TLS may lead to prompt recognition and avoidance of labelling the manifestations as symptoms of the underlying psychosis or attention-seeking behaviour.
“Whiplash” - Injury of the 2nd Cervical Ganglion and Nerve
Authors: Keith William S.
Published: July 2016

Amongst the many patients with persisting neck pain and headache following cervical injuries are a small number in whom the mechanism is compression of the second cervical nerve root and ganglion. This paper describes the clinical features in 14 patients seen by the author. The main features are unilateral pain in the upper cervical and occipital region, tenderness in the suboccipital region, and diminished sensation in the C2 dermatome. The anatomical basis for this syndrome is discussed and illustrated with dissections from a cadaver.
Swallow Syncope
Authors: Guberman A. , Catching J.
Published: July 2016

Swallow syncope is an often misdiagnosed rare disorder due to enhanced vagal tone during eating in patients with underlying esophageal and/or cardiac abnormalities. We present three cases of this disorder, one related to digitalis toxicity and the other two with diffuse esophageal spasm. The investigation, differential diagnosis, prognosis and management of swallow syncope are discussed.
A Controlled Study of Oral Vigabatrin (ɣ-Vinyl GABA) in Patients with Cerebellar Ataxia
Authors: Bonnet A.M. , Esteguy M. , Tell G. , Schechter P.J. , Hardenberg J. , Agid Y.
Published: July 2016

Vigabatrin (ɣ-vinyl GABA; GVG), an irreversible inhibitor of GABA-transaminase, at a daily dose of 2-4 g, and a placebo were each administered orally for 4 months to 14 patients with cerebellar ataxia (9 with Friedreich's ataxia, 5 with olivopontocerebellar atrophy), in a double-blind, placebo-controlled crossover study. For the group as a whole, there was no significant difference between the GVG and placebo periods in any of the parameters of cerebellar symptomatology measured. Individually, one patient showed some improvement after 3 months of treatment with 2 g/day GVG. Tolerance to 4 g/day GVG was poor, whereas 2 g/day was well tolerated. The results suggest that agents which increase central GABA concentrations are not likely to be of benefit to patients with Friedreich's ataxia or olivopontocerebellar atrophy.
Speculations on the Role of Transmissible Agents in the Pathogenesis of Alzheimer's Disease
Authors: Haase Ashley T. , Lewis Elizabeth , Wietgrefe Stephen , Zupancic Mary , Jane Diedrich , Minnigan Hal , Ball Melvyn J.
Published: July 2016

Unconventional agents and conventional viruses provide model systems to investigate the pathogenesis of Alzheimer's disease (AD). The essay which follows examines the hypothetical role of herpes simplex in AD and presents some generally applicable experimental approaches to detecting genes in brain tissues. The concluding section, on parallels between AD and diseases of the brain caused by unconventional viruses, defines strategies for isolating genes related to pathology.
Lewy Body Dementia Without Alzheimer Changes
Authors: Sima A.A.F. , Clark A.W. , Sternberger N.A. , Sternberger L.A.
Published: July 2016

Three patients with clinical Alzheimer's disease were found at postmortem examination to have Lewy-bodies and Lewy-like bodies in the cerebral cortex and the pigmented brainstem nuclei. Neuritic plaques were found in neocortical areas but no neurofibrillary tangles. The distribution of cortical neuronal inclusions correlated with the proposed projection of dopamine terminals. Neuronal cell loss was marked in the ventral tegmental area (paranigral nucleus) and the basal nucleus of Meynert, suggesting a defect in dopaminergic and cholinergic innervation of the cerebral cortex. Immunohistochemical investigations revealed positive staining of cortical Lewy- and Lewy-like bodies for monoclonal antibodies to phosphorylated neurofilaments (03-44, 06-17, 04-7). Also cerebral neurons containing no inclusions showed positivity, suggesting an early neurofilament abnormality, preceding the formation of Lewy-type inclusions.
Leuko-Araiosis: An Ancient Term for a New Problem
Authors: Hachinski V.C. , Potter P. , Merskey H.
Published: July 2016

Recent research with computerized tomography (CT) and magnetic resonance imaging (MR) of the brain has revealed a type of tissue change for which no fitting term exists. The change appears as areas of decreased density on CT and changed signals of the white matter in MR images. Because neither a definite pathological change nor a specific clinical deficit has been linked with the CT and MR changes, a designation is required that limits itself to describing the changes themselves.

We propose “leuko-araiosis”. The Greek root leuko-, signifying “white”, has found wide usage in modern medical terminology: e.g. leucine, leukocyte, leukorrhoea. Also several precedents exist for its application to the white matter of the central nervous system: e.g. leukoencephalitis, leukomyelitis, leukotomy. Araios is an adjective meaning “rarefied”, and the suffix -osis converts the adjective to a noun meaning “rarefaction, diminution of density”, a word used in the Hippocratic Collection to describe a state of excessive porosity of the lung.

A descriptive term precludes premature presuppositions and encourages the search for causes.
Optimum Symptomatic Control of Parkinson's Disease with Dopaminergic Therapy
Authors: Bouchard Sylvie
Published: July 2016

This paper presents a review of the literature on the therapeutic action and the side effects of the two main dopaminergic agents: L-DOPA/decarboxylase inhibitor (L-DOPA/DI) and bromocriptine (Parlodel ®) used either as monotherapy or in combination in patients with Parkinson's disease. The combination of L-DOPA/DI and bromocriptine gives the best therapeutic efficacy (49% improvement) in the total score (bradykinesia, rigidity and tremor). However, treatment by monotherapy or combination gives the same pattern of activity: greatest improvement in tremor, followed by rigidity and bradykinesia. Improvement observed in the short term is not sustained over longer periods of time for monotherapy with either drug. The short-term side effects are similar for each treatment, whereas long-term complications (dyskinesia, end-of-dose deterioration and on-off phenomenon) appear only when levodopa is used, alone (high incidence) or in combination with bromocriptine (low incidence). The overall optimum treatment is obtained with a combination of L-DOPA/DI and bromocriptine.
Antioxidant Therapy in Parkinson's Disease
Authors: Grimes J. David , Hassan Mohamed N. , Thakar Jay
Published: July 2016

It is postulated that endogenous oxidative mechanisms are a major factor in the continuing death of dopaminergic neurons and the progression of Parkinson's disease. Scientific evidence in support of, and negating, the free radical auto-toxicity and dopamine toxicity concepts is reviewed. There is conflicting evidence whether free radicals are involved in the toxicity of l-methyl-4-phenyl-1,2,3,6-tetrahydropyridine (MPTP) and attempts to prevent the toxicity of MPTP with antioxidant therapy have had variable results. The oxidation of dopamine by monoamine oxidase produces toxic metabolites however animal studies with high dose longterm levodopa and MPTP have failed to show clear evidence for autoxidation. Firm supportive evidence is obtained from the monoamine oxidase B inhibitor experience which demonstrated a block of the toxicity of MPTP in animals and probable prolongation of the course of human Parkinson's disease.

The scientific data available is inconclusive but there is significant hope of retarding progressive catecholaminergic neuron degenerative changes by augmenting the free radical scavenging system with antioxidants (such as Vitamin E) and slowing catecholamine oxidation by monoamine oxidase B inhibition. Careful clinical trials with these agents must be performed.
Continuous Dopaminergic Stimulation in Parkinson's Disease
Authors: Obeso J.A. , Luquin M.R. , Vaamonde J. , Grandas F. , Martinez Lage J.M.
Published: July 2016

Complex motor fluctuations and dyskinesias (“on-off” phenomenon) in Parkinson's disease can be corrected by parenteral administration of levodopa, levodopa-methyl-ester, lisuride and apomorphine. Levodopa and levodopa-methyl-ester may only be administered intravenously because of their low solubility. Lisuride and apomorphine are readily absorbed after subcutaneous administration. Repeated or continuous intravenous infusions of levodopa have been given for a few days, using a wearable “jacket-like” pump, with good results. So far, lisuride is the only dopamine agonist used for chronic treatment by continuous infusion. The “on-off” effect is adequately controlled in most patients by subcutaneous lisuride administration (plus oral levodopa). However, adverse effects, particularly psychiatric complications, constitute a major limiting factor for routine applications of this form of treatment. Subcutaneous apomorphine infusion is unlikely to become a standard therapeutic procedure in Parkinson's disease, but it is a very valuable research method to gain further insight into the pathophysiology of motor fluctuations in Parkinson's disease.
Electromyography in Disorders of Muscle Tone
Authors: Eisen Andrew
Published: July 2016

No single clinical electrophysiological test can evaluate disorders of muscle tone. These disorders, symptomatic of a variety of diseases have a multifactorial physiological basis. The several tests used are complimentary each aiming to study different aspects of spinal and supraspinal reflexes which become deranged. The H reflex and F wave (H max/M max and F max/M max ratios) measure motoneuron pool excitability in general. The tendon reflex includes spindle mechanisms bypassed by the H reflex and, with limitations, comparison of H max/M max and T max/M max yields information about the -y system. Tonic vibration of a tendon inhibits the H reflex from the same muscle. The TVR measures autogenous presynaptic inhibition exerted by the la afferents of the muscle. Recurrent inhibition via Renshaw cells is evaluated by studying the effect of collision on the H reflex. Reciprocal inhibition of the la afferents can be assessed by measuring H reflex change induced by stimulating la afferents from antagonists. Changes in the H reflex recovery cycle measure polysynaptic influences on spinal motoneuron excitability. Cutaneomuscular (flexor) reflexes measure poly- and oligosynaptic excitatory drive to spinal motoneurons and the blink reflex evaluates the excitatory drive to brainstem motoneurons. Long loop (segmental) responses can be evaluated by limb pertubation using a torque motor or electrical stimulation applied during voluntary muscle contraction. Finally needle electromyography is a more relevant test in several disorders of muscle tone such as the stiff-man syndrome and Isaacs' syndrome.
Motor, Behavioral and Pharmacologic Findings in Tourette's Syndrome
Authors: Jankovic Joseph , Rohaidy Haydee
Published: July 2016

We studied 112 patients with Tourette's syndrome (TS); the male-to-female ratio was 3.8, the mean age of onset was 7.3 years, and the average duration of symptoms prior to the initial evaluation was 15.2 years. Seventy-nine percent of the patients had at least one family member with motor or vocal tics, and an additional 10 percent had a family member with marked obsessive-compulsive behavior. Simple motor tics occurred as the presenting symptom in about one-third of patients; one-third had multiple motor tics at the onset, and another third started with vocal tics. During the course of the illness all patients developed multifocal motor tics and 86 percent had vocal tics. Verbal and mental coprolalia was present in 44 percent of the patients. Copropraxia was seen in 19 percent of patients, and both coprolalia and copropraxia were more frequent among the males than expected. Attentional deficit disorder was diagnosed in 36 percent of the patients and 32 percent had obsessive-compulsive personality. Sleep disturbances were reported by 62 percent of the patients and polysomnography in 34 patients showed motor and vocal tics during all stages of sleep, sleep apnea, abnormal arousal pattern, and other sleep disturbances. Patients with mild symptoms improved with clonidine or clonazepam, but those with more advanced disorder required fluphenazine, pimozide, haloperidol or tetrabenazine.
Global Arteriovenous Malformation of the Cervical Region
Authors: Holgate R.C. , Lougheed W.M.
Published: July 2016

This case was presented as an interesting example of a global arteriovenous malformation (AVM) involving muscle, bone, extradural and intradural spaces of the cervical region.

A twenty-eight year old male in October, 1976, fell at work sustaining a blow to his lower back. The following day he complained of cervical pain. Physical examination was negative and he had a full range of movement of his cervical spine.

Plain films and tomograms of the cervical spine showed a lytic honeycomblesion of C2 and C3, and a 4 mm. subluxation of C3 on C4 (Fig. 1). The bone scan showed increased flow and decreased static activity in the upper cervical region. The radiological diagnosis was vertebral hemangioma; tumor, infection and histiocytosis were unlikely but important differential considerations.
Structural Lesions of the Brain in Young Schizophrenics
Authors: Averback P.
Published: July 2016

Swollen, degenerate and fragmented neurons of the septal nuclei and the nucleus ansae peduncularis are commonly found in chronic neurological diseases such as Alzheimer’s or Huntington’s disease, and are rarely found in neurologically normal control patients without mental symptoms. These pathological lipid and pigment laden neurons appear to be a common feature of schizophrenia and have been consistently found in a series of unselected young (mean age 38) schizophrenics.
Delayed Encephalopathy Following Carbon Monoxide Intoxication
Authors: Sawa G.M. , Watson C.P.N. , Terbrugge K. , Chiu M.
Published: July 2016

The case history and CT scan of a patient with delayed encephalopathy following carbon monoxide intoxication are described. The patient recovered.

The CT scan revealed symmetrical areas of decreased density at the level of the globus pallidus. This case differs in several ways from other cases of carbon monoxide intoxication in which abnormalities of the CT scan were found.