bluuebunny/crossref_metadata_2025 huggingface dataset:
Hemodynamic and Metabolic Effects of Extracranial Carotid Disease
Authors: Leblanc Richard , Yamamoto Y. Lucas , Tyler Jane L. , Hakim Antoine
Published: July 2016

Cerebral blood flow (CBF), cerebral blood volume (CBV), the CBF/CBV ratio – an index of the hemodynamic reserve capacity – the rate of oxygen metabolism (CMRO2), and the fractional extraction of oxygen by the brain (OEF) were studied by positron emission tomography (PET) in the cortical territory of both internal carotid arteries in 15 cases of transiently symptomatic or progressive extracranial atherosclerotic carotid disease. None of the patients had a major stroke or had a significant neurological deficit except 1 whose damaged hemisphere is excluded from study. All were asymptomatic at the time of PET scanning. Values were obtained in the middle cerebral artery (MCA) distribution, and in the anterior and posterior borderzone regions. Eight cases had unilateral carotid stenosis of 80% or greater and 7 had unilateral or bilateral occlusion of the origin of the internal carotid artery. Results obtained in patients were compared using Student's t-test, to those obtained in neurologically normal, elderly volunteers. Patients with carotid stenosis had a significantly decreased CBF (p<.025) and CBF/CBV ratio (p<.025) selectively in the anterior borderzone regions. This was accompanied by a trend toward elevated OEF and declining CMRO2values. Patients with carotid occlusion had significantly decreased CBF (p<.005), decreased CBF/CBV ratio (p<.005) and decreased CMRO2 (p<.025) in the ipsilateral anterior borderzone and MCA territories. Similar changes were present in the opposite hemisphere of patients with bilateral carotid disease. These results indicate that carotid stenosis is associated with hypoperfusion and diminished hemodynamic reserve capacity in the anterior borderzone, and that carotid occlusion produces more widespread hypoperfusion and metabolic depression.
Sensory Neuron Degeneration in Familial Kugelberg-Welander Disease
Authors: Winder Toni R. , Auer Roland N.
Published: July 2016

A 53 year old man developed symptoms of motor neuron disease in childhood. There was a family history of a similar disorder and it was felt to represent a form of Kugelberg-Welander disease. In addition to the motor deficits, sensory abnormalities in his legs were documented during life. Autopsy revealed anterior horn cell loss throughout the length of the spinal cord, with preservation of the phrenic nucleus. The lumbar dorsal root ganglia showed active degeneration of sensory neurons, with nuclear changes exceeding cytoplasmic ones. The fasciculus gracilis showed Wallerian degeneration. The findings provide direct evidence that sensory neurons can degenerate in some forms of motor neuron disease, and that the “demyelination” or “degeneration” of posterior columns sometimes seen in the various forms of motor neuron disease may actually be secondary to cell body disease in the dorsal root ganglia.
Optimal Indices for Testing Parkinsonian Rigidity
Authors: Teräväinen Heikki , Tsui Joseph K.C. , Mak Edwin , Calne Donald B.
Published: July 2016

We assessed parkinsonian rigidity objectively at the wrist to determine the optimal angular velocity and displacement for detecting abnormality. The wrist was moved passively with a torque motor and the average work done for one complete cycle was computed. This objective rigidity score (ORS) was compared with a clinical rigidity score (CRS). ORS was more pronounced at faster movement velocities in parkinsonian patients, whereas in normal subjects there was only a modest increase in the score. Angular velocities of 140 to 190 degrees/second and displacements of ±25 to ±30 degrees were most sensitive for detecting parkinsonian rigidity and had good correlation with the CRS.
Double-Blind Cross-Over Placebo Controlled Study of Flunarizine in Patients With Therapy Resistant Epilepsy
Authors: Starreveld E. , Beukelaar F. de , Wilson A.F. , McLean D.R. , Findlay Helen P.
Published: July 2016

Twenty-five patients with long-standing therapy resistant epilepsy were studied in an eight-month double- blind cross-over add-on trial with a daily dose of 15 mg flunarizine. In five patients the seizure frequency decreased 50% or more. The mean seizure frequency reduction in the patients on flunarizine was 35%. Particularly the control of secondary generalized seizures improved. Flunarizine did not significantly alter the plasma levels of the regular anticonvulsant drugs. Minimal adverse side effects were reported equally in the flunarizine and the placebo group. In three patients depressive symptoms improved and two patients became free of postictal headaches. Flunarizine appears to be a safe adjuvant anticonvulsant.
Transient Anosognosia for Episodic Hemiparesis: A Singular Manifestation of TIAs and Epileptic Seizures
Authors: Grand'Maison F. , Reiher J. , Lebel M.L. , Rivest J.
Published: July 2016

Anosognosia is a well-known manifestation of non-dominant parietal lobe lesions and typically lasts a few days. That anosognosia may last only a few minutes to a few hours, as observed in six patients, has not been reported. In five patients, transient anosognosia for equally brief left-sided hemiparesis was a manifestation of transient ischemic attacks (TIAs). In the sixth patient, anosognosia for both a left-sided motor seizure and a subsequent brief left hemiparesis could best be explained by an epileptic ictal and post-ictal transient dysfunction of the non-dominant parietotemporal cortex. Prompt recognition of transient anosognosia, whether ischemic or epileptic, is mandatory for proper diagnosis and for rapid initiation of specific therapy.
Brain Death and the Persistent Vegetative State: Similarities and Contrasts
Authors: Young Bryan , Blume Warren , Lynch Abbyann
Published: July 2016

Brain death and the persistent vegetative state (PVS) share the following features: 1.) There is death of neurons in the brain; 2.) Both require an etiology which is capable of causing neuronal death. 3.) The potential for cognition is totally and permanently lost; 4.) Intensive medical support is usually withdrawn. In contrast, the diagnosis of brain death depends on death of the brainstem, while PVS implies permanent and total loss of forebrain function. While brainstem death can be diagnosed clinically, accurate prognosis in PVS requires additional investigation. Thus far, the EEG is the most specific test of neuronal function in the cerebral cortex. Brain death is equivalent to death, while PVS is not; management of the latter is more complex because of medical, social, ethical and legal factors.
MR of Deep Cerebral Venous Thrombosis
Authors: Ashforth R.A. , Melanson D. , Ethier R.
Published: July 2016

Two cases of deep cerebral venous thrombosis are presented with specific reference to the CT and MR findings. The MR findings are discussed, with comparison to the findings of superficial cerebral sino-venous occlusion.
Chorea-Acanthocytosis: Report of a Family and Neuropathological Study of Two Cases
Authors: Alonso Ma. Elisa , Teixeira Fernanda , Jimenez Guillermo , Escobar Alfonso
Published: July 2016

We report three siblings, offspring of normal consanguineous parents, with a progressive neurological illness that began in midlife and was characterized primarily by chorea and leading to death in the fourth decade. The proband had erythrocyte acanthocytosis with normal serum β-lipoprotein. Biopsy of left gastrocnemius muscle showed neurogenic muscular atrophy. There was a decrease in the numbers of large myelinated axons of the sural nerve. Postmortem examination of two cases showed marked atrophy, neuronal loss and gliosis of the caudate nucleus and putamen. Autosomal recessive inheritance is likely in this family.
Molecular Genetics of Familial Alzheimer's Disease
Authors: George-Hyslop P.H. , Haines J.L. , Polinsky R.J. , Tanzi R.E. , Farrer L. , Myers R.H. , Gusella J.F.
Published: July 2016

The molecular genetic strategies aimed at isolating and characterizing the defective gene(s) in familial Alzheimer's disease, the inherent limitations of the techniques and recent progress in the field are reviewed. Three independent groups have found an apparent linkage to chromosome 21 but two other groups have not, suggesting that familial Alzheimer's disease may be etiologically heterogeneous.
Tacrine, a Drug with Therapeutic Potential for Dementia: Post-Mortem Biochemical Evidence
Authors: Francis Paul T. , Bowen David M.
Published: July 2016

A review of biochemical findings is presented which support the idea that Alzheimer's disease represents a condition for which tetrahydroaminoacridine (tacrine) may have a beneficial effect. There is evidence that clinical and histopathologic hallmarks of the disease relate to cholinergic and serotonergic dysfunction, with less obvious abnormalities in other neurotransmitters (aspartate, dopamine, gamma-aminobutyrate, glutamate, noradrenaline and somatostatin). Clincially relevant concentrations of tacrine may ameliorate the above presynaptic deficits without producing harmful (neurotoxic) effects of aspartate and glutamate. The disease seems to be associated with an early and clinically relevant degeneration of some neurons with cortical perikarya that release these amino acid transmitters. Studies are now required on the effect of tacrine on postulated harmful peptide-bond hydrolase activity within and around such cells.
Immune System Response in Alzheimer's
Disease
Authors: McGeer P.L. , Akiyama H. , Itagaki S. , McGeer E.G.
Published: July 2016

Involvement of the immune system in the pathogenesis of Alzheimer's disease was demonstrated in two ways: by the attachment of complement proteins to diseased tissue, and by the activation of cells associated with the immune system. Alzheimer brain tissue was stained immunohistochemically by antibodies to components of the classical, but not the alternative, complement pathway. Antibodies to Clq, C3d, and C4d stained senile plaques, dystrophic neurites, neuropil threads and some tangled neurons. Antibodies to a neoantigenic site on the C5b-9 membrane attack complex stained dystrophic neurites and many tangled neurons, but not senile plaques. Antibodies to Factor P and fraction Bb of Factor B, which are specific for the alternative complement pathway, did not stain Alzheimer brain tissue. The cellular immune response was evaluated by the presence of reactive microglia and by the infiltration of small numbers of T-cells into diseased brain tissue. Reactive microglia were identified by antibodies to HLA-DR, a class II major histocompatibility complex glycoprotein, and by enhanced staining with antibodies to leukocyte common antigen and the FC7RI and FcyRII receptors. T-cells were identified by antibodies to leukocyte common antigen, as well as the CD4 and CD8 surface proteins. Double immunostaining with antibodies to GFAP and MHC class I or class II antigens established that astrocytes, which are GFAP positive, do not express MHC antigens in Alzheimer's disease. Endothelial cells express MHC class I antigens while reactive microglia and some leukocytes express class II antigen.
Acute Transverse Myelopathy in Children
Authors: Adams Coleen , Armstrong Derek
Published: July 2016

Twenty-three children with acute transverse myelopathy (ATM) are reviewed. Antecedent minor trauma or exercise was reported in 10 patients. Despite a positive history in 7 patients no preceding infection was documented. Two patients had a history of less severe ATM followed by recovery prior to a second more severe episode. The most common initial symptom was back pain and the most prominent clinical signs were weakness, sensory level and sphincter disturbances. Myelography and CT myelography at presentation was performed to exclude a compressive lesion. Spinal cord enlargement was demonstrated in 6 of 21 cases. Magnetic resonance imaging (MRI) of the spinal cord, performed in one patient, showed enlargement of the cord. Poor prognostic features were severity of weakness at the time of maximum deficit and a delayed onset of recovery. Maximum motor recovery occurred at a mean of 6½ months but did not occur in one patient until 1½ years. Normal or good outcome was obtained in 64%.
Neurochirurgie et Créativité
Authors: Phéline Christian
Published: July 2016

Les mots de Neurochirurgie et de Créativité, semblent à priori antagonistes. D'une part, le monde de cumul, d'érudition, d'exactitude, de codification de la science neurochirurgicale, et d'autre part, celui de la fantaisie et de la liberté de I'improvisation et de l'aléatoire.

Pourtant, devant la stagnation de certaines «récupérations» cérébrales, nous avons senti la nécéssité d'une démarche supplémentaire affranchie des normes traditionnelles.
Comparison of Long Term Results Between Prolactin Secreting Adenomas and ACTH Secreting Adenomas
Authors: Post Kalmon D. , Habas Jo-Ellen
Published: July 2016

A series of 100 prolactin secreting pituitary adenomas was reviewed and demonstrated an early cure rate of 85.2% with a cure rate of 89% if prolactin was less than 200 ng/ml. For macroadenomas the cure rate was 50% giving an overall cure rate of 71% for the entire group. When long term (> 5 years) followup was obtained a 17% incidence of recurrence was noted for the microadenoma group with a 20% recurrence rate for the macroadenoma group. Secretory dynamic studies were done shortly after surgery and then after a delay. Many showed a return to normal prolactin secretory dynamics suggesting that the underlying hypothalamic regulation is normal in most patients. Abnormal secretory dynamics at 6 weeks post operative testing were not predictive of which patients would relapse as many patients who had abnormal dynamics early did not relapse even during prolonged followup. Conversely a normal response to provocative testing did not preclude late relapse. A similar series of 40 consecutive patients with Cushing's disease was reviewed. Tumor was found in all but three cases. 84% of patients were cured and thus far only one patient (2.5%) has shown late recurrence with this occurring at 62/3 years following surgery with normal stimulatory dynamics present for five years. The implication is that hypothalamic regulation is normal in Cushing's disease as well. The differences in recurrence rates may be reflective of the aggressiveness with which one disease is treated, with the acceptance of a higher incidence of hypopituitarism as a consequence of more radical surgery for Cushing's disease.
Is Atrial Fibrillation an Independent Risk Factor for Stroke?
Authors: Phillips Stephen J.
Published: July 2016

That non-rheumatic atrial fibrillation is an independent risk factor for cerebral infarction has not been established with certainty. The rationale underlying contemporary clinical trials of warfarin therapy for the prevention of stroke in patients who have non-rheumatic atrial fibrillation is that the majority of strokes in such patients are due to cardiogenic cerebral embolism. However, there is evidence to suggest that the increased probability of stroke attributed to this arrhythmia is due to its association with other risk factors such as hypertension, diabetes mellitus, and atherosclerosis. The question of who should be anticoagulated is a major public health issue since atrial fibrillation is present in approximately ten per cent of the general population aged 65 or more years.
Selective Deficits in Alzheimer and Parkinsonian Dementia: Visuospatial Function
Authors: Mohr Erich , Litvan Irene , Williams Jill , Fedio Paul , Chase Thomas N.
Published: July 2016

Deficits in visuospatial cognition are frequently cited as an important component of the cognitive changes accompanying Parkinson's disease. To characterize possible differences between Parkinson's (PD) and Alzheimer's (AD) dementia, patients from both groups, matched for overall dementia severity, age and education, were contrasted neuropsychologically. Visuospatial tasks dissociated from memory, were significantly compromised in both patient groups. Differential impairment was evident on visuospatial abstraction and reasoning (Object Assembly), which was most deficient in PD. Visuospatial cognition associated with memory, classified both patient groups as impaired compared to controls, but AD patients demonstrated substantially lower performance levels than those with PD. Parkinsonian dementia thus appears to have some distinct features compared to Alzheimer's disease, which may indicate differences in underlying pathogenic mechanisms.
A Cluster-Focus of Multiple Sclerosis at Henribourg, Saskatchewan
Authors: Hader Walter J. , Irvine Donald G. , Schiefer H. Bruno
Published: July 2016

This study presents the clinical characteristics of 8 victims of multiple sclerosis from the hamlet of Henribourg, Saskatchewan with a population of less than 75 people. A diligent victim of the disease had observed that six female classmates from the early 1940's had later developed multiple sclerosis. Two male military personnel who had also resided briefly in close proximity, during the same common exposure time, also later developed multiple sclerosis. The mean onset time of developing the disease after leaving the area was 20 years. This cluster-focus suggests a common exposure to an environmental factor or a common infective agent in the etiology of multiple sclerosis.
Neuropsychological Deficits and Sleep in Myotonic Dystrophy
Authors: Broughton R. , Stuss D. , Kates M. , Roberts J. , Dunham W.
Published: July 2016

Eight patients with myotonic dystrophy underwent comprehensive neuropsychological testing and overnight polysomnography to assess whether the waking cognitive impairment might be attributable to sleep structure abnormalities or to sleep-related respiratory problems. Patients showed substantial neuropsychological deficits, fragmented nocturnal sleep and, in half the patients, sleep apnea and/or hypopnea both mainly of central type. There was no statistically significant correlation between the degree of daytime cognitive deficit and the degree of sleep fragmentation or of respiratory problems at night. It was concluded that the neuropsychological deficit in mytonic dystrophy cannot be attributed to a secondary effect of nocturnal sleep apnea or sleep disruption but probably represents a direct effect of CNS lesions.
Bulbo-pontine Paralysis with Deafness: the Vialetto-Van Laere Syndrome
Authors: Abarbanel J.M. , Ashby P. , Marquez-Julio A. , Chapman K.R.
Published: July 2016

A Caucasian girl developed slowly progressive sensory neural deafness and bulbar and spinal muscle weakness typical of the Vialetto-Van Laere syndrome. As the condition progressed the major disabilities became dysphagia, respiratory muscle weakness and postural hypotension. Treatment with gastrostomy feedings, oxygen and fludrocortisone acetate produced worthwhile functional improvement.
Pathological and Molecular Biological Features of a Myelopathy Associated with HTLV-1 Infection
Authors: Power Christopher , Weinshenker Brian G. , Dekaban Gregory A. , Kaufmann John C.E. , Shandling Maureen , Rice George P.A.
Published: July 2016

We report the pathological and molecular biological findings of human T-cell lymphotropic virus type 1 (HTLV-1) infection of the spinal cord in a patient with a chronic progressive myelopathy. Light microscopy disclosed loss of myelin and axons, thickening of blood vessels and a lymphocytic cell infiltrate in the spinal cord especially at the cervical and thoracic levels. Electron microscopy confirmed the vascuolar appearance seen with light microscopy but virus particles were not observed. The HTLV-1 gag gene could be amplified (by polymerase chain reaction) from cervical spinal cord tissue while not from elsewhere in the neuroaxis. The presence of HTLV-1 geonomic material in spinal cord tissue has not been previously reported.
Long-Term Evaluation of Sinemet® CR in Parkinsonian Patients with Motor Fluctuations
Authors: Hutton J. Thomas , Morris Jerry L.
Published: July 2016

The safety and efficacy of Sinemet® CR, a controlled-release formulation of carbidopa/levodopa, were investigated in a three year, open-label trial involving 18 parkinsonian patients with fluctuating motor response. The average daily levodopa dosing frequency did not change significantly during long-term treatment. Efficacy measures generally revealed a gradual progression of parkinsonian disability. Patient diaries of motor fluctuations revealed relative stability of time “on” but with a tendency toward increased time “on with dyskinesias” over the 36 month followup period. There were no adverse laboratory results deemed to be related to Sinemet® CR and no unexpected side effects were observed.
Eosinophilic Granuloma of the Occipital Bone Presenting as Intracranial Venous Hypertension
Authors: Wightman H. , Wheelock B.
Published: July 2016

Intracranial venous sinus thrombosis is an uncommon cause of pseudotumor cerebri. The diagnosis is often not confirmed on the rationale that treatment will not be altered. We report a case presenting a pseudotumor cerebri where the underlying pathology disclosed dural sinus thrombosis resulting from compression by an eosionophilic granuloma of the occipital bone. Routine CT of the head and Technetium-99m brain scan initially demonstrated neither tumor nor thrombosis. Plain skull x-rays subsequently revealed a lytic lesion of the occiput. When reinvestigated with CT using bone density windows the tumor was revealed. Excision of the tumor and a short course of cobalt therapy was curative. Special techniques in nuclear scanning, CT and MRI designed to improve the sensitivity for diagnosing venous sinus thrombosis are described. This case illustrates the importance of establishing a definitive diagnosis and shows the importance of pre-test consultation between clinicians and radiologists to ensure that specific investigative techniques are properly utilized.
Expression of the Immediate Early Gene c-fos in Basal Ganglia: Induction by Dopaminergic Drugs
Authors: Robertson H.A. , Paul M.L. , Moratalla R. , Graybiel A.M.
Published: July 2016

Expression of the immediate early gene c-fos is increased in mammalian neurons by a number of stimuli and the usefulness of this gene as a marker of neuronal activation has been demonstrated in several systems. Directlyacting dopamine agonists of the D1-type (SKF 38393, CY 208-243) and indirectly-acting dopamine gonists (amphetamine, cocaine) all produce a rapid and transient increase in Fos protein levels in varying patterns in striatum and cerebral cortex. irectly-acting dopamine agonists only produce c-fos activation in denervated (supersensitive) striatum whereas cocaine and amphetamine activate c-fos in striatum in naive animals. Remarkably, D2 selective antagonists such as haloperidol, albeit in high doses, also activate c-fos expression. Activation of c-fos and other immediate early genes may play a part in the development of such long-term dopamine-related effects as dyskinetic movements and addiction.
Temporal Lobe Surgery for Epilepsy: Neuropsychological Variables Related to Surgical Outcome
Authors: Leonard Gabriel
Published: July 2016

A retrospective study of 383 patients who had undergone either left or right anterior temporal lobectomy (varying with respect to the amount of hippocampus excised) revealed that there were no significant differences in surgical outcome between those patients having a large and those patients having a small hippocampal removal. Although left temporal-lobe excisions were significantly smaller than right temporal-lobe excisions, both medially and laterally, patients with left temporal-lobe removals had an overall better surgical outcome. Patients in the successful surgicaloutcome group did better than the unsuccessful surgical-outcome group on a variety of cognitive measures, including tests of intelligence, delayed verbal memory, and verbal fluency. The results suggest that, for many patients with medically intractable epilepsy, an anterior temporal lobectomy including the amygdala may suffice to reduce their seizure frequency.
Follow-up study of electronystagmographic findings in friedreich's ataxia patients and evaluation of their relatives
Authors: Monday L. , Lespérance J. , Lemieux B. , Saint-Vincent H.
Published: July 2016

Fourteen Friedreich patients (F group) who had undergone a first electronystagmogram (E.N.G.) reported in 1978, had the same test 12 to 24 months after the first one. In the second study, there are more patients with bilateral hypoactive caloric nystagmus failure of fixation suppression, ocular dysmetria, irregular pendulum tracking and ocular flutter. These signs are probably most representative of the progression of the disease. Nineteen unaffected relatives of these patients (H group) also had an electronystagmogram but no special “familial” electronystagmographic pattern could be identified. Irregular ocular poursuit, nearly invariable in the F group but absent in the H group, was one of the most important differences between patients and their relatives.
Studies on the role of taurine in Friedreich's ataxia
Authors: Lemieux B. , Giguère R. , Shapcott D.
Published: July 2016

New studies were undertaken to verify the previous findings of increased urinary excretion of taurine, in the basal state and after challenge with a taurine load, in Friedreich's disease. Particular attention was paid to possible causes of error such as weight, muscle mass, creatine and creatinine excretion, variability with time and appropriate control groups. Although the overall findings were confirmed, their interpretation is open to question because of all these factors of error. Many possibilities must still be further explored to account for the apparent taurine retention defect observed in many cases of Friedreich's disease.
Free amino acids and calcium, magnesium and zinc levels in Friedreich's ataxia
Authors: Huxtable R.J. , Johnson P. , Lippincott S.E. , Barbeau A.
Published: July 2016

Free amino acid levels and zinc, magnesium and calcium content have been determined in autopsy samples of 9 areas of the brain, two skeletal muscles, and the right ventricle, left ventricle and septum of the heart of a Friedreich's ataxia subject.
Visual Dysfunction with Basal Skull Tumours
Authors: Sharpe James A.
Published: July 2016

Early detection of visual loss caused by tumours of the base of the skull is accomplished by systematic tests of the pregeniculate optic pathway. Even when central acuity is relatively preserved, a relative afferent pupillary defect and reduced color perception yield evidence of impaired optic nerve conduction. Although confrontation tests of the visual fields are useful screening techniques, unexplained symptoms should be pursued with static and kinetic perimetry. Patterns of optic nerve and chiasmal field loss and atrophy of the disc or retinal nerve fibers are imprecise guides to the location of basal skull tumours. Regardless of the pattern of visual field defect, unexplained progressive loss of vision demands intensive neuroradiologic study of the basal cisterns and skull.
Pathological Diversity in Clinical Syndromes of Pituitary Hypersecretion: Its Significance in Evaluating their Surgical Treatment
Authors: Smyth Harley S.
Published: July 2016

Thorough pathological examination disclosed considerable diversity of abnormal cell types and correspondingly different surgical correction rates among patients with apparently similar syndromes of pituitary hypersecretion.The surgical correction of acromegaly in patients with densely granulated growth hormone tumours was threefold that in patients whose tumours showed sparse granulation. Two non-prolactinoma tumour types associated with hyperprolactinemia have aggressive growth patterns; their special therapeutic management is discussed. Of 33 patients with Cushing's disease solitary adenomas were found in only 14, while six patients had proven corticotroph cell hyperplasia. Elective hypophysectomy should replace selective adenomectomy in selected cases of Cushing's disease.
Radiation Therapy of Tumours Involving the Skull Base
Authors: Payne David G.
Published: July 2016

The author reviews the Princess Margaret Hospital (Ontario Cancer Institute) experience with radiation therapy in certain tumours involving the skull base including meningiomas, chordomas, craniopharyngiomas and other less common tumours. Radiation therapy offers an effective supplementary or alternative treatment for such lesions.
Hypopituitarism Resulting from an Intrasellar Carotid Aneurysm
Authors: Ooi T. C. , Russell N.A.
Published: July 2016

We report a 74 year old lady who presented with an exceedingly rare combination of an internal carotid artery aneurysm which was almost entirely intrasellar and associated with hypopituitarism but no neurological deficits. Such a lesion could be misdiagnosed as a pituitary tumour with serious consequences, if surgery is attempted without prior carotid angiography.
Hemiageusia, Hemianaesthesia and Hemiatrophy of the Tongue
Authors: Strong Michael J. , Noseworthy John H.
Published: July 2016

A patient with a submandibular gland carcinoma was found clinically to have a unilateral chorda tympani, lingual and hypoglossal nerve deficit. This unique neurological entity of loss of taste sensation of one-half of the tongue (hemiageusia), hemianaesthesia and hemiatrophy of the tongue, has not previously been reported.
Nemaline Myopathy in an Adult with Primary Hypothyroidism
Authors: Reyes Marcelino G. , Tal Alexander , Abrahamson David , Schwartz Maurice
Published: July 2016

We report a 50 year old woman with primary hypothyroidism and nemaline myopathy to call attention to the rare coexistence of these two disorders.
The Posterior Cerebral Artery Syndrome
Authors: Fisher C.M.
Published: July 2016

Embolic and thrombotic infarction in the territory of the posterior cerebral artery (PCA) is described with emphasis on the stroke and cerebrovascular features rather than special neurological syndromes. Of 47 cases of obstruction at the distal bifurcation of the basilar artery, 43 (95%) were consistent with embolism. The clinical categories and pathological findings are presented. Local embolism, vertebral distal-stump embolism, the dynamics of hemorrhagic infarction and embolus-in-transit are briefly described. The prodromal manifestations of PCA thrombotic occlusion include photopsias, hemianopic blackouts, headache, transient episodes of numbness, episodic lightheadedness, spells of bewilderment and rarely tinnitus. Recognition of these may allow prevention of a stroke. Prodromal photopsias did not closely resemble the scintillating displays of migraineurs. When the stroke occurred, visual complaints ususally predominated. A sensory deficit occurred in one-third of cases. In 25 cases of memory impairment the dominant hemisphere was involved in 24. The kinds of visual hallucinations, simple and formed, are described.
The Treatment of the Restless Legs Syndrome with Clonazepam: A Prospective Controlled Study
Authors: Boghen Dan , Lamothe Lise , Elie Robert , Godbout Roger , Montplaisir Jacques
Published: July 2016

The effect of clonazepam on the restless legs syndrome was studied in a group of 6 patients. Following a drug-free period, 3 patients received clonazepam for 4 weeks followed by placebo for 4 weeks thereafter and 3 patients received the same medication and for the same length of time but in reverse order. The effectiveness of the medication was evaluated by means of a self-rating system in which patients assigned a score daily to the degree of discomfort experienced in the previous 24 hours. Three patients improved on clonazepam but 2 of these also improved on placebo. Clonazepam was not shown to be significantly more effective than placebo in the treatment of RLS.
Glial Fibrillary Acidic Protein (GFAP) in Oligodendrogliomas: A Reflection of Transient GFAP Expression by Immature Oligodendroglia
Authors: Jagadha V. , Halliday W.C. , Becker L.E.
Published: July 2016

Fourteen pure oligodendrogliomas were studied by light- and electronmicroscopy and immunohistochemistry to examine glial fibrillary acidic protein (GFAP) positivity in the tumors. To compare the immunohistochemical staining patterns of neoplastic oligodendroglia and immature oligodendroglia, myelination glia in the white matter of eight normal brains from children under 6 months of age were studied. The tumors possessed light microscopic and ultrastructural features characteristic of oligodendrogliomas. Microtubules were found in the cytoplasm of nine tumors on electronmicroscopy. In one, intermediate filaments and microtubules were observed in occasional tumor cells with polygonal crystalline structures in the cytoplasm. Using the peroxidase-antiperoxidase technique, all specimens were stained for GFAP, vimentin, S-100 and neuron-specific enolase (NSE). In nine tumors, variable numbers of cells with an oligodendroglial morphology reacted positively for GFAP. All tumors were positive for S-100 and negative for vimentin and NSE. The myelination glia in the eight normal brains stained positively for GFAP but not for vimentin. Vimentin is expressed by developing, reactive and neoplastic astrocytes. Thus, GFAP positivity combined with vimentin negativity in both neoplastic and immature oligodendroglia suggests that GFAP positivity in oligodendrogliomas may reflect the transient expression of this intermediate filament by immature oligodendroglia.
The EEG in Alzheimer type Dementia: Lack of Progression with Sequential Studies
Authors: Rae-Grant A.D. , Blume W.T. , Lau K. , Fisman M. , Hachinski V. , Merskey H.
Published: July 2016

Our findings dispel the commonly held belief that the EEG always worsens progressively in dementia of the Alzheimer's type. In a continuing cohort analytical study of dementia, 139 patients with Alzheimer's disease and 148 controls were studied for EEG abnormalities and progression. EEGs were read without knowledge of the previous EEGs or clinical condition, and classified according to the presence of diffuse delta or theta, bisynchronousspikes, projected activity, and focal activity. EEGs were significantly different in the two groups. EEG scores generally worsened over 1-4 years, but most of the subjects showed no alteration in their EEG scores. A few patients with Alzheimer's disease showed improvement of EEG findings.
Investigation of the Polymorphic Region in the 5' Flanking Region of the Insulin Gene in Patients with Alzheimer's Disease
Authors: Fisman Michael , Watt Valerie M.
Published: July 2016

A potential relationship between Alzheimer's Disease (AD) and insulin gene expression was suggested by the observation that patients with AD have altered levels of fasting blood sugar and insulin. Since polymorphisms in the region 5' to the insulin gene have been associated with blood glucose levels, we have studied this polymorphism in AD patients. Subjects were 19 nondiabetic AD patients with symptoms of aphasia and apraxia and a family history of AD; and 20 age and sex-matched nondiabetic controls without family history of AD. The 5' polymorphic region of the insulin gene was analyzed by restriction enzyme digestion of DNA extracted from whole venous blood. We did not observe a correlation between the size of the 5' polymorphic region and AD.
An Attempt to Produce Cholinergic Hypofunction in Rat Brain using Choline Mustard Aziridinium Ion: Neurochemical and Histological Parameters
Authors: Colhoun E.H. , Myles L.A. , Rylett R.J.
Published: July 2016

Deficiencies in cholinergic nerve function have been clearly documented in patients with Alzheimer's disease. The lack of this neurotransmitter may be responsible for early memory loss in patients with the disease. Choline mustard Az ion has been used in our laboratory to produce cholinergic hypofunction in rat brain. Injections of the compound into the medial septal nucleus and dorsal hippocampus produced tissue lesions. The lesions were dose and time-dependent. Lesions produced in the medial septum resulted in transmitter depression in the hippocampus. These results suggest non-specific tissue damage because 5-HT levels were lower than normal. Other strategies for getting choline mustard Az ion into rat brain are being investigated to circumvent the apparent ability of this compound and other nitrogen mustard analogs of choline to produce non-specific tissue damage when injected directly into brain tissue.
Muscarinic Binding and Choline Acetyltransferase in Postmortem Brains of Demented Patients
Authors: Waller Steven B. , Ball Melvyn J. , Reynolds Mark A. , London Edythe D.
Published: July 2016

Postmortem human brain samples were taken from non-neurological controls as well as demented subjects who died with Alzheimer's disease (AD), multi-infarct dementia (MID), or a combination of AD and MID dementia (MIXED). Choline acetyltransferase (Ch AT) activity was measured radiometrically using [ 1 -l4C]acetyl-coenzyme A as the substrate, muscarinic binding was assayed with [3H]quinuclidinyl benzilate, and the proportion of binding associated with high affinity agonist sites was measured by carbamylcholine displacement of the radioligand.

Relative to control, ChAT activity was significantly reduced (p ≤0.01) in samples taken from the temporal, frontal, and hippocampal areas of demented patients. A small elevation in Bmax was noted in the hippocampal endplate (p ≤0.01) (AD vs. control) and the H1-subiculum region (p ≤0.05) (AD vs. all other groups). In addition, the percentage of binding associated with high affinity agonist sites was greater in the frontal cortex of AD and MID samples (p≤0.05), compared with MIXED and control, and in the temporal cortex of the AD group compared with all other groups (p≤0.05). The results suggest a regionally specific upregulation of cerebral muscarinic receptors in dementia, especially in AD.
Cerebral Glucose and Dopa Metabolism in Movement Disorders
Authors: Martin W.R. Wayne , Hayden Michael R.
Published: July 2016

The development of positron emission tomography (PET) has enabled us to perform in vivo measurements of certain aspects of regional cerebral function. Regional cerebral glucose metabolism may be readily quantified with [18F] fluoro-2-deoxyglucose (FDG) and presynaptic dopaminergic function may be studied with the labelled dopa analog 6-[18F] fluoro-L-dopa. We have applied a model to the analysis of 6-FD/PET data with which in vivo age-related changes in dopaminergic function may be demonstrated in normal subjects. With this technique, we have studied a series of asymptomatic MPTP-exposed subjects and have shown evidence of subclinical nigrostriatal pathway damage. Studies of regional cerebral glucose metabolism with FDG in early Huntington's disease have shown a characteristic impairment in caudate function which precedes the development of caudate atrophy. In addition, some asymptomatic individuals who are at risk for HD have caudate hypometabolism. We feel that, at the present time, PET provides information which is complementary to the clinical examination in establishing a diagnosis of HD. In the future these studies may also help in the investigation of at risk individuals
Vestibular Control of Muscular Tone and Posture
Authors: Markham Charles H.
Published: July 2016

The vestibulospinal system helps to maintain upright posture and head stability. The semicircular canals and their short latency connections to the neck motoneurons, largely via the medial vestibulospinal tract, respond to angular accelerations so as to stabilize the head in space. The paired otolith organs, the utricles placed approximately horizontally, and the saccules vertically, respond to linear acceleration including gravity. Their influence leads, via the lateral vestibulospinal tract, to excitation of ipsilateral extensor motoneurons of the limbs and trunk, and to inhibition of reciprocal flexor motoneurons. Linear displacement of the otoliths leads to bracing of the limbs and body so as to maintain upright posture, and to extend the limbs so as to help in landing after sudden falls.
Treatment of Spasticity with Tizanidine in Multiple Sclerosis
Authors: Lapierre Y. , Bouchard S. , Tansey C. , Gendron D. , Barkas W.J. , Francis G.S.
Published: July 2016

Spasticity is a frequent and often disabling symptom in MS patients. Current drugs used as antispastic agents include Dantrolene Sodium, Baclofen and Diazepam. Tizanidine (5-chloro-4-(2imidazolin-2 yl amino) -2, 1, 3-benzothialdiazole) is a new antispasticity agent that has purported central action. A double blind placebo controlled trial was performed to study the efficacy of this drug in MS patients. Sixty-six patients entered an eight week therapeutic trial and fifty-nine completed the trial. Patients were assessed at 0, 2, 3 and 8 weeks of therapy for clinical effects. Electrophysiologic tests were performed at 0 and 8 weeks.

A statistically significant benefit was noted in spastic muscle groups in the legs with concomitant significant reduction in hyperactive stretch reflexes and ankle clonus. Side effects most frequently cited included dry mouth and drowsiness. Two patients developed elevated liver function test that decreased with cessation of therapy. Other clinical details, side effects and electrophysiologic data will be presented.

Tizanidine appears to reduce clinical spasticity and hyperreflexia in MS patients although no change in functional status was detected. Tizanidine may well serve as an alternate antispastic agent, alone or in combination with other agents.
Local Treatment of Spasmodic Torticollis with Botulinum Toxin
Authors: Tsui Joseph K.C. , Fross Robin D. , Calne Susan , Calne Donald B.
Published: July 2016

Fifty-six patients with spasmodic torticollis were treated with local injections of botulinum toxin. The drop out rate was 21%. The remaining 44 patients were followed for a period of 3 to 21 months. Thirty-two patients (76%) had pain relief out of 42 presenting with pain; 37 (66%) improved in the amount of sustained movements of torticollis. The efficacy was reproducible after repeated injections.
Method of Addition of Bromocriptine to the Drug Regime of Patients with Advanced Parkinson’s Disease
Authors: Grimes J. David , Hassan Mohamed N.
Published: July 2016

Using a method of a gradual increase of bromocriptine with a concomitant reduction of Sinemet® (levodopa 250 mg + carhidopa 25 mg), 19 patients with advanced Parkinson’s disease have been treated for periods of up to 22 months and 16 of them have shown improvements of varying degrees. Eighteen patients were able to tolerate bromocriptine addition, with early transient adverse effects occurring in seven cases. In contrast to several previously reported studies, it was found necessary to withdraw bromocriptine in only one case.

With the drugs currently available, bromocriptine has a role in the management of patients with advanced Parkinson’s disease. The method described here may allow a greater number of patients to be given a trial with this drug.
Multiple Sclerosis and Associated Diseases: A Relationship to Diabetes Mellitus
Authors: Warren S. , Warren K.G.
Published: July 2016

One hundred multiple sclerosis (MS) patients and hospital controls were compared for coexisting illnesses, and other illnesses among their family members. The patients and controls were compared for allergy, migraine, and cancer among themselves and for a family history of these illnesses. The findings were negative. However a relationship was found between multiple sclerosis and diabetes mellitus. More MS patients than controls were either diabetic or reported a blood relative with diabetes. MS patients who reported another blood relative with MS were also more likely to report a family history of diabetes mellitus than MS patients who had no other relative with multiple sclerosis.
Treatment of Fisher’s Variant of Guillain Barré Syndrome by Exchange Transfusion
Authors: Irvine Allan T. , Tibbles John
Published: July 2016

A case of Fisher’s variant of Guillain Barré Syndrome is presented. Treatment with exchange transfusion coincided with clinical improvement. This is the first case of Fisher’s variant treated in this manner.
Immunofluorescent Staining of Rat Brain Glial Cells With Multiple Sclerosis Serum
Authors: Singh Vuendra K. , Mashal Robert D.
Published: July 2016

Antibodies directed against glial cells may be involved in autoimmunity in multiple sclerosis (MS). Using a tissue culture system, the presence of glial cell antibodies in MS-patient serum was detected through immunofluorescent technique. Thirty one of 73 MS-sera were strongly positive for anti-glial cells, 13 were equivocal and 29 were negative. The antibody staining was either cytoplasmic or associated with cell surface membrane, and involved IgG type of antibody.
Porphyric Neuropathy: An Ultrastructural and Quantitative Case Study
Authors: Thorner P.S. , Bilbao J.M. , Sima A.A.F. , Briggs S.
Published: July 2016

We report a case of acute neuropathy in a 46 year old female with porphyria variegata. Histologic, electron microscopic, and quantitative examinations of peripheral nerves were performed at onset of the neuropathy and at autopsy. The results revealed severe qualitative and quantitative changes in myelinated and unmyelinated fibers showing features indicative of an axonopathy with a distribution in keeping with a dying-back phenomenon.
Meningitis and Brain Abscess Due to Clostridium Perfringens And Clostridium Paraputrificum Following Orbital Trauma
Authors: Girouard Yves , Delage Gilles , Mathieu Jean-Pierre , Larbrisseau Albert
Published: July 2016

An 8 year old boy developed cerebral abscess and purulent meningitis due to Clostridium perfringens and Clostridium paraputrificum after trauma to the left orbit. The patient made a satisfactory recovery with surgical treatment and antibiotic therapy. A review of the literature revealed that meningitis due to Clostridia is rare and usually related to penetrating trauma, and that penetrating trauma to the orbit is associated with significant central nervous system morbidity.
Familial Benign Intracranial Hypertension and Depression
Authors: Coffey C. Edward , Ross Donald R. , Massey E. Wayne , Olanow C. Warren
Published: July 2016

Two sisters developed benign intracranial hypertension (BIH) two weeks following the resolution of a major depressive episode. The association of BIH and a major affective disorder in genetically related individuals has not been previously reported to our knowledge. Both conditions are associated with disturbances in the hypothalamic-pituitary-adrenal axis. Falling corticosteroid levels in a resolving depression may result in impaired cerebrospinal fluid absorption and subsequent BIH.
Neonatal Myasthenia Gravis in the Infant of an Asymptomatic Thymectomized Mother
Authors: Olanow C. Warren , Lane Russell J.M. , Hull Keith L. , Roses Allen D.
Published: July 2016

A case of neonatal myasthenia gravis is reported in the infant of an asymptomatic thymectomized mother with comparably elevated acetylcholine receptor (AChR) antibody titers. The mother remained asymptomatic despite elevated antibody titers while the infant became asymptomatic in association with the disappearance of the AChR antibody. It is suggested that the AChR antibody plays an essential role in the development of neonatal myasthenia gravis. It is also suggested that a thymic factor is necessary for the development of clinical symptomatology accounting for the lack of correlation between the clinical state of the mother and infant.
Urodynamic Evaluation of Patients with Hereditary Ataxias
Authors: Vezina J.G. , Bouchard J.P. , Bouchard R.
Published: July 2016

Seventeen patients with Friedreich’s ataxia or spastic ataxia were subjected to an urodynamic evaluation. Fifty-three per cent (53%) of the patients presented with urinary symptoms consisting of urgent micturition and urgency incontinence. Cystometric evaluation showed a lack of inhibition of the detrusor in 7 patients (41%). Abnormal electric hyperactivity of the external sphincter was documented in 6 cases (37.5%) by electromyography. Some hypotheses are presented to explain the etiology of these abnormal findings.