bluuebunny/crossref_metadata_2025_split huggingface dataset:
Recurrent Cerebral Infarction during Anticoagulation Therapy in Patients with Mitral Valve Prolapse
Authors: Shuaib Ashfaq , Murphy William F. 
Published: July 2016

ABSTRACT:Mitral valve prolapse has been associated with an increased risk of transient or lasting ischemic events. Recurrence is uncommon after initiation of antiplatelet or anticoagulant therapy. In this communication we report two patients, both female, who had mitral valve prolapse as the major risk factor for cerebrovascular disease and who developed cerebral infarction despite anticoagulation. The cerebral infarctions were bilateral and extensive in one patient and led to the patient's death. In the second case, three infarctions resulted in moderate disability.
Two Types of Spheroid Bodies in the Nigral Neurons in Parkinson's Disease
Authors: Yamada Tatsuo , Akiyama Haruhiko , McGeer Patrick L. 
Published: July 2016

ABSTRACT:Dendritic spheroid bodies (SBs) and Lewy bodies (LBs) were identified in comparable numbers in the substantia nigra pars compacta (SBC) of nine parkinsonian cases and one case of striatonigral degeneration but were not found irt cases of Huntington's disease or neurologically normal controls. The immunohistochemical profile of the SBs in dystrophic dendrites of nigrostriatal dopaminergic neurons was remarkably similar to that of the LBs found within dendrites or free of the SNC neuropil. Both types of inclusions stained positively with antibodies to tyrosine hydroxylase, ubiquitin and microtubule-associated protein-2 (MAP2), and negatively for Tau-2, although they had different ultrastructural appearances. A few intracellular LBs were stained by antibodies to neurofilament proteins (NFs) 68, 160, and 200 kD, but dendritic SBs and extracellular LBs were not so stained. These data indicate that dendritic SBs and extracellular LBs may have a common molecular pathogenetic origin in Parkinson's disease. On the other hand, the SBs seen in the pars reticulata (SNR) and in the distal nigrostriatal axons even in control cases were generally stained by antibodies to NFs and ubiquitin but not to MAP2. This latter staining pattern in similar to that shown by SBs in the anterior horn in ALS and in the cerebellum of neurologically normal brains and is believed typical of axonal as opposed to dendritic SBs.
Myotonic Dystrophy: An Electrophysiological Study of Cognitive Deficits
Authors: Ragazzoni Aldo , Pinto Francesco , Taiuti Rosanna , Silveri Maria Caterina 
Published: July 2016

ABSTRACT:Patients with Myotonic Dystrophy (MyD) frequently suffer from a dysfunction of the primary sensory pathways, as documented by abnormalities of short-latency evoked potentials. Impairment of intellectual functions has been less extensively investigated. Short-latency brainstem auditory evoked potentials (BAEPs) as well as long-latency auditory event-related potentials (ERPs) were recorded from 5 female and 6 male patients affected by MyD. A simple discrimination (“oddball”) paradigm was used to record ERPs to tones from Fz, Cz, Pz. Both BAEPs and ERPs were significantly altered as compared to normals. BAEP abnormalities were detected in 9 patients and ERP components N2 and P3 were delayed or absent for all patients, who nonetheless correctly discriminated between tones. These data indicate that CNS dysfunction in MyD involves not only primary sensory systems but also neural mechanisms underlying cognitive events and ERP generation.
Accessory Nerve Palsy: A Review of 23 Cases
Authors: Berry H. , MacDonald E.A. , Mrazek A.C. 
Published: July 2016

ABSTRACT:In a series of 23 patients, the commonest cause of accessory nerve palsy was surgical trauma at the time of lymph node biopsy. The less common causes were penetrating or blunt trauma and a few were of spontaneous onset. There was involvement of adjacent motor sensory nerves in about half of the patients. The prognosis was better following blunt trauma, stretch injuries and after a spontaneous onset. The anatomical relationships of the accessory nerve and aspects of the clinical picture and management are discussed.
Complications of the Transsphenoidal Approach to Sellar Lesions
Authors: Reddy Kesava , Fewer Derek , West Michael 
Published: July 2016

ABSTRACT:Transsphenoidal surgery is currently performed extensively for lesions of the sella turcica. The mortality and morbidity of this surgical approach are minimal, and the results satisfactory overall. Only a few studies have addressed the complications of this approach. The authors present a retrospective study of 200 transsphenoidal procedures in 196 patients over an eleven year period. There was one death due to meningitis. Major morbidity consisted of intraventricular hemorrhage in three patients, false aneurysm of the internal carotid artery in one patient and thalamic infarction in another patient. Minor morbidity details are presented. The pathogenesis of the most serious of these complications along with possible preventive measures are discussed.
Current Status of Radiosurgery for Arteriovenous Malformations
Authors: Schwartz Michael , O'Brien Peter , Davey Phillip , Young Charlene , Willinsky Robert , Catton Charles 
Published: July 2016

ABSTRACT:Cerebral arteriovenous malformations (AVM), regardless of the mode of discovery, have an annual risk of hemorrhage of approximately 4 percent. A progressive obliterative vasculitis culminating in the occlusion of an AVM may be induced by the administration of radiation doses of approximately 20 Gy given in a single fraction. The process takes about two years and occlusion occurs in approximately 80% of patients so treated. Such a dose may be accurately administered to AVMs up to 3 cm in diameter with very little radiation imparted to the adjacent brain by means of multiple highly collimated radially arranged cobalt sources (the Gamma Knife) or by means of a modified linear accelerator turned through an arc or arcs with the target AVM as the centre of rotation. The Gamma Knife and the modified linear accelerator have nearly equal accuracy. Recent experience with modified linear accelerators indicates efficacy equal to the Gamma Knife. Both devices are effective treatment for small AVMs but the cost of modifying a pre-existing linear accelerator is only a few percent of the acquisition and installation costs of the Gamma Knife.
The Association between Polycystic Kidney Disease and Cerebral Aneurysms
Authors: Fehlings Michael G. , Gentili Fred 
Published: July 2016

ABSTRACT:Rupture of cerebral aneurysms is an important cause of morbidity and mortality in patients with adult polycystic kidney disease (PKD). In the present paper, we review the literature regarding the association of cerebral aneurysms and PKD and emphasize, by means of a case report, the importance of familial clustering of aneurysms in patients with PKD. We conclude that the prevalence of intracranial aneurysms in patients with PKD approaches 40% and is likely significantly higher in patients with PKD and a positive family history for cerebral aneurysm. We recommend that screening cerebral angiography be strongly considered in all patients with PKD and a family history of cerebral aneurysm or subarachnoid hemorrhage.
New Approaches to the Treatment of Age-Related Brain Disorders
Authors: Da Prada M. 
Published: July 2016

ABSTRACT:Two novel reversible enzyme inhibitors involved in monoamine metabolism are described. The novel and reversible inhibitors are the catechol-O-methyl-transferase (COMT) inhibitor, Ro 40-7592 (3,4-dihydroxy-4'-methyl-5-nitrobenzophenone), and the monoamine oxidase type-B (MAO-B) inhibitor, Ro 19-6327 (N-(2-aminoethyl)-5-chloro-2-pyridine carboxamide HC1). These may be of special therapeutic benefit in Parkinson's and Alzheimer's diseases.
Anomalous Gene Expression in Alzheimer Disease: Cause or Effect
Authors: McLachlan D.R.C. , Lukiw W.J. , Mizzen C. , Percy M.E. , Somerville M.J. , Sutherland M.K. , Wong L. 
Published: July 2016

ABSTRACT:Altered chromatin conformation and increased amounts of aluminum have been observed in the brains of patients with Alzheimer disease. These factors have been shown to affect gene regulation. In this report, we describe how these changes may selectively alter the pool size of the human light chain neurofilament gene and play a fundamental role in the expression of this disease.
Treatment of Alzheimer's Disease: Hopes and Reality
Authors: Gauthier S. , Gauthier L. , Bouchard R. , Quirion R. , Sultan S. 
Published: July 2016

ABSTRACT:The relative failure of symptomatic therapeutic trials for Alzheimer's disease using non-selective cholinergic agonists brings about the need for longitudinal studies with parallel designs aimed at disease stabilization, using trophic substances or amyloid suppressors.
Preoperative Electroencephalographic Investigation in Frontal Lobe Epilepsy: Electroencephalographic and Electrocorticographic Recordings
Authors: Quesney L.F. 
Published: July 2016

ABSTRACT:The first section of this article deals with specific anatomic and pathophysiologic factors which contribute to a poor EEG localization of the interictal epileptic abnormality and to the unreliable seizure onset localization commonly reported in patients with frontal lobe epilepsy. The localizing effectiveness of long term EEG monitoring was reviewed in four different groups of frontal lobe epileptic patients who underwent preoperative EEG investigation with extracranial and intracranial electrodes. The results of this study reveal a continuum distribution of interictal epileptic disturbances, ranging from focal abnormalities to lobar or multi-lobar epileptogenesis. A frontal lobe localization of the seizure generator based on ictal recordings obtained with extracranial electrodes is rather poor and much more reliable results can be obtained by depth-electroencephalography.
Introduction à l'Etude Clinique des Crises Epileptiques Rétrorolandiques
Authors: Baucaud J. , Talairach J. , Munari C. , Giallonardo T. , Brunet P. 
Published: July 2016

ABSTRACT:An introduction to the clinical study of postrolandic epileptic seizures. We reviewed, in 145 epilectics studied with SEEG, 800 clinical and electrographic seizures originating from the post-rolandic areas (590 spontaneous Sz and 260 induced by stimulation). The intra-cranial electrodes were implanted using a technique described by Talairach et al. (1974). Seizure onsets were recorded in the centro-parietal region (64 patients) in the partietal49 and in the occipital region.15 Additionally in 15 patients, mixed Sz onset were recorded. One hundred and eight (108) patients underwent surgical removal of their epileptic focus. (69 on the right, 39 on left.) 65% were cured (Sz free or occas Sz, f/up 3 years). The main ictal cal features are discussed. Emphasis is placed on the role of the operculo-insular cortex in the functional organization in man, based on Sz arising from the supra-temporal (fronto-pariental) cortex. The study of the pattern of onset and spread of seizures originating in the post-rolandic areas and of their clinical correlates allow a topographic differential diagnosis.
Localization of Epileptic Foci in Children
Authors: Blume Warren T. , Kaibara Masako 
Published: July 2016

ABSTRACT:Electroencephalographic and neuro-imaging data were correlated with the lobe of cortical resective surgery among 48 patients age 16 years or less with medically intractable partial seizures. Decisions about surgery were determined by clinical judgement and therefore our results reflect the weight of these factors in clinical practise. Neuroimaging disclosed a lesion over the operated lobe in 32 patients (67%). Multiple EEGs demonstrated a most active spike focus over the operated lobe in 32 patients (67%). Although only 34 patients had a recorded seizure, this clearly arose over the operated lobe in 23 (68% of the 34 patients; 48% of series). Persistent focal delta activity appeared in 20 of the 48 patients (42%). No instance was encountered in which false lateralization of regional or focal interictal EEG spikes or delta when normal apiculate phenomena were excluded. Thus, the scalp EEG remains a valuable guide to epileptogenesis, even among young patients, and should continue to play a major role in determining the region of cortical resection. Effectiveness of cortical resection for seizure control was clearly better among patients with a single major lesion and among those with normal intelligence.
Relative Localizing Value of Common Tests used in the Preoperative Investigation of Epileptic Patients
Authors: Saint-Hilaire Jean-Marc , Richer Francois , Turmel André , Rouleau Isabelle , Bouvier Guy 
Published: July 2016

ABSTRACT:We have compared the relative localizing value of common tests in the presurgical evaluation of epilepsy in 73 patients with depth electrode (SEEG) confirmed epileptogenic foci and excellent surgical outcome. We found the tests of abnormality (CT, amytal, neuropsychology) to be frequently non-informative and often discordant with SEEG. The EEG was concordant with the SEEG in 38% of cases and provided lateralization in 78%. EEG was less localizing than ictal behavior in frontals but not in temporals. The need for SEEG is rather evident in these results but could become attenuated by the development of the localizing power of scalp EEG.
Electrophysiological Analysis of Human Neocortex In Vitro: Experimental Techniques and Methodological Approaches
Authors: Avoli M. , Hwa G.G.C. , Kostopoulos G. , Olivier A. , Villemure J.-G. 
Published: July 2016

ABSTRACT:In this review we summarize a number of technical and methodological approaches that have been used in our laboratory to study human brain slices maintained in vitro. The findings obtained in the course of these studies appear to be relevant in establishing he mechanisms that underlie physiological phenomena of the human brain such as synaptic plasticity or responses to neuroactive drugs. Moreover, these data are important for understanding certain fundamental mechanisms of epilepsy. In this respect, however, we aution that the mechanisms that apply to different forms of clinical epilepsy might be ifficult to find given the variability present in the pathogenesis of human epilepsy.
Bilateral Pathological Damage in Temporal Lobe Epilepsy
Authors: Babb Thomas L. 
Published: July 2016

ABSTRACT:Patients with drug-refractory temporal lobe epilepsy can be treated successfully with surgical resection of one temporal lobe, especially when the resection includes the hippocampus. Although intrahippocampal recordings usually localize seizure onsets to one hippocampus, there are bilaterally-independent interictal spikes, occasional contralateral seizure onsets and post-resection seizures that implicate contralateral damage and epileptogenicity. Post-mortem onquantified studies of both hippocampi in epileptics have revealed incidences of bilateral hippocampal sclerosis, mostly being asymmetric. The present paper reports on two post-mortem cases of bilateral, asymmetric cell loss in patients with physiologically-verified hippocampal epilepsy. In one patient the damage was severe bilaterally, but only slightly greater damage in one hippocampus. In the second patient, damage in one hippocampus was as severe as in the first patient; however the contralateral hippocampus appeared undamaged. However, cell counts revealed losses of over 30% in three different hippocampal subregions, indicating a mild form of asymmetric bilateral damage in patient two.
Evidence that Charcot-Marie-Tooth disease with tremor coincides with the Roussy-Levy syndrome
Authors: Barbieri F. , Filla A. , Ragno M. , Crisci C. , Santoro L. , Corona M. , Campanella G. 
Published: July 2016

AbstractWe report data on 3 members of a family affected by a dominantly inherited disorder closely resembling Roussy-Levy syndrome (RLS). Electrophysiological findings showed a marked decrease of motor and sensory conduction velocities and EMG signs of mild neurogenic damage. Light and electron microscopy of sural nerve biopsy showed a hypertrophic neuropathy with diffuse onion-bulb formations and marked decrease of large size fibers. Teased fiber preparations evidenced reduced internodal lengths and segmental demyelination. Other data from the literature on RLS are reviewed and discussed. The hypothesis that RLS is not a disease entity but a hypertrophic-type of Charcot-Marie-Tooth disease with essential tremor (HMSN type 1) is strongly supported.
Evidence that Charcot-Marie-Tooth disease with tremor coincides with the Roussy-Levy syndrome
Authors: Chouza C. , Caamaño J.L. , De Medina O. , Bogacz J. , Oehninger C. , Vignale R. , De Anda G. , Novoa E. , De Bellis R. , Cardozo H. , Crispino B. , Romero S. , Correa H. , Feres S. 
Published: July 2016

ABSTRACT:Four members of a family with consanguineous relationships, the proband and his three children (2 sons and 1 daughter) are affected with Familial Spastic Ataxia and with Ehlers-Danlos' Syndrome with platelet aggregation dysfunction. In the four cases, this exceptional association appears remarkably homogeneous both in clinical and laboratory studies. The two syndromes are of dominant-autosomic transmission and probably originated in a new mutation which presumably maintained a genetic linkage. Spastic ataxia is characterized by a precocious onset and a slow evolution. The first-born son shows a dominant pyramidal syndrome with mild ataxia suggesting that it is a transitional form of familial spastic paraplegia. The Ehlers-Danlos syndrome pertains to form II or “mitis” with moderate skin hyperelasticity and joint hypermobility. The abnormal platelet aggregation curves have the same profile in all the patients. The first-born son also presents a mitral valve prolapsus as we may find either in Ehlers-Danlos syndrome or in spastic ataxia.
The neuropathology of “typical” Friedreich's ataxia in Quebec
Authors: Lamarche J.B. , Lemieux B. , Lieu H.B. 
Published: July 2016

AbstractWe present the pathological data from the autopsies performed on 6 Friedreich's disease patients since the start of the Quebec Cooperative Study. All patients met the strict diagnostic criteria of the QCSFA. The anatomical lesions found in the peripheral and central nervous system were similar in all 6 cases and do not differ from those described in the literature. The clinical findings correlate closely with the histological lesions found in the peripheral nervous system and spinal cord. The evidence of segmental demyelination and remyelination in the spinal ganglia and posterior roots further supports the dying-back axonopathy hypothesis.
Cardiac malic enzyme in Friedreich's disease
Authors: Barbeau A. , Charbonneau M. , Cloutier T. , Lemieux B. 
Published: July 2016

AbstractWe measured the activity of cytosolic and of mitochondrial malic enzyme in the hearts from 4 patients with Friedreich's disease and from two non-ataxic control subjects. There was a wide variability in the results and the slight overall decreases in both enzyme activities were not considered to be statistically significant. From these and other results, we conclude that deficient mitochondrial malic enzyme activity is not a constant or primary feature of Friedreich's disease.
Spinal Cord Infarction in Disease and Surgery of the Aorta
Authors: R.T Ross 
Published: July 2016

ABSTRACT:Diseases of the aorta and surgery of the aorta can produce spinal cord damage. There are major variations in blood supply to the spinal cord between individuals. The spinal cord may be tamponaded by increased spinal fluid pressure subsequent to clamping the aorta. Both of these factors may contribute to spinal cord infarction. The available methods and procedures to protect the spinal cord during surgery are discussed.
A Pilot Study on the Use of Botulinum Toxin in Spasmodic Torticollis
Authors: Tsui J.K. , Eisen A. , Mak E. , Carruthers J. , Scott A. , Calne D.B. 
Published: July 2016

ABSTRACT:Dystonic torticollis has been treated with local injections of botulinum toxin in a single blind study of 12 patients. A significant decrease of abnormal movements was recorded, and pain improved. Further studies are desirable to define the optimum dosage and site for injections, and the long term effects of repeated injections.
Interstitial Irradiation of Skull Base Tumours
Authors: Bernstein Mark , Gutin Philip H. 
Published: July 2016

ABSTRACT:The rationale for interstitial irradiation of tumours in and around the skull base is reviewed, and the experience accumulated with pituitary adenomas, meningiomas, and chordomas is summarized. Intracystic irradiation for craniopharyngiomas is also reviewed.
Photo-Dynamic Therapy: Cavitary Photo-Illumination of Malignant Cerebral Tumours Using a Laser Coupled Inflatable Balloon
Authors: Muller Paul J. , Wilson Brian C. 
Published: July 2016

ABSTRACT:Interest in photodynamic therapy of malignant brain tumours has been growing in recent years as intra-operative laser applications become more available.We have developed an inflatable balloon which can be coupled to an argon dye pump laser in order to deliver light to a brain tumour cavity created by the subtotal resection of tumour. Eight patients with primary malignant brain tumours have been treated with photodynamic therapy (PDT) using this device. The 8 patients tolerated the treatment well; morbidity attributable to the PDT was acceptably low.
1985 Silversides Lecture: Unusual Vascular Events in the Territory of the Posterior Cerebral Artery
Authors: Fisher C.M. 
Published: July 2016

ABSTRACT:There is an unusual type of vascular episode in the territory of the posterior cerebral artery which remains relatively unknown. Ten cases are presented in which a posterior cerebral artery deficit developed suddenly in dramatic fashion with headache, visual symptoms, sensory and motor deficits and signs of third nerve involvement. Nine of the patients were female and one was male. Seven were under the age of 33. In all instances there was a permanent neurologic sequela, usually a hemianopia. A similar case was described in 1901. The nature of the underlying process remains obscure, but the evidence favors accompanied migraine in which a particularly severe attack results in permanent damage. The term “catastrophic migraine” is suggested.
The Use of Shunting Devices for Cerebrospinal Fluid in Canada
Authors: Hoffman Harold J. , Smith May S.M. 
Published: July 2016

ABSTRACT:The use of diversionary CSF shunting devices for the treatment of hydrocephalus has been surveyed in Canada. Various factors influencing the operation of shunting devices, the characteristics of commercial shunts and the causes of shunt malfunction are described. Suggestions are made as to how to reduce the incidence of shunt malfunction.
Creutzfeldt-Jakob Disease: Correlation of Focal Electroencephalographic Abnormalities and Clinical Signs
Authors: Eggertson Douglas E. , Pillay Neelan 
Published: July 2016

ABSTRACT:Three patients are described with pathologically verified Creutzfeldt-Jakob disease (CJD) who presented with localizing clinical signs accompanied by focal electroencephalographic abnormalities including periodic lateralized epileptiform discharges (PLEDS). With further progression of the disease, diffuse background slowing and continuous bisynchronous periodic discharges appeared in all three cases. There was good correlation between the initial focal clinical manifestations and the EEG findings.
Malignant Intracranial Fibrous Histiocytomas. Histologic, Ultrastructural and Immunohistochemical Studies of Two Cases
Authors: Sima A.A.F. , Ross R.T. , Hoag G. , Rozdilsky B. , Diocee M. 
Published: July 2016

ABSTRACT:Two cases of malignant intracranial fibrous histiocytoma are presented. In Case 1 the tumour arose from the meninges and showed a disseminated spread throughout the neuroaxis. In the second case the tumour appeared to arise from within the brain substance. In this case surgical intervention and radiotherapy appeared to have achieved a cure, since no residual tumour was found at autopsy.The tumours were examined using ultrastructural and immunohistochemical techniques, which appeared advantageous in delineating this rare tumour from other intracranial neoplasms.