bluuebunny/crossref_metadata_2025_split huggingface dataset:
Congenital Malformations Associated with Maternal Use of Valproic Acid
Authors: Huot Celine , Gauthier Marie , Lebel Marc , Larbrisseau Albert 
Published: July 2016

ABSTRACT:Two children born with birth defects after intrauterine exposure to valproic acid are reported. The mothers took the drug throughout pregnancy as sole treatment for primary generalized epilepsy. The first baby showed facial dysmorphism, arachnodactyly and triphalangeal thumbs. The second had facial dysmorphism, severe laryngeal hypoplasia, tracheomalacia and an aberrant innominate artery that caused tracheal compression. A left superior vena cava, abnormal pulmonary lobulation, and unilateral hydronephrosis were also found at autopsy. Valproic acid has probable teratogenic potential in humans but the number of reported cases is few and the spectrum of anomalies is broad so it is not possible to delineate a definite fetal valproate syndrome.
Hand Wasting Due to Mid-Cervical Spinal Cord Compression
Authors: Goodridge A.E. , Feasby T.E. , Ebers G.C. , Brown W.F. , Rice G.P.A. 
Published: July 2016

ABSTRACT:Three patients presented with hand wasting and weakness secondary to mid-cervical spinal cord compression. This was due to cervical spondylosis in two patients and a meningioma in one case. This phenomenon is probably similar to that seen with foramen magnum lesions and may be due to spinal cord ischemia distal to the compression, secondary to venous stasis.
A Syndrome of Brain, Inner Ear and Retinal Microangiopathy
Authors: MacFadyen D.J. , Schneider R.J. , Chisholm I.A. 
Published: July 2016

ABSTRACT:We report the seventh case of a self-limiting syndrome of brain, inner ear and retinal microangiopathy which affects adult women producing mild to moderate mental status and personality changes, mild pyramidal and cerebellar dysfunction, patchy and asymmetrical visual loss secondary to non-inflammatory retinal infarctions, and asymmetrical neurosensory hearing loss. There is no clinical or laboratory evidence of involvement beyond the brain, the eyes and the ears. Magnetic resonance imaging or brain biopsy in previously reported cases best outline the brain pathology: multiple microinfarcts. All cases have been treated with steroids or cyclophosphamide. The etiology is unknown but may be related to systemic lupus erythematosus.
Comparison of Surgical and Conservative Management in 208 Patients with Acute Spinal Cord Injury
Authors: Tator C.H. , Duncan E.G. , Edmonds V.E. , Lapczak L.I. , Andrews D.F. 
Published: July 2016

ABSTRACT:The role of surgery in the management of acute spinal cord or cauda equina injuries remains controversial. The present study analyzed ten admission features and three outcome variables in 208 patients treated in an Acute Spinal Cord Injury Unit, 116 (56%) of whom underwent at least one spinal operation. The surgical and non-surgical groups showed no significant differences in the following seven clinical features: age, sex, distance travelled to the Unit, time interval between trauma and admission, type of accident, severity of injuries to the spinal cord, and severity of associated injuries. However, the two groups showed significant differences in level and type of vertebral column injury, and in the frequency of pre-existing spinal abnormalities. These differences were due to management policies which selected certain injuries for surgical or non-surgical treatment. One-third of the operative procedures were performed primarily for neural decompression, one-third primarily for reduction of bony structures and one-third for fusion. However, 95% of the operative patients had a fusion at the initial operation. Operative treatment was associated with a lower overall mortality rate (6.1%) than non-operative (15.2%), despite a higher frequency of thrombo-embolic complications in the surgical group. Overall, there was no difference between operated and non-operated patients in length of stay or neurological recovery. Surgical management of patients with acute spinal cord injury appears safe in terms of mortality rate and neurological recovery, but it has not been proven to improve the latter.
Microsurgical Discectomy: A Prospective Study of 200 Patients
Authors: Schutz H. , Watson C.P.N. 
Published: July 2016

ABSTRACT:This report describes the results of microsurgical discectomy in a prospective study of 200 consecutive patients with intractable sciatica, followed independently by a pain clinic. Post operative morbidity was minimal, the complication rate very low, and patients were discharged home early. Leg pain was relieved in almost all patients, but back pain was relieved in only 72% of patients. The rate of return to work at one, three and twelve months post-operatively was 50%, 83% and 95% respectively. Microsurgical discectomy is an effective and safe procedure.
Pathology of Spontaneous Dissection of Intracranial Arteries
Authors: Deck John H.N. 
Published: July 2016

ABSTRACT:Spontaneous dissection of a major subarachnoid artery is an uncommon cause of stroke in young and apparently healthy individuals. Such dissection does not correlate well with systemic conditions such as emboli, thrombotic or hemorrhagic tendencies or hypertension, nor with disease of blood vessels such as atherosclerosis, fibromuscular dysplasia, cystic medial necrosis or Moya-Moya disease. Unusual exertion has been implicated as a factor in some cases. Gap defects were found in the internal elastic lamina near the site of dissection in three of four cases of spontaneous dissection reported here. It is suggested that such defects, because of their unusual size or number, may be responsible for initiating dissection in susceptible individuals. A detailed study of the major cerebral arteries in cases of dissection and control cases to document the size and frequency of such defects could shed light on the pathogenesis of spontaneous dissection. The subsequent course of events tends to differ in the internal carotid and vertebro-basilar systems. In most cases, the dissection in the internal carotid system is sub-intimal leading to thrombosis and cerebral infarction, while in the vertebrobasilar system dissection lies between the media and adventitia leading to subarachnoid hemorrhage. It is not known what structural differences of the two arterial systems may underlie this difference in the pattern of arterial dissection.
Treatment of Dopaminomimetic Psychosis in Parkinson's Disease with Electroconvulsive Therapy
Authors: Hurwitz T.A. , Calne D.B. , Waterman K. 
Published: July 2016

ABSTRACT:Two Parkinsonian patients with chronic nonconfusional dopaminomimetic psychosis were treated with a course of electroconvulsive therapy. In both cases sustained remission of psychosis was obtained in the face of ongoing administration of dopaminomimetics.
Current Status of Artificial Vision by Electrocortical Stimulation
Authors: Girvin John P. 
Published: July 2016

ABSTRACT:The history of the provision of artificial vision by electrocortical stimulation has its origin in observations over 50 years ago. However, the realistic modern stimulation of human visual cortex is only 20-years-old. No useful device has been devised as yet and it therefore remains purely experimental. The primary objective of such a prosthetic device is that of independent mobility. This would require the appreciation of half-tone pictures, the detection of depth and discontinuities in outlines, and a significant quality of resolution. Some of the problems which threaten resolution in such a device and the factors which must be overcome in order to achieve a useful visual prosthesis are discussed.
Jakob-Creutzfeldt Disease Associated with Wernicke Encephalopathy
Authors: Gaytan-Garcia S. , Gilbert J.J. , Deck J.H.N. , Kaufmann J.C.E. 
Published: July 2016

ABSTRACT:Wernicke disease (WD) is a complication of alcoholism and malnutrition and usually presents acutely and is characterized by disturbances of consciousness, paralysis of the external ocular muscles, and ataxia. The disease results from deficiency of vitamin B 1, or thiamine, an essential coenzyme in intermediate carbohydrate metabolism. On the other hand, Jakob-Creutzfeldt disease (J-C) results from infection with an unconventional agent with a long incubation period and is characterized by a rapidly progressive dementia and histologically by a spongiform encephalopathy associated with neuronal destruction and pronounced astrogliosis. Combination of both diseases has not been reported in the literature previously and their relationship is uncertain. We present 3 cases with this interesting association and consider their relationship.
Cognitive Enhancing Agents: Current Status in the Treatment of Alzheimer's Disease
Authors: Waters Cheryl 
Published: July 2016

ABSTRACT:Extensive recent literature on drugs used to enhance cognitive functioning, reflects the growing social problem of dementia. Many clinical trials have been undertaken with variable success. In most cases the disorder studied has been Alzheimer's disease. The pharmacological approach has been designed to rectify the presumed pathophysiological processes characteristic of the condition. Agents tested include cerebral vasodilators, cerebral metabolic enhancers, nootropics, psychostimulants, neuropeptides and neurotransmitters with a special emphasis on drugs used to enhance cholinergic function. Ethical and practical issues concerning clinical drug trials in dementia will be discussed.
Cerebellar Atrophy in Epileptic Patients
Authors: Botez M.I. , Attig Ezzedine , Vézina Jean Lorrain 
Published: July 2016

ABSTRACT:High-resolution CT scans of the brain and posterior fossa were performed on 106 phenytoin (PHT)- treated epileptics, 28 de novo epileptics and 43 control subjects. A higher incidence of cerebellar and brainstem (CBS) atrophy was observed in chronic PHT- or PHT+ phenobarbital-treated epileptics compared to the two other groups. Some control subjects and de novo epileptics presented mild CBS atrophy, whereas moderate to severe atrophy was noted exclusively in chronically-treated patients. In attempting to delineate the etiology of CBS atrophy, epileptic patients were divided in three groups: 55 subjects with normal CT scans, 30 with both cerebral and CBS atrophy, and 49 with pure CBS atrophy. Their ages, length of illness, number of generalized seizures, number of other seizures, and amount of PHT received during their lifetime were assessed. Statistical analysis revealed that posterior fossa atrophy in epileptics was significantly correlated with both the length of the illness and the amount of PHT ingested during the patient's lifetime. The number of seizures appears to not be related to CBS atrophy.
Origin and Diffusion of the Myotonic Dystrophy Gene in the Saguenay Region (Quebec)
Authors: Bouchard Gérard , Roy Raymond , Declos Manon , Mathieu Jean , Kouladjian Kevork 
Published: July 2016

ABSTRACT:A very high prevalence (approximately 1/475 in 1985) of myotonic dystrophy (Steinert disease) is observed in the Saguenay region, which is located in the north-east part of the Province of Quebec. For various reasons, however, the literature on the subject generally associates a high degree of selective disadvantage with this gene, which seems to contradict the Saguenay data. Using a computerized regional population register, we have reconstituted patients' genealogies and family biographies. We have thus been able to study the origin of the gene and to compare the demographic behavior of patients and controls. On the whole, patients seem to be very little disadvantaged compared to controls, in terms of reproduction as well as of geographical and occupational mobility.
Computed Tomography, Magnetic Resonance Imaging and Pathological Correlations in a Case of Binswanger's Disease
Authors: Mascalchi M. , Inzitari D. , Dal Pozzo G. , Taverni N. , Abbamondi A.L. 
Published: July 2016

ABSTRACT:The results of 3 computed tomography (CT) examinations carried out over a 7 year period and of a post-mortem magnetic resonance (MR) study showed aspects of a white matter disease in a hypertensive patient suffering from vascular dementia. Histopathology revealed the primary cause of dementia to be a white matter degeneration sparing the U fibers. Rarefaction of both the myelin sheaths and the axons was present together with severe thickening of the medullary arteries. These findings support the existence of Binswanger's disease (BD) as a distinct variety of arteriosclerotic dementia. CT and MR imaging are valuable aids for diagnosis. However, since there are many other causes of CT and MR demonstrated diffuse white matter degeneration in the elderly, a conclusive diagnosis of BD requires pathological confirmation.
Trends in Blood Pressure, Osmolality and Electrolytes after Subarachnoid Hemorrhage from Aneurysms
Authors: Disney Lew , Weir Bryce , Grace Michael , Roberts Paul 
Published: July 2016

ABSTRACT:Daily trends in blood pressure, osmolality and electrolytes were analyzed in a series of 173 operated aneurysm cases who had subarachnoid hemorrhage (SAH) and were admitted within 4 days of the ictus. High blood pressure was associated with a greater risk of mortality and the development of clinically significant vasospasm (VSP). High osmolality shortly after admission was related to mortality but not VSP. Changes in sodium and potassium had no obvious relationship to mortality or VSP.
Multiple Intra-Cranial Hemorrhages After Carotid Endarterectomy
Authors: Shuaib Ashfaq , Hunter K. Michael , Anderson Melvin A. 
Published: July 2016

ABSTRACT:Intra-cerebral hemorrhage is an unusual and often fatal complication of endarterectomy (CEA). Previous cerebral infarction, intra-operative or post operative hypertension, and the use of anti-coagulants are some of the defined major risk factors. Hemorrhage usually occurs early after CEA, and a second hemorrhage has only once been reported. We wish to report a patient who developed three separate hemorrhages following CEA. This patient had a long history of poorly controlled hypertension. We believe that poorly controlled hypertension, in addition to other reported factors, may be an important separate risk factor for post-CEA hemorrhage.
Routine Screening of Blood and Urine for Severe Anticonvulsant Reactions in Asymptomatic Patients is of Doubtful Value
Authors: Camfield Peter , Camfield Carol , Dooley Joseph , Farrell Kevin , Langevin Pierre , Humphreys Peter 
Published: July 2016

ABSTRACT:On rare occasions anticonvulsant medication may cause severe or fatal toxic reactions. This position paper, approved by the Canadian Association for Child Neurology, examines the value of routine blood and urine screening to prevent catastrophic reactions in asymptomatic patients. The basic assumptions of such a screening program are faulty or unproven. Although not definitive, available studies of screening do not appear to support its value. It is recommended that routine blood and urine screening be discontinued in asymptomatic patients. Until such time as further research is able to identify patients at special risk, it is suggested that patients be carefully informed of the early symptoms of severe toxic reactions and report these immediately to a physician.
Clustering of Parkinson's Disease in Southern Israel
Authors: Herishanu Y.O. , Goldsmith J.R. , Abarbanel J.M. , Weinbaum Z. 
Published: July 2016

ABSTRACT:On three adjacent kibbutzim (collective rural communities) in the Negev (Southern Israel) 13 parkinsonian patients were found among a population of 592 persons 40 years or older. The clinical picture was not different from that of patients from other areas with idiopathic parkinsonism. Long term residence in the kibbutzim is characteristic of this population. In the past most of the drinking water has been supplied by wells from a common aquifer. From other patients with Parkinson's disease in the Negev, we estimated the age-specific incidence for the region. The incidence is about five times greater in each of these kibbutzim than in the remainder of the Negev. Although associations with rural residence and well water use have been reported elsewhere, clusters of this sort have not been reported. They strongly suggest that a common environmental factor exists.
Growth Hormone Rebound After Cessation of SMS 201-995 Treatment in Acromegaly
Authors: Charest L. , Comtois R. , Beauregard H. , Serri O. 
Published: July 2016

ABSTRACT:We studied a 42-year-old woman who had persistent active acromegaly despite conventional therapies. She was treated for 6 months with SMS 201-995. Her mean plasma growth hormone GH values decreased during treatment from 9.1 ± 1.2 to 6.6 ± 1.2 µg/L. One month after the withdrawal of SMS 201-995, the plasma GH level increased to 24.4 µg/L (P < 0.001). This elevation was clinically silent and transitory, as GH levels decreased 8 months later to 6.9 ± 1.3 µg/L. Furthermore, at the beginning of therapy, her intractable headache was completely relieved; however, it progressively resumed under therapy. In conclusion, cessation of SMS 201-995 may be followed in some acromegalic patients by a rebound of plasma GH levels. This rebound suggests that SMS 201-995 decreases GH levels by an inhibition of its release from the pituitary. Furthermore, SMS 201-995 may relieve intractable headache in some acromegalic patients, but tolerance to the analgesic effect may develop.
Cortical Glutaminase, β-Glucuronidase and Glucose Utilization in Alzheimer's Disease
Authors: McGeer E.G. , McGeer P.L. , Akiyama H. , Harrop R. 
Published: July 2016

ABSTRACT:Large pyramidal neurons of rat and human neocortex stain immunohistochemically for phosphate-activated glutaminase (PAG). In a limited number of postmortem brains, we find large reductions in cortical PAG activity in Alzheimer's disease (AD). This finding is consistent with histological evidence that pyramidal neurons are affected in AD. The reductions are greater than those found in the same samples in choline acetyltransferase (ChAT) but the possible deleterious effects of coma and similar premortem factors on human PAG activity have yet to be assessed. The activity of (β-glucuronidase, a lysosomal enzyme which occurs in reactive astrocytes, is elevated in the same samples. Positron emission tomography (PET) studies, using18F-fluorodeoxyglucose (FDG), have demonstrated significant deficiencies in glucose metabolism in the cortex in AD, with the parietal, temporal and some frontal areas being particularly affected. We found in serial scans of 13 AD cases, including one relatively young (44-46 year old) familial case, an exacerbation of the defect over time in most cases. We have found a negative correlation between the regional metabolic rates for glucose (LCMR(s)) measured premortem and the (β-glucuronidase activities measured postmortem on a few AD cases that have come to autopsy. The correlations between LCMR(s) and PAG and ChAT activities tend to be positive. The results are consistent with previous suggestions that decreased LCMR(s) in AD reflect local neuronal loss and gliosis.
Ultrastructural Studies of the Cells Forming Amyloid Fibers in Classical Plaques
Authors: Wisniewski H.M. , Wegiel J. , Wang K.C. , Kujawa M. , Lach B. 
Published: July 2016

ABSTRACT:Three-dimensional reconstruction and ultrastructural studies of classical plaques from the cortex of patients with Alzheimer's disease showed that microglial cells of the plaques are the amyloid-forming cells. The amyloid star of the single plaque represents the product of five or six microglial cells covering about 80% of the amyloid star surface. The amyloid fibers appear to be formed within altered cisterns of the endoplasmic reticulum. Distended cisterns form channels filled with amyloid fibers. Numerous vesicles derived from the Golgi apparatus appear to be attached to or fused with the amyloid-filled channels. Reconstruction of the amyloid star and the microglia cell pole that forms the amyloid star reveals three different zones of distribution of cytoplasmic organelles and amyloid deposits. The peripheral zone comprises channels filled with loosely packed amyloid fibers arranged in a parallel manner. The transient zone consists of a mixture of fusing amyloid channels and products of disintegration of cytoplasmic pockets, dense bodies and fragments of cellular membranes. The core of the amyloid star is composed of condensed, densely packed amyloid fibers that are free of cellular debris. Formation of the three zones supports the idea that the microglia/macrophages are not phagocytes but instead are the cells manufacturing the amyloid fibers.
Surgical Management of Giant Pituitary Adenomas
Authors: Mohr Gérard , Hardy Jules , Comtois Ronald , Beauregard Hughes 
Published: July 2016

ABSTRACT:During the past 25 years, 77 giant pituitary adenomas have been treated surgically, including suprasellar extensions of type C in 66 cases and of type D in 11 cases. Non-secreting adenomas were present in 53 and secreting adenomas in 24 cases. All patients except 3 presented with significant visual field defects; including bitemporal hemianopia, superior quadranopia or unilateral temporal hemianopia, contralateral blindness in 73% of the cases, and one case with sudden bilateral blindness due to acute pituitary apoplexy. A single transsphenoidal procedure was carried out in 74% of the patients while 11 patients (7%) required re-operations for recurrent or residual tumor. Only 3 patients required a subsequent transcranial procedure. Complications included 1 CSF-leak, 1 empty-sella syndrome and 4 fatal post-operative hematomas. We prefer the transsphenoidal route even in very large or giant pituitary adenomas, since it allows rapid and adequate decompression of the optic nerves and chiasm, avoids major pituitary insufficiency in 60% of the cases and is associated with low morbidity-mortality rates.
Differential Response to Aminergic Stimuli and Biological Behavior of Growth Hormone Secreting Pituitary Adenomas
Authors: Fanghanel Guillermo , Larraza Oscar , Villalobos Martha , Fanghanel Leticia , Velasco Marcos , Velasco Francisco 
Published: July 2016

ABSTRACT:Growth hormone (GH) serum levels in response to the administration of aminergic drugs and thyroliberine (TRH) were determined in a group of 34 acromegalics. Administration of bromocriptine (10 mg single oral dose) was followed by a decrease in GH below 60% control values in 35% of the cases. Administration of diazepam (10 mg single oral dose) to those cases not responding to bromocriptine induced a decrease in GH in 58% of the cases and an increase in GH in 42%. Administration of cyproheptadine (24 mg/day for one month) to those cases not responding to bromocriptine or with increased GH after the administration of diazepam, decreased GH in 75%, while increased GH in 25% of the cases. TRH 200 (Xg single I.V. dose induced increase of 128% GH basal level in 65% of cases (TRH positive) which correlated with more benign clinical course, decreased GH levels in response to bromocriptine, increased PRL levels, PRL-GH mixed secreting adenomas in immunohistochemistry studies, presence of granulated cells in electron microscopy studies and normalization of GH in the majority of surgically treated cases. By contrast, TRH negative cells correlated with aggressive tumor growth, lack of response to bromocriptine, normal PRL levels, pure GH secreting adenomas by immunohistochemistry, poorly granulated cells and lack of response to surgical treatment. Results suggest that there is more than one type of acromegaly that might be distinguished by the aminergic control on GH secretion.