bluuebunny/crossref_metadata_2025_split huggingface dataset:
Neurofibrillary Tangles in the Dementia of “Normal Pressure” Hydrocephalus
Authors: Ball Melvyn J. 
Published: July 2016

SUMMARY:Routine neuropathological examination could not explain the dramatic improvement exhibited by one patient with “normal pressure” hydrocephalus after shunting. The improved patient contrasted remarkably with the unchanged condition of four others also shunted successfully. The five brains were analysed by quantitative morphometry to determine the degree of neurofibrillary tangle formation in mesial temporal neurons. The density of tangle-bearing nerve cells in the four unimproved cases was markedly greater than in age-matched control brains from nineteen normal subjects, and fell in the same range as that of eight dements with neuropathologically confirmed Alzheimer's disease. The density of the one who recovered was within normal limits.The duration of dementia before shunting, and the total duration of dementia in these five patients rank in the same order as their degree of neurofibrillary formation. Furthermore, a positive linear correlation exists between the Tangle Indices and the total duration of dementia. The data suggest that early diagnosis may improve the chances of reversing the dementia of normal pressure hydrocephalus before histological alterations prove too severe.
Some Neurophysiological Effects of Cerebellar Stimulation in Man
Authors: Upton Adrian R.M. , Cooper Irving S. 
Published: July 2016

SUMMARY:This paper presents the results of neurophysiological studies of the effects of cerebellar stimulation on H reflexes, late reflexes, blink reflexes, evoked potentials and EEG patterns in 18 human subjects (Male 13, Female 5, Age 25.8 ±10.0, Epileptic 9, Cerebral Palsy 9).In addition to the effects of cerebellar stimulation on the H reflex studies on salens we assessed V1 and V2, “late” responses (Upton et al., 1971), cortical somatosensory evoked potentials (SSEP) after median nerve stimulation, and visual evoked responses (VER) after flash stimulation. Experiments were extended to assess recovery curves of all the potentials and we examined the effects of changes on the rate or voltage of cerebellar stimulation.Cerebellar stimulation was inhibitory to all the responses except the visual evoked potentials. Serial studies in five patients produced consistent results. Preoperative and postoperative results were compared in two patients with no significant difference in the results in the absence of cerebellar stimulation.Ipsilateral cerebellar stimulation (CS) had the greatest inhibitory effects on H, V1 and V2 responses in the arm and leg whereas contralateral CS produced the greatest effects on cortical SSEPs. There was a greater bilateral effect on SSEPs and reflex responses after right CS than left CS and this may be the first indication of “dominance” in the cerebellar hemispheres. Cerebellar stimulation in patients on diphenylhydantoin produced minimal effects on SSEP“s and this observation has led to further studies in patients taking diphenylhydantoin.Recovery of amplitude of the reflex and cortical responses took eight to 30 minutes after one minute of cerebellar stimulation. Serial CS of one minute on and one minute off produced increasing inhibition of SSEP's and reflexes for up to five stimulations. Recovery after cessation of cerebellar stimullion was associated with rebound excitation in six patients, the rebound being noted in the amplitude of H reflexes and SSEP's as well as in the frequency of paroxysmal spike and wave discharges in the EEG.The correlation of the results of such quantitative neurophysiological tests with clinical improvement may allow prediction of clinical results after cerebellar stimulation. These techniques have already been used to measure the threshold of stimulation and may allow optimal stimulation characteristics to be assessed. The prolonged neurophysiological effects of stimulation may allow the use of maximum effective intervals between optimal epochs of stimulation so that any cerebellar damage can be minimized.
Friedreich's Ataxia: Preliminary Results of some Genealogical Research
Authors: Barbeau A. , Le Siege M. , Breton G. , Coallier R. , Bouchard J.P. 
Published: July 2016

SUMMARY:A preliminary genealogical investigation of all the known ancestors from the year 1608 of 4 apparently unrelated French Canadian kindreds with Friedreich’s ataxia reveals that the original ataxia gene in the province of Quebec was present within a core of no more than 10 families living in Quebec City in the mid-1600's.
Nerve Conduction Studies and Electromyography in Friedreich's Ataxia
Authors: Peyronnard J.M. , Lapointe L. , Bouchard J.P. , Lamontagne A. , Lemieux B. , Barbeau A. 
Published: July 2016

SUMMARY:Twenty-six of 50 patients were investigated with nerve conduction studies and electromyography using a standard protocol and were compared to the findings in 50 normal control subjects. Almost all cases of typical Friedreich's ataxia had absent sensory action potentials (SAP) in the digital (92%) or sural (96%) nerves. The others had markedly decreased S.A.P's. In these same patients motor conduction auvelocities were either normal or only slightly decreased. In the second, atypical group of 9 patients, the motor conduction velocities were considerably decreased.Because of the absence of sensory action potentials in Friedreich's ataxia, and that the absence was noted in our very mild cases, it is proposed that this measure be used to facilitate early diagnosis.
Glucose and Insulin Metabolism in Friedreich's Ataxia
Authors: Shapcott D. , Melancon S. , Butterworth R.F. , Khoury K. , Collu R. , Breton G. , Geoffroy G. , Lemieux B. , Barbeau A. 
Published: July 2016

SUMMARY:Our prospective survey of 50 ataxic patients confirms the previous finding of frequent clinical or chemical diabetes in Friedreich's ataxia. Eighteen percent of our typical cases have clinical diabetes and 40% at least an abnormal glucose tolerance curve. However, this finding does not appear to be specific to that form of ataxia. Furthermore, we have shown that most patients with ataxia have normal or low fasting insulin levels, but a hyperinsulinic response to a glucose load.
Friedreich's Ataxia 1976 — An Overview
Authors: Barbeau A. 
Published: July 2016

SUMMARY:The prospective investigation of 50 cases of possible Friedreich's ataxia has permitted the clinical and biochemical delineation of the typical disease and an hypothesis on its pathogenesis. A tentative definition of the disorder could read: “Friedreich's ataxia is a progressive degenerative disease always inherited in an autosomal recessive fashion and characterized by a cardiomyopathy and a ganglioneuropathy with dying back phenomenon. It is probably secondary to a defect in the membrane transport of taurine and β -alanine and/or a defect in the regulation of pyruvate oxidation.” The existence of two pathogenetically distinct distinct entities with the same phenotype is a strong possibility.
Lissencephaly
Authors: Norman Margaret G. , Roberts Maureen , Sirois J. , Tremblay L.J.M. 
Published: July 2016

SUMMARY:The first reported case of lissencephaly resulting from a consanguinous union strengthens the supposition that in some cases, it is transmitted as an autosomal recessive trait. Comparison of this case with a sporadically occuring case of lissencephaly, with different cortical morphology, suggests that lissencephaly may be an example of either varying gene expressivity or gene-tic heterogeneity. Lissencephaly and pachygyria may eventually be shown to be due to different causes, some inherited, some acquired. The classical examples of lissencephaly are different morphologically from a case in which antenatal cytomegalovirus infection had produced a small smooth brain. This suggests that antenatal viral infections are destructive rather than teratogenic.
Disorders of Neuronal Migration
Authors: Barth Peter G. 
Published: July 2016

Abstract:Neuronal migration constitutes one of the major processes by which the central nervous system takes shape. Detailed knowledge about this important process now exists for different brain regions in rodent and monkey models as well as in the human. In the human, distinct genetic, chromosomal and environmental causes are known that affect neuronal migration, often in a morphologically distinct pattern, but the underlying pathological mechanisms are largely unknown. This review is intended to integrate our basic knowledge of the field with the accumulated intelligence on a large number of disorders and syndromes that represent the human part of the story.
Distribution of Extracellular Matrix Proteins in Primary Human Brain Tumours: An Immunohistochemical Analysis
Authors: Rutka James T. , Myatt Craig A. , Giblin Jane R. , Davis Richard L. , Rosenblum Mark L. 
Published: July 2016

Abstract:Using immunohistochemical techniques, we localized several glycoproteins of the extracellular matrix in paraffin-embedded sections of 4 normal brain and 38 primary intracranial tumour specimens. All specimens were positively immunostained to various degrees by monoclonal antibodies to type IV collagen and procollagen III and by antisera to laminin and fibronectin. Staining was consistently most intense at sites of contact between neuroepithelial and mesenchymal or leptomeningeal elements; there was no demonstrable staining within or between neuroepithelial elements in the neuropil. Tumour cells from meningiomas and from the sarcomatous portion of a gliosarcoma were positively immunostained for fibronectin and laminin. The integrity of the glial limitans externa was demonstrated by the positive linear reaction product produced by immunostains for type IV collagen and laminin, even in the most malignant gliomas. The deposition of extracellular matrix glycoproteins at the glial-mesenchymal interface observed in this study of primary human brain tumours is a manifestation of one of the interactions between tumour and stromal cells in the central nervous system. A loss of coordination and an alteration in the interactions between epithelial cells and stromal cells across extracellular matrices such as basement membranes are thought to be fundamental steps in the development and progression of cancer. Further characterization studies focusing on other markers of the extracellular matrix are needed to elucidate completely the function of this structure in the central nervous system.
Massive Cerebral Edema Associated with Meningioma
Authors: Jagadha Venkita , Deck John H.N. 
Published: July 2016

Abstract:Cerebral edema is a well-known complication of malignant astrocytoma and metastatic brain tumour. Though far less frequent, the association of cerebral edema with meningioma is also recognized; this is unpredictable and sometimes out of proportion to tumour size. We describe three cases where the mass effect was due to severe edema, rather than the tumour itself and was responsible for a catastrophic clinical presentation. The mechanisms responsible for the development of severe edema with meningioma which have been previously proposed are reviewed. They include tumour size, location, histologic subtype, cellularity, mitotic activity, vascularity and the presence of excretory-secretory activity. None of these consistently correlate with cerebral edema. Our cases suggest that spontaneous hemorrhage into the tumour may be the common factor responsible for severe cerebral edema. It is stressed that though a decidedly rare complication, cerebral edema secondary to meningioma should be entertained in the differential diagnosis of acute neurologic deterioration of these patients.
Astrocytomas: Old and Newly Recognized Variants, Their Spectrum of Morphology and Antigen Expression
Authors: Kepes John J. 
Published: July 2016

Abstract:The relationship between the morphology of astrocytomas and their prognosis is complex, with the localization of the tumour, the predominant cell type and the degree of anaplasia all playing an important part in determining the patient's future. Since many astrocytomas have a diversity of patterns, small needle biopsies taken from just one or a few areas may fail to elucidate the principal features of a given tumour. In addition to the astrocytoma subtypes listed in the WHO's International Histological Classification of brain tumours, new entities have been observed in the last few years. These include lipidized forms, such as the relatively benign pleomorphic xanthoastrocytoma and the highly malignant lipidized glioblastoma. Neoplastic astrocytes are capable of forming basal laminae and of phagocytosis, and often contain alpha-1-antitrypsin, features that may lead to confusing them with histiocytes. They may be arranged in a whorled pattern imitating meningiomas, their myxoid intercellular stroma may assume the morphology of cartilage and closely packed tumour cells in “epitheloid” astrocytomas come close to imitate metastatic carcinoma. Some astrocytomas contain cells indistinguishable from those of granular cell tumours of other tissues. The presence of reactive lymphocytes in astrocytomas and reactive astrocytes in malignant lymphomas can be the source of confusion between those two kinds of neoplasms.
Triphasic Waves: Clinical Correlates and Morphology
Authors: Sundaram Mecheri B.M. , Blume Warren T. 
Published: July 2016

Abstract:Twenty-six (41%) of 63 consecutive patients with triphasic waves had various types of metabolic encephalopathies while 37 patients (59%) had non-metabolic encephalopathies, usually senile dementia. Triphasic waves were not found to be specific for any single type of metabolic encephalopathy.Etiology was more closely linked to conscious level at recording than any morphological or distributional feature of the triphasic waves themselves. Thus, all 31 alert patients had non-metabolic encephalopathies while all 13 comatose patients had metabolic encephalopathies.The second, positive, component (Wave II) most often had the highest voltage while equally maximal Waves I and II occurred next most commonly. Triphasic waves were most often maximally expressed anteriorly.Among patients with metabolic encephalopathies, a posterior-anterior delay or lag of the wave II peak occurred more commonly than did the better known anterior-posterior lag. Lags occurred with both metabolic and nonmetabolic conditions, but were more common with the former. No difference in quantity or mode of appearance existed between the metabolic and non-metabolic groups when matched for conscious level.Prognosis for patients with either metabolic or non-metabolic encephalopathies was unfavourable. Only 4 of 24 metabolic and one of 35 non-metabolic patients were well at follow-up over 2 years later.Forty percent of EEGs with sharp and slow wave complexes (slow spike waves) had sporadically-appearing triphasic waves. The relative amplitudes of the 3 components differed from triphasic waves in other conditions: equally maximal Waves II and III were the most usual form.
Fenestration of the Supraclinoid Internal Carotid Artery
Authors: Findlay J. Max , Chui Mario , Muller Paul J. 
Published: July 2016

Abstract:A twenty-eight year old woman presenting with subarachnoid hemorrhage was found at angiography to have a left anterior cerebral-anterior communicating artery aneurysm. Also identified was a fenestration of the right supraclinoid internal carotid artery with an associated accessory middle cerebral artery. This appears to be the second reported case of fenestration of the intracranial internal carotid artery. Fenestrations of cerebral vessels and their possible embryologic origins are briefly reviewed.
Colour Vision Abnormalities in Multiple Sclerosis
Authors: Harrison Alexandra C. , Becker Werner J. , Stell William K. 
Published: July 2016

ABSTRACT:A battery of colour vision tests was employed to evaluate visual function in patients with multiple sclerosis (M.S.). Colour deficits were found in 45% of patients tested with the Ishihara plates and 42.5% of patients tested with the FM 100-Hue test. 65% of M.S. patients failed at least one of the tests.The colour vision deficits were not restricted to patients with optic neuritis or with visual evoked potential (VEP) abnormalities and there was no significant correlation between an abnormal VEP latency and a colour vision deficit. Colour vision testing may be a useful option to consider in the investigation of M.S. patients, even if there is no other evidence of visual system involvement.
CT Findings in Late-Onset Epilepsy
Authors: Sayette V. de la , Cosgrove R. , Melanson D. , Ethier R. 
Published: July 2016

ABSTRACT:We reviewed the CT findings of 387 patients with new-onset seizures after the age of 50. Seizures were generalized in 212 patients, focal in 160, and indeterminant in 15. CT scanning revealed cerebral atrophy in 113 cases, ischemic lesions in 75, cerebral neoplasms in 20, and no abnormality in 177 cases. Tumours were found in only three patients with generalized seizures, and all three had focal neurological deficits at the time of CT diagnosis, while 17 neoplasms were discovered in patients with a focal seizure disorder. The majority of patients with late-onset epilepsy have a normal CT scan with cerebral atrophy being the most common abnormality detected. Cerebral vascular disease appears to be the most frequently identified cause of late-onset epilepsy, while cerebral neoplasms are uncommon.
A Technique for Rapid Non-Suture Vascular Anastomosis
Authors: Gentili Fred , Lougheed W.M. , Yoshijima Sunao , Hondo Hideki , MacKay Margot B. 
Published: July 2016

ABSTRACT:In an attempt to overcome some of the difficulties inherent in vascular anastomosis using standard suture methods, we have developed a technique that allows for rapid non-suture anastomosis of blood vessels. The technique has been used in over 400 anastomoses, including interposition vein grafts in rat, rabbit and dog vessels ranging in size from 0.7 mm to 3 mm in diameter. Animals have been followed for over 1 year ostoperatively with serial angiography and histological evaluation including scanning electron microscopy. Stress studies have compared the biomechanical properties of both non-suture and suture anastomosis. The overall patency rate in non-suture end-to-end anastomoses using an interposition vein graft was 85% at 1 year follow up. Histological evaluation revealed a healing pattern that was temporally similar to that of the suture technique. Stress tests revealed no significant difference between suture and non-suture technique. These studies reveal that the non-suture technique compares favourably with the suture technique, and demonstrate the feasibility of applying this technique to man.
Multicore Myopathy: Not Always a Benign Entity
Authors: Shuaib Ashfaq , Martin Justin M.E. , Mitchell L. Brent , Brownell A. Keith W. 
Published: July 2016

ABSTRACT:Four patients with Multicore Myopathy, a rare morphologically distinct myopathy, are described. Although previously considered to be a non-progressive or only slowly progressive myopathy, progression to significant disability was seen in three of our cases. The association of cardiac disease with Multicore Myopathy has not been previously emphasised. All four patients in this study had a cardiomyopathy, and heart disease was the cause of death in two of the patients. Multicore Myopathy is not always a benign entity. Cardiac involvement, when present, adversely affects prognosis.
Cerebral Metastases from Malignant Melanoma
Authors: Mendez Ivar M. , Del Maestro Rolando F. 
Published: July 2016

ABSTRACT:Six hundred and fifty-two patients with histologically proven primary malignant melanoma have been followed by the London Regional Cancer Centre from 1960 to 1985. Neurological signs and symptoms secondary to metastases to the brain developed in fifty-five patients (8.4%). The median age was 49 years; 71% were male and 29% female. Multiple lesions were found in 61% and a single metastasis in 39%. The most common site for the primary lesion was the trunk in males (44%) and the lower limb in females (37%). Six month survival for patients with a single metastasis was 58% if surgical excision was possible and 25% of these patients survived greater than two years. In patients with multiple metastases that received radiotherapy, survival times of greater than six months were found in 12% of the patients. Patients with a single metastasis appear to benefit by being managed by surgical removal of the lesion.
Hypomelanosis of Ito. Neurological Complications in 34 Cases
Authors: Pascual-Castroviejo Ignacio , López-Rodriguez Luisa , Medina María de la Cruz , Salamanca-Maesso Cipriano , Herrero Carmen Roche 
Published: July 2016

ABSTRACT:We studied 34 Spanish children with hypomelanosis of Ito. This disease has an incidence of 1 per 1000 new patients consulting a paediatric neurological service, or 1 per 8000-10,000 unselected patients in a children's hospital. About 94% of our patients show noncutaneous abnormalities. Mental retardation (IQ below 70) was present in 64.7%; another 14.7% had an IQ between 70 and 90, usually associated with poor school performance. Four children exhibited autistic behaviour. Seizures of various types were present in 53% of cases. Other skin alterations in addition to the typical hypomelanosis were observed in 38% of our cases: caf6-au-lait spots, angiomatous nevi, nevus marmorata, nevus of Ota, Mongolian blue spot, heterochromia of the iris or hair, and other nonspecific pigmentations. Other associated disorders occur inconsistently and include macrocephaly, microcephaly, h6mihypertrophy, kyphoscoliosis, coarse facial features, genital anomalies, inguinal hernia, congenital heart disease, hypertelorism, and abnormalities of the teeth, feet and eyes. Autosomal dominant inheritance is demonstrated in some but not all cases.
Cerebral Arteriovenous Malformations with Associated Arterial Aneurysms: Hemodynamic and Therapeutic Considerations
Authors: Kondziolka Douglas , Nixon Bruce J. , Lasjaunias Pierre , Lasjaunias Pierre , Tucker William S. , TerBrugge Karel , Spiegel Sanford M. 
Published: July 2016

ABSTRACT:The common vascular anomalies of cerebral aneurysm and arteriovenous malformation may exist independently, or together as part of a closely related hemodynamic pairing. Resection or embolization of an AVM may be followed by a decrease in local blood flow, and lead to regression of a suitably situated proximal aneurysm. However, aneurysms located outside the angioarchitecture of the AVM, which remain flow-unrelated to the malformation, will likely not regress, and may in fact enlarge. Two cases are presented which demonstrate these vascular relationships, in order to better understand the regional hemodynamics of these anomalies prior to surgical or endovascular treatment planning.
Myopathy in Primary Systemic Amyloidosis
Authors: Roke M.E. , Brown W.F.E. , Boughner D. , Ang L.C. , Rice G.P.A. 
Published: July 2016

ABSTRACT:Involvement of the peripheral nervous system by amyloidosis is common. It is less well recognized that amyloid can directly infiltrate and weaken skeletal muscle. We report a case of a 73-year-old woman, known to have cardiac amyloidosis, who developed profound weakness secondary to amyloid myopathy. Review of the 8 other well documented cases in the literature has revealed a rather homogeneous syndrome. Proximal weakness, muscle stiffness, pseudohypertrophy and myalgia constitute the principal features. This syndrome usually develops in cases with well recognized generalized amyloidosis. Amyloid is deposited within the basal lamina of blood vessels and muscle fibers.