bluuebunny/crossref_metadata_2025_split huggingface dataset:
Tizanidine Versus Baclofen in the Treatment of Spasticity in Patients with Multiple Sclerosis
Authors: Bass B. , Weinshenker B. , Rice G.P.A. , Noseworthy J.H. , Cameron M.G.P. , Hader W. , Bouchard S. , Ebers G.C. 
Published: July 2016

ABSTRACT:Tizanidine (Sirdalud) was compared to baclofen (Lioresal) in a randomized, double-blind, cross-over trial. Each medication was introduced over a three week titration period and then maintained at the highest tolerated dose for five weeks. The two treatment phases were separated by a one week drug withdrawal and a two week washout period. Sixty-six patients entered the trial and forty-eight completed both treatment phases. At the end of the trial, neurologists and physiotherapists thought that baclofen was superior on the basis of perceived efficacy and tolerance (p≤0.05). Although the efficacy of tizanidine or baclofen was judged as good to excellent by 24 and 39% of patients respectively, this difference was not statistically significant. Muscle weakness was the most common adverse effect. This was significantly more troublesome in patients treated with baclofen. Somnolence and xerostomia were more common in patients treated with tizanidine. Both baclofen and tizanidine appear to be useful adjuncts in the treatment of spasticity in patients with multiple sclerosis. Preference of either drug is tempered principally by side-effects.
Multimodal Evoked Potential Studies in Leukodystrophies of Children
Authors: De Meirleir L.J. , Taylor M.J. , Logan W.J. 
Published: July 2016

ABSTRACT:Evoked potentials were studied in 22 children with leukodystrophy [10 metachromatic leukodystrophy (MLD), 4 Pelizaeus-Merzbacher (PM), 3 Krabbes, 2 adrenoleukodystrophy (ALD), and one each of Alexander's, Canavan's and multiple sulphatase deficiency (MSD) diseases]. The ABRs were abnormal in all patients (except for the younger ALD), but varied with the type of leukodystrophy. The PM and Krabbes patients had abnormal ABRs with a loss of the rostral waves, accompanied in Krabbes with delayed I-III interpeak latencies; in MLD, ALD and MSD prolonged interpeak latencies were found. Three patients who had no clinical signs, but were positively diagnosed as MLD on the basis of absent arylsulphatase A, also had abnormal ABRs. The SEPs were abnormal in all patients. Cortical SEPs were absent in 16 and abnormal in 5 who were in the earlier stages of their disease. Cervical SEPs were within normal limits except for the Krabbes and MLD patients studied, who showed peripheral slowing. The VEPs were normal in only 6 and, unlike the ABRs and SEPs, did not seem to covary with clinical severity across the various leukodystrophies but did correlate with disease progression. Thus, multimodal EPs are useful in the diagnostic differentiation of the leukodystrophies.
Harrington Rod Instrumentation: A Cause of Brown-Sequard Syndrome
Authors: Van Orman C.B. , Darwish H.Z. 
Published: July 2016

ABSTRACT:We describe a child with Brown-Sequard syndrome beginning at C8-T1, immediately following Harrington rod instrumentation for idiopathic scoliosis. We believe this is most likely related to either a common variation of the branches of the anterior spinal artery to one side or due to rupture of the ipsilateral spinal vein. This possible complication should be considered in patients undergoing Harrington rod instrumentation.
Electrophysiological Monitoring During Acoustic Neuroma and Other Posterior Fossa Surgery
Authors: Linden R.D. , Tator C.H. , Benedict C. , Charles D. , Mraz V. , Bell I. 
Published: July 2016

ABSTRACT:Techniques used to monitor the function of the seventh and eighth cranial nerves during acoustic neuroma and other posterior fossa surgery are reviewed. The auditory brainstem response (ABR), electrocochleogram (ECochG) and direct recording from the auditory nerve (CNAP) were compared. The best technique is the ECochG, although in many cases, the CNAP should be used as a back-up technique. The CNAP is especially useful for the identification of the auditory nerve. Both can provide real-time feedback on the physiological integrity of the auditory nerve. The ABR may be helpful in monitoring brainstem function. For some procedures, optimal monitoring requires the combined recording of all three techniques.Monopolar constant-voltage intracranial stimulation of the facial nerve is helpful for the identification and preservation of the facial nerve. Audio monitoring of spontaneous electromyographic activity provides real-time feedback on the effect of surgical manipulation of the nerve. Monitoring of ephaptic transmission in the facial nerve during microvascular decompression for hemifacial spasm aids in the identification of the offending vessel.
Immunocytochemical Analysis of Intermediate Filaments in Human Ependymal Tumors
Authors: Izukawa D. , Lach B. 
Published: July 2016

ABSTRACT:The peroxidase anti-peroxidase technique was used for localization of glial fibrillary acidic protein (GFAP) and vimentin (VM) in 19 ependymal tumors in order to determine if a unique pattern of intermediate filament (IF) expression could be demonstrated. Cytokeratin (CK) immunoreactivity was examined in a subgroup of 7 tumors with papillary pattern. Nineteen non-ependymal neuroectodermal tumors were used as controls. Ependymomas, subependymomas and astrocytomas were positive for both IF. Oligodendrogliomas, oligodendroglial portions of mixed gliomas and the majority of medulloblastomas were negative for GFAP and VM. Areas of poor differentiation in all tumors demonstrated little expression of any IF. A composite ependymoma/choroid plexus papilloma showed the presence of GFAP, VM and CK in the papillomatous portion only. Four papillary ependymomas were negative for CK. This study emphasizes the parallel distribution of GFAP and VM in well differentiated ependymomas and other glial tumors and casts doubt upon the concept of VM as a marker for de-differentiation in neuroectodermal neoplasia.
Magnetic Resonance Imaging in the Assessment of Disease Activity in Multiple Sclerosis
Authors: Paty Donald W. 
Published: July 2016

ABSTRACT:Magnetic resonance imaging (MRI) has been shown to be a good method of visualizing the lesions in MS. We have studied several applications of MRI to the evaluation of patients and experimental models. In diagnosis, MRI is the most sensitive test for the demonstration of dissemination of lesions in space. Pathological correlation studies show that MRI reliably measures the extent of chronic demyelination. Experimental studies show that MRI detects acute inflammatory lesions and measures their evolution. MRI also is a reliable measure of the extent of the MS process, serial MRI scans detect evidence of disease activity in MS not always disclosed by clinical evaluation. MRI will have an enormous future impact on the evaluation of patients in clinical studies and in understanding the evolution of pathological processes.
Botulinum Toxin Injections in the Treatment of Blepharospasm, Hemifacial Spasm, and Eyelid Fasciculations
Authors: Kraft Stephen P. , Lang Anthony E. 
Published: July 2016

ABSTRACT:Seventy-six patients with blepharospasm (mean age 56.9 years) received 248 injection treatments with botulinum A exotoxin (mean 3.1 treatments per patient): 87.0% of treatments led to total relief of spasms for a mean interval of 14.1 weeks. The average duration of response remained fairly constant over the first six injection series, although patients with the most severe spasms had shorter intervals than patients with less severe symptoms. Twenty patients with hemifacial spasm (mean age 56.9 years) received 44 treatments (mean 1.9 treatments per patient): In 93.1% of cases there was total relief of periocular and perioral spasms, with a mean interval of 17.4 weeks. The average duration of response for the third series of treatments was much shorter than the mean durations for the first two treatments. Side effects were always transient and included ptosis (23.3%), dry eyes (18.1%), tearing (5.5%), and strabismus (1.4%). No patient had a systemic reaction to the drug. Chronic benign eyelid fasciculations were also successfully treated in 3 patients with single treatments.
Changes in Serum Anticonvulsant Levels with Febrile Illness in Children with Epilepsy
Authors: Goulden Keith J. , Camfield Peter R. , Camfield Carol S. , Tibbles John A.R. , Dooley Joseph M. , Fraser Albert D. , Renton Kenneth W. 
Published: July 2016

ABSTRACT:Changes in anticonvulsant serum levels during intercurrent illness may cause toxicity or decreased seizure control in children with epilepsy. We studied prospectively the effect of intercurrent illness and its treatment in 111 children being treated with AC monotherapy. Free fraction and total serum AC levels were determined when the child was well, on the fifth day of any illness with fever and one month after recovery. There were 55 episodes of febrile illness in 39 children during the study period. Twelve illnesses were associated with significant increases or decreases in serum AC levels; 7 children became clinically toxic; 1 child had increased seizures during illness. The mechanisms of AC level changes appeared to include interaction with antibiotics, with antipyretics or with viral illness. Amoxycillin and acetaminophen did not appear to interact with the AC's used. Physicians caring for children with epilepsy should be aware of the frequency and complexity of potential interactions between intercurrent febrile illness and anticonvulsant medication.
Interactions entre recherches fondamentale et clinique. Deux exemples tirés d'une expérience personnelle
Authors: Albe-Fessard D. 
Published: July 2016

ABSTRACT:Basic research helps to understand clinical knowledge which in turn leads to new fundamental research. Two examples are taken from the studies of the anomalies existing in the brain of patients suffering from Parkinson disease or deafferentation Pain. The researches performed to solve these two problems for the past 40 years (in particular by the author's working group) are replaced in the international context. In Man the precision of stereotaxic technique was improved by using the organisation of the somato-sensory thalamic projection described in animals by the physiologists. In the thalamus of Parkinsonian patients, the presence of cells bursting at tremor frequency led to search for the relations existing between the striato-nigral system and the motor thalamus. In the patients suffering from deafferentation Pain, a special role is attributed to the medial thalamus, whose characteristics were formerly studied in animals. The origin of the deafferentation Pain is nowadays studied in an animal model of this disease.
Hemodynamic and Metabolic Effects of Extracranial Carotid Disease
Authors: Leblanc Richard , Yamamoto Y. Lucas , Tyler Jane L. , Hakim Antoine 
Published: July 2016

ABSTRACT: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

ABSTRACT: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

ABSTRACT: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

ABSTRACT: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

ABSTRACT: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

ABSTRACT: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.
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

ABSTRACT: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

ABSTRACT: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

ABSTRACT: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

ABSTRACT: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

ABSTRACT: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

ABSTRACT: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.