bluuebunny/crossref_metadata_2025_split huggingface dataset:
Myasthenia gravis and myasthenic syndromes
Authors: Engel Andrew G. 
Published: January 2005

AbstractMore than a decade ago myasthenic symptoms were observed in rabbits immunized with acetylcholine receptor (AChR) {119} and AChR deficiency was found at the neuromuscular junction in human myasthenia gravis (MG) {36}. By 1977 the autoimmune character of MG and the pathogenic role of AChR antibodies had been established by several measures. These included the demonstration of circulating AChR antibodies in nearly 90% of patients with MG {87}, passive transfer with IgG of several features of the disease from human to mouse {149}, localization of immune complexes (IgG and complement) on the postsynaptic membrane {30}, and the beneficial effects of plasmapheresis {20, 123}. Substantial subsequent progress has occurred in understanding the structure and function of AChR and its interaction with AChR antibodies. The relationships of the concentration, specificities, and functional properties of the antibodies to the clinical state in MG have been carefully analyzed, and the mechanisms by which AChR antibodies impair neuromuscular transmission have been further investigated. The clinical classification of MG has been refined, the role of the thymus gland in the disease has been further clarified, and new information has become available on transient neonatal MG. The prognosis for generalized MG is improving, but there is still no consensus on its optimal management. Novel therapeutic approaches to MG are now being explored in animal models. Recognition of the autoimmune origin of acquired MG also implied that myasthenic disorders occurring in a genetic or congenital setting had a different cause. As a result, a number of congenital myasthenic syndromes have come to be recognized and investigated. Finally, an acquired disorder of neuromuscular transmission different from MG, the Lambert‐Eaton myasthenic syndrome, has also been shown to have an autoimmune basis. In this syndrome, active zone particles of the presynaptic membrane are direct or indirect targets of the pathogenic autoantibodies.
Nuclear magnetic resonance imaging in evaluation of clinical stroke syndromes
Authors: DeWitt L. Dana , Buonanno Ferdinando S. , Kistler J. Philip , Brady Thomas J. , Pykett PhD Ian L. , Goldman Mark R. , Davis Kenneth R. 
Published: January 2005

AbstractProton nuclear magnetic resonance (NMR) imaging was performed on normal volunteers and patients with various types of clinical strokes. True three‐dimensional volumetric data were obtained for subsequent reconstruction of images at various orientations, including transverse, coronal, and sagittal, and for specific matching to x‐ray computed tomographic planes. A variety of radiofrequency pulse sequences was used to generate images weighted by the NMR parameters spin density (ρ) and spin‐lattice (T1) and spin‐spin (T2) relaxation times. Quantitative T1 data and qualitative T2 information were obtained from appropriate sequences. Proton NMR images embodying T1 information provided adequate delineation of normal intracranial anatomical structure and a high level of gray matter–white matter contrast; high lesion detection sensitivity in various clinical forms of stroke was seen in images providing both T1 and T2 information. T1 and T2 relaxation times were prolonged in embolic, thrombotic, and watershed infarctions as well as in lacunae; shortening of T1 and T2 was found in hematomas. Serpiginous vessels in an arteriovenous malformation were seen that had particular pulse sequences.
Galactose neuropathy: Impact of chronic endoneurial edema on nerve blood flow
Authors: Myers Robert R. , Powell Henry C. 
Published: January 2005

AbstractPeriperal neuropathy induced by galactose feeding results in endoneurial edema with increased tissue pressure and ultimate demyelination of nerve fibers. To assess the role of ischemia in the pathogenesis of this neuropathy, nerve blood flow was measured 6 months after the start of galactose ingestion, between the onset of edema and nerve fiber changes. Measurement was carried out by a noninvasive technique involving the use of [14C]iodoantipyrine as a radioactive tracer of tissue perfusion. A significant decline in nerve blood flow was found, which correlated positively with increased nerve water content as quantified by a microgravimetric technique. We sought to establish the pathogenic role of nerve edema in the development of schwannopathy and demyelination by morphological examination. It was found that Schwann cells appeared normal in areas of the peripheral nervous system in which edema does not occur, such as spinal ganglia and roots, whereas in regions in which there was edema, massive glycogen accumulation in Schwann cells and demyelination occurred. These findings suggest that edema, rather than hyperactivity in the sorbitol pathway, is responsible for the pathological changes in galactosemic neuropathy and that ischemia resulting from edema and increased endoneurial fluid pressure is the mechanism responsible for fiber injury.
Quality‐specific taste changes in multiple sclerosis
Authors: Catalanotto Frank A. , Dore‐Duffy Paula , Donaldson James O. , Testa Marcia , Peterson Margaret , Ostrom Karin M. 
Published: January 2005

AbstractTaste sensitivity in 79 patients with multiple slcerosis (MS) and 65 age‐ and sex‐matched control subjects was measured with a sip‐and‐spit, suprathreshold scaling, magnitude estimation procedure using six concentrations each of sodium chloride, sucrose, citric acid, and quinine hydrochloride. Results were analyzed with a taste scoring system and by plotting psychophysical functions (log concentration versus log magnitude estimate) normalized to 1.0 M sucrose. Gender did not affect taste scores, but age was inversely related, so the results were analyzed by an analysis of covariance with age as the covariant. There was a significant alteration in taste sensitivity in the subjects with MS for sodium chloride and quinine hydrochloride stimuli but not for sucrose and citric acid; these results were confirmed by a separate analysis of the psychophysical functions. Some of the MS taste scores correlated with MS functional and physical disability scores. Taste sensitivity was not correlated with clinical history or presence of facial symptoms.
Neonatal Citrobacter meningitis: Pathogenesis of cerebral abscess formation
Authors: Foreman Susan D. , Smith Edward E. , Ryan Nell J. , Hogan Gwendolyn R. 
Published: January 2005

AbstractIn 2 premature infants with brief survival, the major pathological feature of Citrobacter meningitis consisted of vasculitis and infarction with necrosis and liquefaction of large portions of the white matter of the hemispheres. Areas of decreased density demonstrated on computed tomographic head scan represented infarction with necrosis, liquefaction, and cavitation and not a classic abscess. This finding has clinical significance, because surgical drainage of liquefied infarcts is rarely indicated and could further damage the relatively preserved cortex.
Idiopathic dystonia‐parkinsonism with marked diurnal flucuation of symptoms
Authors: Sunohara Nobuhiko , Mano Yukio , Ando Kazuya , Satoyoshi Eijiro 
Published: January 2005

AbstractStudies were performed on 5 patients with idiopathic dysotnia‐parkinsonism, each of whom had minor dystonic movements and parkinsonian symptoms with marked diurnal fluctuation. Levels of homovanillic acid and 5‐hydroxyindoleacetic acid were not defferent from those in controls. Considerable improvement in dystonic movements and parkinsonian symptoms was obtained with a small dose of L‐dopa, trihexyphenidyl hydrochloride, amantadine, or bromocriptine hydrochloride. Electrophysiological investigation confirmed that the dystonic movements, which became remarkably worsened on attempted movement, contributed importantly to the diurnal fluctuation of symptoms.
Comparison of survival times of mice inoculated with brain tissue from various neurological diseases
Authors: Greenham Leighton W. , Peacock David B. 
Published: January 2005

AbstractMice inoculated intracerebrally before the age of 5 days with homogenates of autopsied brain tissue from patients with Creutzfeldt‐Jakob (C‐J), Alzheimer's, or Pick's diseases showed highly significant decreases in life span when compared with sham‐inoculated control mice. With C‐J disease there was strong indication of horizontal transmission of the agent to unionculated mice caged with the inoculated mice. While the results with C‐J disease were not unexpected in view of the known infectious etiology of the disease, the results obtained with Alzheimer's and Pick's diseases suggest that previously undetermined infectious or toxic agents may be associated with these who diesease, which currently have unresolved etiologies. Mice inoculated with autopsied brain tissue from patients with multiple scelrosis or subacute sclerosing panencephalitis had life spans similar to those of the control mice.
Inflammatory myopathies: Part 1
Authors: Mastaglia Frak L. , Ojeda Victor J. 
Published: January 2005

AbstractThe inflammatory myopathies have diverse clinical and pathological features and multiple etiologies. Some are confined to a single muscel or group of muscles (e.g., orbital myositis and localized nodular myositis) while others are diffuse. Infective forms may be due to viral, bacterial, fungal, protozoal, or parasitic organisms. Viruses may cause acute self‐limited forms of myositis and have been isolated from muscle in some cases of acute rhabdomyolysis and inclusion body myositis. They have also been implicated in some cases of acute rhabdomyolysis and inclusion body myositis. They have also been implicated in some cases of congenital myopathy and in polymyositis and dermatomyositis, but there is no evidence of viral invasion of muscle in these conditions. In polymyositis and dermatomyositis there are derangements in humoral and cellular immune function, and recent evidence suggests an underlying disturbance of immunoregulation. The roles of genetic factors, drugs, and Toxoplasma infection have been under scrutiny. There is increasing recognition of immunological and pathological differences in polymyositis and juvenile and adult dermatomyositis, and in cases with associated connective tissue diseases, suggesting different underlying pathogenetic mechanisms. Inclusion body myositis, eosinophilic myositis, and granulomatous myositis can be separated from the other idiopathic inflammatory myopthies because of distinctive clinical and pathological features and this may also reflect different mechanisms of muscle injury. Recent developments in the treatment of the idiopathic inflammatory myopathies include the use of plasmapheresis and total‐body irradiation in cases that are resistant to corticosteroids and immunosuppressive drugs.
Atypical presentation of progressive supranuclear palsy
Authors: Davis P. H. , Bergeron C. , McLachlan D. R. 
Published: January 2005

AbstractFour pathologically documented cases of profressice supranuclear palsy are reported. Two patients exhinited severe dementia and 2 parkinsonism; none had the classic opthalmoplegia. On retrospective analysis, clues to the diagnosis included early prominent gait disturbance, apraxia of eyelid opening in 1 patient, lack of tremor, poor response to levodopa‐carbidopa, and severe rigidity with a posture of neck extension terminally in 1 patient. The clinical presentation of progressive supranuclear palsy, therefore, is not as stereotyped as previously thought, and the diagnosis can be overlooked if one adheres rigidly to the classic diagnostic criteria.
Fatal infantile mitochondrial myopathy and renal dysfunction caused by cytochrome c oxidase deficiency: Immunological studies in a new patient
Authors: Zeviani Massimo , Nonaka Ikuya , Bonilla Euardo , Okino Eizo , Moggio Maurizio , Jones Sara , DiMauro Salvatore 
Published: January 2005

AbstractA 3‐month‐old female infant had profound generalized weakness, de Toni‐fanconi‐Debre syndrome, and lactic acidosis. She required asisted ventilation and died at the age of 8 months. Muscle biosy showed accumulated of mitochondria, glycogen, and lipid droplets. Histochemical reaction and immunocytochemical stain for cytochrome c oxidase showed very weak normal in intrafusal fibers of the muscle spindle. In crude extracts of the patient's muscle, cytochrome c oxidase activity was undetectabel and enzyme‐linked immunosorbent assay showed decreased reaction at all dilutions of antiserum. These data indicate that the amount of immunoreactive enzyme protein is markedly decreased in muscle of patients with fatal infantile cytochrome c oxidase deficiency and renal dysfunction.
The initial diagnosis of multiple sclerosis: Clinical impact of magnetic resonance imaging
Authors: Gebarski Stephen S. , Gabrielsen Trygve O. , Gilman Sid , Knake James E. , Latack Joseph T. , Aisen Alex M. 
Published: January 2005

AbstractThirty patients in whom the initial diagnosis of multiple sclerosis was clinically entertained underwent cranial magnetic resonance imaging (MRI) in close temporal relationship to cranial x‐ray computed tomography (CT), electrodiagnostic studies (visual evoked responses, brainstem auditory evoked responses, and somatosensory evoked responses), and cerebrospinal fluid analyses (Oligoclonal bands, myelin basic protein, and IgG/albumin ratio). In 26 of the 30 patients, MRI demonstrated lesions consistent with multiple sclerosis that corresponded, at least in part, with the clinically expected neuroanatomical lesion distribution. Two of the 4 patients with normal MRI had normal electrodiagnostic studies and cerebrospinal fluid analyses, and the other 2 had a single abnormal or equivocal electrodiagnostic study. All 26 patients with abnormal MRI had at least one other abnormal laboratory test. CT revealed only the largest lesions, and in the patients with abnormal CT, MRI demonstrated even more lesions. MRI, in this limited series, proved to be a strong tool in the initial diagnosis of multiple sclerosis; it may prove to be the single best test, with a sensitivity exceeding that of electrodiagnostic studies and cerebrospinal fluid analysis.
The critical role Wernicke's area in sentence repetition
Authors: Selnes Ola A. , Knopman David S. , Niccumm Nancy , Rubens Alan B. 
Published: January 2005

AbstractThe anatomical correlates of persistent deficits in sentence repetition were studied in a group of 32 right‐handed patients with fluent aphasia caused by left hemisphere ischemic stroke. Patients whose ability to repeat remained impaired by 6 month after onset almost withoutexveption had damage to Wernicke's area had impaired repetition 6 months after onset. The most consistent lesion correlating with chronic impairment of repetition, therefore, was one destroying parts or all of Wernicke's area. Lesion outside of this area typically resulted in milder impairment of repetition that resolved within a few months. Only 5 patients had no repetition impairment when tested early (1 month after onset). These patients all had lesions anterior to the rolandic fissure, some including Broca's area.
Nucleus tractus solitarius lesions elevate pulmonary arterial pressure and lymph flow
Authors: Darragh Teresa M. , Simon Roger P. 
Published: January 2005

AbstractThe effects of lesions of the nucleus tractus solitarius (NTS) on pulmonary vascular pressure and pulmonary lymph flow were investigated in 6 halothane‐anesthetized sheep. After lesions of the NTS were created using bilateral thermocoagulation, pulmonary artery pressure rose to 150% of baseline and remained elevated for the 3‐hour duration of the experiment. Systemic and left atrial pressures did not change. Sham NTS lesions and lesions lateral of NTS produced no changes. These experiments demonstrate that lesions of the central nervous system can alter pulmonary vascular pressures and transcapillary fluid flux independently of effect upon the systemic circulation. These findings may findings may have relevance for the understanding of neurogenic pulmonary edema in humans.
Cerebral cortical function in infants at risk for sudden infant death syndrome
Authors: Clancy Robert R. , Spitzer Alan R. 
Published: January 2005

AbstractCerebral cortical function was prospectively examined by electroencephalography (EEG) in 3 subgroups of 257 infants at risk for sudden infant death syndrome (SIDS). Group 1 consisted of apparently healthy infants with near‐miss SIDS episodes; Group 2 consisted of siblings of SIDS victims; and Group 3 consisted of neurologically suspect infants with apnea. The usual abundance and distribution of sharp EEG transients (SETs) were determined from 69 Group 1 infants. EEGs were interpreted as abnormal in the presence of ictal apnea, excessively abundant SETs, or immaturity of EEG background for conceptional age. Ninety percent of infants in Group 1 had entirely normal EEGs. There was no significant difference in the abundance or distribution of SETs between infants with normal breathing patterns and those with excessively periodic respirations. Nonictal apnea was recorded in 7% of Group 1 infants. The unexpected diagnosis of ictal apnea was confirmed in 2 Group 1 infants (1.2%), and 5 (2.9%) had excessive SETs but no recorded seizures. Only 4 infants (2.3%) had abnormally immature EEGs for conceptional age. Nonictal apnea occurred in 5 of 33 (15.2%) Group 3 infants and ictal apnea was confirmed in 2 others (6.1%). We conclude that the majority of Group 1 and 2 infants have normal cerebral cortical activity between and during apnea and that central nervous system cortical immaturity, as measured by EEG, plays no important role in the pathogenesis of SIDS. SETs are commonly recorded in these infants and must be conservatively interpreted. However, an EEG examination was critical in establishing the unexpected diagnosis of ictal apnea in a small percentage of Group 1 and 3 infants and materially influenced subsequent evaluation, management, and prognosis.
Acute traumatic midbrain hemorrhage
Authors: Ropper Allan H. , Miller Douglas C. 
Published: January 2005

AbstractA syndrome resulting from traumatic midbrain hemorrhage is described in 5 patients, 3 of whom had intracranial pressure measurements and 2 of whom had pathological examinations. The major clinical signs, evident immediately after injury, are coma, dilated (4 to 8 mm diameter) pupils that are fixed to light and dark, diminished limb tone, and impaired horizontal eye movements. The hemorrhages are single and linear or oval‐shaped in the midsagittal rostral midbrain, extending several millimeters bilaterally. The appearance of the hematoma may be delayed on computed tomographic scanning, but early abnormalities of wave V of the brainstem auditory evoked potentials aid in diagnosis. Pathological features suggest that secondary brainstem distortion occurred soon after injury, perhaps resulting from a transiently raised intracranial pressure, but shearing injuries caused by acceleration–deceleration forces were also found.
A quantitation of myelin‐associated glycoprotein and myelin basic protein loss in different demyelinating disease
Authors: Gendelman Howard E. , Pezeshkpour Gholam H. , Pressman Norman J. , Wolinsky Jerry S. , Quarles Richard H. , Dobersen Michael J. , Trapp Bruce D. , Kitt Cheryl A. , Aksamit Allen , Johnson Richard T. 
Published: January 2005

AbstractThe loss of myelin‐associated glycoprotein (MAG) and myelin basic protein (MBP) was compared by quantitative immunocytochemistry in demyelinating lesions of measles encephalomyelitis (ME), multiple sclerosis (MS), and progressive multifocal leukoencephalopathy (PML). Serial sections from paraffin‐embedded tissue were reacted with antisera for MAG and MBP, and areas of staining loss were compared morphometrically. Lesions in ME showed MAG loss equal to that of MBP, lesions of PML showed MAG loss greater than that of MBP, and MS lesions showed a mixture of patterns. These data demonstrate distinctive patterns of MAG and MBP loss in these three diseases.
Cerebal norepinephrine depletion enhances recovery after brain ischemia
Authors: Busto Raul , Harik Sami I. , Yoshida Shinichi , Scheinberg Peritz , Ginsberg Myron D. 
Published: January 2005

AbstractMonoamine neurotransmitters, especially norepinephrine (NE), may have an important role in the pathophysiological aspects of postischemic cerebral dysfunction. In previous studies of post‐decapitation–induced ischemia, we found that NE depletion caused a delay in glycogen breakdown but did not influence any of the other known biochemical abnormalities that accompany brain ischemia. In this study, we have turned to a model of transient incomplete and diffuse forebrain ischemia in the rat to examine the effects of cerebral NE depletion on the recovery after brain ischemia of levels of high‐energy phosphate compounds, products of intermediary oxidative metabolism, and free fatty acids. We found that a unilateral lesion of the locus ceruleus and the resultant depletion of NE in the ipsilateral cerebral cortex had no effect on sham‐operated controls nor on rats subjected to ischemia alone. However, in rats subjected to ischemia followed by 15 minutes of recirculation, the NE‐depleted cerebral cortex had significantly higher phosphocreatine and adenosine triphosphate levels and energy charge, and lower adenosine monophosphate and docosahexaenoic acid concentrations. With longer periods of recirculation, these side‐to‐side differences were not apparent. These results suggest that activity of the central NE systems during transient brain ischemia has deleterious effects on the biochemical recovery of the cerebral cortex from severe ischemic insults.
Chickenpox with delayed contralateral hemiparesis caused by cerebral angiitis
Authors: Kamholz John , Tremblay Gerald 
Published: January 2005

AbstractChickenpox and herpes zoster ophthalmicus are caused by the same virus. Herpes zoster ophthalmicus can be followed by contralateral hemiparesis, which is thought to be caused by spread of varicella‐zoster virus to blood vessels contiguous to the trigeminal nerve and its branches. We report what we believe to be the first case of a patient with chickenpox followed by hemiparesis in whom there was angiographic evidence of an associated vasculitis similar to that found with herpes zoster ophthalmicus.
Acute improvement in exacerbating multiple sclerosis produced by intraenous administration of mannitol
Authors: Stefoski D. , Davis F. A. , Schauf C. L. 
Published: January 2005

AbstractThe mode of action of adrenocorticotropic hormone (ACTH) treatment in exacerbating multiple sclerosis was studied by short‐term infusions of agents that mimic specific and limited pharmacological actions of ACTH and observing for temporally phase‐locked clinical changes. The study was double blinded, and agents were administered while the patients were being treated with a standard course of 10‐day intramuscular ACTH therapy (40 U twice daily). Antiedema, alkalotic–hypocalcemic, extraadrenal, and sodium‐retaining actions were studied using infusions of mannitol, sodium bicarbonate, ACTH, and sodium chloride, respectively. Seven of 8 patients receiving placebo infusions (2.5% glucose) showed no significant clinical change and 1 exhibited an equivocal improvement. Five of 9 patients receiving mannitol showed definite signs of clinical improvement phase‐locked to drug administration, with subsequent gradual reversal to baseline. Similar improvements occurred with infusions of NaHCO3 in 5 of 8 patients and of ACTH in 4 of 8 patients. Three of 7 patients given NaCl infusion showed possible mild improvements. The results indicate that mannitol and NaHCO3 induced transient acute improvement in signs at the 95% confidence level in patients with exacerbating multiple sclerosis, with ACTH having a similar effect at the 90% confidence level. These agents mimic some of the known effects of ACTH, which may be important in the therapeutic action of ACTH in multiple sclerosis. A possible role for mannitol and high‐dose ACTH in the treatment of demyelinating disease warrants further study.