bluuebunny/crossref_metadata_2025_split huggingface dataset:
Spinocerebellar engeneration with parkinsonian features: A clinical and pathological report
Authors: Weir Roger L. , Fan Kuang J. 
Published: January 2005

AbstractA 20‐year‐old patient had spinocerebellar ENgeneration and parkinsonian tremor that improved with L‐dopa treatment. Postmortem examination revealed posterior column atrophy in the spinal cord and severe ENgeneration in the substantia nigra. Atrophic changes were noted in ENep cerebellar nuclei and superior cerebellar peduncles. This study confirms the relationships between clinical parkinsonism, substantia nigra atrophy, and the therapeutic effect of L‐dopa.
Experimental viral infections of the facial nerve and geniculate ganglion
Authors: Davis Larry E. 
Published: January 2005

AbstractAlthough the cause of Bell's palsy is in most cases unknown, a number of viral infections have been associated with the disease. To determine if human viruses could experimentally infect the facial nerve or ganglion, six different viruses were inoculated into newborn hamsters by intracerebral, intralabyrinthine, or intraperitoneal routes. The facial nerve within the temporal bone and the geniculate ganglion were studied by light and immunofluorescent microscopy for the presence of viral infection. Herpes simplex, rubeola, mumps, and reovirus infected the neurons in the geniculate ganglia, while vaccinia virus infected the stroma cells of the facial nerve and ganglia. Influenza virus did not infect either the nerve or ganglia. These studies indicate that several human viruses can experimentally infect the facial nerve and geniculate ganglia, lending support to the hypothesis that Bell's palsy is caused by a virus.
Ultrastructure of tuberous sclerosis: Cortical tuber and subependymal tumor
Authors: Trombley Irene K. , Mirra Suzanne S. 
Published: January 2005

AbstractUltrastructural examination of two biopsy cases of tuberous sclerosis, a subependymal tumor and a cortical tuber, revealed giant cells with astrocytic features in both cases. These included multipolar processes containing glial filaments, glycogen, and membrane‐bound dense bodies, as well as formation of hemidesmosomes with pia and vascular basement membranes. Megamitochondria were also seen within glial processes. In addition to the numerous glial‐glial contacts encountered in both cases, rare neuroglial junctions suggesting aberrant synapse formation were observed within the cortical tuber. This observation, in concert with the organization of multiple small neurites along large, aberrant astrocyte processes found within the tuber, mimics features of neuroembryonic development described in lower mammals and nonhuman primates. Such a parallel in ultrastructural morphology supports a primarily dysplastic nature for the lesions in tuberous sclerosis.
Binaural interaction in brainstem potentials of human subjects
Authors: Levine Robert A. 
Published: January 2005

AbstractBinaural interaction in the short‐latency averaged auditory evoked potentials (AEPs) can be assessed from the binaural difference waveform (BD). The BD is derived by computing the difference between the AEP evoked by simultaneous clicks from both earphones and the sum of two other AEPs: one evoked by clicks from the right earphone alone and the other evoked by clicks from the left earphone alone. Once the contributions of acoustic cross talk and the middle ear reflex are eliminated, the BD can be considered to represent neural binaural interaction. This interaction begins after wave III and has its first major peak during the downslope of wave V. The relative amplitude of this peak to wave V is constant over a wide range of click rates and levels. The BD may have more localizing value than the AEPs alone since the BD probably represents a subpopulation of generators of the AEPs, which show binaural interaction at the single cell level.
Ataxic hemiparesis from a midbrain mass
Authors: Bendheim Paul E. , Berg Bruce O. 
Published: January 2005

AbstractA 10‐year‐old boy with leukemia developed ipsilateral corticospinal and cerebellar signs: the ataxic hemiparesis syndrome. Computed tomography revealed a mass lesion in the contralateral rostral midbrain. Previous reports of this syndrome placed the lesion in the basis pontis. The anatomical substrate of the ataxic hemiparesis syndrome includes the basis pontis, rostral midbrain, and possibly other areas within the central nervous system.
Methioninemia and myopathy: A new disorder
Authors: Gaull Gerald E. , Bender Adam N. , Vulovic Dimitrije , Tallan Harris H. , Schaffner Fenton 
Published: January 2005

AbstractA 7½‐year‐old girl with hypermethioninemia, myopathy, and mental deficiency (IQ = 65) is described. The increased methionine was not associated with deficiency of methionine adenosyltransferase, which was normal or increasedin liver, muscle, erythrocytes, and cultured fibroblasts. Methionyl‐tRNA synthetase in fibroblasts was normal. The hypermethioninemia and a concurrently increased blood S‐adenosylmethionine declined on a diet low in methionine. There was a diffuse, symmetrical, moderate proximalmuscle weakness, but muscle atrophy was not discernible, and the deep tendon reflexes were hypoactive but obtainable. Electromygraphic abnormalites were not detected. Electron microscopy of muscle revealed 3 to 6 small myelin figures in the region of the I band in nearly every fiber, with occasional myelin figures at other sites also. These myelin figures were more numerous and smaller than those seen accompanying nonspecific and may reflect a more specific pathological change. Electron microscopy of liver revealed three nonspecific lesions in all hepatocytes: (1) numerous megamitochondria with crystalloid deposit in the matrix; (2) increased numbers of small vesicles of smooth endoplasmic reticulum; and (3) loss of plasma membrane microvilli, with extensive bleb for formation and shedding of eytoplasm into Disse's space.
The ocular motor system
Authors: Spector Robert H. , Troost B. Todd 
Published: January 2005

AbstractRecent advances in ocular motor neurophysiology are reviewed with emphasis on brainstem control of horizontal saccadic and pursuit eye movements. Certain aspects of the control of vertical gaze and the role of the cerebellum and medial longitudinal fasciculus in normal and pathological states are also discussed. Current nomenclature and concepts are clarified so that they may be applied to the understanding of clinical ocular motor phenomenology.
Saccadic eye movement strategies in patients with homonymous hemianopia
Authors: Meienberg Otmar , Zangemeister Wolfgang H. , Rosenberg Michael , Hoyt William F. , Stark Lawrence 
Published: January 2005

AbstractInfrared oculographic recordings from three patients with hemianopia due to an occipital lesion showed that these patients employed a consistent set of (presumably unconscious) compensatory strategies to find and fixate objects. For targets in the blind hemifield, patients at first used a staircase strategy consisting of a series of stepwise saccadic search movements. This is safe but slow. When retested later, one patient had adopted a more efficient strategy employing one large saccadc calculated to overshoot the target. Other strategies for finding targets in the blind hemifield were employed in response to specific situations presented by our experiments: a predictive strategy using past experience to anticipate where the target would be found, and special strategies for recovering a lost target and for awaiting the reappearance of the target. To fixate targets in the seeing hemifield, our subjects undershot the target to prevent losing it in the blind hemifield, then held it off‐fovea on the seeing side of the macula.
Characterization of immune complexes in progressive rubella panencephalitis
Authors: Coyle P. K. , Wolinsky Jerry S. 
Published: January 2005

AbstractIn two patients with the slow virus disease progressive rubella panencephalitis, high levels of immune complexes were found in serum and, in one case, spinal fluid. These complexes contained immunoglobulin G. Serum complexes sedimented over a wide density range compared with the more homogeneous spinal fluid complexes. Complexes from both serum and spinal fluid contained antibody directed against rubella virus. Serum complexes were also shown to contain rubella antigen (or antigens) that appeared to differ between the two patients.
Polyneuropathy with monoclonal gammopathy: Studies of 11 patients
Authors: Dalakas Marchinos C. , Engel W. King 
Published: January 2005

AbstractAmong patients with chronic idiopathic nonfamilial polyneuropathy studied 3 to 21 years after onset, we identified 11 cases associated with monoclonal gammopathy (MCG) (5 with IgGK, 4 with IgMK, 2 with IgMλ). The patients, aged 29 to 80 years, presented with sensorimotor polyneuropathy of insidious onset and slow, nonfluctuating progression, delayed motor and sensory nerve conduction, and increased cerebrospinal fluid (CSF) protein. None of the patients in the initial or follow‐up study (mean, 9.2 years) had or developed signs of multiple myeloma, malignant plasma cell dyscrasia, macroglobulinemia syndrome, amyloidosis, neoplasia, or other associated illness.The CSF revealed abnormalities of protein electrophoresis or immunoelectrophoresis in 9 of the 11 patients. Three of 5 sural nerve biopsies studied by immunofluorescence demonstrated deposition in nerve fibers of the light chain characteristic of the abnormal circulating immunoglobulin. The findings suggest that these patients form a distinct subset of the dysimmune neuropathies. Although the immunoglobulin deposition and abnormal protein patterns in serum and CSF could be secondary to nerve damage, we propose that an immunopathological mechanism underlies the neuropathy. Immunosuppressants had minimal to marked beneficial effect in 4 of 5 patients, indicating that this polyneuropathy is potentially treatable.
Computed tomography in the diagnosis of Canavan's disease
Authors: Rushton Alan R. , Shaywitz Bennett A. , Duncan Charles C. , Geehr Robert B. , Manuelidis Elias E. 
Published: January 2005

AbstractComputed tomography (CT scan) demonstrated a symmetrical decrease in white matter attenuation of the cerebral hemispheres of two young children with macrocephaly and normal neurological examination. Subsequent developmental delay led to brain biopsy, which documented Canavan's disease (spongy degeneration of the brain, Van Bogaert‐Bertrand type). The CT scans obtained from these patients with proved Canavan's disease appeared to be quite characteristic in differentiating this disorder from Alexander's disease and adrenoleukodystrophy (Schilder's disease). The CT scan may decrease the necessity for diagnostic brain biopsy in these white matter disorders.
Physiological observations in the median nerve during carpal tunnel surgery
Authors: Yates S. K. , Hurst L. N. , Brown W. F. 
Published: January 2005

AbstractIn entrapment of the median nerve in the carpal tunnel, it has been claimed that release of the flexor retinaculum is accompanied by immediate improvement in conduction time across the caarpal tunnel segment. We observed no such immidiate changes in conduction time intraoperatively in eight subjects following retinaculum release or neurolysis. Neurolysis led to conduction block in some median nerves, cating doubt on the value of that procedure.
Myeloma protein synthesis within the CNS by plasma cell tumors
Authors: Siegal Tali , Shorr Joshua , Lubetzki‐Korn Isabelle , Soffer Dov , Naparstek Elizabeth , Tur‐Kaspa Ron , Abramsky Oded 
Published: January 2005

AbstractTwo patients had centrl nervous system involvement from plasma cell tumor, onr with leptomeningeal infiltraion and the other with solitary intracranial plasmacytoma. In both, cerebrospinal fluid (CSF) electrophoresis revealed IgG index consistent with local synthesi of the myeloma protein. the CSF findings were helpful in the diagnosis and evaluation of tumor recurrence.
SSPE, measles virus, and the matrix protein: Report of a case with unusual immunochemical findings
Authors: Mandelbaum David E. , Hall William W. , Paneth Nigel , Wolff Robert R. , DeVivo Darryl C. 
Published: January 2005

AbstractWe report a case of atypical subacute sclerosing panencephalitis (SSPE) in which the diagnosis was confirmed by a new radioimmunoprecipitation method. Antimeasles antibody was absent in the cerebrospinal fluid (CSF) when measured by conventional immunoassay techniques, there was no clinical response to isoprinosine, and the patient showed a selective absence of CSF antibody response to the matrix protein and only a partial serum antibody response to this measles polypeptide. The value of this sensitive immunoprecipitation method in profiling the selective antibody responses to the several measles polypeptides and the advisability of using isoprinosine, an immunopotentiating agent, to treat SSPE are discussed.
Instrumental neutron activation analysis of brain aluminum in Alzheimer disease and aging
Authors: Marchkesbery William R. , Ehmann William D. , Hossain T. I. M. , Alauddin Mohammad , Goodin Daniel T. 
Published: January 2005

AbstractInstrumental neutron activation analysis procedures were used to determine the aluminum content of various brain regions in histologically verified Alzheimer disease (AD) and in controls. The grand mean aluminum level for 74 AD specimens was 0.372 ± 0.058 μg/gm and for 137 adult controls, 0.467 ± 0.033 μg/gm, both on a wet weight basis. No difference was found at the bulk sample level between AD and adult controls, corrected for age and sex, or when frontal, temporal, and hippocampal specimens were compared. Control specimens (infancy to 85 years) showed an increase in brain aluminum concentration with age. Comparison of freez‐dried to wet weight ratios of AD and controls revealed a small increase in water content in AD brains.
Multiple sclerosis: Heterology among early T cells and TG cells
Authors: Traugott Ute , Scheinberg Labe C. , Raine Cedric S. 
Published: January 2005

AbstractCirculating early T cells and TG cells were estimated simultaneously in 100 patients with multiple sclerosis and 21 controls. The results were analyzed from individual patients, from patients grouped according to disease activity, and from some patients studied longitudinally. Previously documented fluctuations in these two T cell populations were confirmed. By comparing the fluctuations and by examining the distribution of TG cells in suspensions of isolated early and isolated late T cells, it was found that TG cells are a T cell subpopulation distinct from early T cells.
Brainstem auditory evoked responses and alpha‐patten coma
Authors: Hari Riitta , Sulkava Raimo , Haltia Matti 
Published: January 2005

AbstractA young comatose man with an alpha‐pattern electroencephalogram is reported. The brainstem auditory evoked responses were severely abnormal, suggesting a pontomesencephalic lesion which was confirmed at autopsy.
D‐lactic acidosis simulating a hypothalamic syndrome after bowel bypass
Authors: Carr Daniel B. , Shih Vivian E. , Richter James M. , Martin Joseph B. 
Published: January 2005

AbstractA 36‐year‐old man had one year of periodic symptoms suggestive of episodic hypothalamic dysfunction: hypersomnia, thirst, ravenous hunger and gorging behavior, pallor, and irritability. However, neuroendocrine testing proved normal. A mild transient acidosis at the onset of his attacks and a history of bowel bypass five years earlier prompted metabolic screening. Markedly increased urinary D‐lactic and phenolic acids were present, as were intermittent elevations of plasma D‐lactic acid during two symptomatic episodes. Prompt and sustained clinical remission coincided with disappearance of abnormal organic acid excretion during oral antibiotic therapy. D‐Lactic acidosis must be considered in the differential diagnosis of otherwise unexplained neurological syndromes, particularly in patients with altered bowel anatomy.
Multiple sclerosis: Trial of a synthetic polypeptide
Authors: Bornstein M. B. , Miller A. I. , Teitelbaum D. , Arnon R. , Sela M. 
Published: January 2005

AbstractA synthetic polypeptide, copolymer I (COP I), composed of alanine, glutamic acid, lysine, and tyrosine, has been demonstrated to be nonecephalitogenic and nontoxic in laboratory animals, yet it is capable of suppressing experimental allergic encephalomyelitis. A preliminary open trial examined the ability of COP I to alter the course of disease in 12 patients with chronic progressive and 4 with exacerbating‐remitting multiple sclerosis (MS). After therapy for as long as two years or more, no undersirable side reaction was noted in any patient. Three patients with chronic progressive MS and 2 with exacerbating‐remitting disease are better. These results, which may represent simply a placebo effect or may be a significant response, are now being examined in randomized, placebo‐controlled, double‐blind pilot trials.
Generalized paroxysmal fast activity: Electroencephalographic and clinical features
Authors: Brenner Richard P. , Atkinson Ruth 
Published: January 2005

AbstractWe reviewed electroencephalographic and clinical features of 20 patients with generalized paroxysmal fast activity (GPFA). All had seizure disorders and all but 3 had more than one seizure type. Mental retardation was present in 17 patients. In most cases no cause for the seizures could be found.GPFA occurred almost exclusively in sleep. Fifteen patients had clinical seizures associated with this activity; in 8 the seizures were tonic. Additional generalized epileptiform abnormalities were present in 18 patients, 10 of whom had slow spike and wave complexes. Only 4 patients had normal background activity.
Dopaminergic deficiency causes delayed visual evoked potentials in rats
Authors: Onofrj Marco , Bodis‐Wollner Ivan 
Published: January 2005

AbstractFlash and pattern visual evoked potentials (VEPs) to two temporal frequencies of stimulation were studied in nineteen rats. The effect of a tyrosine hydroxylase inhibitor and of a dopamine receptor blocker on the VEP was explored in ten animals. Significant latency change occurred following injection of either drug, while only the hydroxylase inhibitor reduced the VEP amplitude. These changes were not caused by the anesthesia used in these experiments, although the same anesthetics in higher doses did depress VEP amplitudes. When dopamine blockade was followed by administration of apomorphine, a dopamine agonist, VEP delays could be partially reversed. Besides conduction defects of myelinated axons, synaptic malfunction may also cause delays in sensory evoked potentials.
Kuru with incubation periods exceeding two decades
Authors: Prusiner Stanley B. , Gajdusek D. Carleton , Alpers Michael P. 
Published: January 2005

AbstractThe clinical characteristics of kuru in 15 patients are described. All the patients had a history of joint pains preceding difficulty walking. The severity of the neurological dysfunction varied from mild truncal and limb ataxia necessitating the use of a stick for walking to terminal illness accompanied by marked ataxia, rigidity, spasticity, and dementia. All the patients with kuru in this study had an apprehensive, frightened facial expression. Most of the patients examined showed diminished or absent optokinetic nystagmus bilaterally. Apprehensive facies and diminished optokinetic nystagmus have not previously been described in kuru. The other clinical features of the current patients with kuru are similar to those recorded twenty years ago. Epidemiological surveillance, anamnesis, and missionary reports strongly suggest that all the patients described in this study were exposed to the kuru agent more than two decades ago through ritualistic cannibalism. Thus the incubation period or time interval from exposure to the onset of clinical illness exceeds two decades, while the duration of illness is two years or less. The cause of these extraordinarily long incubation periods is unknown but may result in part from exposure to small doses of the kuru agent through an inefficient oral route.
Misonidazole neuropathy: A clinical, electrophysiological and histological study
Authors: Melgaard B. , Hansen H. Sand , Kamieniecka Z. , Paulson O. B. , Pedersen A. Gersel , Tang Xiaofu , Trojaborg W. 
Published: January 2005

AbstractWe studied eight patients with carcinoma of the pharynx and larynx (five cases) or lungs (three cases) who, during treatment with the radiosensitizing drug misonidazole, developed peripheral neuropathy dominated by severe sensory symptoms and signs mainly localized to the lower extremities. The symptoms partially subsided within months after cessation of therapy. Electrophysiological and histological findings indicated an axonal neuropathy with loss of large fibers and secondary demyelination. The neurotoxic property of misonidazole limits its therapeutic use.
The laboratory diagnosis of mild myasthenia gravis
Authors: Kelly John J. , Daube Jasper R. , Lennon Vanda A. , Howard Frank M. , Younge Brian R. 
Published: January 2005

AbstractWe report our experience with single‐fiber electromyogrphy (SF‐EMG), acetylcholine receptor antibody assays, and Lancaster Red‐Green tests of ocular motility with edrophonium chloride in the diagnosis of myasthenia gravis (MG). Over a period of almost 3 years, 43 patients in whom repetitive‐stimulation nerve conduction tests were nondiagnostic had SF‐EMG studies; 34 (79%) were positive. Examination of more proximal muscles yielded a higher likelihood of positive results. Anti‐acetylcholine receptor antibodies were detected in 25 (71%) of 35 patients whose sera were assayed, and Lancaster Red‐Green tests of ocular motility were positive in 13 (81%) of 16 patients studied. No single test was superior in diagnosing MG, but the three were complementary. If patients with mild MG are tested by all three methods, at least 95% should have laboratory confirmation of MG.
Sleep‐induced nonobstructive hypoventilation associated with diaphragmatic paralysis
Authors: Thorpy Michael J. , Schmidt‐Nowara Wolfgang W. , Pollak Charles P. , Weitzman Elliot D. 
Published: January 2005

AbstractA patient with adult‐onset spinal muscular atrophy causing mild proximal limb muscle weakness had sleep‐induced nonobstructive hypoventilation due to diaphragmatic paralysis. Nocturnal dyspnea and daytime somnolence were associated with frequent arousals from sleep due to arterial oxygen desaturation. The nocturnal use of a cuirass respirator improved the oxygen saturation, resulting in less disrupted sleep, relief of the somnolence, and improved daytime ventilation.
Involuntary movements in chorea‐acanthocytosis: A comparison with Huntington's chorea
Authors: Shibasaki Hiroshi , Sakai Tetsuo , Nishimura Hiroyuki , Sato Yuji , Goto Ikuo , Kuroiwa Yoshigoro 
Published: January 2005

AbstractThe involuntary movements in three patients with chorea‐acanthocytosis (CA) and three with Huntington's chorea (HC) were studied by polygraphic recordings and jerk‐locked averaging technique. Choreic movements in patients with CA were suppressed by mental calculation, voluntary muscle contraction, and instruction to stop them, whereas movements in HC were generally enhanced by these tasks. Backward averaging of the EEG time‐locked to the choreic movement of an extremity or the trunk demonstrated a cortical slow negativity before the movement, similar to the Bereitschaftspotential (“readiness potential”), in cases of CA but not those of HC. Involuntary movements in CA appear to differ from those in HC and might be generated at least in part by a mechanism similar to that of voluntary movement.