bluuebunny/crossref_metadata_2025_split huggingface dataset:
Progressive multifocal leukoencephalopathy and magnetic resonance imaging
Authors: Levy Jody D. , Cottingham Karen L. , Campbell Robert J. , Moore Gregory K. , Gyorkey Ference , Ashizawa Tetsuo , Goldman Arnold M. 
Published: January 2005

AbstractProgressive multifocal leukoencephalopathy developed in a homosexual man with underlying Hodgkin's disease. Computed tomography and magnetic resonance imaging of the brain demonstrated multiple lesions, more in gray than white matter. Brain biopsy established the diagnosis of progressive multifocal leukoencephalopathy. Magnetic resonance imaging was found useful for detecting brain lesions and for localizing an accessible lesion for biopsy.
Uptake of radiolabeled ions in normal and ischemia‐damaged brain
Authors: Dienel Gerald A. , Pulsinelli William A. 
Published: January 2005

AbstractThe regional concentrations of nine radiochemicals were measured in rat brain after induction of cerebral ischemia to identify tracers concentrated by brain undergoing selective neuronal necrosis. Transient (30 minute) forebrain ischemia was produced in the rat; 24 hours after cerebral recirculation the radiochemicals were injected intravenously and allowed to circulate for 5 hours. The brain concentrations of the radiochemicals in dissected regions were determined by scintillation counting. Forebrain ischemia of this nature will produce extensive injury to striatal neurons but will spare the great majority of neocortical neurons at 24 hours. The regional concentrations of these radiochemicals varied considerably in both control and ischemic animals. In postischemic animals, 4 radionuclides (63Ni, 99TcO4, 22Na, and [3H]tetracycline) were concentrated in the irreversibly damaged striatum in amounts ranging from 1.4 to 2.4 times greater than in normal tissue. The concentrations of 65Zn, 59Fe, 32PO4, and 147Pm in postischemic brain were similar to or less than those in normal brain. The concentration of [14C]EDTA was increased in injured and uninjured brain of postischemic rats. Autoradiographic analysis of the distribution patterns of some of these ions in normal animals showed that 99TcO4, 22Na, 65Zn, and 59Fe were distributed more uniformly throughout the brain than were 32PO4, 63Ni, and 147Pm. At 24 or 48 hours after ischemia, 63Ni, 99TcO4, and 22Na were preferentially concentrated in the damaged striatum and hippocampus, whereas 65Zn, 59Fe, 32PO4, and 147Pm did not accumulate in irreversibly injured tissue. Of the radiochemicals tested to date, Ni, TcO4, and tetracycline may be useful for diagnosing ischemic brain injury in humans, using positron emission tomography.
Successful treatment with tocainide of recessive generalized congenital myotonia
Authors: Streib Erich W. 
Published: January 2005

AbstractA patient with recessive generalized congenital myotonia and severe, disabling weakness underwent various forms of treatment while being monitored electrophysiologically. Phenytoin, verapamil, and acetazolamide were ineffective, but tocainide yielded good results. Improvement was dose‐dependent, and was limited by irritability and action tremor when the patient was taking 1,600 mg per day.
Narcolepsy following cerebral hypoxic ischemia
Authors: Rivera Victor M. , Meyer John S. , Hata Takashi , Ishikawa Yoshiki , Imai Akira 
Published: January 2005

AbstractA 51‐year‐old man with non‐HLA‐DR2 histocompatibility developed classic signs and symptoms of the narcoleptic tetrad soon after recovering from an episode of cardiopulmonary insufficiency, which occurred during induction of surgical anesthesia. Symptoms included excessive daytime sleepiness, hypnagogic hallucinations, sleep paralysis, and cataplexy. The diagnosis was confirmed by repeated polysomnographic examinations in the sleep laboratory. Cerebral hemodynamic changes during the onset of sleep showed remarkable increases of cerebral blood flow during the onset of rapid‐eye‐movement sleep similar to those reported previously in patients with narcolepsy. Magnetic resonance imaging showed focal regions of abnormal spin‐echo signals in the ventral pons.
Clinical correlation with anti‐peripheral‐nerve myelin antibodies in Guillain‐Barré syndrome
Authors: Koski Carol L. , Gratz Edward , Sutherland John , Mayer Richard F. 
Published: January 2005

AbstractAnti‐peripheral‐nerve myelin antibodies (anti‐PNM Ab) can be detected in the serum of all patients with acute‐phase Guillain‐Barré syndrome (GBS) thus far tested. Correlation of the titer of this antibody with the clinical course would help to establish a role for the humoral immune system in the pathophysiology of GBS. In this study, anti‐PNM Ab levels were measured in serial serum samples of 7 patients with GBS with an assay that detects antibodies bound to peripheral nerve myelin antigens by fixation of the first component of complement. Although the titers of anti‐PNM Ab detected in these patients varied between 0 and 256 U/ml, the antibody titer was always highest on admission (35 to 256 U/ml) and rapidly declined during a one‐to‐three‐week period. Disappearance of antibodies or very low levels of them correlated with cessation of progression and considerable clinical improvement as documented by increased pulmonary vital capacity and muscular strength. Low but measurable antibody titers (5 to 12 U/ml) were frequently found up to four months following the acute neurological deficit. The close temporal relationship between anti‐PNM Ab titer and the clinical course in GBS suggests that antibody most likely participates through complement activation in peripheral nerve demyelination.
Adult‐onset autosomal dominant limb‐girdle muscular dystrophy
Authors: Chutkow Jerry G. , Heffner Reid R. , Kramer Andrew A. , Edwards John A. 
Published: January 2005

AbstractWe describe a kindred with a rare autosomal dominant myopathy limited to the limb‐girdle muscles, beginning insidiously any time from the late second through the sixth decades and followed by slow progression. Pelvifemoral precedes scapulohumeral weakness, and proximal appendicular involvement antedates limited distal paresis. Expressivity varies and includes an asymptomatic myopathy (preclinical or subclinical) and a nonmanifesting carrier state that extends well into the eighth decade. A variety of nonspecific changes are present in muscle on light, enzyme histochemical, and electron microscopic examination; of these changes, “rimmed” or autophagic vacuoles are the most characteristic. We identified one very similar previously reported genealogy. The similarities between the two unrelated families clearly establish this dystrophic process as a distinct genetic entity; their differences suggest genetic heterogeneity.
Immunoreactive myelin basic protein in cerebrospinal fluid of patients with peripheral neuropathies
Authors: Cornblath David R. , Griffin John W. , Tennekoon Gihan I. 
Published: January 2005

AbstractThe presence of myelin basic protein (MBP)–like material in cerebrospinal fluid (CSF) usually reflects breakdown of central nervous system myelin. Immunoreactive MBP levels were measured in 70 CSF specimens from 66 patients with a variety of peripheral neuropathies. Immunoreactive MBP was present in CSF in 70% (16 of 23) of patients with biopsy‐proved chronic demyelinating polyneuropathies, whereas in neuropathies producing primarily axonal damage it was present in only 22% (5 of 23). In Guillain‐Barré syndrome, 45% (9 of 20) of patients had immunoreactive MBP in the CSF. We conclude that MBP‐like material is present in the CSF of most patients with longstanding demyelinating polyradiculoneuropathies and probably reflects detection of peripheral nervous system P1 protein.
Perforant pathway changes and the memory impairment of Alzheimer's disease
Authors: Hyman B. T. , Van Hoesen G. W. , Kromer L. J. , Damasio A. R. 
Published: January 2005

AbstractThe perforant pathway is a large neuronal projection that arises from layers II and III of the entorhinal cortex of the parahippocampal gyrus. It is the principal source of cortical input to the hippocampal formation. In 11 cases of Alzheimer's disease, we have found that neurofibrillary tangles develop in the cells of origin of the perforant pathway. In addition, the termination zone of the perforant pathway, in the outer two thirds of the molecular layer of the dentate gyrus, contains a distinct layer of neuritic plaques. None of the 8 control subjects had such changes. These profound alterations effectively disconnect the hippocampal formation from the association and limbic cortices. Because of the central role of the hippocampus and parahippocampal gyrus in learning, it is likely that pathological changes in the perforant pathway, by precluding normal hippocampal operation, account for some aspects of the memory impairment in Alzheimer's disease.
Subacute sensory neuronopathy secondary to dorsal root ganglionitis in primary Sjögren's syndrome
Authors: Malinow K. , Yannakakis G. D. , Glusman S. M. , Edlow D. W. , Griffin J. , Pestronk A. , Powell D. L. , Ramsey‐Goldman R. , Eidelman B. H. , Medsger T. A. , Alexander E. L. 
Published: January 2005

AbstractSensory neuropathies, particularly trigeminal neuropathy, have been recognized as neurological complications of Sjögren's syndrome, but the pathogenesis has not been established. We describe a woman with primary Sjögren's syndrome who developed a progressive debilitating subacute sensory neuronopathy. Results of electrophysiological studies were consistent with involvement of the trigeminal and dorsal root ganglia. A thoracic dorsal root ganglion biopsy showed lymphocytic infiltration and degeneration of ganglion cells. We believe that this is the first description of biopsy‐documented dorsal root ganglionitis in a subacute sensory neuronopathy associated with Sjögren's syndrome and that the finding suggests an immunopathogenic basis.
Primary central nervous system lymphoma in acquired immune deficiency syndrome: A clinical and pathological study
Authors: So Yuen T. , Beckstead Jay H. , Davis Richard L. 
Published: January 2005

AbstractTwenty cases of primary lymphoma of the centratl nervous system associated with acquired immune deficiency syndrome were seen over a period of four years and were studied clinically and pathologically. Biopsy established the diagnosis in 11 cases, and autopsy confirmed it in 9. Multicentricity was demonstrated in all cases for which there was adequate autopsy material. Both large‐cell immunoblastic and small noncleaved lymphomas were seen, and marker studies in 5 patients established that the lymphomas were of B‐cell origin. Neurological symptoms and signs, cerebrospinal fluid characteristics, and radiographic appearance were reviewed. The clinical and radiographic picture is nonspecific and histological confirmation is essential for diagnosis. Although the tumor appears to be radiosensitive, prognosis is extremely poor, with an average survival of less than two months.
Effect of testosterone on muscle protein synthesis in myotonic dystrophy
Authors: Griggs Robert C. , Halliday David , Kingston William , Moxley Richard T. 
Published: January 2005

AbstractMuscle wasting in myotonic dystrophy may result from decreased muscle anabolic processes rather than from increased catabolism. Male patients with myotonic dystrophy often have low levels of circulating androgens, and androgen administration has been shown to increase their muscle mass. We have studied the effect of testosterone enanthate administration (3 mg/kg weekly for 3 months) on muscle and whole body protein synthesis in 6 male patients with myotonic dystrophy. Muscle protein synthesis was estimated from the rate of isotope incorporation into muscle protein obtained by quadriceps muscle biopsy during a primed continuous infusion of L‐{1‐13C}leucine. Testosterone administration resulted in a significant increase in muscle protein synthesis in all patients. Whole body protein synthesis did not increase, indicating that protein synthesis in other tissues may have declined. Muscle ribonucleic acid content rose significantly in response to testosterone administration, suggesting that testosterone initiated its effect by hormone receptor interaction with muscle nuclei.
Japanese encephalitis: Idenufication of inflammatory cells in cerebrospinal fluid
Authors: Johnson Richard T. , Intralawan Pensri , Puapanwatton Siriraj 
Published: January 2005

AbstractFifteen patients with acute encephalitis were studied during the epidemic season of Japanese encephalitis (JE) in Northern Thailand; 13 of 15 proved to have the encephalitis. In serial cerebrospinal fluids and blood samples, mononuclear cells were identified using monoclonal antibodies. In cerebrospinal fluid from the patients with Japanese encephalitis, T cells predominated with a 4.2:1 ratio of T helper/inducer cells to T suppressor/cytotoxic cells; B cells and macrophages were often present but in small numbers compared to their presence in blood. Distribution of cell types did not vary between the first and fifth day of hospitalization, were similar in fatal and nonfatal cases, and were unaffected by administration of steroids.
Zinc in multiple sclerosis. II: Correlation with disease activity and elevated plasma membrane–bound zinc in erythrocytes from patients with multiple sclerosis
Authors: Ho Shih‐Yieh , Catalanotto Frank A. , Lisak Robert P. , Dore‐Duffy Paula 
Published: January 2005

AbstractPrevious studies have shown that zinc levels in erythrocytes are significantly elevated in patients with multiple sclerosis (MS). To examine the correlation between erythrocyte Zn levels and disease activity, we measured erythrocyte Zn levels longitudinally. Levels were dramatically decreased during a clinically documented exacerbation of MS. To determine the localization of increased Zn levels in MS erythrocytes, we employed standard techniques for the isolation of nonhemoglobin erythrocyte membrane ghosts. Patients with MS had three times more Zn in ghost material than did controls. Chloroform–methanol extraction in erythrocyte ghosts followed by determination of Zn levels indicated that most of the membrane‐bound Zn was associated with the lipid‐soluble fraction. Non‐lipid‐associated Zn and total membrane protein concentration were similar in MS and control samples. Results suggest that mechanisms which govern cellular availability, compartmentalization of Zn, or the binding of Zn to cell surface membranes may be altered in patients with MS, and that these mechanisms vary with disease activity.
Changes in epileptic spike configuration associated with attainment of seizure control
Authors: Frost James D. , Kellaway Peter , Hrachovy Richard A. , Glaze Daniel G. , Mizrahi Eli M. 
Published: January 2005

AbstractThis study provides preliminary evidence that computer‐derived parameters of interictal spike waveforms can be used to assess seizures hazard in a quantitative manner. Thirteen children with spike foci and simple or complex partial seizures were studied before and during treatment with carbamazepine or phenobarbital; spike parameters were determined from 5‐ to 30‐minute electroencephalographic samples obtained during non–rapid eye movement sleep. Measurements included spike amplitude, duration, and sharpness, and a mathematical combination of these three parameters (the composite spike parameter). Achievement of seizure control was associated with a decrease in spike amplitude, duration, and composite spike parameter, and an increase in normalized sharpness, compared to baseline values. These result suggest that this method may provide a prospective means for estimating drug efficacy that can be applied shortly after attainment of steady‐state serum drug levels.
Inflammatory demyelinating peripheral neuropathies associated with human T‐cell lymphotropic virus type III infection
Authors: Cornblath David R. , McArthur Justin C. , Kennedy Peter G. E. , Witte Arnold S. , Griffin John W. 
Published: January 2005

AbstractNine patients with inflammatory demyelinating polyneuropathies (IDP) were found to have human T‐cell lymphotropic virus type III (HTLV‐III) infection. The 8 men, 6 of whom were homosexual, and 1 woman, a former intravenous drug user, presented with progressive weakness. Two had lymphadenopathy but all were otherwise asymptomatic. Six had chronic IDP and 3 had Guillain‐Barré syndrome. In addition to an elevated cerebrospinal fluid (CSF) protein level (mean, 193 mg/dl), most patients had cerebrospinal fluid pleocytosis (mean, 23 cells/mm3), a distinctive feature. All had reduced T4:T8 T‐cell ratios. Results of nerve conduction studies were characteristic of demyelination. Nerve biopsies revealed intense inflammatory cell infiltrates and macrophage‐mediated demyelination. The patients recovered either spontaneously or following treatment with corticosteroids or plasmapheresis. During a mean interval of 20 months after presentation, only 1 patient had developed acquired immune deficiency syndrome. Patients with HTLV‐III infection have disordered immune function, and the mechanism of the development of the IDP is likely to be immunopathogenic. As a result of our experience, we suggest that all patients with IDP be tested for evidence of HTLV‐III infection. We also found, although in uncontrolled trials, that treatment with either prednisone or plasmapheresis was followed by clinical improvement; since plasmapheresis is not likely to further depress cell‐mediated immunity, we suggest that it be the initial therapy.
Latencies of visually guided saccades in unilateral hemispheric cerebral lesions
Authors: Pierrot‐Deseilligny Charles , Rivaud Sophie , Penet Christiane , Rigolet Marie‐Hélène 
Published: January 2005

AbstractLatencies of lateral visually guided saccades were studied in 60 patients without hemianopia who had unilateral focal lesions clearly visible on computed tomographic (CT) scan that were variously located in both cerebral hemispheres. Significantly asymmetrical latencies were found in 29 patients whose lesions had damaged the deep and posterior frontal region near the corpus callosum and/or, just inferior to this region, the anterior part of the internal capsule. In the 31 other patients, including those with lesions of the frontal eye fields (FEF), latencies were not significantly asymmetrical and the lesions spared the entire region just described. These topographical features suggest that the asymmetry of latencies is due to damage in a certain portion of the efferent pathways descending from the FEF. A significant increase in bilateral latency was observed in most patients whose lesions had damaged the posterior part of the parietal cortex and/or the underlying white matter. The parietal lobe could therefore exert an excitatory bilateral action on the triggering of visually guided saccades, probably mediated via the superior colliculus. A significant decrease in the bilateral or ipsilateral latency was often found in patients whose lesions had damaged the FEF or the underlying white matter. The frontal lobe could therefore exert a predominantly inhibitory bilateral action on this triggering, probably also mediated via the superior colliculus. However, an increase in contralateral latency in some patients with subcortical frontal lesions indicates that FEF also probably have an excitatory action. This action could be transmitted directly (or indirectly via the superior colliculus) to the reticular premotor structures by tracts decussating partly through the corpus callosum.
Cortical blindness: Etiology, diagnosis, and prognosis
Authors: Aldrich Michael S. , Alessi Anthony G. , Beck Roy W. , Gilman Sid 
Published: January 2005

AbstractWe examined 15 patients with cortical blindness, reviewed the records of 10 others, and compared these 25 patients to those in previous studies of cortical blindness. Although cerebrovascular disease was the most common cause in our series, surgery, particularly cardiac surgery, and cerebral angiography were also major causes. Only 3 patients denied their blindness, although 4 others were unaware of their visual loss. Electroencephalograms (EEGs) were performed during the period of blindness in 20 patients and all recordings were abnormal, with absent alpha rhythm. Visual evoked potentials recorded during blindness were abnormal in 15 of 19 patients, but did not correlate with the severity of visual loss or with outcome. Bioccipital lucencies were found in computed tomographic (CT) scans of 14 patients; none of the 14 regained good vision. Recovery of vision was poor in all 8 patients who had a spontaneous stroke, but fair or good in 11 of the other 17 patients. Prognosis was best in patients under the age of 40 years, in those without a history of hypertension or diabetes mellitus, and in those without associated cognitive, language, or memory impairments. We conclude that (1) the prognosis in cortical blindness is poor when caused by stroke; (2) EEGs are more useful than visual evoked potentials for diagnosis; and (3) bioccipital abnormalities shown on CT scan are associated with a poor prognosis.
Brain extraction of a calcium channel blocker
Authors: Grotta James C. , Pettigrew L. Creed , Lockwood Alan H. , Reich Catherine 
Published: January 2005

AbstractDihydropyridine calcium channel blockers may be effective treatment for acute cerebral ischemia, but the uptake of these drugs into the brain is unknown. A 0.2‐ml bolus of [14C]nicardipine hydrochloride and [3H] water was injected into the common carotid arteries of 7 normal and 7 ischemic rats. The corrected first‐pass extraction of nicardipine, compared to water, was calculated to be 30.7% into the hemispheres and 42.3% into the hippocampi. The uptake was greater into the ischemic hemispheres (p ≤ 0.001). These data suggest that dihydropyridines are available to binding sites and calcium channels in neurons.
Pontine ataxic hemiparesis studied by a high‐resolution magnetic resonance imaging system
Authors: Nabatame Hidehiko , Fukuyama Hidenao , Akiguchi Ichiro , Kameyama Masakuni , Nishimura Kazumasa , Torizuka Kanji 
Published: January 2005

AbstractUsing a high‐resolution magnetic resonance imaging system, we studied 3 patients who had exhibited pontine ataxic hemiparesis. In each patient, the ischemic lesions responsible for the contralateral ataxia were located in the dorsomedial basis pontis. The isointensity bands in the ischemic area on T2‐weighted images showed the spared transverse fibers originating from the contralateral pontine nuclei, and this may explain the cause of the unilateral ataxia.
Linkage of adrenoleukodystrophy to a polymorphic DNA probe
Authors: Aubourg Patrick R. , Sack George H. , Meyers Deborah A. , Lease John J. , Moser Hugo W. 
Published: January 2005

AbstractLinkage studies between X‐linked adrenoleukodystrophy and a cloned deoxyribonucleic acid fragment (St14), which detects polymorphisms in the distal end of the long arm of the X chromosome (Xq27–28), have shown no recombination in six families. The lod score for these data (and another kindred reported earlier) [2] is 13.766 at recombination fraction (θ) = 0.0. These data permit assignment of adrenoleukodystrophy carrier status in family members at risk, supplementing the chemical measurement of very‐long‐chain fatty acids.