bluuebunny/crossref_metadata_2025_split huggingface dataset:
A case control study of Creutzfeldt‐Jakob disease: Association with physical injuries
Authors: Kondo Kiyotaro , Kuroiwa Yoshigoro 
Published: January 2005

AbstractSixty cases of Creutzfeldt‐Jakob disease reported from 902 neurological clinics throughout Japan between 1975 and 1977 were examined in a retrospective case control study. Histories were obtained from patients, sex‐matched neighbors, and spouses. No association was observed in either sex with five socioeconomic variables; exposures to eight species of animals; ingestion of raw meat or quadruped brains; eighteen diseases, allergies, or immunizations; tooth extraction; blood transfusion; or lumbar puncture. Surgical operations within five years before disease onset were reported for 25.9% and 7.8% of male patients and pooled controls and for 24.2% and 9.6% of female patients and controls. Mechanical injuries were observed for the same period in 33.3% and 9.8% of male patients and controls and in 18.2% and 13.5% of female patients and controls. The operations and mechanical injuries varied in all groups as to body parts affected and nature of the lesions. Physical injuries including operations may be a predisposing factor to the development of Creutzfeldt‐Jakob disease.
Defective mitogenic responses in myasthenia gravis and multiple sclerosis
Authors: Dropcho Edward J. , Richman David P. , Antel Jack P. , Arnason Barry, G. W. 
Published: January 2005

AbstractUsing in vitro lymphocyte proliferation induced by the phytomitogen concanavalin A (ConA), we investigated immune function and regulation in patients with myasthenia gravis (MG) and multiple sclerosis (MS). Unfractionated peripheral blood mononuclear cells of normal individuals responded to a wide range of ConA concentrations; the T cell fraction responded to a lesser degree and only to high concentrations. These findings suggest the presence of two receptors for ConA, one of high affinity present on a non‐T cell accessory cell and the other of low affinity present on T cells. Contrasting defects in the level of response of unfractionated lymphocytes and T cells were found in patients with MG and MS. The peak response of T cells in the MG patients was 22.6 ± 9.6 ± 103 cpm (mean ± SEM) compared with 54.6 ± 6.5 × 10(3) for controls (p < 0.05), while the response of unfractionated lymphocytes did not differ from that in controls. For MS patients, the unfractionated lymphocyte response was diminished: 56.3 ± 2.8 × 103 cpm versus 70.5 ± 4.5 × 103 for controls (p < 0.05), while the T cell response was normal. These results indicate a defect in the direct T cell response in MG; in contrast, in MS the response requiring T cell‐accessory cell interaction is abnormal.
Convulsive syncope in blood donors
Authors: Lin James T.‐Y. , Ziegler Dewey K. , Lai Chi‐Wan , Bayer William 
Published: January 2005

AbstractSyncope with and without convulsion was studied in unselected blood donors in a community blood center. Convulsive syncope occurred in 0.03% of all blood donors and was more commonly observed when nursing personnel were alerted to its possible occurrence. It was more common in men. Although tonic extensor spasm was the most common convulsive movement, other complex convulsive phenomena occurred, some simulating epileptic seizure. No statistical difference in changes of pulse or blood pressure was found between subjects with convulsive versus nonconvulsive syncope. Similarly, no difference was found between subjects with tonic spasm and those with other convulsive phenomena, nor between those with “early” and those with “delayed” reactions. Marked individual variation may exist in the susceptibility of the central nervous system to ischemia. Some individuals appear to be predisposed to development of seizures in situations of global cerebral ischemia such as occur in hypotension and bradycardia.
Solitary intracranial plasmacytoma: Two patients with extended follow‐up
Authors: Krumholz Allan , Weiss Howard D. , Jiji Violet H. , Bakal Daniel , Kirsh Milton B. 
Published: January 2005

AbstractSolitary neoplastic proliferation of plasma cells (plasmacytoma) rarely occurs in the central nervous system. The longest follow‐up in nine previously reported patients was 5 years. We have followed two patients with solitary intracranial plasmacytoma for 8 and 13 years, respectively. At the time of biopsy diagnosis, neither showed involvement of the adjacent calvarium or evidence of a systemic plasma cell dyscrasia. The first patient underwent partial excision of a mass in the right occipital lobe that showed no gross or microscopic involvement of the dura. The second patient had partial resection of a mass arising from dura and invading the left temporal lobe. Postoperative irradiation was given to both patients. No clinical or radiological sign of recurrent brain tumor has developed in either patient during the extended follow‐up period. Neither patient has had laboratory or clinical evidence of a systemic plasma cell dyscrasia. Solitary intracranial plasmacytoma may be a “curable” brain tumor.
Neuraminidase deficiency and accumulation of sialic acid in lymphocytes in adult type sialidosis with partial β‐galactosidase deficiency
Authors: Tsuji Shoji , Yamada Takamichi , Tsutsumi Atsuko , Miyatake Tadashi 
Published: January 2005

AbstractNeuraminidase deficiency has been demonstrated in cultured skin fibroblasts of patients who have adult type sialidosis with partial β‐galactosidase deficiency. A substantial amount of residual enzyme activity has been observed in leukocytes, however. To explain this discrepancy, the nature and distribution of the enzyme were studied. Neuraminidase activity was higher in lymphocytes than in granulocytes of normal controls. In patients' lymphocytes, neuraminidase activity was profoundly decreased and total sialic acid contents were increased 2.3‐fold. Two neuraminidases, one sonication‐labile and the other sonication‐stable, were found in lymphocytes; the former was predominant in cultured skin fibroblasts. The defective enzyme in this disorder was found to be the sonication‐labile neuraminidase in both cultured skin fibroblasts and lymphocytes.
Necrotizing arteritis and spinal subarachnoid hemorrhage in Sjögren syndrome
Authors: Alexander Elaine L. , Craft Carolyn , Dorsch Carole , Moser Robert L. , Provost Thomas T. , Alexander Garrett E. 
Published: January 2005

AbstractA 37‐year‐old woman with primary Sjögren syndrome developed mixed cryoglobulinemia and systemic vasculitis. Subarachnoid hemorrhage occurred as a result of necrotizing anterior spinal arteritis. Although rarely seen in mixed cryoglobulinemia, central nervous system complications have recently been documented in Sjögren syndrome. The patient's serum contained antibodies to the Ro(SSA) cytoplasmic antigen, and these antibodies were concentrated in the cryoglobulin fraction. Anti‐Ro(SSA) antibodies are associated with the occurrence of vasculitis in patients with Sjögren syndrome, which suggests that the spinal arteritis and subarachnoid hemorrhage in this patient may have been directly related to the underlying connective tissue disorder.
Hind limb somatosensory evoked potentials in the monkey: The effects of distal axonopathy
Authors: Arezzo J. C. , Schaumburg H. H. , Vaughan H. G. , Spencer P. S. , Barna J. 
Published: January 2005

AbstractComputer‐averaged short‐latency somatosensory evoked potentials (SLSEP) to unilateral stimulation of the peroneal nerve were recorded from surface electrodes overlying the peripheral nerve, cauda equina, spinal cord, brainstem, and contralateral sensorimotor region. Seven monkeys were studied under normal conditions and at various stages of distal axonopathy induced by systematic acrylamide intoxication. With the use of a noncephalic reference, a series of five small‐amplitude positive components were identified that precede the initial cortical activity. On the basis of timing and topography of the components, the source of the first one, SLSEP1, was localized to the lumbar dorsal root fibers and that of the second, SLSEP2, to the ascending spinal tracts, principally the gracile fasciculus. Bipolar recordings of the SLSEP2 overlying the rostral extreme of the cervical spinal cord provided a sensitive measure of the onset of distal axonopathy. Changes in the timing of this component antedated both abnormalities of spinal or peripheral nerve conduction and behavioral signs of intoxication. The initial alteration was specific to stimulation of the hind limb and was associated with early morphological change limited to the terminal and preterminal portions of the long axons in the gracile fasciculus.
Burn encephalopathy in children
Authors: Mohnot Dhanpat , Snead O. Carter , Benton John W. 
Published: January 2005

AbstractAmong 287 children with burns treated over a recent two‐year period, 13 (5%) showed evidence of encephalopathy. The major clinical symptoms were an altered sensorium and seizures. The majority of symptoms began later than 48 hours after the burn and were accompanied by multiple metabolic aberrations including hypocalcemia. Three children had a relapsing course, and 1 had temporarily enlarged cerebral ventricles. Eleven children improved to normal. In the majority of instances, burn encephalopathy probably reflects central nervous system dysfunction resulting from complex metabolic, hematological, and hemodynamic abnormalities rather than from a single metabolic abnormality.
Stability of monoamine metabolites in human cerebrospinal fluid
Authors: Langlais Philip J. , Bird Edward D. , McEntee William J. 
Published: January 2005

AbstractConcentrations of 3‐methoxy‐4‐hydroxyphenylglycol (MHPG), homovanillic acid (HVA), and 5‐hydroxyindoleacetic acid (5‐HIAA) were measured in human lumbar crerospinal fluid following storage at room temperature and 4°C for intervals up to 72 hours. These monoamine metabolites were quantitated using a high‐performance liquid chromatographic—electrochemical detector technique. No significant difference could be detected in any of the three metabolites stored at room temperature for up to 48 hours. Additionof ascorbic acid to CSF had no measurable effect on the levels of HVA and 5‐HIAA. Repeated thawing and freezing produced no detectable change in metabolite levels.The results indicate that these monoamine metabolites are stable at room temperature for up to 48 hours after lumbar puncture and that the addition of an antioxdant, such as ascorbic acid, is not necessary. The data also demonstrate that immediate refrigeration or freezing of samples is not required for maintenance of metabolite levels.
Neuroimmunomodulation: Neural anatomical basis for impairment and facilitation
Authors: Brooks William H. , Cross Richard J. , Roszman Thomas L. , Markesbery William R. 
Published: January 2005

AbstractRats with bilateral electrolytic lesions of specific limbic nuclei show alterations in lymphoid cell number and in lymphocyte activation induced in vitro by concanavalin A (Con A). The number of splenocytes decreases after lesioning in the anterior hypothalamus (p < 0.001), ventromedial hypothalamus (p < 0.002), and mamillary bodies (p < 0.001). The number of thymocytes decreases after lesioning of the anterior hypothalamus (p < 0.001) and increases after hippocampal lesioning (p < 0.001). Spleen cell responsivensess to Con A decreases subsequent to lesioning of the anterior hypothalamus, whereas reactivity was enhanced after lesion placement in the mamillary bodies (p < 0.002), hippocampus (p < 0.001), and amygdaloid complex (p < 0.001). Thymocyte mitogen reactivity is increased by lesions of the hipocampus (p < 0.001), and amygdaloid complex (p < 0.001). These effects manifest themselves maximally 4 days after lesioning, with a return to normal by day 14. These preliminary data indicate that quantitative and qualitative lymphocyte functions are altered by ablation of selected brain nuclei, thereby suggesting the presence of neural modulation of immune function.
Pergolide in late‐stage Parkinson disease
Authors: Lang A. E. , Quinn N. , Brincat S. , Marsden C. D. , Parkes J. D. 
Published: January 2005

AbstractTwenty‐six patients with late‐stage Parkinson disease were given 0.4 to 15 mg of pergolide mesylate daily in addition to, or as replacement for, levodopa or bromocriptine therapy. Despite treatment with individually determined optimum doses of levodopa, bromocriptine, and anticholinergics, they had shown response failure or fluctuating response. Forty percent (11 patients) were unable to tolerate pergolide. Nausea and vomiting, somnolence, and psychiatric disturbances were the most frequent side effects. Eleven of the remaining patients improved on pergolide, 2 were unchanged, and 2 were slightly worse. Among the patients who benefited, pergolide improved dose‐related response fluctuations more than non‐dose‐related fluctuations, with a reduction in number and duration of “off” periods and improvement in quality of sleep and early morning akinesia but little change in freezing episodes. Despite treatment failure in many cases, pergolide is at present the best available drug for specific latestage management problems.
A defect in catecholamine metabolism in kinky‐hair disease
Authors: Grover Warren D. , Henkin Robert I. , Schwartz Michael , Brodsky Nancy , Hobdell Elizabeth , Stolk Jon M. 
Published: January 2005

AbstractThe measurement of blood and cerebrospinal fluid levels of copper, dopamine β‐hydroxylase, dopamine, norepinephrine, and epinephrine in a patient with kinky‐hair disease indicated a block in the conversion of dopamine to norepinephrine. The decreased activity of dopamine β‐hydroxylase was not corrected by increasing serum copper levels. A second patient with kinky‐hair disease had similar clinical and biochemical findings on initial examination. With oral supplemental copper therapy, the second patient attained levels within the control range for blood and cerebrospinal fluid copper, dopamine β‐hydroxylase, dopamine, and norepinephrine, demonstrating a state of genetic heterogeneity for this syndrome.
Ross river virus‐induced demyelination: I. Pathogenesis and histopathology
Authors: Seay Alan R. , Wolinsky Jerry S. 
Published: January 2005

AbstractRoss River virus (strain T48) infection in mice causes and encephalomyelitis characterized by focal, primary demyelination in the cerebellum, brainstem, and spinal cord. Maximal serum and brain content of virus occurs on days 2 and 4, respectively. Virus is not detectable in serum after day 3 or in brain after day 9. Histopathological lesions are present by day 2 and consist of perivascular macrophage and polymorphonulcear leukocyte infiltration, focal necrosis in the internal granule cell layer, and myelin disruption. Mononuclear cell infiltrates are present by day 5. Foci of demyelination in the presence of preserved axons become more widespred by day 8, and early partial remyeliantion occurs by day 13. Immunosuppression reduces the mononuclear dell infiltration but does not alter the demyelination. Althogh the mechanism of Ross River virus‐induced demyelination is not known, these findings suggest that it is not imnune mediated.
Pseudoepileptic seizures: Methods and video analysis to aid diagnosis
Authors: Luther J. Scott , McNamara James O. , Carwile Sandra , Miller Patrica , Hope Victor 
Published: January 2005

AbstractThe purposes of this study were to obtain a detailed description of the clinical features of pseudoepileptic (PE) seizures, to try to reproduce these events in the laboratory during a single recording, and to establish a framework useful to the clinician for evaluating patients whose behavior suggests physiological seizures, but about which doubt remains. We analyzed 37 episodes of PE seizures recorded in 30 patients during a single three‐hour video/EEG recording. The PE seizures occurred spontaneously or were induced by sequential activation procedures. The historical information together with behavioral observations show that the PE seizures mimicked primary generalized seizures in 15 episodes, elementary partial seizures with secondary generalization in 21 episodes, and complex partial seizures in a single episode. Our data underscore the usefulness of the video/EEG recording method and suggest activation techniques which, combined with detailed historical information, aid in establishing the diagnosis of PE seizures.
Progression of carotid disease after endarterectomy: A Doppler ultrasound study
Authors: Norrving Bo , Nilsson Bengt , Olsson Jan‐Edvin 
Published: January 2005

AbstractThe clinical prognosis and evolution of carotid lesions after unilateral endarterectomy were determined in 64 patients examined 1 to 13 years after surgery (mean observation period, 6 years). Surgery mainly was confined to symptomatic patients with a stenosis only on the appropriate side. Average annual stroke rate was 1.6% on the operated and 0.8% on the nonoperated side. Direct Doppler examination at follow‐up revealed a recurrent stenosis (≥50%) or occlusion in 36% of the operated carotid arteries, not significantly different from the proportion of progressive carotid lesions on the nonoperated side (27%). In total, 43.8% of the patients had developed new lesions in one or both carotid arteries, as compared to previous angiographic findings. About 30% of the progressive lesions were associated with symptoms of transient ischemic attacks or stroke, as opposed to 5.5% of vessels without progression of lesions (p < 0.001). The incidence of recurrent stenosis on the operated side is considerably higher than that previously reported for symptomatic recurrent stenosis, but may represent the natural course of carotid disease in this population, in which carotid surgery thus should not be regarded as definitive treatment.
Preoperative CT diagnosis of mesial temporal sclerosis for surgical treatment of epilepsy
Authors: Wyler Allen R. , Bolender Nicole‐Fr. 
Published: January 2005

AbstractA computerized tomographic (CT) scanning technique has been devised by which the anatomy of the mesial temporal lobe can be examined in epileptics who are being considered for temporal lobectomy. Among 25 patients examined with this technique, 17 have had the anatomy of their mesial temporal lobe verified at craniotomy, showing close correlation between surgical findings and the high‐resolution cerebrospinal fluid–enhanced CT scans. Atrophy was present in 4 patients, whereas chronic herniation of the mesial temporal structures over the free edge of the tentorium was present in 12; in 1 patient the structures were normal. The correlation of anatomical lesion with an electroencephalographically (EEG) defined epileptogenic focus will improve the outcome of surgery for epilepsy. This CT technique aids in identifying the epileptogenic temporal lobe for patients in whom the focus is ambiguous by EEG criteria alone.
Comparison between fascicular and whole sural nerve biopsy
Authors: Pollock Martin , Nukada Hitoshi , Taylor Peter , Donaldson Ivan , Carroll Grant 
Published: January 2005

AbstractSural nerve regeneration and sensation were evaluated in 16 subjects five or more years after fascicular or whole nerve biopsy. Conduction studies demonstrated successful nerve regeneration in all subjects who had whole nerve biopsy. Postbiopsy interrogation revealed no long‐term pain or paresthesias but a high incidence of tactile‐induced dysesthesias. No significant difference was seen when areas of sural sensory loss were compared in fascicular and whole nerve biopsy groups. We conclude that whole nerve biopsy should be recommended in preference to fascicular biopsy since it is simpler, has greater diagnostic potential, and allows for a more complete morphological evaluation.
Decreased pyruvate dehydrogenase complex activity in Huntington and Alzheimer brain
Authors: Sorbi Sandro , Bird Edward D. , Blass John P. 
Published: January 2005

AbstractThe activity of the pyruvate dehydrogenase complex (PDHC) was reduced in affected areas of brain from patients with Huntington disease (caudate, putamen) and Alzheimer disease (frontal cortex) where choline acetyltransferase (CAT) activity was low. PDHC was also deficient in an area (Huntington hippocampus) where CAT was not significantly reduced. The activity of fumarase, an inner mitochondrial marker, was normal in all areas examined. The activities of PDHC and CAT correlated well in caudate, putamen, and amygdala but not in hippocampus or frontal cortex. Both total activity and activation of PDHC were below normal in fibroblasts from 4 patients with C‐21 trisomy Down syndrome, who are at very high risk to develop Alzheimer disease. However, no abnormality of PDHC was detected in Huntington or Alzheimer fibroblasts. Deficiency of PDHC may play a role in the pathophysiology of Huntington and Alzheimer diseases, although it does not appear to be a primary defect. Loss of tissue oxidative capacity may relate to the reduction in cerebral metabolic rate and blood flow which are characteristic of many dementing illnesses.
Prenatal symmetrical thalamic degeneration with flexion spasticity at birth
Authors: Parisi J. E. , Collins G. H. , Kim R. C. , Crosley C. J. 
Published: January 2005

AbstractThe unusual occurrence of spasticity at birth with symmetrical thalamic damage was found in a male infant delivered at 36 weeks' gestation following an episode of traumatically induced premature labor at 32 weeks. The infant was found to be spastic in flexion with increased stretch reflexes, depressed primitive reflexes, and moderate flexion contractures. Computerized tomographic scans showed bilateral nonenhancing thalamic densities. Neuropathologically, the lateral thalamic nuclei and the red nucleus showed neuron loss, astrocytosis, and, as confirmed by electron microprobe analysis, calcified neurons. The striatum was uninvolved. These findings closely resemble those reported as “symmetrical thalamic degeneration in infancy” and are strongly reminiscent of the pattern of thalamic involvement frequently seen in status marmoratus. It would appear that there is a period during perinatal life in which the lateral thalamus can be rendered vulnerable to hypoxic‐ischemic injury, and that the thirty‐second week of gestation must be included within this period.
Pupil‐sparing oculomotor palsies with internal carotid—posterior communicating artery aneurysms
Authors: Kissel John T. , Burde Ronald M. , Klingele Terence G. , Zeiger H. Evan 
Published: January 2005

AbstractThe charts of 84 patients admitted to the Neurosurgery Service of the Washington University Medical Center between January, 1960, and July, 1981, with aneurysms at or near the junction of the internal carotid and posterior communicating arteries (ICA‐PoCA) were reviewed. Special emphasis was placed on the pupillary size and reactivity of 51 patients with oculomotor nerve involvement. Seven initially had normal pupils, representing 8% of the total group and 14% of those presenting with oculomotor palsies. In 4 of these patients pupillary involvement developed within 5 days, and in I pupillary involvement developed in 4 months. Pupillary sparing appears to be more common than previously appreciated in patients with ICA‐PoCA aneurysms and oculomotor nerve involvement. Patients with acute somatic oculomotor paresis should be observed closely for at least one week for the development of pupillary involvement. Arteriography may be indicated more frequently than previously recommended.