bluuebunny/crossref_metadata_2025_split huggingface dataset:
Interleukin‐2 levels in serum and cerebrospinal fluid of multiple sclerosis patients
Authors: Gallo P. , Piccinno M. , Pagni S. , Tavolato B. 
Published: January 2005

AbstractA sensitive enzyme‐linked immunosorbent assay method was employed to measure interleukin‐2 (IL‐2) levels in cerebrospinal fluid (CSF) and sera from 30 patients with multiple sclerosis (MS) and 8 patients with other neurological diseases. Detectable levels of IL‐2 were found in 6 sera and 9 CSF samples of 21 patients with acute relapse of MS. However, only 3 patients showed measurable IL‐2 both in CSF and in serum. IL‐2 was not detected in specimens from 9 patients with chronic progressive MS, whereas high levels were found in 2 CSF samples from patients with aSeptembertic meningitis. Our data suggest that systemic activation of a T‐cell population is present in some MS patients; moreover, an active immune mechanism involving IL‐2 production takes place within the central nervous system.
Gray‐matter degeneration in presenile alzheimer's disease
Authors: Prohovnik Isak , Smith Gwenn , Sackeim Harold A. , Mayeux Richard , Stern Yaakov 
Published: January 2005

AbstractPrevious comparisons between presenile Alzheimer's disease (AD) and senile dementia of the Alzheimer type (SDAT) did not control for disease severity and duration. In the current study, 18 patients with each diagnosis were matched for disease duration, cognitive dysfunction, and behavioral symptoms (using the modified Mini‐Mental Status [mMMS] examination and the Blessed Dementia Rating Scale [BDRS]). Regional cerebral blood flow (rCBF) was quantified by the 133xenon inhalation technique, and several indices of tissue perfusion were examined. The two variables of primary interest were relative gray‐matter weight and a gray‐matter perfusion index, the initial slope index. Presenile onset was associated with loss of gray‐matter relative weight (35% in presenile patients versus 39% in senile patients and healthy control subjects, p = 0.006), with neither perfusion nor disease severity differences between the two dementia samples. This loss of gray matter was significantly related to both severity and duration of disease in the patients with presenile AD, but not in patients with SDAT. These findings lend support to previous suggestions of greater degenerative process in presenile AD and confirm the need to examine and control age of onset in future investigations of AD. Further, correlation analysis suggests greater proportion of common variance among clinical and physiological indices in presenile AD.
The open opercular sign: Diagnosis and significance
Authors: Tatum William O. , Coker Steven B. , Ghobrial Mona , Abd‐Allah Shamel 
Published: January 2005

AbstractFour children with varying clinical manifestations, but with the unifying feature of severe developmental delay, had bilateral enlargement of the sylvian fissure confirmed by magnetic resonance imaging (MRI). Subsequently, we examined 125 consecutive MRI scans of the heads of pediatric patients, looking for this insular exposure, and did not find it. Pathological correlation in 1 child revealed arhinencephaly and abnormal gyral formation; another is known to have migrational abnormalities. We suggest that the open operculum is a sign of arrested development and is associated with other anomalies and a poor prognosis.
Activated suppressor cell function in severely disabled patients with multiple sclerosis
Authors: Antel J. P. , Freedman M. S. , Brodovsky S. , Francis G. S. , Duquette P. 
Published: January 2005

AbstractDefective suppressor cell function has previously been demonstrated in patients with multiple sclerosis (MS) with progressive disease and moderate degrees of disability. In the present study activated suppressor cell function was assessed in patients with documented progressive disease who, at the time of study, had experienced severe disability (Kurtzke score ≥ 6.5) for at least 2 years. We found that mean suppressor levels were significantly increased in this patient group compared with the suppressor levels in the MS patient group with progressive disease but only moderate disability (Kurtzke score of ≤ 6.0 within 2 years of study) (59 ± 8% vs 19 ± 7%, respectively, p < 0.01). The mean value in the latter group was significantly reduced compared with the mean value for normal control subjects (47 ± 4%, p < 0.01), a finding consistent with previous reports. The results of this study indicate that suppressor cell function, as measured by our assay system, need not be defective in MS patients who have become severely disabled from the progressive form of the disease. Whether the patients who are now severely disabled from progressive MS passed through a phase of disease associated with the same suppressor defects as found in the progressive patients currently with moderate disability will remain speculative until long‐term longitudinal studies are performed.
Impaired human leukocyte antigen–restricted measles virus–specific cytotoxic T‐cell response in subacute sclerosing panencephalitis
Authors: Dhib‐Jalbut Suhayl , Jacobson Steven , McFarlin Dale E. , McFarland Henry F. 
Published: January 2005

AbstractThe capacity of peripheral blood lymphocytes to proliferate in response to measles virus and to generate measles virus–specific cytotoxic T lymphocytes (CTL) was examined in 4 patients with subacute sclerosing panencephalitis (SSPE). The lymphoproliferative response to measles virus was obtainable in the 4 SSPE patients. In contrast, the CTL response to measles virus was reduced in 3 of the 4 SSPE patients. This defect appeared to be in the generation of the measles virus–specific CTLs, since measles virus–infected target cells from the patients could be lysed by human leukocyte antigen–matched peripheral blood lymphocytes from healthy individuals. The SSPE patients with reduced measles virus CTL response had a normal ability to generate mumps virus, influenza virus, or alloantigen–specific CTLs. The lysis of measles virus–infected targets that was observed with these SSPE patients could be reduced by depleting the effectors of natural killer cells or by performing cold target blocking with K562 cells, indicating that the lysis of the measles virus–infected targets was probably mediated by natural killer cells. These results demonstrate a reduction in the cell‐mediated immune response to measles virus as measured by the generation of measles virus–specific CTLs in 3 of the 4 SSPE patients studied. This defect could relate to the persistence of measles virus in these patients.
Viral infectious complementary‐DNA studies may identify nonviral genes critical to central nervous system disease
Authors: Ohara Yoshiro , Roos Raymond P. 
Published: January 2005

AbstractA major interest of modern science and medicine is the delineation of genes that cause disease. In the case of cancer, the study of viral oncogenic genes led to the recognition of similar human genes that play an important role in this disease. In an analogous fashion, the identification of viral genes important in central nervous system disease may lead to the recognition of related cellular genes that are important in nonviral central nervous system disease. New molecular techniques now provide tools for identification of pathogenic viral genes and elucidation of mechanisms of disease production. Positive‐strand RNA viruses such as picornaviruses provide an especially attractive model system for studies of central nervous system disease–producing genes. A limitation in molecular studies of these viruses has resulted from an inability to use restriction enzymes, since these enzymes are active against DNA and not RNA. This limitation has recently been overcome with the preparation of infectious picornavirus complementary‐DNA. This review highlights the importance of infectious complementary‐DNA in pathogenesis studies and provides a glimpse of the impact of such studies on neurology.
Peripheral nerve pathological findings in familial amyloid polyneuropathy: A correlative study of proximal sciatic nerve and sural nerve lesions
Authors: Hanyu Norinao , Ikeda Shu‐Ichi , Nakadai Akira , Yanagisawa Nobuo , Powell Henry C. 
Published: January 2005

AbstractTo analyze the peripheral nerve pathological abnormalities in familial amyloid polyneuropathy, a correlative pathological study was carried out on the spinal nerve roots, proximal sciatic nerves, sural nerves, and brachial plexuses from 3 patients with the disease in Japan. The spinal nerve roots appeared to be unaffected except for amyloid deposition on the epineurium. In sciatic nerves and brachial plexuses the nerve lesions had a multifocal distribution, showing prominent interstitial edema in the endoneurium frequently adjacent to deposits of amyloid; in these regions the nerve fibers were severely depleted. A teased‐fiber study revealed that segmental demyelination was the predominant type of nerve fiber abnormality. However, these findings were not seen in the sural nerves; instead a diffuse fiber loss with axonal degeneration was observed. It is suggested that multifocal lesions in the proximal portions of the long extremity nerves could summate distally to produce a symmetrical polyneuropathy in the disease. In addition to a spaceoccupying effect of amyloid deposits in the endoneurium, severe endoneurial edema associated with amyloid deposition in blood vessels and the endoneurial interstitium may induce ischemia in nerve fibers, thus causing the progressive polyneuropathy in this disorder.
Multicenter trial of naloxone in alzheimer's disease
Authors: Henderson V. W. , Roberts E. , Wimer C. , Bardolph E. L. , Chui H. C. , Damasio A. R. , Eslinger P. J. , Folstein M. F. , Schneider L. S. , Teng E. L. , Tune L. E. , Weiner L. P. , Whitehouse P. J. 
Published: January 2005

AbstractIn a double‐blind, placebo‐controlled, crossover study of intravenously administered naloxone hydrochloride, 54 subjects with clinically ascertained Alzheimer's disease tested at three university centers showed no significant improvement in neuropsychological performance after 1‐mg or 10‐mg doses; 15 patients at 1 center were similarly unimproved after receiving 30 mg naloxone (single blind). Findings fail to support claims that naloxone monotherapy ameliorates cognitive impairments of Alzheimer's disease.
Vascular involvement in mitochondrial myopathy
Authors: Sakuta Ryoichi , Nonaka Ikuya 
Published: January 2005

AbstractElectron microscopic examination of muscle specimens taken at biopsy in 6 patients with complex I deficiency and 1 patient with an unknown primary chemical defect who had the clinical characteristics of mitochondrial myopathy, encephalopathy, lactic acidosis, and stroke‐like episodes (MELAS) revealed striking abnormalities in blood vessels in 5. Abnormalities consisted of an increased number of enlarged mitochondria with complicated cristae in the pericytes of capillaries, endothelial cells, and smooth muscle cells of the small arteries, including terminal arterioles and precapillary sphincters, predominantly in smooth muscle cells. On statistical analysis, the number of mitochondria and the ratio of mitochondrial area to the total area of the smooth muscle cells were increased approximately tenfold (p < 0.001). Although stroke‐like episodes were not present, similar mitochondrial abnormalities in blood vessels were found in 1 patient who had the encephalomyopathic form of complex IV deficiency and in 2 patients in whom the primary chemical defects could not be clearly defined. Such abnormalities in small arteries might be responsible for the occasional occurrence of transient cerebral ischemia causing stroke‐like episodes and progressive mental deterioration.
A quantitative investigation of the substantia nigra in Huntington's disease
Authors: Oyanagi Kiyomitsu , Takeda Shigeki , Takahashi Hitoshi , Ohama Eisaku , Ikuta Fusahiro 
Published: January 2005

AbstractTo elucidate the quantitative and topographical changes of neurons in the substantia nigra in Huntington's disease (HD), sections from 4 HD patients and 8 age‐matched control subjects were stained with cresyl violet and the numbers and localization of pigmented and nonpigmented neurons in the substantia nigra were examined. This study revealed a decrease of about 40° (p < 0.01 or p < 0.05) in neuronal number and a shrinkage of both pigmented and nonpigmented neurons in the substantia nigra in HD. Pigmented neuron number was decreased markedly in the ventral cell group. Loss of the neurons occurred in the medial and lateral thirds, but relative sparing of the neurons was seen in the central part of the substantia nigra. In contrast, loss of nonpigmented neurons was relatively uniform in the substantia nigra although it was more severe in the central part. We conclude that a primary neuronal degeneration occurs in the substantia nigra in HD.
Lipid‐containing cells in brains of normal and hypoxic infant monkeys: A quantitative and ultrastructural study
Authors: Goddard‐Finegold Jan , Sloper John J. , Esiri Margaret M. 
Published: January 2005

AbstractThe signficance of lipid‐containing cells found at autopsy in the white matter of infant brains is controversial, particularly with respect to their postulated role as markers of the “sudden infant death syndrome.” To determine whether such cells are indicative of prior nonlethal hypoxic insult, we quantitated them in the brains of control infant monkeys and in two groups of infant monkeys that were subjected to 30 minutes of hypoxic insult. One group consisting of monkeys that died less than 48 hours after the hypoxia, and the other of those that survived 7 to 13 days following the insult. The quantification of lipid‐containing cells was undertaken in frozen brain sections stained with Oil red O; sections of brains from 4 perfusion‐fixed animals were evaluated by electron microscopy.Lipid‐containing cells were found in the corpus callosum, in the septum, and in periventricular white matter in both posthypoxic and control animals. There was a relationship between numbers of lipid‐containing cells and the age of the animal; animal with large numbers were less than 28 days old. Decreases in numbers of lipid‐containing cells correlated with advancing myelination as well as with age. Electron microscopic evaluation revealed lipid droplets in the cytoplasm of cells with irregularly shaped nuclei, densely clumped chromatin, occasional microtubules, and narrow cytoplasmic processes.We suggest that lipid‐containing cells in the white matter of the brains of infants are related to age and to maturational factors and, in the absence of other pathologic signs, are not related to prior hypoxic injury.
Benign childhood epilepsy with occipital paroxysms: A 15‐year prospective study
Authors: Panayiotopoulos Chrysostomos P. 
Published: January 2005

AbstractEighteen of 418 children who had onset of epilepsy before the age of 13 years showed clinical and electroencephalographic evidence of benign childhood epilepsy with occipital paroxysms. They represented one‐fifth of all benign age‐ and localization‐related idiopathic epilepsies seen. Some patients were followed as long as 15 years. There was a preponderance in females and peak age at onset of epilepsy was 5 years. In 16 children, the seizures were infrequent and sometimes prolonged and consisted mainly of tonic deviation of the eyes and vomiting, often with evolution to unilateral or generalized convulsions. Seizures were only nocturnal in 11 and nocturnal and diurnal in another 5 children. Prognosis was excellent; 5 children had only one fit. Remission usually occurred 1 to 2 years after onset and no seizures occurred after the age of 12 years. The remaining 2 children had frequent diurnal episodes consisting of visual hallucinations, postictal headache, and occasional nocturnal hemiconvulsions. Their prognosis was less favorable. Electroencephalographic abnormalities in all 18 patients consisted of repetitive spike and slow‐wave discharges confined to the occipital regions and attenuated when the eyes were open. These outlasted clinical remission for many years, sometimes up to the age of 16. Fixation‐off sensitivity was demonstrated frequently. Based on these findings, a unifying definition for benign childhood epilepsy with occipital paroxysms is proposed.
Magnetic resonance imaging morphology of the corpus callosum in monozygotic twins
Authors: Oppenheim Jeffrey S. , Skerry John E. , Gazzaniga Michael S. 
Published: January 2005

AbstractRecent reports describe wide variations in the size and shape of the human corpus callosum. To investigate genetic influences on this variability, magnetic resonance images from 5 pairs of monozygotic twins and 10 unrelated control subjects were analyzed. Measurements of size and shape revealed greater similarity in twin pairs than in randomly paired controls. The results are consistent with the view that the anatomy of the corpus callosum, while clearly influenced by nongenetic factors, is under considerable genetic control.
Aberrant guanosine triphosphate–beta‐tubulin interaction in Alzheimer's disease
Authors: Khatoon Sabiha , Campbell Susan R. , Haley Boyd E. , Slevin John T. 
Published: January 2005

AbstractGuanosine triphosphate (GTP) is an absolute requirement for tubulin polymerization in situ. The nucleotide photoaffinity probe 8‐azidoguanosine 5′‐triphosphate (8N3GTP) has been shown to be a biological mimic of GRP in this system and, also, an effective active site probe of the exchangeable GRP binding site. Using [32P]8N3GTP we demonstrate that the exchangeable GTP site of the beta subunit of tubulin is available to added guanine nucleotide in normal aged brain homogenates, whereas it is variably unavailable in Alzheimer's diseased brain. Inability of 8N3GTP to photolabel beta tubulin appears to be associated with neurofibrillary tangle density. These results support the hypothesis that microtubule formation is abnormal in brains affected by Alzheimer's disease.
Chronic progressive multiple sclerosis: Serial magnetic resonance brain imaging over six months
Authors: Koopmans R. A. , Li D. K. B. , Oger J. J. F. , Kastrukoff L. F. , Jardine C. , Costley L. , Hall S. , Grochowski E. W. , Paty D. W. 
Published: January 2005

AbstractEight patients in the chronic progressive stage of multiple sclerosis were studied prospectively with magnetic resonance imaging of the head and neurological examination every 2 weeks for 6 months. There were no clinical relapses and disability ratings did not change between the start and completion of the study. Despite the clinical inactivity, a total of 86 active events (new, reappearing, or enlarging lesions) were identified by magnetic resonance imaging over the 6 months, with 50 (51) of 98 follow‐up scans showing evidence of one or more active lesions. New and changing lesions were indistinguishable in appearance, distribution, and temporal pattern from those previously seen in patients who had relapsing and remitting multiple sclerosis. However, as noted previously, the patients in the chronic progressive stage had many more confluent lesions than did the group in relapse. These results strongly suggest that the frequently observed clinical evolution of multiple sclerosis into a chronic progressive course is not accompanied by a fundamental change in the disease process. In fact the chronic progressive stage seems associated with more active changes demonstrated by scans than does relapsing and remitting multiple sclerosis. This study also confirms our previous experience that serial magnetic resonance imaging is much more sensitive than clinical examination in detecting disease activity in multiple sclerosis.
Isolation of herpes simplex virus type 1 during first attack of multiple sclerosis
Authors: BergstOum Tomas , Andersen Oluf , Vahlne Anders 
Published: January 2005

AbstractHerpes simplex virus type 1 was isolated from the cerebrospinal fluid of a patient during the first attack of multiple sclerosis. This is the first virus to be isolated from the central nervous system of a living patient with MS. The virus was identified as herpes simplex virus type 1 by restriction endonuclease analysis and by an enzytme immunoassay using monoclonal antibodies. Antibodies against type 1 but not type 2 were detected in consecutive samples of serum and cerebrospinal fluid. The patient has since entered a progressive phase of multiple sclerosis. The isolated type 1 strain might be of pathogenetic relevance to the development of multiple sclerosis in this patient.
Increased replication of HTLV‐I in HTLV‐I–associated myelopathy
Authors: Yoshida Mitsuaki , Osame Mitsuhiro , Kawai Hisaomi , Toita Masami , Kuwasaki Nanako , Nishida Yoshihiko , Hiraki Yukako , Takahashi Kouichi , Nomura Koichiro , Sonoda Shunro , Eiraku Nobutaka , Ijichi Shinji , Usuku Koichiro 
Published: January 2005

AbstractTo estimate the replication of the human T‐cell leukemia virus type I (HTLV‐I) in patients with HTLV‐I‐associated myelopathy (HAM), or tropical spastic paraparesis (TSP), HTLV‐I DNA integrated into lymphocyte genomes was analyzed by Southern blot hybridization. HTLV‐I DNA was detected in 125 (82%) of 153 patients and most showed random integration. This incidence was much higher than the 29% found in asymtopmatic carriers. Therefore, HAM/TSP development is associated with a high level of HTLV‐I replication. In addition, lymphocytes from 3 patients with HAM/TSP showed monoclonal integration of HTLV‐I DNA, indicating adult T‐cell leukemia.
Cisplatin‐induced Neurotoxicity with Seizures in Frogs
Authors: Blisard Karen S. , Harrington Deborah A. 
Published: January 2005

AbstractCis‐diamminedichloroplatinum II (cisplatin) given by injection to adult frogs (Rana pipiens) reslted in tonic‐clonic seizures 3 to 5 weeks later. The seizures could be induced multiple times; the animals appeared entirely normal between seizures. In the spinal cord there was vacuolation in the anterior grey horns. Ultrastructurally, the vacuoles consisted of swollen astrocytic processes in the neuropil and around neurons. Generalized edema with swelling of perivascular astrocyte foot processes was not seen. Systemic administration of cisplatin to frogs results in neurotoxicity with seizures and astrocytic swelling in the spinal cord.
Human cytotoxic T‐cell recognition of a synthetic peptide of myelin basic protein
Authors: Richert John R. , Robinson Eve D. , Deibler Gladys E. , Martenson Russell E. , Dragovic Ljubo J. , Kies Marian W. 
Published: January 2005

AbstractPrevious studies with a panel of myelin basic protein (MBP)–specific human T‐cell clones suggested a clustering of epitopes in the middle and at the C terminus of the molecule. The current study demonstrates that 19 of 40 clones recognize a synthetic peptide corresponding to residues 152 to 170 of the human MBP molecule and that 9 clones recognize a synthetic peptide corresponding to residues 86 to 105. Myelin basic protein–specific cytotoxic activity was restricted to the clones that recognized peptide 152–170, and this peptide served as a preferential cytotoxic T‐cell target when attached to an autologous B‐cell line. The specificity of MBP‐directed cytotoxic activity appears to be much more restricted than the specificity demonstrated for proliferative activity.
Cardiogenic brain embolism and lupus anticoagulant
Authors: Young Seymour M. , Fisher Marc , Sigsbee Ann , Errichetti Annmarie 
Published: January 2005

AbstractThe lupus anticoagulant, an acquired immunoglobulin associated with an increased tendency for thrombosis, has been linked to the occrence of cerebral ischemia presumably related to in situ thrombosis. Cardioembolic cerebral ischemic events have rarely been reported. We encountered 2 patients with focal cerebral ischemia, substantial mitral valvular masses, and a circulating lupus anticoagulant. In each, diagnostic evaluation supported a cardioembolic etiology. These findings illustrate the need for evaluating patients with cerebral ischemic events for a cardioembolic source when a circulating lupus anticoagulant is present.