bluuebunny/crossref_metadata_2025_split huggingface dataset:
Continuous muscle fiber activity, peripheral neuropathy, and thymoma
Authors: García‐Merino A. , Cabello A. , Mora J. S. , Liaño H. 
Published: January 2005

AbstractTwo patients, one of them with myasthenia gravis, presented symptoms of continuous muscle fiber activity syndrome before discovery of a thymoma. Peripheral neuropathy was present in both patients, with axonal and demyelinating lesions in sural nerve biopsy. The syndrome remained unchanged or whose after thymectomy. Both patients died of associated complications.
Regional variation in brain lactate in leigh syndrome by localized 1H magnetic resonance spectroscopy
Authors: Detre John A. , Wang Zhiyue , Bogdan Andrew R. , Gusnard Debra A. , Bay Carolyn A. , Bingham Peter M. , Zimmerman Robert A. 
Published: January 2005

AbstractLocalized water‐suppressed 1H magnetic resonance spectroscopy was performed in an 11‐month‐old infant with Leigh syndrome. Spectra obtained from the basal ganglia, occipital cortex, and brainstem showed elevations in lactate, which were most pronounced in regions where abnormalitites were seen with routine T2‐weighted magnetic resonance imaging. This approach has allowed us to examine metabolism in brain tissue directly and noninvasively, and may provide a sensitive means for evaluating metabolic disease and the response to therapy in the brain.
Anti‐Ri: An antibody associated with paraneoplastic opsoclonus and breast cancer
Authors: Luque F. Antonio , Furneaux Henry M. , Ferziger Reuven , Rosenblum Marc K. , Wray Shirley H. , Schold S. Clifford , Glantz Michael J. , Jaeckle Kurt A. , Biran Haim , Lesser Martin , Paulsen William A. , River Mary E. , Posner Jerome B. 
Published: January 2005

AbstractThe serum and cerebrospinal fluid (CSF) of 8 women with ataxia, 6 of whom also had eye movement abnormalities believed to be opsoclonus, were found to contain a highly specific antineuronal antibody we call anti‐Ri. Seven of the 8 women also had or developed cancer: carcinoma of the breast in 5, adenocarcinoma in an axillary lymph node in 1, and carcinoma of the fallopian tube in 1. Four patients presented with the neurological disorder; the cancer was diagnosed first in the other 4. Immunohistochemical studies using serum or CSF from all 8 patients revealed a highly specific antibody interaction with central nervous system neuronal nuclei but not with glial or other cells; the titer ranged from 1:5,000 to 1:320,000 in serum and from 1:2,000 to 1:16,000 in CSF. Biotinylated IgG from the patients' serum reacted with the tumors of 3 of 4 patients with anti‐Ri antibody but not with breast cancers from patients without anti‐Ri antibody. Immunoblots against cerebral cortex neuronal extracts identified protein antigens of 55‐kd and 80‐kd relative molecular mass. Serum titers by immunoblot ranged from 1:500 to more than 1:40,000 and CSF titers, from 1:10 to 1:2,000. The relative amount of anti‐Ri was always higher in CSF than in serum. The antibody was not present in sera from normal individuals; patients with breast cancer without opsoclonus; other patients with opsoclonus; or patients with other paraneoplastic syndromes related to breast, ovarian, or small‐cell lung cancer. We conclude that the presence of anti‐Ri antibody identifies a subset of patients with paraneoplastic ataxia and eye movement disorders (opsoclonus) who usually suffer from breast or other gynecological cancer; the antibody when present is a useful marker for an underlying malignancy.
Parent‐child concordance in multiple sclerosis
Authors: Sadovnick A. D. , Bulman D. , Ebers G. C. 
Published: January 2005

AbstractIt is now recognized that susceptibility to multiple sclerosis (MS) is determined in part by genetic factors. The gene loci influencing MS susceptibility are largely unidentified. In an attempt to better understand the mode of transmission, parent‐child concordance for MS was studied in two large, population‐based MS clinic populations. Among 75 parent‐child pairs concordant for MS, we found 40 mother‐daughter pairs, 13 mother‐son pairs, 21 father‐daughter pairs, and 1 father‐son pair. Controlling for the known female preponderance in MS, the data show a paucity of father‐son pairs. These data have implications for understanding the mechanisms of inheritance for MS susceptibility as well as for risk counseling in families of patients.
The hypothalamic lateral tuberal nucleus in Alzheimer's disease
Authors: Kremer Berry , Swaab Dick , Bots Gerard , Fisser Bart , Ravid Rivka , Roos Raymund 
Published: January 2005

AbstractThe hypothalamic lateral tuberal nucleus was investigated in 5 young patients, aged 45 to 64 years, with Alzheimer's disease (AD) and in 5 age‐matched control subjects. Combining conventional histopathological and immunocytochemical staining with neuronal counts, a peculiar form of neuronal pathology was characterized. Although neurons and neurites in the lateral tuberal nucleus of AD specimens were heavily stained by Alz‐50, silver and thioflavine‐S stains disclosed few neurofibrillary tangles or neurites. The numbers of neurons in the lateral tuberal nucleus of patients with AD (67,450; SEM = 5,050) were no different from those of control subjects (58,900; SEM = 2,450). In the AD patients, few plaques were present and were almost exclusively of the amorphous variety. We conclude that neurons in the lateral tuberal nucleus show an early stage of AD‐related cytoskeletal pathology (Alz‐50 positivity), but without plaques or neuronal death.
Anatomical left‐right asymmetry of language‐related temporal cortex is different in left‐ and right‐handers
Authors: Steinmetz Helmuth , Volkmann Jens , Jäncke Lutz , Freund Hans‐Joachim 
Published: January 2005

AbstractAsymmetry of the planum temporale, a language‐related intrasylvian area on the superior temporal gyrus, is the most remarkable anatomical left‐right asymmetry of the human brain. The in vivo application of magnetic resonance morphometry in 52 healthy volunteers (26 dextrals and 26 sinistrals) revealed that planum temporale asymmetry than right‐handers. Thus, a structural‐functional relation exists in cerebral asymmetry. The correlation is likely to reflect language representation. Because familial sinistrality influenced the anatomical pattern in left‐handers and planum temporale asymmetry is already present in the newborn, prenatal factors must play an important role in the development of functional laterality.
A selective loss of somatostatin in the hippocampus of patients with temporal lobe epilepsy
Authors: Robbins Richard J. , Brines Michael L. , Kim Jung H. , Adrian Thomas , De Lanerolle Nihal , Welsh Susan , Spencer Dennis D. 
Published: January 2005

AbstractAlthough neuropeptides have been demonstrated to be hippocampal neuromodulators in laboratory animals, their role in human hippocampal physiology or pathophysiology remains to be defined. The concentrations of somatostatin, cholecystokinin octapeptide, vasoactive intestinal polypeptide, and dynorphin A 1‐17 were determined in hippocampal tissue resected from patients with cryptogenic temporal lobe epilepsy, a common seizure disorder originating in or near the hippocampus. Control tissue was obtained from cadavera or epilepsy patients in whom the hippocampus was removed during the resection of temporal lobe tumors. Peptide determinations were performed on extracts of punch biopsy specimens taken from six different hippocampal regions. A significant decrease in immunoreactive somatostatin concentration was identified in the dentate gyrus and in region cornu ammonis 4 of cryptogenic temporal lobe epilepsy specimens. No significant changes were present in any other hippocampal region or in the levels of other peptides. In situ hybridization studies performed on cryostat sections from similar patients confirmed a marked loss of neurons expressing the somatostatin gene, which was restricted to the dentate hilus. The density of specific 125I‐somatostatin binding to cryostat sections, as determined by semiquantitative in vitro autoradiography, was significantly increased in the dentate gyrus of the cryptogenic epilepsy patients, compared with tumor control specimens. We conclude that a loss of somatostatin‐producing interneurons with an upregulation of dentate somatostatin receptors is a specific and characteristic element in the pathophysiology of human crytogenic temporal lobe epilepsy.
Correction of non–24‐hour sleep/wake cycle by melatonin in a blind retarded boy
Authors: Palm Lars , Blennow Gösta , Wetterberg Lennart 
Published: January 2005

AbstractA 9‐year‐old, blind boy with severe mental retardation with a chronic sleep/wake disturbance had a circadian rhythm of 24.75 hours and an internal desynchronization of the endogenous rhythms. Treatment with oral melatonin given at 6PM induced a regular sleep/wake pattern. Melatonin, in this patient, convincingly entrained the endogenous rhythm to the appropriate chronological 24‐hour day.
Identification of early recurrence of primary central nervous system tumors by [18F]fluorodeoxyglucose positron emission tomography
Authors: Glantz Michael J. , Hoffman John M. , Coleman R. Edward , Friedman Allan H. , Hanson Michael W. , Burger Peter C. , Herndon PhD James E. , Meisler William J. , Schold S. Clifford 
Published: January 2005

AbstractAs aggressive neurosurgery and adjuvant therapy have become standard care for most patients with primary central nervous system (CNS) tumors, limitations of posttreatment neuroimaging techniques have become more apparent. Interpretation of computed cranial tomography (CT) and magnetic resonance imaging (MRI) in patients with brain tumors is complicated by changes related to surgery, corticosteroids, radiation, and chemotherapy. We investigated the role of 18F‐2‐fluoro‐2‐deoxy‐D‐glucose (FDG) positron emission tomography (FDG‐PET) in these difficult diagnostic situations by obtaining FDG‐PET scans in 5 patients following temporal lobectomy for epilepsy, in 5 patients with recurrent anaplastic gliomas before and after corticosteroid therapy, and in 5 patients after the development of histologically confirmed radionecrosis. We also obtained postoperative FDG‐PET scans in 32 consecutive patients undergoing initial resection of a primary brain tumor. Our results indicate that glucose uptake as detected by FDG‐PET scanning with[18F]fluorodeoxyglucose is not increased in the postoperative period; is not affected by steroid therapy; and accurately predicts early recurrence of tumor, supplementing other predictors of tumor behavior, including extent of resection, histological diagnosis, and postoperative CT. Thus PET using [18F] fluorodeoxyglucose can contribute to the optimum management of patients with primary brain tumors.
Antiepileptogenic effects of conventional anticonvulsants in the kindling model of epilepsy
Authors: Silver Jon M. , Shin Cheolsu , McNamara James O. 
Published: January 2005

AbstractWe sought to determine whether the clinically effective anticonvulsant drug valproate exhibited antiepileptogenic properties in the kindling model (we use the term anticonvulsant to mean suppression of seizure, and antiepileptogenic to mean suppression of development of epilepsy). We compared and contrasted valproate with two other anticonvulsan drugs, phenobarbital and carbamazepine. We investigated the effects of these drugs on the development of kindling that is, the number of stimulation‐induced afterdischarges required to induce enhanced seizure susceptibility in rats Valproate exhibited powerful antiepileptogenic effects as evident in a dose‐dependent increase in the number of afterdischarges required to induce kindling. These effects were not due to retained valproate or an active metabolite merely masking the expression of kindled seizures. By contrast, carbamazepine was devoid of any antiepileptogenic effects despite exhibiting marked anticonvulsant effects. Like valproate, phenobarbital exhibited both antiepileptogenic and anticonvulsant properties, but its antiepileptogenic properties were significantly less pronounced. The antiepileptogenic effects of valproate and phenobarbital strengthen the candidacy of these agents for the clinical studies needed to investigate pharmacological prevention of the development of epilepsy in high‐risk groups.
Change in pattern of muscle activity following botulinum toxin injections for torticollis
Authors: Gelb Douglas J. , Yoshimura Don M. , Olney Richard K. , Lowenstein Daniel H. , Aminoff Michael J. 
Published: January 2005

AbstractTwenty patients with torticollis had electromyographic studies of their neck muscles performed before and after a series of local injections of botulinum toxin. The pattern of muscle activity changed after the injections, and this effect persisted even after head position had returned to baseline. Patients who did not experience any clinical benefit from the injections also demonstrated a change in the pattern of muscle activity. These results suggest that the underlying abnormality in torticollis usually involves a general motor program for head position, rather than the activity of individual neck muscles.
Eosinophilia myalgia syndrome: I. Immunocytochemical evidence for a T‐cell–mediated immune effector response
Authors: Emslie‐Smith Alison M. , Engel Andrew G. , Duffy Joseph , Bowles Carolyn A. 
Published: January 2005

AbstractSpecimens of muscle and fascia from 13 patients fulfilling the Centers for Disease Control criteria for the eosinophilia myalgia syndrome (EMS) were studied by quantitative immunocytochemical analysis. The immunolocalization of CD3, CD4, CD8, CD22, and CD56 markers, the γδ T‐cell receptor, major histocompatibility complex (MHC) class I complex and class II antigens, and complement membrane attack complex (MAC) were examined. The distribution and relative proportions of T cells and T‐cell subsets, B cells, macrophages, and eosinophils were determined at perivascular, perimysial, endomysial, and fascial sites of accumulation. At all sites, T cells were predominant, CD8+ cells outnumbered CD4+ cells 6‐ to 20‐fold, and between 60 and 80% of T cells were activated. B cells and eosinophils each accounted for less than 3% of inflammatory cells. Very few cells expressed either the γδ T‐cell receptor or natural killer cell markers. As in dermatomyositis (DM), MHC class I antigen complex expression was increased on many structurally normal muscle fibers, but in contrast to DM, microvascular MAC deposits were not a feature of EMS. The findings implicate a cellular immune response directed against a connective tissue component in EMS.
Suppression of experimental autoimmune encephalomyelitis by oral administration of myelin antigens: IV. Suppression of chronic relapsing disease in the lewis rat and strain 13 guinea pig
Authors: Brod Staley A. , al‐Sabbagh Ahmad , Sobel Raymond A. , Hafler David A. , Weiner Howard L. 
Published: January 2005

AbstractOral administration of proteins is a long‐recognized method of inducing antigen‐specific peripheral immune tolerance. We previously showed that oral administration of myelin basic protein suppresses monophasic experimental autoimmune encephalomyelitis in the Lewis rat when it is given in association with immunization and prior to disease onset. As a potential therapy for human autoimmune disease, it is crucial to determine whether oral tolerance can ameliorate an ongoing immune response. We therefore asked whether oral administration of myelin antigens, after sensitization and disease expression has occurred, could affect immunological, clinical, or pathological features of experimental autoimmune encephalomyelitis. Chronic relapsing experimental autoimmune encephalomyelitis was induced in the Lewis rat and strain 13 guinea pig by immunization with whole guinea pig cord homogenate, complete Freund's adjuvant, and Mycobacterium tuberculosis. Following recovery from the first attack, animals were orally given bovine myelin, guinea pig myelin, or guinea pig myelin basic protein three times per week for up to 3 months. Animals receiving myelin products orally had decreased severity and frequency of clinical relapses, decreased delayed‐type hypersensitivity responses to myelin antigens, diminished inflammation in the central nervous system (CNS), and decreased areas of CNS demyelination. In the rat, guinea pig myelin basic protein was as effective as guinea pig myelin in ameliorating the disease and also resulted in decreased serum anti–myelin basic protein antibody levels. No exacerbation of disease or worsening of pathological findings occurred in the animals given myelin products. These results demonstrate that oral administration of myelin antigens can suppress chronic relapsing experimental autoimmune encephalomyelitis and have direct relevance to therapy of human demyelinating disorders such as multiple sclerosis.
Benefits of monitoring plasma levodopa in Parkinson's disease patients with drug‐induced chorea
Authors: Sage Jacob I. , Mark Margery H. , McHale Denise M. , Sonsalla Patricia K. , Vitagliano Dawn 
Published: January 2005

AbstractWe studied the temporal patterns of chorea and plasma levodopa profiles in 30 patients with Parkinson's disease whose motor fluctuations were difficult to characterize and treat on the basis of observation alone. We were able to determine whether chorea was associated with high levodopa concentrations or low levodopa concentrations or both. We found the following patterns of levodopa‐associated chorea: chorea due to inadequate levodopa levels, chorea due to biphasic levodopa absorption, chorea associated with either rapid or slow levodopa absorption, and chorea due to long‐duration levodopa absorption mimicking a sustained‐release preparation. Seven patients benefited after their dosing schedules were rearranged as a result of information gained from monitoring. We conclude that any patient with levodopa‐associated chorea who cannot be regulated on the basis of observation alone should be studied with simultaneous plasma levodopa measurements and clinical monitoring to detect an unusual plasma levodopa pattern that may be improved by adjustment of dosing schedule.
Epilepsy surgery in the posterior cortex
Authors: Blume Warren T. , Whiting Sharon E. , Girvin John P. 
Published: January 2005

AbstractFourteen (74%) of 19 patients obtained a significant reduction in seizures after posterior corticectomy; 6 (32%) were seizure‐free over a median follow‐up of 3.7 years (range, 1 to 14 years). Surgery included limited resections of the occipital lobe in 16 patients, posterior temporal region in 11, and posterior portion of parietal lobe in 7. Surgical failure related to probable multiple areas of epileptogenesis (4 patients), or limited resections (2 patients) to preserve visual fields (2 patients) and to avoid dyslexia (1 patient). Of 14 patients without a complete hemianopia preoperatively, 6 (43%) developed a new or increased visual field deficit, 2 (14%) of which were hemianopia. Four (36%) of 11 occipital lobe resections resulted in a new or increased visual field deficit: quadrantanopia in 3 and hemianopia in 1. Visual phenomena were the most common initial ictal symptoms, occurring in 13 (68%) of the 19 patients. Twelve patients had complex partial seizures: in 2, always without warning; in 7, always following an aura, usually visual; and in 3 patients, with or without warning. Scalp electroencephalography identified the origin of most recorded seizures in 12 (63%) of the 19 patients. A principal interictal spike focus appeared in 15 patients (79%), and always correlated with the epileptogenic lobe as defined by scalp and/or subdural‐recorded seizures (14 patients) or by clinical analysis and computed tomography (1 patient).
Motor responses after transcranial electrical stimulation of cerebral hemispheres with a degenerated pyramidal tract
Authors: Fries Wolfgang , Danek Adrian , Witt Thomas N. 
Published: January 2005

AbstractMotor responses were evoked in the thenar muscles by transcranial electrical cortex stimulation in 5 stroke patients with an isolated lacuna in the internal capsule, in whom wallerian degeneration of the pyramidal tract was demonstrated in vivo. Suprathreshold stimulation of the affected hemisphere elicited bilateral motor responses; whereas, stimulation at identical intensities of the undamaged hemisphere yielded strictly unilateral responses in the contralateral hand, like the responses of all normal control subjects. Focused magnetic brain stimulation was performed in 1 patient and gave identical results. Because muscular excitability to cortical stimulation is preserved in spite of pyramidal tract disruption, other pathways must bypass the lesion. Because of the bilaterality of responses, we suggest polysynaptic corticoreticulospinal connections.
Neocortical damage during HIV infection
Authors: Wiley Clayton A. , Masliah Eliezer , Morey Mitchel , Lemere Cindy , DeTeresa Richard , Grafe Marjorie , Hansen Lawrence , Terry Robert 
Published: January 2005

AbstractClinical and pathologicals evidence of subcortical central nervous system (CNS) damage is observed commonly in patients with human immunodeficiency virus (HIV) encephalitis. Whether other CNS regions are also affected has not been well studied. We report neocortical damage in patients with HIV encephalitis. Using quantitative techniques, we demonstrate statistically significant thinning of the neocortex, with a loss of large cortical neurons. Qualitative and quantitative assessments of neocortical neuropil reveal a loss of synaptic density and vacuolation of dendritic processes. Failure to demonstrate an association of these changes with the presence of HIV antigens suggests that neocortical damage may be an indirect effect of HIV infection of the CNS.
Positron emission tomography suggests that the rate of progression of idiopathic parkinsonism is slow
Authors: Bhatt Mohit H. , Snow Barry J. , Martin W. R. Wayne , Pate Brain D. , Ruth Thomas J. , Calne Donald B. 
Published: January 2005

AbstractWe performed sequential positron emission tomography scans with 6‐[18F]fluoro‐L‐dopa in 9 patients with idiopathic parkinsonism and 7 age‐matched normal control subjects to compare changes in the nigrostriatal dopaminergic pathway over time. The mean interval between the scans was 3.3 years for the group with idiopathic parkinsonism and 3.9 years for the control subjects. The scans were analyzed by calculating the ratio of striatal to background radioactivity. Both groups showed statistically significant reductions of striatal uptake over the interval. The rate of decrease was almost identical in each group (p = 0.6). We infer that the usual rate of loss of integrity of the dopaminergic nigrostriatal pathway in patients with idiopathic parkinsonism is slow and the rate of change between the two groups was comparable.
Magnetic resonance imaging–based volume studies in temporal lobe epilepsy: Pathological correlations
Authors: Cascino Gregory D. , Jack Clifford R. , Parisi Joseph E. , Sharbrough Frank W. , Hirschorn Kathryn A. , Meyer Frederic B. , Marsh W. Richard , O'Brien Peter C. 
Published: January 2005

AbstractWe performed a prospective study correlating magnetic resonance imaging volume measurements of the hippocampal formation with histopathology in 24 patients with intractable partial epilepsy who subsequently underwent an anterior temporal lobectomy for their seizure disorder. Patients with mass lesions verified pathologically were excluded from this study. In 71% of patients, quantitative hippocampal formation atrophy correctly lateralized the temporal lobe of seizure origin; in 29%, the volume study was indeterminant. The severity of the pathological alterations in the hippocampus correlated with the hippocampal formation volume determination. Mesial temporal sclerosis was identified in the surgically excised temporal lobe in 15 patients. The magnetic resonance imaging volume studies indicated hippocampal atrophy in the temporal lobe resected in 14 of the 15 patients. Magnetic resonance imaging‐based volume measurements of the hippocampal formation increase the diagnostic yield of magnetic resonance imaging scanning in patients with intractable partial epilepsy related to mesial temporal sclerosis.