bluuebunny/crossref_metadata_2025_split huggingface dataset:
New anatomical and functional imaging methods
Authors: Prichard James W. , Brass Lawrence M. 
Published: January 2005

AbstractPowerful new methods for imaging both brain anatomy and brain function are appearing at an increasing rate. The modern era of minimally invasive, highly informative, neurological diagnostic imaging methods began with the introduction of x‐ray computed tomography in the 1970s. More recently, positron emission tomography and single‐photon emission computed tomography have been used extensively in research on normal and pathological brain function, and they are finding specific medical applications. Nuclear magnetic resonance methods are in widespread use for neurological diagnosis only a decade after they became available. Rapid development of new techniques based on the same principles, and implementable on clinical instruments with relatively minor modifications, will expand the range of nuclear magnetic resonance measurement capabilities considerably in the near future. These technological innovations and others yet to come have major implications for the practice of neurology. The most important one is an increase in relative value among clinical diagnostic skills of history taking and mental status examination, which will remain largely beyond the reach of technology.
Skeletal muscle toxoplasmosis in patients with acquired immunodeficiency syndrome: A clinical and pathological study
Authors: Gherardi R. , Baudrimont M. , Lionnet F. , Salord J.‐M. , Duvivier C. , Michon C. , Wolff M. , Marche C. 
Published: January 2005

AbstractThe present article describes the clinical pathological findings in 5 human immunodeficiency virus (HIV)‐infected patients with muscle toxolasmosis. The patients had marked lymphopenia (5/5), with less than five CD4+ cells/mm3 (3/3), when they developed fever (5/5), and multiorgan failure (5/5), including diffuse ecephalitis, pneumonia, pancytopenia, and myopathy. Muscle involvement; included weakness and wasting (4/5), myalgias (3/5), and high serum creatine kinase levels (3/3). Serology for toxoplasmosis showed high IgG titers in 3 patients (3/4). Anti‐Toxoplasma therapy resulted in complete recovery in 2 patients. Muscle toxoplasmosis was detected by biopsy (3/5) or postmortem evaluation (2/5), and was identified using immunocytochemistry and electron microscopy. Toxoplasma cysts were detected in 0.5 to 4%of muscle fibers close to or remote from necrotic fibers and inflammatory infitrates. Muscle fibers strongly expressed the major histocompatibility complex class I antigen (2/2) as in polymyositis. We suggest that Toxoplasma gondii should be sought by muscle biopsy in patients who have acquired immunodeficiency syndrome with fever, encephalitis, multiorgan dysfunction, and elevated serum creatine kinase levels of obscure origin.
Familial adult‐onset muscular dystrophy with leukoencephalopathy
Authors: van Engelen Baziel G. M. , Leyten Quint H. , Bernsen Pieter L. J. A. , Gabreëls Fons J. M. , Joosten Ed M. G. , ter Laak Henk J. , Ruijs Mechelien B. M. , Hamel Ben C. J. , Cruysberg Johan R. M. , Barkhof Frederik , Valk Jaap 
Published: January 2005

AbstractWe report on 3 siblings with an adult‐onset, predominantly distal muscle weakness. In the female index patient this was associated with epilepsy and a progressive spastic ataxic gait, while the 2 other siblings had no appreciable clinical nervous system involvement. Additional investigations revealed muscular dystrophy and leukoencephalopathy in all 3 siblings. We conclude that this familial adult‐onset muscular dystrophy associated with leukoencephalopathy represents a newly recognized autosomal recessive syndrome.
Long‐term treatment response and fluorodopa positron emission tomographic scanning of parkinsonism in a family with dopa‐responsive dystonia
Authors: Nygaard Torbjoern G. , Takahashi Hirohide , Heiman Gary A. , Snow Barry J. , Fahn Stanley , Calne Donald B. 
Published: January 2005

AbstractDopa‐responsive dystonia (DRD) is one form of childhood‐onset idiopathic torsion dystonia. Adult‐onset parkinsonism has appeared in several previously unaffected members in families with DRD suggesting that this may be an additional phenotypical expression of the disease. We report a family with DRD in which 2 women and 1 man, unaffected by dystonia, developed tremor‐onset parkinsonism after age 50 years. The women continue on a low dosage of levodopa after 9 and 13 years of treatment, with a stable, nearly complete, symptomatic response. This contrasts to the typical long‐term treatment complications observed in patients with Parkinson's disease. We assessed nigrostriatal dopaminer‐gic function in the proband, with typical DRD, and the 2 women with parkinsonism using 6‐{18F}fluoro‐L‐dopa positron emission tomography. All 3 had normal striatal 6‐{18F}fluoro‐L‐dopa uptake. These observations provide compelling evidence that “benign” adult‐onset parkinsonism may be an expression of the disease in some members of families with DRD and does not support consideration of the DRD gene as a risk factor for development of Parkinson's disease. There may be considerable clinical heterogeneity in DRD depending on the age at onset.
Suppression of postischemic epileptiform activity with MK‐801 improves neural outcome in fetal sheep
Authors: Tan William K. M. , Williams Chris E. , Gunn Alistair J. , Mallard Carina E. , Gluckman Peter D. 
Published: January 2005

AbstractTo determine the effect of suppression of epileptiform activity that develops after hypoxic‐ischemic injury in the immature brain, chronically instrumented near‐term fetal sheep (119–133 days) were subjected to 30 minutes of complete cerebral ischemia: 6 were given a 0.3‐mg/kg bolus of MK‐801 at 6 hours after the insult followed by continuous infusion of 1 mg/kg over the next 36 hours, and were compared to 6 control sheep. Electrocorticographic activity and edema within the parasagittal region of the cortex were quantified with real‐time spectral analysis and impedance measurements, respectively. Histological outcome was assessed 72 hours later. The intense epileptiform activity seen from 9 ± 2 to 30 ± 3 hours in the control group was completely suppressed in the MK‐801‐treated group. The onset of secondary cortical edema was delayed from 9.4 ± 1.1 hours to 14.8 ± 0.7 hours (p < 0.01). Neuronal damage was reduced, particularly in the lateral cortex and hippocampus (p < 0.05). Infarction of the parasagittal cortex was not prevented. These results suggest that N‐methyl‐D‐aspartate‐mediated epileptiform activity that develops after a global hypoxic‐ischemic insult worsens neuronal outcome in the immature brain.
Childhood peripheral neuropathy with autoantibodies to myelin glycoprotein Po
Authors: Jelloun‐Dellagi S. Ben , Dellagi K. , Burger D. , Younes‐Chennoufi A. Ben , Hentati F. F. , Steck A. , Hamida M. Ben 
Published: January 2005

AbstractThe serum of a 4‐year‐old child with severe hypertrophic peripheral neuropathy contained high‐titer polyclonal antibodies, mainly of IgG class, reacting with the 30‐kd Po glycoprotein of peripheral nerve. This was demonstrated by indirect immunofluorescence, immunoblot analysis of myelin proteins, and enzyme‐linked immunosorbent assay with purified Po glycoprotein. The antibodies did not react with myelin‐associated glycoprotein, sulfated glycuronic acid‐containing paraglo‐boside (SGPG and SGLPG), or other peripheral nerve glycolipids or gangliosides. To our knowledge this is the first report of strong antibody activity specifically directed against Po. This antibody may play a causative role in the pathogenesis of the peripheral neuropathy in this patient.
The audiospinal reaction in parkinsonian patients reflects functional changes in reticular nuclei
Authors: Delwaide P. J. , Pepin J. L. , de Noordhout A. Maertens 
Published: January 2005

AbstractAudiospinal facilitation using the soleus H‐reflex as a test was compared in 16 control subjects and 23 parkinsonian patients. In the patients, facilitation was significantly reduced during the 75 to 150 msec after the conditioning stimulation. This reduction was seen bilaterally even in patients with a hemisyndrome. It was corrected by L‐dopa but not by anticholinergic agents. Facilitation at the 75‐msec delay showed an inverse linear correlation with the bradykinesia intensity (r = ‐0.7, p < 0.01). The results argue in favor of a reduced excitability of the nucleus reticularis pontis caudalis from which a reticulospinal tract emanates as effector of the audiospinal facilitation. This would represent another example of dysfunction of reticular nuclei in Parkinson's disease.
Crossed aphasia with visceral situs inversus
Authors: Cohen Laurent , Gény Christian , Hermine Oliver , Gray Francoise , Degos Jean‐Denis 
Published: January 2005

AbstractA patient presenting the association of anomalous cerebral dominance for language, as revealed by crossed aphasia, and partial visceral situs inversus is described. Although it cannot be excluded that the two abnormalities coincided by chance, it is suggested that they both resulted from a single aberration of development.
Delayed peripartum vasculopathy: Cerebral eclampsia revisited
Authors: Raps Eric C. , Galetta Steven L. , Broderick Magie , Atlas Scott 
Published: January 2005

AbstractOver a 2‐Year Period, we treated 4 postpartum patients with acute neurologic problems appearing 4 to 9 days after delivery. Three patients either had no proteinuria or edema and therfore did not meet all accepted clinical criteria for the diagnosis of eclampisa. Magnetic resonance imaging (MRI) of the brain in all patients demonstrated high‐signal foci most prominent in the parietooccipital regions and the subcortical white matter. Cerebral angiogram in 2 patients revealed diffuse vasospasm. In 3 patients, MRI abnormalities resolved. MRI and angiographic abnormalities in our patients are identical to those of patients meeting accepted criteria for eclampisa. Our experience suggest that the current criteria for the diagnosis of eclampsia are too stingent, both in terms of clinical picture and days postpartum to cerebral mainfestations.
Isolation of HTLV‐II from a patient with chronic, progressive neurological disease clinically indistinguishable from HTLV‐I‐associated myelopathy/tropical spastic paraparesis
Authors: Jacobson Steven , Lehky Tanya , Nishimura Masataka , Robinson Susan , McFarlin Dale E. , Dhib‐Jalbut Suhayl 
Published: January 2005

AbstractAn increasing spectrum of diseases has been shown to be associated with the human T‐cell lymphotropic virus type I (HTLV‐I), most notably a chronic, progressive myelopathy termed HTLV‐I‐associated myelopathy/tropical spastic paraparesis and adult T‐cell leukemia. HTLV‐II is a close relative of HTLV‐I and is structurally similar but molecularly distinct. This virus is endemic in Amerindian populations and a high seroprevalence rate has been observed in intravenous drug abusers. Here, for the first time, we have identified a patient with a chronic, progressive neurological disease clinically indistinguishable from HTLV‐I‐associated myelopathy/tropical spastic paraparesis from whom we have isolated and characterized HTLV‐II in the absence of any other detectable human retrovirus. Antibodies to HTLV were deteced in both serum and cerebrospinal fluid, with typical HTLV‐II banding patterns on Western blots. HTLV‐II viral sequences were detected in high copy number from peripheral lymphocytes by polymerase chain reaction techniques, and cloning and sequencing of the virus revealed a 99.5% homology with prototype HTLV‐II. These results serve to alert the medical community to the possibility that in addition to HTLV‐I, HTLV‐II may be associated with a neurological disorder.
Mechanical hyperalgesias in neuropathic pain patients: Dynamic and static subtypes
Authors: Ochoa José L. , Yarnitsky David 
Published: January 2005

AbstractTwo behavioral kinds of mechanical hyperalgesia can be clearly discerned by clinical criteria in patients with neuropathic syndromes, i.e., a dynamic type, elicitable by lightly stroking the symptomatic skin, and a static type, elicitable by steadily applying gentle pressure on it. Of 28 patients studied, 19 had dynamic and 18 had static type mechanical hyperalgesia (9 expressed both types). Experimental compression–ischemia nerve block totally abolished the dynamic hyperalgesia in all patients except in 2, in whom it was markedly diminished. Disappearance of dynamic hyperalgesia occurred contemporaneously with block of myelinated A fibers, as expressed by abolition of the sensations of touch and cold, monitored quantitatively. Static hyperalgesia, however, outlasted A‐fiber block in 15 of 18 patients; the phenomenon persisted during the stage when only unmyelinated fibers were available for impulse conduction. It is thus concluded that, at the primary afferent level, dynamic hyperalgesia is mediated by myelinated fibers, whereas static hyperalgesia depends on unmyelinated afferents. These two kinds of hyperalgesia represent discrete pathophysiological entities with distinct clinical connotations.
Clinical worsening in multiple sclerosis is associated with increased frequency and area of gadopentetate dimeglumine–enhancing magnetic resonance imaging lesions
Authors: Smith M. E. , Stone L. A. , Albert P. S. , Frank J. A. , Martin R. , Armstrong M. , Maloni H. , McFarlin D. E. , McFarland H. F. 
Published: January 2005

AbstractIt is now well established that clinically stable patients with relapsing–remitting multiple sclerosis have ongoing disease activity when evaluated by serial gadolinium–enhanced (Gd‐DTPA) magnetic resonance imaging (MRI) scans. Despite this, the relationship between clinical disease and MRI lesions, though suspected, has not been extensively documented. The relationship between Gd‐DTPA MRI lesions, and clinical disease was examined in this study of 9 patients with mild relapsing–remitting multiple sclerosis (Expanded Disability Status Scale [EDSS] <3.5) who had 24 to 37 monthly Gd‐DTPA MRI scans, neurological examinations, and EDSS score assignments. The area and frequency of Gd‐DTPA lesions were examined during months with and without clinical worsening as measured by EDSS. Forty‐one episodes of clinical worsening were noted during the study. A significant association was observed between these periods of clinical worsening and MRI parameters, including increases in total number, number of new lesions, and the total area of enhancement. Logistic regression analysis showed a signficant effect of the number and area of Gd‐DTPA MRI lesions on both the onset and continuation of clinical worsening, confirming an imporant relationship between clinical disease and an increase in cerebral Gd‐DTPA MRI activity. A relationship with long‐term disability was suggested, but cannot be confirmed without longer follow‐up of these patients.
Genetic susceptibility and head injury as risk factors for Alzheimer's disease among community‐dwelling elderly persons and their first‐degree relatives
Authors: Mayeux Richard , Ottman Ruth , Tang Ming‐Xin , Noboa‐Bauza Lourdes , Marder Karen , Gurland Barry , Stern Yaakov 
Published: January 2005

AbstractWe performed a community‐based study to investigate the relationship of genetic susceptibility and head injury to Alzheimer's disease(AD) in 138 patients with AD and 193 healthy elderly control subjects. Data concerning presence or absence of dementia and certain exposures were also obtained from 799 first‐degree relatives of the patients and 1,238 first‐degree relatives of the control subjects. Adjusting for age, gender, and other risk factors, the odds ratio for AD associated with head injury was 3.7 (95% confidence interval [CI], 1.4–9.7). The association was highest for head injuries that occurred after age 70. The risk of AD was higher in first‐degree relatives of patients with onset prior to age 70 than in relatives of control subjects (risk ratio [RR] = 2.5; 95% CI, 1.1–5.6). The risk was not increased for relatives of patients with onset of AD at age 70 or older. Compared with relatives without head injury, the risk of AD was increased among both head‐injured relatives of patients (RR = 5.9; 95% CI, 2.3–14.8) and head‐injured relatives of control subjects (RR = 6.9; 95% CI, 2.5–18.9). Our results are consistent with the hypothesis that severe head injury and genetic susceptibility are associated with AD. Both associations concur with current concepts regarding the role of amyloid in AD. Although we regard head injury, like genetic susceptibility, to be a putative risk factor for AD, the temporal relationship between head injury and AD warrants further investigation.
Pretectal eyelid retraction and lag
Authors: Galetta Steven L. , Gray Lawrence G. , Raps Eric C. , Schatz Norman J. 
Published: January 2005

AbstractWe report 2 patients who had eyelid retraction and eyelid lag with minimal impairment of vertical gaze. In both patients, magnetic resonance imaging demonstrated a circumscribed unilateral lesion immediately rostral and dorsal to the red nucleus involving the lateral periaqueductal gray area. Our clinical and radiological findings support the existence of a premotor eyelid control center in the region of the nucleus of the posterior commissure.
Intracerebral cytokine messenger RNA expression in acquired immunodeficiency syndrome dememtia
Authors: Wesselingh Steven L. , Power Christopher , Glass Jonathan D. , Tyor William R. , McArthur Justin C. , Farber Joshua M. , Griffin John W. , Griffin Diane E. 
Published: January 2005

AbstractThe pathogenesis of the dementia associated with human immunodeficieny virus (HIV) infection is unclear, but has been postulated to be due to indirect effects of HIV infection including the local production of cytokines. To determine which cytokines are produced in the nervous system and to identify and correlations with dementia, cytokine and HIV messenger RNA expression was analyzed by reverse transcriptase‐polymerase chain reaction in the brains from 24 HIV‐ infected patients with and without dementia and 9 HIV uninfected control subjects. Levels of tumor necrosis factor‐α messenger RNA were significantlyhigher and levels of interleukin (IL)‐4 messenenger RNA were significantly lower in demented compared to nondemented HIV‐infected parients. Demented patients als had lower IL‐1β levels than did nondemented patients. No significant differences were detected in the amounts of leukemia inhibitory factor, IL‐6, transforming growth factor β1 and β2 monokine induced bygamma interferon‐2 (MIC‐2), or interferon‐Y Messenger RNSs IL‐10 and IL‐2 messenger RNAs wre undetectable in all brains examined. Cytokine messenger RNA levels in nondemented HIV‐positivew patients were similar to those in HIV‐negative control subjects. HIV transcripts were more abundant in subcortical white matter than in the basal ganglia, cortex, or deep white matter. Our findings suggest a possible role for tumor necrosis factor‐α in the development of neurological dysfunction. Increased levels of tumor necrosis factor‐α in the development of neurological dysfunction. Increased levels of tumor necrosis factor‐α messenger RNA were not associated with increased levels of IL1β messenger RNA, suggesting differential regulation of these monokines in acquired immunodeficiency syndrome dementia. Levels of IL‐4 messenger RNA were decreased in dementia and the loss of this and other macrophage downregulatory factors in demented patients may contribute to the increased expression of tumor necrosis factor‐α.
Phenotypic heterogeneity in families with the myoclonic epilepsy and ragged‐red fiber disease point mutation in mitochondrial DNA
Authors: Graf W. D. , Sumi S. M. , Copass M. K. , Ojemann L. M. , Longstreth W. T. , Shanske S. , Lombes A. , DiMauro S. 
Published: January 2005

AbstractTwo families with a point mutation in mtDNA associated with myoclonic epilepsy and ragged‐red fiber disease showed pronounced clinical heterogeneity. The mothers of the two families had adult‐onset myopathy with ragged‐red fibers, partial deficiency of cytochrome c oxidase, and sensory neuropathy. Members of the first family had variable clinical features of progressive ataxic‐myoclonic encephalomyopathy and of the other family, primarily adult‐onset myiopathy. There was a point mutation from A to G at uncleotide pair 8344 located in the tRNALys gene of the tmDNA of all patients tested, three in family 1, and the mother of Family 2.This clinical heterogeneity may reflect the effects of varying proportions of mutant and wild‐type mt DNA in the different organ systems in each individual.
Magnetic resonance imaging of brain and the neuromotor disorder in endemic cretinism
Authors: Ma Tai , Lian Z. C. , Qi S. P. , Heinz E. Ralph , DeLong G. Robert 
Published: January 2005

AbstractNeurological endemic cretinism, resulting from severe iodine deficiency, is characterized by mental deficiency, deafmutism, and a spastic–rigid motor disorder. Its neuropathology and pathophysiology have been investigated very little. We report the clinical and magnetic resonance imaging brain scan findings of 3 adult Chinese cretins. All show an apparent magnetic resonance imaging abnormality in the globus pallidus and substantia nigra, with hyperintensity on T1‐weighted images and hypointensity on T2‐weighted images. The motor abnormality, characterized by truncal and proximal limb–girdle rigidity and spasticity, with relative sparing of the hands and feet, is analogous to other extrapyramidal disorders. Endemic cretinism is a unique form of cerebral palsy of potential interest for students of human neuromotor development.
Adhesion molecule expression on cerebrospinal fluid T lymphocytes: Evidence for common recruitment mechanisms in multiple sclerosis, aseptic meningitis, and normal controls
Authors: Svenningsson Anders , Hansson Göran K. , Andersen Oluf , Andersson Rune , Patarroyo Manuel , Stemme Sten 
Published: January 2005

AbstractThe expression of T‐cell surface antigens was investigated in the cerebrospinal fluid (CSF) and peripheral blood of 11 patients with multiple sclerosis, 6 patients with aseptic meningitis, and 16 healthy subjects. A panel of monoclonal antibodies to adhesion and activation proteins was used in combination with an anti‐CD3 antibody in dual‐color flow cytometry. The problem of low cell numbers in the CSF from normal individuals was overcome by use of a modified staining procedure in microtiter plates, enabling analysis of as few as 5,000 cells. The majority of T cells in the CSF of the three patient groups exhibited the phenotype of memory cells (CD45RO+). CSF T cells also expressed significantly higher levels of several adhesion and activation molecules, including very late activation (VLA) antigens 3 through 6, lymphocyte function‐associated (LFA) antigen 1, LFA‐3, CD2, CD26, and CD44. Comparison between the different categories revealed that peripheral blood T cells from patients with multiple sclerosis expressed significantly lower amounts of the VLA integrins 4 and 5 as well as their common β subunit CD29, compared with normal control subjects. No differences between patients with multiple sclerosis and control subjects could, however, be seen regarding the distribution of memory/naive cells or CD4+/CD8+ cells in peripheral blood. Our data support a hypothesis that memory T cells with a high expression of several adhesion molecules are selectively recruited to the central nervous system compartment, under both pathological and normal conditions. We also provide evidence for an altered expression of adhesion molecules on peripheral blood T cells in patients with multiple sclerosis that is independent of the memory cell phenotype as defined by the expression of the CD45RO epitope.