bluuebunny/crossref_metadata_2025_split huggingface dataset:
Patterns of plasma levels of catechols in neurogenic orthostatic hypotension
Authors: Goldstein David S. , Polinsky Ronald J. , Garty Moshe , Robertson David , Brown Robert T. , Biaggioni Italo , Stull Robin , Kopin Irwin J. 
Published: January 2005

AbstractPatients with neurogenic orthostatic hypotension can have deficits in sympathetic neural function at any of several levels of the sympathetic neuraxis. We determined whether patterns of plasma levels of dopa, norepinephrine, dihydroxyphenylglycol, and dihydroxyphenylacetic acid would distinguish patients with orthostatic hypotension associated with multiple system atrophy, pure autonomic failure, or deficiency of dopamine‐β‐hydroxylase. Plasma levels of catechols were normal in most patients with multiple system atrophy, consistent with relatively intact peripheral sympathetic neurons; in contrast, most patients with pure autonomic failure had decreased levels of all four catechols, consistent with degenerative loss of sympathetic nerve endings. Patients with deficiency of dopamine‐β‐hydroxylase had increased levels of dopa and dihydroxyphenylacetic acid and markedly decreased levels of norepinephrine and dihydroxyphenylglycol, suggesting compensatory increases in sympathetic nerve activity in the absence of norepinephrine biosynthesis. Subgroups of patients with pure autonomic failure or multiple system atrophy had low levels of norepinephrine with normal levels of dopa, dihydroxyphenylglycol, and dihydroxyphenylacetic acid, consistent with normal catecholamine biosynthesis and decreased postganglionic sympathetic nerve traffic or decreased exocytotic release from sympathetic nerve endings. The results demonstrate the value of examining patterns of plasma levels of catechols to elucidate mechanisms of neurogenic orthostatic hypotension.
Peripheral neuropathy in amyotrophic chorea‐acanthocytosis
Authors: Vita G. , Serra S. , Dattola R. , Santoro M. , Toscano A. , Venuto C. , Carrozza G. , Baradello A. 
Published: January 2005

AbstractWe investigated involvement of the peripheral nervous system in 6 patients with amyotrophic chorea‐acanthocytosis. Electromyographic and neurographic findings, and pathological changes as demonstrated by examination of biopsy specimens of muscle and sural nerve indicate that most patients had an axonal sensorimotor polyneuropathy with more pronounced involvement of the distal portion of the nerves. Results obtained in one patient raised the question of an anterior horn cell disorder.
Neurological and neuropshychological manifestations of HIV‐1 infection: Association with AIDS‐related complex but not asymptomatic HIV‐1 infection
Authors: Janssen Robert S. , Saykin Andrew J. , Cannon Lyn , Campbell John , Pinsky Paul F. , Hessol Nancy A. , O'Malley Paul M. , Lifson Alan R. , Doll Lynda S. , Rutherford George W. , Kaplan Jonathan E. 
Published: January 2005

AbstractTo determine whether neurological and neuropsychological abnormalities are associated with clinical manifestations of human immunodeficiency virus type 1 (HIV‐1) infection in men who do not have acquired immunodeficiency syndrome (AIDS), we performed a historical prospective and cross‐sectional study. One hundred HIV‐1 seropositive homosexual or bisexual men, of whom 26 had AIDS‐related complex, 31 had generalized lymphadenopathy, and 43 had no signs or symptoms of HIV‐1 infection, and 157 HIV‐1 seronegative men were enrolled from a cohort of 6,701 men who were originally recruited between 1978 and 1980 for studies of hepatitis B virus infection. Evaluation included medical history, physical examination, and neuropsychological tests. Of 26 HIV‐1 seropositive subjects with AIDS‐related complex, 11 (42%) reported neurological, cognitive, or affective symptoms compared with 30 (19%) of 157 HIV‐1 seronegative subjects (relative risk ≈ 2.2, p = 0.02). On neuropsychological testing, subjects with AIDS‐related complex performed at a significantly lower level than the HIV‐1 seronegative group (p = 0.001). A significantly higher percentage of subjects with AIDS‐related complex (8[31%] of 26) than HIV‐1 seronegative subjects (19 [12%] of 157) had abnormal results on two or more neuropsychological tests (rate ratio = 2.5, p = 0.03). symptoms and impairment on neuropsychological tests were correlated only within the group who had AIDS‐related complex. Subjects with generalized lymphadenopathy and subjects who had no signs or symptoms of HIV‐1 infection were not different from HIV‐1 seronegative subjects with respect to symptoms or performance on neuropsychological tests. Absolute T‐helper lymphocyte count and estimated duration of HIV‐1 infection were not associated with neuropsychological test results. These findings suggest that neurological and neuropsychological abnormalities are associated with AIDS‐related complex but not with asymptomatic HIV‐1 infection.
Neuropathological changes in early HIV‐1 dementia
Authors: McArthur Justin C. , Becker P. Scott , Parisi Joseph E. , Trapp Bruce , Selnes Ola A. , Cornblath David R. , Balakrishnan Janaki , Griffin John W. , Price Donald 
Published: January 2005

AbstractEarly pathological abnormalities in human immunodeficiency virus (HIV‐1)–related dementia have not been well documented. We report a homosexual man with fatigue and intermittent diarrhea in whom early HIV‐1–related dementia was demonstrated during neurological screening in the Multicenter AIDS Cohort Study. Within 4 months he died of massive epistaxis, and the brain revealed astrocytosis of white matter and mild pallor of myelin staining in the absence of inflammation, multinucleated giant cells, and brain atrophy.
Divisional oculomotor nerve paresis caused by intrinsic brainstem disease
Authors: Ksiazek Susan M. , Repka Michael X. , Maguire Albert , Harbour Robert C. , Savino Peter J. , Miller Neil R. , Sergott Robert C. , Bosley Thomas M. 
Published: January 2005

AbstractTwo patients with findings ascribable to superior branch oculomotor nerve paresis and one patient with findings ascribable to an inferior branch paresis are described. In each patient the paresis was caused by an intra‐axial midbrain lesion. These patients represent the first described cases of divisional oculomotor nerve paresis caused by intrinsic brainstem disease.
Molecular mimicry and lyme borreliosis: A shared antigenic determinant between Borrelia burgdorferi and human tissue
Authors: Aberer E. , Brunner C. , Suchanek G. , Klade H. , Barbour A. , Stanek G. , Lassmann H. 
Published: January 2005

AbstractThe pathogenesis of chronic manifestations in Lyme borreliosis, a disease induced by Borrelia burgdorferi, is at present unresolved. By testing monoclonal antibodies directed against various borrelia antigens, we found an antigenic determinant shared by the 41 kDa flagella protein and human tissue, especially prominent on myelinated fibers of human peripheral nerve, on nerve cells and axons of the central nervous system, as well as on certain epithelial cells (including joint synovia) and on heart muscle cells. Immune reactions against such a shared antigen could play a pathogenetic role in chronic organ manifestations of Lyme borreliosis.
Central pontine myelinolysis and pontine lesions after rapid correction of hyponatremia: A prospective magnetic resonance imaging study
Authors: Brunner John E. , Redmond Janice M. , Haggar Allan M. , Kruger Davida F. , Elias Stanton B. 
Published: January 2005

AbstractThe rate at which profound hyponatremia should be corrected is the focus of a recent clinical debate. We prospectively studied neurological outcomes with serial magnetic resonanace imaging in 13 hyponatremic subjects with serum sodium concentrations of less than 115 mmol/L (mean initial serum sodium concentration, 103.7; range, 93–113 mmol/L). All subjects were corrected to mildly hyponatremic levels at 24 hours and ultimately underwent an increase in serum sodium concentration of 25 mmol/L without development of hypernatremia. Magnetic resonance imaging revealed the development of pontine lesions in 3 patients. The correction rate of hyponatremia over the first 24 hours was significantly faster in patients with pontine lesions (mean ± SD, 1.25 ± 0.4 mmol/(L ± hr) versus 0.74 ± 0.3 mmol/(L ± hr); ±<0.05). Initial sodium concentration was also significantly lower in the pontine lesion group (97.3 ± 6.7 vs 105.6 ± 5.2 mmol/L,p ± <0.05). We conclude that the correction rate of hyponatremia plays a significant role in the pathogenesis of pontine lesions in individuals with profound hyponatremia who undergo large increases in sodium concentration as a result of severe initial hyponatremia.
Hyponatremia is associated with cerebral ischemia in patients with aneurysmal subarachnoid hemorrhage
Authors: Hasan Djo , Wijdicks Eelco F. M. , Vermeulen Marinus 
Published: January 2005

AbstractThe association between hyponatremia and cerebral ischemia was investigated in a consecutive series of 208 patients with subarachnoid hemorrhage who had a daily fluid intake of at least 3 L and in whom fluid restriction, to correct hyponatremia, was not applied. Hyponatremia occurred in 70 (34%) of the 208 patients. The occurrence of cerebral ischemia in patients with hyponatremia, 17 (24%) of 70 patients, was significantly higher than in patients without hyponatremia, 17 (12%) of 138 patients (χ2 = 4.028, p = 0.045). Cerebral ischemia was not more often fatal in hyponatremic patients than in patients without hyponatremia. We conclude that patients with hyponatremia are at increased risk of developing cerebral ischemia even if fluid restriction is not applied.
The diagnosis and treatment of cerebral mycotic aneurysms
Authors: Brust John C. M. , Dickinson P. C. Taylor , Hughes James E. O. , Holtzman Robert N. N. 
Published: January 2005

AbstractSeventeen patients were treated for 28 documented cerebral mycotic aneurysms. Initial neurological symptoms were attributable to aneurysm rupture in only 7 patients, and in 3 of them symptoms did not suggest subarachnoid hemorrhage. Six patients presented with embolic infarction and 1 with meningitis; in 3 patients it was uncertain if aneurysm rupture occurred. Four patients had rupture of at least one aneurysm while receiving appropriate antibiotic treatment and another had rupture at the conclusion of therapy. Of 20 aneurysms followed angiographically or with computed tomography during medical treatment, 10 became smaller or disappeared and 10 remained unchanged or enlarged, 1 with fatal rupture. Eight ruptured aneurysms were surgically excised; 2 of the patients with ruptured aneurysms died and 2 had residual aphasia or cognitive impairment. All 4 patients whose only surgery was for an unruptured aneurysm made uneventful recoveries. Recognizing the retrospective and anecdotal nature of our data and the differing views of previous investigators, we recommend: (1) that careful neurological examination, computed tomography, and (unless contraindicated) lumbar puncture be performed on any patient with endocarditis; (2) that those with neurological abnormalities not attributable to systemic toxicity, including pleocytosis in the cerebrospinal fluid or apparent infarction on computed tomographic scans, undergo four‐vessel cerebral angiography; (3) that single accessible mycotic aneurysms in medically stable patients be promptly excised, with individualization of multiple or proximal aneurysms; and (4) that repeat angiography be performed at the conclusion of antibiotic therapy in patients requiring long‐term anticoagulation. Our data do not allow us to predict whether performing repeat angiography on all neurologically abnormal patients or initial angiography on all endocarditis patients would do more harm than good.
Mitogen accumulation in von Recklinghausen neurofibromatosis
Authors: Ratner Nancy , Lieberman Michael A. , Riccardi Vincent M. , Hong Dingming 
Published: January 2005

AbstractMost, but not all, patients with von Recklinghausen neurofibromatosis develop tumors (neurofibromas) that contain large numbers of Schwann cells and fibroblasts. To begin to understand the molecular events that contribute to cell proliferation in these benign tumors, we have analyzed extracts of neurofibromas to determine whether they contain mitogens for Schwann cells or fibroblasts, or both. Schwann cell and fibroblast mitogens are present in neurofibroma extracts. All the neurofibromas analyzed contain a Schwann cell mitogen similar to a neuronal cell surface molecule known to stimulate Schwann cell proliferation during normal development; this mitogen also stimulates fibroblast proliferation. Basic fibroblast growth factor is present in 60% of tumors evaluated. Accumulation of mitogenic substances may contribute to the growth of neurofibromas.
A long‐term prospective study of optic neuritis: Evaluation of risk factors
Authors: Sandberg‐Wollheim Magnhild , Bynke Hans , Cronqvist Sten , Holtås Stig , Platz Per , Ryder Lars P. 
Published: January 2005

AbstractEighty‐six patients with monosymptomatic optic neuritis of unknown cause were followed prospectively for a median period of 12.9 years. At onset, cerebrospinal fluid (CSF) pleocytosis was present in 46 patients (53%) but oligoclonal immunoglobulin in only 40 (47%) of the patients. The human leukocyte antigen (HLA)‐DR2 was present in 45 (52%). Clinically definite multiple sclerosis (MS) was established in 33 patients. Actuarial analysis showed that the cumulative probability of developing MS within 15 years was 45%. Three risk factors were identified: low age and abnormal CSF at onset, and early recurrence of optic neuritis. Female gender, onset in the winter season, and the presence of HLA‐DR2 antigen increased the risk for MS, but not significantly. Magnetic resonance imaging detected bilateral discrete white matter lesions, similar to those in MS, in 11 of 25 patients, 7 to 18 years after the isolated attack of optic neuritis. Nine were among the 13 with abnormal CSF and only 2 belonged to the group of 12 with normal CSF (p = 0.01). Normal CSF at the onset of optic neuritis conferred better prognosis but did not preclude the development of MS.
Immunocytochemical and ultrastructural studies of Pick's disease
Authors: Murayama Shigeo , Mori Hiroshi , Ihara Yasuo , Tomonaga Masanori 
Published: January 2005

AbstractCerebral cortical changes in 10 cases with Pick's disease were studied immunocytochemically and ultrastructurally. All cases contained Pick's argentophilic bodies and ballooned neurons. The antibodies against phosphorylated tau proteins that intensely stained all Pick bodies recognized numerous neuronal processes around Pick body–bearing cells and focal portions in the perikarya of ballooned neurons. Monoclonal and polyclonal anti‐ubiquitin antibodies stained not only some Pick bodies with variable intensity, but also the perikarya of all ballooned neurons. Ultrastructurally, Pick bodies consisted of accumulation of randomly oriented, approximately 15‐nm straight filaments and paired twisted profiles with a minimal diameter of 13 nm, maximal diameter of 26 nm, and twist periodicity of 120 nm. These Pick body–type filaments were also observed in the perikarya of ballooned neurons and neuronal processes around Pick body–bearing cells. Our studies demonstrate, for the first time, the characteristic pathological feature of neuropil in Pick's disease. Pick body–bearing cells and ballooned neurons show unique immunocytochemical and ultrastructural properties that may be a clue to the pathogenesis of Pick's disease.
Normal pressure hydrocephalus associated with cauda equina neurinoma
Authors: Ohta Kouichi , Gotoh Fumio , Amano Takahiro , Obara Katsuyuki 
Published: January 2005

AbstractA 65‐year‐old woman with normal pressure hydrocephalus experienced improved gait and hydrocephalus following surgical resection of a cauda equina neurinoma. Fibrinogen, detected in the cerebrospinal fluid, may be involved in the pathophysiological process of normal pressure hydrocephalus.
Peduncular hallucinosis associated with isolated infarction of the substantia nigra pars reticulata
Authors: McKee A. C. , Levine D. N. , Kowall N. W. , Richardson E. P. 
Published: January 2005

AbstractWe report the clinicopathological study of an 83‐year‐old man who abruptly developed complex visual hallucinations, disordered sleep, and mild cognitive impairment that persisted until his death 6 months later. Postmortem neuropathological examination including serial sections through the diencephalon, midbrain, and pons disclosed isolated bilateral infarcts confined to the medial substantia nigra pars reticulata. The findings suggest that destruction of the pars reticulata may be the essential feature to the development of peduncular hallucinosis.
Polyclonal IgM anti‐GM1 ganglioside antibody in patients with motor neuron disease and variants
Authors: Salazar‐Grueso Edgar F. , Routbort Mark J. , Martin Jay , Dawson Glyn , Roos Raymond P. 
Published: January 2005

AbstractRecent studies reported the presence of anti‐ganglioside antibodies in occasional patients with motor neuron disease. We found polyclonal serum IgM anti‐GM1 antibodies by an anti‐GM1 enzyme‐linked immunosorbent assay (ELISA) in 9 (19%) of 48 patients with motor neuron disease. A comparable frequency of IgM anti‐GM1 antibodies was found in 4 (10%) of 40 sera from patients with other neurological disease. Three (17%) of 18 sera from the patients with motor neuron disease and 2 (17%) of 12 sera from patients with other neurological diseases had anti‐GM1 immunostaining as shown by thin layer chromatography immunoblot. One patient with a lower motor neuron variant of motor neuron disease or motor axonopathy without multifocal conduction block had a markedly elevated polyclonal IgM anti‐GM1 ELISA titer (> 1:64,000) with prominent immunostaining of GM1, moderate immunostaining of GM2, and weak and inconsistent immunostaining of GD1b by thin layer chromatography immunoblot. Treatment with prednisone resulted in clinical improvement despite increasing anti‐GM1 antibody titers. These data indicate that patients with motor neuron disease have measurable levels of anti‐ganglioside antibodies as frequently as patients with other neurological diseases. This contrasts with a small subgroup of patients with a lower motor neuron variant of motor neuron disease or motor axonopathy who have markedly elevated levels of serum anti‐ganglioside antibodies and a clinical syndrome that is treatable with immunosuppression.
Prevalence of primary Sjögren's syndrome in patients with multiple sclerosis
Authors: Miró J. , Peña‐Sagredo J. L. , Berciano J. , Insúa S. , Leno C. , Velarde R. 
Published: January 2005

AbstractSixty‐four consecutive patients with clinically or laboratory‐supported definite multiple sclerosis (MS) were evaluated prospectively for evidence of primary Sjögren's syndrome (SS). This diagnosis was established when a patient had objective keratoconjunctivitis sicca, xerostomia, or both together with positive labial salivary gland biopsy. We found 2 patients (3.1%) with clinical evidence of primary SS. Whether this association is fortuitous or whether there is pathogenetic linkage between MS and primary SS remains to be established.
Glutamate dysfunction and selective motor neuron degeneration inamyotrophic lateral sclerosis: A hypothesis
Authors: Plaitakis Andreas 
Published: January 2005

AbstractRecent studies provided evidence for a generalized defect in glutamate metabolism in patients with amyotrophic lateral sclerosis, associated with widespread alterations in the central nervous system levels of this excitatory amino acid putative transmitter. Present data support the hypothesis that altered presynaptic glutamatergic mechanisms may be responsible for a neuroexcitotoxic cell loss in this disorder. High local concentrations of glycine, released from glycinergic terminals, may disrupt adaptive processes contributing to abnormal potentiation of excitatory transmission mediated by glutamate receptors and resultant selective degeneration of motor neurons. These considerations offer new therapeutic strategies for amyotrophic lateral sclerosis.
The effect of mild hyperthermia and hypothermia on brain damage following 5, 10, and 15 minutes of forebrain ischemia
Authors: Minamisawa Hiroaki , Smith Maj‐Lis , Siesjö Bo K. 
Published: January 2005

AbstractThe present study examines the effects of mild hypothermia and hyperthermia on the density and distribution of ischemic brain damage, and compares these effects to those induced by variations in the duration of ischemia. Body temperatures were maintained at 35°C, 37°C, and 39°C before, during, and after ischemia, and brain temperatures were held at similar values with a technique that in preliminary experiments proved to avoid intracerebral temperature gradients or overheating of surface structures. In all animals, brain damage was assessed by histopathological analysis of perfusion‐fixed brains after six to seven days of recovery. Our results confirm previous findings showing that a decrease in temperature of only 2°C significantly reduces damage to several selectively vulnerable neuronal populations. The results also showed that an increase in temperature of 2°C significantly enhances brain damage. In general, a rise in temperature had effects similar to an increased duration of the ischemia. In some areas, such as the CA1‐subiculum sectors of the hippocampus, temperature and ischemic duration altered damage in a gradual manner, but in others, such as the caudoputamen, there was a steplike change from virtually no to virtually complete damage. In some areas, the effects of hypothermia and hyperthermia appeared symmetrical around the normal temperature of 37°C. Hyperthermia had some seemingly “specific” effects, however, notably the tendency to induce pannecrosis (“infarction”) in the neocortex and caudoputamen, and to cause damage to the substantia nigra pars reticulata. The results underscore the potentially devastating effects of fever in patients with cerebrovascular disease.
A computer simulation of conduction block: Effects produced by actual block versus interphase cancellation
Authors: Rhee Edward K. , England John D. , Sumner Austin J. 
Published: January 2005

AbstractA reduction in compound muscle action potential (CMAP) amplitude and area following proximal versus distal stimulation is the accepted clinical hallmark of conduction block; however, quantitative criteria for determining conduction block remain ambiguous. In this study, digitized records of individual motor unit action potentials (MUAPs) elicited by incremental stimulation in vivo were arithmetically combined in a computer simulation of CMAP generation. Through simulation of possible phase interaction patterns of individual MUAPs, we have shown that abnormal temporal dispersion alone can produce reductions in CMAP area of up to 50%, values that are commonly thought to represent conduction block. Furthermore, by simulating conduction block without excessive temporal dispersion in defined subpopulations of axons, we have demonstrated the importance of the fastest conducting (largest MUAP) axons in determining CMAP amplitude and area. In conclusion, measurements of CMAP amplitude and area in determining conduction block may be misleading if there is significant abnormal temporal dispersion, and quantitation of the degree of conduction block is difficult without knowledge of which subpopulations of axons are affected.