bluuebunny/crossref_metadata_2025_split huggingface dataset:
The Golgi apparatus of moptor neurons in amyotrophic lateral sclerosis
Authors: Mourelatos Zissims , Yachnis Anthony , Rorke Lucy , Mikol Jacqueline , Gonatas Nicholas K. 
Published: January 2005

AbstractThe Golgi apparatus plays a key role in the posttranslational processing of ploypeptides destined for secretion, incorporation into plasma memberanes, and fast axoplasmic transport. Dispersion of fragmentation of the Golgi apparatus, experimentally induced by microtuble‐disrupting agents, is associated with decreased secretion of immunoglobulins and insulin. The golgi apparatus is also involved in targeting of lysosomal enzymes and in the endocytosis of certain hormones, receptors, and toxins. There is a paucity of information on this important organelle in human neuropathological conditions. Using an organelle‐specific antiserum we have examined by immunocytochemistry the Golgi apparatus of motor neurons in the spinal cord in 4 patients with amyotrophic lateral sclerosis and 1 patient with Werding Hoffmann's disease, I with infantile neuronal degeneration, 1 with adult‐type familial bulbospinal atrophy, I with motochondrial myopathy with cytochrome c oxidase deficiency, 1 with centronuclear myopathy, and 1 with Duchenne'smuscular dystrophy, and in 9 age‐matched control subjects. In all motor neuronopathies examined and in the patient with mitochondrial myopathy, 20 to 85% of neurons counted had “fragmented” golgi apparatus. In age‐matched control subjects and the other 2 patients with myopathies, 0 to 1.65% of motor neurons had fragmented Golgi apparatus. These findings suggest that the Golgi apparatus of motor neurons is involved in patients with amyotrophic lateral sclerosis and relation motor neuron diseases, and perhaps in patients with certain fatal primary myopathies.
Epidemiology of multiple sclerosis in U.S. veterans: V. Ancestry and the risk of multiple sclerosis
Authors: Page William F. , Durtzke John F. , Murphy Frances M. , Norman James E. 
Published: January 2005

AbstractSelf‐reported ancestry data for the U.S. population from the 1980 decennial census and multiple sclerosis (MS) risk data derived from a large series of World War II white male veterans with MS and matched controls were aggregated on a state level and analyzed to determine the relationship between ancestry and MS risk. A significant portion of the state‐by‐state variation in MS risk is explainable statistically by differences in ancestry among state populations, even when geographic latitude is included in analyses. In the main, Swedish and other Scandinavian ancesty is most consistently associated with places with increased MS risk. In some analyses, Italian, French, and (to a lesser extent) Scottish ancestries are also associated with increased risk, whereas English and Dutch ancestries are each associated with decreased risk, but most of these non‐Scandinavian correlations may effect predomininatly geography per se. These findings provide evidence that ancestry of the resident population, a confounded measure of genetic susceptibility and cultural evironment, is part of the complicated picture of MS as a disease of place.
A positron emission tomography study of essential tremor: Evidence for overactivity of cerebellar connections
Authors: Jenkins I. H. , Bain P. G. , Colebatch J. G. , Thompson P. D. , Findley L. J. , Frackowiak R. S. J. , Marsden C. D. , Brooks D. J. 
Published: January 2005

AbstractThe origin of essential tremor is unknown. Animal models have suggested that the inferior olivary nucleus may act as a tremor generator. We used positron emission tomography to study changes in regional cerebral blood flow associated with involuntary postural tremor and passive wrist oscillation in patients with essential tremor. Activation due to voluntary wrist oscillation and arm extension without tremor was studied in normal control subjects. The essential tremor group had bilaterally increased cerebellar blood flow at rest (without tremor) compared with the control group. Involuntary postural tremor was associated with further bilateral cerebellar activation, and also contralateral striatal, thalamic, and sensorimotor cortex activation. Voluntary wrist oscillation, maintained arm extension without tremor, and passive wrist oscillation were all associated with significant ipsilateral rather than bilateral cerebellar activation. We conclude that essential tremor is associated with increased bilateral cerebellar activity both at rest and during tremor.
The spectrum of immune responses to Campylobacter jejuni and glycoconjugates in Guillain‐Barré syndrome and in other neuroimmunological disorders
Authors: Enders Uwe , Karch Helge , Toyka Klaus V. , Michels Marco , Zielasek Jürgen , Pette Martin , Heesemann Jürgen , Hartung Hans‐Peter 
Published: January 2005

AbstractAn acute infectious illness frequently precedes the Guillain‐Barré syndrome. Recently, Campylobacter jejuni was claimed to be a predominant precipitating agent that may also trigger a humoral immune response to glycoconjugates of peripheral myelin in Guillain‐Barré syndrome. Because of conflicting reports, we determined the frequency of a recent infection with C. jejuni in 38 patients with Guillain‐Barré syndrome using a highly sensitive and specific immunoblot technique, and of the presence of circulating antibodies to gangliosides. We detected IgM and/or IgG C. jejuni directed antibodies in 15 of 38 patients with Guillain‐Barré syndrome. In contrast, only 7 of 39 healthy control subjects, 3 of 20 patients with multiple sclerosis, and 2 of 72 patients with neuroborreliosis showed IgA or IgM antibody responses to C. jejuni. In Guillain‐Barré syndrome, C. jejuni–specific antibodies were predominantly directed to outer membrane proteins of one specific serotype, Lior 11, whereas the most common serotype associated with enteritis in Germany is Lior 4. Two of 27 patients with Guillain‐Barré syndrome had ganglioside‐specific IgA antibodies; 1 of 32 patients, antibodies of IgM; and 4 of 31 patients, antibodies of IgG class. There was no correlation between severity, type (axonal versus demyelinating), and outcome of the disease and the presence or absence of a humoral immune response to C. jejuni or to glycoconjugates. Our findings do not support previous suggestions that a preceding C. jejuni infection heralds a poorer outcome or that antibodies to gangliosides carry prognostic significance. The predilective humoral immune response to outer membrane proteins of one specific C. jejuni serotype raises new important questions concerning the immunopathogenic link between an acute C. jejuni infection and subsequent Guillain‐Barré syndrome.
Inhibition of experimental autoimmune encephalomyelitis by an antibody to the intercellular adhesion molecule ICAM‐1
Authors: Archelos Juan J. , Jung Stefan , Mäurer Mathias , Schmied Mascha , Lassmann Hans , Tamatani Takuya , Miyasaka Masayuki , Toyka Klaus V. , Hartung Hans‐Peter 
Published: January 2005

AbstractExperimental autoimmune encephalomyelitis (EAE) was induced in Lewis rats by active immunization with myelin from guinea pig spinal cord by the encephalitogenic myelin basic protein or by adoptive transfer using myelin basic protein‐specific CD4‐positive T cells. Treatment with purified monoclonal antibody (1A‐29) to the intercellular adhesion molecule‐1 and its F(ab')2 fragments efficiently suppressed active EAE. Control treatment with an irrelevant antibody or saline did not alter the course of the disease. Histological sections of the central nervous system showed a pronounced reduction of inflammatory infiltrates during treatment with antibody to intercellular adhesion molecule‐1. In the adoptive transfer model of EAE, 1A‐29 had only a minor effect. Proliferation assays on lymph node cells ex vivo from 1A‐29– and saline‐treated animals were performed. Administration of 1A‐29 suppressed antigen‐specific T‐cell proliferation. The differential effects in EAE versus adoptive transfer EAE suggest that 1A‐29 acts predominantly on the induction phase of the immune response and, to a lesser extent, on the transendothelial migration of T cells. We conclude that intercellular adhesion molecule‐1–dependent pathways are critically involved in the pathogenesis of EAE and that antibodies to leukocyte adhesion molecules could be a novel therapeutic approach to autoimmune disease of the central nervous system.
Abnormal eye movements in Creutzfeldt–Jakob disease
Authors: Grant Michael P. , Cohen Mark , Petersen Robert B. , Halmagyi G. Michael , McDougall Alan , Tusa Ronald J. , Leigh R. John 
Published: January 2005

AbstractWe report 3 patients with autopsy‐proven Creutzfeldt–Jakob disease who, early in their course, developed abnormal eye movements that included periodic alternating nystagmus and slow vertical saccades. These findings suggested involvement of the cerebellar nodulus and uvula, and the brainstem reticular formation, respectively. Cerebellar ataxia was also an early manifestation and, in 1 patient, a frontal lobe brain biopsy was normal at a time when ocular motor and cerebellar signs were conspicuous. As the disease progressed, all saccades and quick phases of nystagmus were lost, but periodic alternating gaze deviation persisted. At autopsy, 2 of the 3 patients had pronounced involvement of the cerebellum, especially of the midline structures. Creutzfeldt‐Jakob disease should be considered in patients with subacute progressive neurological disease when cognitive changes are overshadowed by ocular motor findings or ataxia.
Human immunodeficiency virus type 1 infections and myopathy: Clicical relevance of zidovudine therapy
Authors: Grau Josep M. , Masanés Ferrán , Pedrol Enric , Casademont Jordi , Fernández‐Solá Joaquim , Urbano‐Márquez Alvaro 
Published: January 2005

AbstractFifty consecutive patients infected with human immunodeficiency virus type 1 (HIV‐1) were evaluated regarding the prevalence of HIV‐related myopathies and the relevance of zidovudine‐related mitochondrial myopathy. Disease stage, total lifetime intake of zidovudine, anthropometric and nutritional parameters, muscle strength, and histochemical and immunohistochemical findings in muscle specimens were recorded. The series was divided into two groups, patients with a total lifetime intake of zidovudine under 200 gm and those with a total lifetime intake over 200 gm. A control group included 50 healthy people matched for age and sex. HIV‐related myopathy was defined by the presence of at least one of the classic pathological reactions in muscle, while idovudine‐related myopathy was defined by the presence of raged red fibers in any percentage. Lower values of the nutritional parameters were detected in the HIV cohott, when compared with normal control values. HIV‐related myopathy was detected in 13 (26%) of the 50 patients. There were no differneces between groups except for the development of mitochondrial myopathy that occurred in 1 of the 26 patients in Group I and in 16 of the 24 in Group II. Six patients who had a total intake of more than 200 gm of zidovudine and demonstrated red ragged fibers in their muscle speciments were absolutely asymptomatic. There was a positive correlation between total intake of zidovudine and the percentage of red ragged fibers in muscle biopsy specimens.
Human positron emission tomographic [18F]Fluorodopa studies correlate with dopamine cell counts and levels
Authors: Snow B. J. , Tooyama I. , McGeer E. G. , Yamada T. , Calne D. B. , Takahashi H. , Kimura H. 
Published: January 2005

AbstractPostmortem counts of dopaminergic cell densities in the substantia nigra (5 subjects) and striatal levels of dopamine (DA) and its metabolites (6 subjects) were determined on 1 parkinsonian (PD), 3 progressive supranuclear palsy (PSP), 1 amyotrophic lateral sclerosis, and 1 Alzheimer's case who had been positron emission tomography scanned with 6‐[18F]fluorodopa during life. [18F]Fluorodopa uptake rate constants, which presumably depend on the number of functioning striatal DA terminals, were strictly proportional to cell densities (significant correlation with zero intercept) and also correlated significantly with striatal DA levels but with an intercept indicating greater losses of DA than of terminals in PSP and PD. Postmortem data on 6 PD, 1 PSP, and 9 neuronally normal controls substantiated the significant correlation between cell counts and DA levels, with the latter being the more depressed in pathological cases.
Functional integration of cortical grafts placed in brain infarcts of rats
Authors: Grabowski M. , Brundin P. , Johansson B. B. 
Published: January 2005

AbstractFive to 6 days after a right middle cerebral artery occlusion, a cell suspension of fetal neocortex was grafted into the infarcted area of adult spontaneously hypertensive rats. Three to 17 months later, functional integration of the grafts into the afferent somatosensory pathway was tested using the 2‐[14C]deoxyglucose method for estimation of glucose utilization. Grafted rats (n = 8) and control rats (n = 5) with no arterial occlusion were stimulated in the left vibrissal region resulting in an increased glucose utilization in the left trigeminal sensory nucleus and the right ventroposterior nucleus of the thalamus, whereas the same regions in a group (n = 5) of nonstimulated grafted rats were not activated. Glucose uptake in the right somatosensory cortex of control rats was 96 ± 5 (mean ± SEM) μmol/100 gm/min. Neocortical grafts consumed less glucose than cortex in control rats but the vibrissae‐stimulated group displayed a 110% higher value than the nonstimulated grafted group (32 ± 5 vs 15 ± 2, p < 0.05). We conclude that graft glucose metabolism is increased following stimulation of the host somatosensory pathway, which demonstrates that transplanted neurons can be functionally integrated with neural circuitries of the host after an ischemic insult.
The T‐cell response to myelin basic protein in familial multiple sclerosis: Diversity of fine specificity, restricting elements, and T‐cell receptor usage
Authors: Joshi Narendra , Usuku Koichiro , Hauser Stephen L. 
Published: January 2005

AbstractIndirect evidence suggests that an autoimmune response to myelin basic protein (MBP) may be involved in the pathogenesis of multiple sclerosis (MS). In MS, several reports have suggested that restricted T‐cell populations respond to MBP, as in inbred rodents with the MS disease model experimental allergic encephalomyelitis. In experimental allergic encephalomyelitis, the T‐cell repertoire to MBP varies between strains, and in MS it is likely that the response to MBP is also best defined under conditions where genetic differences between subjects are controlled. In this report, the fine specificity of the T‐cell response to MBP was assessed in three families, each with multiple individuals affected with MS. We found that (1) comparable frequencies of MBP‐reactive T‐cell lines were obtained from peripheral blood of MS patients and their healthy siblings. Human leukocyte antigen (HLA) identical sibling pairs discordant for MS had similar frequencies of MBP‐reactive T‐cell lines. (2) A broad spectrum of MBP epitopes was recognized by T‐cell lines from all individuals studied. Within a family, the fine specificity of MBP recognition showed little or no overlap between individuals, even between HLA identical siblings. (3) Recognition of MBP epitopes occurred in the context of different HLA class II alleles. At least four DR alleles each served as restricting elements for recognition of P82—101 or the carboxy terminal region of MBP, two regions thought to be important in the human T‐cell response to the molecule. No relationship between the use of a particular DR allele and a response to a particular region of MBP could be established. (4) Apparently random expression of different T‐cell receptor Vβ gene families by MBP‐reactive T‐cell lines was present, with little overlap by different lines reponding to the same region of MBP, or between different individuals belonging to the same family. These results demonstrate that the frequency and fine specificity of T‐cell responses to MBP in peripheral blood is independent of the MS phenotype.
Failure of 3,4‐diaminopyridine to reverse conduction block in inflammatory demyelinating neuropathies
Authors: Bergin Peter S. , Miller David H. , Hirsch Nicholas P. , Murray Nicholas M. F. 
Published: January 2005

Abstract3,4‐Diaminopyridine was administered to 6 patients with inflammatory demyelinating neuropathies in whom partial conduction block was demonstrable. Four had Guillain‐Barré syndrome and 2 had chronic inflammatory demyelinating neuropathy. Nerve conduction studies were performed before the administration of a single oral dose of 3,4‐diaminopyridine, and at regular intervals thereafter. Neither resolution of conduction block nor clinical benefit were seen.
Positron emission tomographic imaging of the dopamine transporter with 11C‐WIN 35,428 reveals marked declines in mild Parkinson's disease
Authors: Frost J. James , Rosier Annemie J. , Reich Stephen G. , Smith Justin S. , Ehlers Michael D. , Snyder Solomon H. , Ravert Hayden T. , Dannals Robert F. 
Published: January 2005

AbstractParkinson's Disease (PD) is characterized by a selective loss of nigrostriatal dopaminergic neurons that results in a marked reduction of dopaminergic nerve terminals in the striatum. Recently, 11C‐WIN 35,428, a cocaine analogue that specifically labels the dopamine transporter, was developed and can be used to label dopaminergic nerve terminals in vivo by positron emission tomography. In healthy control subjects, binding of 11C‐WIN 35,428 is highest in the striatum. In addition, 2 symmetrical focal areas of low binding were observed in the midbrain. The cerebellum functioned as an appropriate region for nonspecific binding. The binding of 11C‐WIN 35,428 in patients with PD (Hoehn‐Yahr II) was compared with that in healthy control subjects by using the (region‐cerebellum)/cerebellum ratio for data acquired 34 to 82 minutes after injection. In control subjects, this ratio varied, at approximately 2, in the striatum. In patients with PD, binding in the posterior putamen was reduced by 78%, whereas the anterior putamen and the caudate nucleus showed a reduction of 59 and 39%, respectively. The reduction in 11C‐WIN 35,428 binding was highest in the midbrain (84%). The high specific/nonspecific binding ratio and the pronounced reduction in binding in mild PD may permit detection of even earlier stages of PD or presymptomatic individuals with dopaminergic cell loss.
Ictal single‐photon emission computed tomography demonstrates localized epileptogenesis in cortical dysplasia
Authors: Kuzniecky Ruben , Mountz James M. , Wheatley Gregory , Morawtz Richard 
Published: January 2005

AbstractWe investigated the use of ictal single‐photon emission computed tomography (SPECT) in 2 children with intractable epilepsy. Ictal scalp electroencephalographic studies failed to localize the epileptic focus and interictal data demonstrated widespread lateralized abnormalities. Ictal SPECT studies with technetium 99m–hexamethylpropyleneamineoxime demonstrated focal areas of hyperperfusion in the frontal lobes. Resection of the abnormal areas shown by SPECT was carried out despite evidence of large areas of epileptogenesis demonstrated by scalp and subdural electroencephalographic studies. Pathological material revealed cortical dysplasia. Ictal SPECT provides functional evidence for localized epileptogenesis in focal cortical dysplasia.
Polysomnographic sleep measures in Parkinson's disease patients with treatment‐induced hallucinations
Authors: Comella Cynthia L. , Tanner Caroline M. , Ristanovic Ruzica K. 
Published: January 2005

AbstractPrior studies of sleep in Parkinson's disease (PD) have been compromised by inadequate comparison groups, mixed medication regimens, and absence of quantitative data collection. This is the first study to compare polysomnographic sleep measures in PD patients on only dopaminergic medications with and without hallucinations. We performed two consecutive nights of polysomnography in 10 nondepresed, nondemented PD patients, 5 with and 5 without hallucinations. All patients were being treated with carbidopa/levodopa and a dopaminergic agonist only. Hallucinators and nonhallucinators were group‐matched for age, PD duration, severity, and medication doses. Both groups had abnormal sleep records. In particular, there was a reduction in K‐complexes and spindle formation, and the frequent occurrence of motor activation during rapid eye movement (REM) sleep consistent with REM behavior disorder. The hallucinator group had a significantly lower sleep efficiency (0.25 in hallucinators vs 0.61 in nonhallucinators, p = 0.006), a reduced total REM sleep time (mean total REM sleep time, 3 minutes in hallucinators vs 50 in nonhallucinators; p = 0.005), and a reduced REM percentage (mean, 5% in hallucinators vs 20% in nonhallucinators; p = 0.011). This study demonstrates that advanced PD patients treated with dopaminergic agents have abnormal sleep patterns and that those with dopaminergic‐induced hallucinations have significantly greater REM aberrations than nonhallucinating PD patients.
Unverricht‐Lundborg disease: Absence of nonallelic genetic heterogeneity
Authors: Cochius J. I. , Figlewicz D. A. , Kälviäinen R. , Nousiainen U. , Farrell K. , Patry G. , Söderfeldt B. , Frydman M. , Lerman P. , Andermann F. , Andermann E. , Rouleau G. A. 
Published: January 2005

AbstractUnverricht‐Lundborg disease is a clinically recognizable form of progressive myoclonus epilepsy. Recently, in several families of both Finnish and Mediterranean extraction segregating Unverricht‐Lundborg disease, the gene for this disease was linked to the same region of the long arm of chromosome 21. We performed linkage analysis in eight families, including four of neither Baltic nor Mediterranean origin, using a polymorphic (CA)n repeat marker for the human liver‐type 6 phosphofructokinase (PFKL) gene, previously mapped to 21q22.3. No recombinations were observed between the disease phenotype and the PFKL marker and a maximum lod score of 5.63 was obtained. These findings confirm tight linkage between PFKL and the gene for Unverricht‐Lundborg disease and strongly suggest a lack of nonallelic genetic heterogeneity of the disease.
Significance of reactive lyme serology in multiple sclerosis
Authors: Coyle P. K. , Krupp Lauren B. , Doscher Carol 
Published: January 2005

AbstractNineteen of 283 consecutive patients evaluated in the Multiple Sclerosis (MS) Comprehensive Care Center had a borderline or positive B. burgdorferi serology. In 8 patients a repeat serology was nonconfirmatory. Cerebrospinal fluid was examined in 10 seropositive patients and showed anti‐B. burgdorferi antibodies without intrathecal production in 5 patients. Antibiotic treatment did not prevent subsequent neurological relapses. The finding of reactive Lyme serology in an MS patient with no suggestive features of the infection is unlikely to indicate neurological Lyme disease.