bluuebunny/crossref_metadata_2025 huggingface dataset:
X‐linked emery‐dreifuss muscular dystrophy can be diagnosed from skin biopsy or blood sample
Authors: Mora M. , Cartegni L. , Di Blasi C. , Barresi R. , Bione S. , di Barletta M. Raffaele , Morandi L. , Merlini L. , Nigro V. , Politano L. , Donati M. A. , Cornelio F. , Cobianchi F. , Toniolo D.
Published: January 2005

We have raised an anti‐emerin polyclonal antibody against a fusion protein encompassing most of the hydrophilic portion of emerin. Using this antibody, we have analyzed emerin expression in Emery‐Dreifuss muscular dystrophy (EDMD) patients and controls, by immunocytochemistry, in peripheral blood mononuclear cells and lymphoblasts. Emerin was localized on the surfaces of nuclei in control skeletal muscle and skin but was absent or reduced in patient skeletal muscle, was absent from the skin of patients, and was expressed only in a few nuclei in a patient's mother. Immunoblot of peripheral blood cells from EDMD patients showed absence of the emerin band, altered‐size emerin, or a protein of normal molecular mass but slightly reduced quantity. The diagnosis of X‐linked EDMD is normally confirmed by genegic analysis of the STA gene coding for emerin. We propose immunocytochemical evaluation of emerin expression in skin biopsies as a sensitive and more convenient tool for diagnosing X‐linked EDMD and, in particular, for distinguishing it from the autosomal dominant form. This technique may be applied to suspected EDMD patients, especially sporadic cases or those with incomplete clinical phenotype, and also suspected carriers. Immunoblot of peripheral blood cells is also useful, but it may not unequivocally identify carriers and some patients.
Hypoxia—ischemia causes abnormalities in glutamate transporters and death of astroglia and neurons in newborn striatum
Authors: Martin Lee J. , Brambrink Ansgar M. , Lehmann Christoph , Portera‐Cailliau Carlos , Koehler Raymond , Rothstein Jeffrey , Traystman Richard J.
Published: January 2005

The neonatal striatum degenerates after hypoxia–ischemia (H‐I). We tested the hypothesis that damage to astrocytes and loss of glutamate transporters accompany striatal neurodegeneration after H‐I. Newborn piglets were subjected to 30 minutes of hypoxia (arterial O2 saturation, 30%) and then 7 minutes of airway occlusion (O2 saturation, 5%), producing cardiac arrest, followed by cardiopulmonary resuscitation. Piglets recovered for 24, 48, or 96 hours. At 24 hours, 66% of putaminal neurons were injured, without differing significantly thereafter, but neuronal densities were reduced progressively (21–44%). By DNA nick‐end labeling, the number of dying putaminal cells per square millimeter was increased maximally at 24 to 48 hours. Glial fibrillary acidic protein–positive cell body densities were reduced 48 to 55% at 24 to 48 hours but then recovered by 96 hours. Early postischemia, subsets of astrocytes had fragmented DNA; later postischemia, subsets of astrocytes proliferated. By immunocytochemistry, glutamate transporter 1 (GLT1) was lost after ischemia in the astroglial compartment but gained in cells appearing as neurons, whereas neuronal excitatory amino acid carrier 1 (EAAC1) dissipated. By immunoblotting, GLT1 and EAAC1 levels were 85% and 45% of control, respectively, at 24 hours of recovery. Thus, astroglial and neuronal injury occurs rapidly in H‐I newborn striatum, with early gliodegeneration and glutamate transporter abnormalities possibly contributing to neurodegeneration.
Short‐ and long‐duration responses to levodopa during the first year of levodopa therapy
Authors: Nutt John G. , Carter Julie H. , Van Houten Laura , Woodward William R.
Published: January 2005

We examined the response to 2–hour levodopa infusions in 18 Parkinson's disease subjects before starting long‐term levodopa treatment and after 6 and 12 months of treatment using tapping speed as an index of bradykinesia. The long‐duration response (LDR), measured as the increase in baseline (overnight without levodopa) tapping speed, increased by 29 ± 18 at 6 months and by 35 ± 24 at 12 months. The magnitude of the short‐duration response (SDR) to a 2‐hour levodopa infusion after an overnight levodopa withdrawal did not differ at 6 and 12 months (16 ± 18 and 20±13 taps/min) from that before long‐term levodopa (21±taps/min). However, when levodopa was withheld for 3 days, it was evident that the SDR magnitude was increasing in magnitude (19, 23, and 31 taps/min at 0, 6, and 12 months). Duration of SDR did not change. A diurnal motor pattern with faster tapping speeds in the morning and slower in the evening was apparent on the days no levodopa was administered. These observations indicate (1) the LDR is responsible for much of the sustained response to levodopa during the first year of treatment, (2) the SDR magnitude increases but is obscured by the LDR, and (3) a diurnal pattern of motor function is present that may be partially responsible for the poorer motor function in the afternoons and evenings.
Increased 3‐nitrotyrosine in both sporadic and familial amyotrophic lateral sclerosis
Authors: Beal M. Flint , Ferrante Robert J. , Browne Susan E. , Matthews Russell T. , Kowall Neil W. , Brown Robert H.
Published: January 2005

The pathogenesis of neuronal degeneration in both sporadic and familial amyotrophic lateral sclerosis (ALS) associated with mutations in superoxide dismutase may involve oxidative stress. A leading candidate as a mediator of oxidative stress is peroxynitrite, which is formed by the reaction of superoxide with nitric oxide. 3‐Nitrotyrosine is a relatively specific marker for oxidative damage mediated by peroxynitrite. In the present study, biochemical measurements showed increased concentrations of 3‐nitrotyrosine and 3‐nitro‐4‐hydroxyphenylacetic acid in the lumbar and thoracic spinal cord of ALS patients. Increased 3‐nitrotyrosine immunoreactivity was observed in motor neurons of both sporadic and familial ALS patients. Neurologic control patients with cerebral ischemia also showed increased 3‐nitrotyrosine immunoreactivity. These findings suggest that peroxynitrite‐mediated oxidative damage may play a role in the pathogenesis of both sporadic and familial ALS.
Inflammatory central nervous system demyelination: Correlation of magnetic resonance imaging findings with lesion pathology
Authors: Brück Wolfgang , Bitsch Andreas , Kolenda Herbert , Brück Yvonne , Stiefel Michael , Lassmann Hans
Published: January 2005

Magnetic resonance imaging (MRI) is widely used to evaluate and monitor disease activity in inflammatory demyelinating central nervous system (CNS) diseases such as multiple sclerosis. The present study aimed at correlating MRI findings with histological parameters in 6 cases of biopsy‐proven inflammatory demyelination of the CNS. The earliest stages of demyelinating activity manifested as almost isointense lesions with a massive gadolinium‐DTPA (Gd‐DTPA) enhancement in T1‐weighted scans. In T2‐weighted scans, early active lesions formed a border of decreased intensity compared with the lesion center and the perifocal edema. The morphological correlate of this pattern in our patients was activated macrophages in the zone of myelin destruction at the plaque border. Late active lesions were hypointense in T1 and hyperintense in T2 scans. Inactive demyelinated and remyelinating lesions were hyperintense in T2 scans and enhanced inhomogenously after Gd‐DTPA application. T1 scans revealed major differences in the degree of hypointensity that correlated with the extent of axonal damage, extracellular edema, and the degree of demyelination or remyelination.
Inhibition of axonal growth from sensory neurons by excess nerve growth factor
Authors: Conti Anna M. , Fischer Stephanie J. , Windebank Anthony J.
Published: January 2005

Fifteen‐day embryonic rat dorsal root ganglion (DRG) neurons were exposed to 1 to 200 ng/ml nerve growth factor (NFG). Maximal neurite outgrowth was obtained with 10 to 20 ng/ml. Neurite outgrowth was reduced to 89% of maximal by increasing NGF to 50 ng/ml, to 66% by 100 ng/ml, and to 18% by 200 ng/ml NGF. Identical effects were seen with mouse 2.5S NGF and recombinant human NGF. Neuron cell counts demonstrated that significant cell death did not occur. In time course experiments, significant inhibition, compared with control, began within 1 hour of adding 200 ng/ml and 3 hours of adding 50 ng/ml NGF. The inhibitory effect of NGF on neurite outgrowth was reversed within 3 hours when DRG were incubated with 5 ng/ml NGF after treatment with 50 or 200 ng/ml NGF medium for 12 hours. The inhibition demonstrated for neurons did not occur in PC12 cells; axonal growth was not inhibited by up to 1,000 ng/ml NGF. Excess brain‐derived neurotrophic factor or neurotrophin‐3 did not inhibit neurite outgrowth. We conclude that high concentrations of NGF produces specific and reversible arrest of neurite outgrowth from sensory neurons. This observation has important clinical implications, because these inhibitory concentrations have been exceeded when NGF has been administered into the central nervous system of humans and animals.
Association of IgM type anti‐GM1 antibodies and muscle strength in chronic acquired demylelinating polyneuropathy
Authors: Bech Einar , Andersen Henning , Ørntoft Torben F. , Jakobsen Johannes
Published: January 2005

The pathogenetic role of anti‐GM1 in chronic acquired demyelinating polyneuropathy (CADP) is uncertain. An association between antibodies and disease activity has not yet been established. In 8 patients with CADP followed longitudinally, anti‐GM1 antibodies were monitored with a standardized enzyme‐linked immunosorbent assay technique and muscle performance with isokinetic dynamometry. During a mean observation period of 24 months, strength improved in 6 of 8 patients by a median value of 54.5%, and anti‐GM1 fell in all 6 patients; the reduction being 43%. In 2 patients, muscle performance deteriorated by 30 and 8%, whereas anti‐GM1 titers increased by 10 and 9%, respectively. The relative change in anti‐GM1 was inversely related to muscle performance. Clinical scoring of muscle performance according to the Medical Research Council scale failed to show an association with anti‐GM1. It is concluded that anti‐GM1 antibodies are closely realted to disease activity, and that the close association indicates a role of anti‐GM1 in the pathogenesis of CADP.
Diffusion‐weighted MRI in transient global amnesia: Elevated signal intensity in the left mesial temporal lobe in 7 of 10 patients
Authors: Strupp Michael , Brüning Roland , Wu Ren Hua , Deimling Michael , Reiser Maximilian , Brandt Thomas
Published: January 2005

Prompted by the findings of previous studies with positron emission tomography and single photon emission computed tomography, which demonstrated hypoperfusion or hyperperfusion in the left temporal lobe in isolated patients with transient global amnesia (TGA), we compared the sensitivity of diffusion‐weighted (DW) magnetic resonance imaging (MRI) with that of conventional T1‐ and T2‐weighted MRI in patients with TGA. Ten patients with the typical syndrome of a pure TGA were included in the study. For all patients, a coronal DW sequence, a steady‐state free precession (SSFP) sequence, and conventional T1‐ and T2‐weighted turbo spin‐echo sequences were obtained. Seven of the 10 patients had elevated signal intensity in the left hippocampal region on DW MRI; moreover, 3 of these 7 patients exhibited bilateral signal abnormality in this sequence. All conventional T1‐ and T2‐ weighted images as well as all follow‐up studies were normal. The signal elevation in DW MRI correlates with a decrease in the interstitial space and with cellular edema in the temporal lobe during TGA. The underlying pathomechanism causing this cellular edema cannot be clearly outlined using DW MRI. Our data are, however, compatible with spreading depression. This is the first study to show that DW MRI is a sensitive MRI method for evaluating TGA, especially in the early stage of the disease.
Progressive atrophy of cerebellum and brainstem as a function of age and the size of the expanded CAG repeats in the MJD1 gene in Machado‐Joseph disease
Authors: Onodera Osamu , Idezuka Jiro , Igarashi Shuichi , Takiyama Yoshihisa , Endo Kotaro , Takano Hiroki , Oyake Mutsuo , Tanaka Hajime , Inuzuka Takashi , Hayashi Tsunemi , Yuasa Tatsuhiko , Ito Jusuke , Miyatake Tadashi , Tsuji Shoji
Published: January 2005

Machado‐Joseph disease (MJD) is an autosomal dominat neurodegenerative disease characterized by cerebellar ataxia associated to varying degrees with pyramidal signs, extrapyramidal signs, or peripheral amyotrophy. It is caused by unstable expansion of the CAG repeat in the MJD1 gene on chromosome 14q32.1. To determine how the neurodegenerative process in the central nervous system of patients with MJD correlates with the size of expanded CAG repeats in the MJD1 gene and other factors, we performed detailed quantitative analyses of findings of magnetic resonance imaging of the central nervous system of 21 patients with MJD of various ages and with various sizes of expanded CAG repeats. We found that atrophy of the brainstem and cerebellar vermis in MJD patients is closely correlated not only with the size of expanded CAG repeat in the MJD1 gene but also with patient age, which suggests that the neurodegenerative process in MJD is regulated by the size of expanded CAG repeats as well as by the patient age.
Increased sensitivity of fibroblasts from amyotrophic lateral sclerosis patients to oxidative stress
Authors: Aguirre T. , van Den Bosch L. , Goetschalckx K. , Tilkin P. , Mathijis G. , Cassiman J. J. , Robberecht W.
Published: January 2005

Although the cause of amyotrophic lateral sclerosis (ALS) is unkown, free radical toxicity is thought to play a pathogenic role. We investigated whether cells from ALS patients are more vulnerable to exogenously induced oxidative stress than cells from controls. We therefore studied the sensitivity of fibroblasts from patients with sporadic ALS (SALS), and from patients with familial ALS (FALS) associated with copper/zinc superoxide dismutase (Cu/ZnSOD) mutations (SOD1‐FALS), to the free radical‐generating agents 3‐morpholinosydnonimine (SIN‐1) and hydrogen peroxide (H2O2), and to serum withdrawal. SOD1‐FALS and SALS fibroblasts were significantly more sensitive than controls to SIN‐1 but not to serum withdrawal. In addition, SOD1‐FALS fibroblasts were more sensitive to H2O2 than SALS fibroblasts and than fibroblasts of controls. These results suggest that the mechanism underlying both SOD1‐FALS and SALS jeopardizes the cell's defense against free radical stress, and that SOD1‐FALS cells are particularly sensitive to H2O2. The latter finding is compatible with biochemical data on the increased affinity of the mutated Cu/ZnSOD for H2O2.
Paroxysmal tonic upgaze: A reappraisal of outcome
Authors: Hayman M. , Harvey A. S. , Hopkins I. J. , Kornberg A. J. , Coleman L. T. , Shield L. K.
Published: January 2005

Paroxysmal tonic upgaze (PTU) of childhood is a distinctive neuro‐ophthalmological syndrome of unknown etiology and pathogenesis that is characterized by episodes of sustained upward deviation of the eyes, often with incomplete downward saccades on attempted downgaze. It is generally regarded as having a benign outcome. We observed 16 children with PTU, from 10 months to 11 years from onset (mean, 5.4 years), to study the natural history and possible etiology. Five cases were from two unrelated families. Onset of PTU occurred either during or after an intercurrent infection or vaccination in 5 children. No antecedent was identifiable in the rest. PTU had completely resolved in 10 children (62%) (mean age at offset, 2.5 years), whereas 2 children intermittently manifest a modified from of the disorder. At follow‐up, 11 children (69%) had developmental delay, intellectual disability, or language delay and 9 (56%) had ocular motility problems other than PTU. Only 3 children (19%) had normal development and neurological findings. PTU is a heterogeneous syndrome with respect to associations and outcome and may simply be an age‐dependent manifestation of a variety of disorders affecting corticomesencephalic control of vertical eye movement. This disorder may be an early sign of more widespread neurological dysfunction.
Optic neuritis heralding varicella zoster virus retinitis in a patient with acquired immunodeficiency syndrome
Authors: Meenken Christina , van Den Horn Gerardus J. , de Smet Marc D. , van Der Meer Jan T. M.
Published: January 2005

We report on a 29‐year‐old severely compromised acquired immunodeficiency syndrome patient who developed retrobulbar optic neuritis 5 weeks after an episode of cutaneous herpes zoster infection. During the optic neuritis, varicella zoster virus could be demonstrated in the cerebrospinal fluid. The neuritis responded well to treatment with foscarnet, but, 3 weeks into therapy, varicella zoster retinitis developed. Additional treatment with intravenous acyclovir stopped progression of the retinitis and resulted in healing of the retinal lesions. This case suggests that retrobulbar optic neuritis can be regarded as a prodrome of imminent acute retinal necrosis. Early recognition and prompt therapy with combined antivirals may prevent the development of this devastating ocular complication of varicella zoster infection.
Functional evaluation of inhibition of autonomic transmitter release by autoantibody from lambert eaton myasthenic syndrome
Authors: Houzen Hideki , Hattori Yuichi , Kanno Morio , Kikuchi Seiji , Tashiro Kunio , Motomura Masakatsu , Nakao Youko , Nakamura Tatsufumi
Published: January 2005

The effects of the anti–voltage‐gated Ca2+ channel (VGCC) antibody obtained from patients with Lambert‐Eaton myasthenic syndrome (LEMS) on autonomic neurotransmission were studied in in‐vitro experiments. The releases of acetylcholine (ACh) and norepinephrine from the autonomic nerves were evaluated by changes in the contractile responses of guinea pig taenia caeci and left atria to electric field stimulation, respectively. Incubations for 6 hours with LEMS serum and IgG, both of which contain anti‐VGCC antibody, markedly suppressed the parasympathetic response but did not affect the sympathetic response. Pharmacological experiments with specific blockers to the VGCC subtypes showed that the Q‐type VGCC is closely linked to the genesis of the parasympathetic responses. We suggest that the anti‐VGCC antibody from the LEMS patients specifically reduces the ACh release from the parasympathetic nerve by binding to the Q‐type VGCC.
Codon 219 lys allele of PRNP is not found in sporadic Creutzfeldt‐Jakob disease
Authors: Shibuya Satoshi , Higuchi June , Shin Ryong‐Woon , Tateishi June , Kitamoto Tetsuyuki
Published: January 2005

The polymorphism at codon 219 of the prion protein gene (PRNP) was found in the general Japanese population with 6% allele frequency. Herein, we examined 85 cases of sporadic Creutzfeldt‐Jakob disease (CJD) for the codon 219 polymorphism. The codon 219Glu/Lys heterozygous polymorphism was not found in these CJD cases. In addition, we examined 43 patients with dementia of non‐CJD origin, and 4 were found to have the codon 219 Glu/Lys heterozygous polymorphism with a similar allele frequency as in the general population. Thus, the codon 219Glu/Lys heterozygous polymorphism might be uniquely excluded from sporadic CJD.
The apolipoprotein E ε4 allele is not a significant risk factor for frontotemporal dementia
Authors: Geschwind Dan , Karrim Juliana , Nelson Stanley F. , Miller Bruce
Published: January 2005

Frontotemporal dementia (FTD) is the most common early‐onset non‐Alzheimer's dementia (non‐AD). Although the role of the ε4 allele of apolipoprotein E (ApoE) has been well established in AD, studies of ApoE allele distribution in patients with FTD have produced variable results. We studied 33 rigorously diagnosed FTD patients, including several who wre pathologically confirmed, and compared the fequency of the ε4 allele in patients with FTD with the frequency in those with early‐onset AD (EOAD), in those with late‐onset AD (LOAD), and in non‐demented elderly controls. The frequency of ApoE ε4 was 21% in patients with FTD, significantly less than the ApoE ε4 frequency in those patients with EOAD (38%) and those with LOAD (40%), but not significantly different from the ApoE ε4 frequency in elderly controls (13%).
Role of collateral flow on cerebral hemodynamics in patients with unilateral internal carotid artery occlusion
Authors: van Everdinge K. J. , Visser G. H. , Klijn C. J. M. , Kappelle L. J. , van Der Grond J.
Published: January 2005

The objective of this study was to evaluate the role of collateral blood flow via the anterior and posterior communicating arteries (ACoA and PCoA) and via the ophthalmic artery (OphA) on cerebral hemodynamics, metabolism, and border zone infarcts in 57 patients with unilateral symptomatic occlusions of the internal carotid artery. Collateral flow via the ACoA and PCoA was determined with magnetic resonance angiography (MRA) and collateral flow via the OphA with transcranial Doppler (TCD). Volume flow was studied with MRA, metabolism with 1H MR spectroscopy, CO2 reactivity with TCD, and the incidence of border zone infarcts with MRI. Compared with controls, patients had deteriorated volume flow, metabolism, and CO2 reactivity. No differences were found between patients with and patients without collateral flow through the ACoA and/or PCoA, or between patients with or without collateral flow via the OphA. Patients without collateral flow via any of these collaterals had decreased volume flow in the middle cerebral artery, decreased N‐acetylaspartate/choline, and increased lactate/ N‐acetylaspartate, compared with the other patients. Patients with symptomatic internal carotid artery occlusion have deteriorated cerebral hemodynamics and metabolism. Different collateral flow patterns via the ACoA, PCoA, or OphA have no effect on the hemodynamic and metabolic parameters, as long as one of these pathways is present.
The influence of nuclear background on the biochemical expression of 3460 Leber's hereditary optic neuropathy
Authors: Cock H. R. , Tabrizi S. J. , Cooper J. M. , Schapira A. H. V.
Published: January 2005

The role of mitochondrial DNA (mtDNA) mutations in the pathogenesis of Leber's hereditary optic neuropathy (LHON) has yet to be characterized. Several clinical feature of the disease imply that nuclear genes might also be involved in its expressions. We have confirmed the presence of a severe NADH:coenzyme Q1 reductase (complex I) defect in association with the A3460G mtDNA LHON mutation in cultured fibroblasts compared with age‐matched controls. This defect was not seen in clonal fibroblasts with 0% mutant mtDNA developed from a heteroplasmic A3460G LHON subject, confirming the association between the A3460G mutation and the complex I defect. Cybrids prepared from the fusion of enucleated fibroblasts homplasmic for the A3460G mutation with 206 (osteosarcoma) cells lacking mtDNA (ρo) also had a severe deficiency of complex I activity. However, in A3460G LHON fusion cybrids containing a different nuclear background, A549 ρO (lung derived), this biochemical defect was not apparent in all the clones studied. These results suggest that the nuclear environment can influence the expression of the biochemical defect in LHON patients with the A3460G mutation.
The α‐synuclein Ala53Thr mutation is not a common cause of familial Parkinson's disease: A study of 230 European cases
Authors: Vaughan Jenny , Durr Alexandra , Tassin Johann , Bereznai Benjamin , Gasser Thomas , Bonifati Vincenzo , De Michele Giuseppe , Fabrizio Edito , Volpe Gianpiero , Bandmann O. , Johnson William G. , Golbe Lawrence I. , Breteler Monique , Meco Giuseppe , Agid Yves , Brice Alexis , Marsden C. David , Wood Nicholas W.
Published: January 2005

We report the results of a screen of 230 European familial index cases of Parkinson's disease for the recently described Ala53Thr mutation in the α‐synuclein gene in an autosomal dominant Parkinson's disease kindred. No mutations were found from this broad white population, and we therefore conclude that although of great interest, this mutation is a very rare cause of familial Parkinson's disease.
Infrequent detection of human herpesvirus 6 DNA in peripheral blood mononuclear cells from multiple sclerosis patients
Authors: Mayne Michael , Krishnan Jay , Metz Luanne , Nath Avindra , Auty Anthony , Sahai Beni M. , Power Christopher
Published: January 2005

Several studies have suggested an association between human herpesvirus 6 (HHV‐6) infection and multiple sclerosis. As HHV‐6 is predominantly a T‐cell tropic virus, we examined the frequency of detection of HHV‐6 genome in peripheral blood mononuclear cells from relapsing–remitting (n = 32) and chronic progressive (n = 14) patients and from healthy (n = 17) and neurological (n = 7) controls. Two sensitive polymerase chain reaction assays were used to target different regions within the HHV‐6 genome. Depending on the polymerase chain reaction assay used, the detection of HHV‐6 genome ranged from 11.7 to 23.5% (controls), 3.1 to 23.0% (relapsing–remitting), and 14.2 to 28.5% (chronic progressive). Although these observations do not exclude a pathogenic role for HHV‐6 in multiple sclerosis, they indicate a lack of correlation between HHV‐6 infection of peripheral blood mononuclear cells and the development of multiple sclerosis.
β‐Chemokines MCP‐1 and RANTES are selectively increased in cerebrospinal fluid of patients with human immunodeficiency virus–associated dementia
Authors: Kelder Wendy , McArthur Justin C. , Nance‐Sproson Tish , McClernon Daniel , Griffin Diane E.
Published: January 2005

Human immunodeficiency virus–associated dementia (HAD) is associated with increased numbers of activated central nervous system (CNS) macrophages. Chemokines, which regulate infiltration of macrophages, were measured in the cerebrospinal fluid (CSF) of human immunodeficiency virus (HIV)‐negative and HIV‐positive individuals with and without neurological disese. Monocyte chemotactic protein (MCP)‐1 and RANTES (but not MCP‐3), macrophage inflammatory protein (MIP)‐1α, MIP‐1β, or interleukin‐8 (IL‐8) was higher in HAD. MCP‐1 correlated with CSF viral load and severity of dementia, and it increased over time in patients who developed dementia.
Consequences of neonatal seizures in the rat: Morphological and behavioral effects
Authors: Holmes Gergory L. , Gairsa Jean‐Luc , Chevassus‐Au‐Louis Nicolas , Ben‐Ari Yehezkel
Published: January 2005

Whereas neonatal seizures are a predictor of adverse neurological outcome, there is controversy regarding whether seizures simply reflect an underlying brain injury or can cause damage. We subjected neonatal rats to a series of 25 brief flurothyl‐induced seizures. Once mature the rats were compared with control littermates for spatial learning and activity level. Short‐term effects of recurrent seizures on hippocampal excitation were assessed by using the intact hippocampus formal preparation and long‐term effects by assessing seizure threshold. Brains were analysed for neuronal loss, sprouting of granule cell axons (mossy fibers), and neurogenesis. Compared with controls, rats subjected to neonatal seizures had impaired learning and decreased activity levels. There were no differences in paired‐pulse excitation or inhibition or duration of afterdischarges in the intact hippocampal preparation. However, when studied as adults, rats with recurrent flurothyl seizures had a significantly lower seizure threshold to pentylenetrazol than controls. Rats with recurrent seizures had greater numbers of dentate granule cells and more newly formed granule cells than the controls. Rats with recurrent seizures also had sprouting of mossy fibers in CA3 and the supragranular region. Recurrent brief seizures during the neonatal period have long‐term detrimental effects on behavior, seizure susceptibility, and brain development.
Aicardi‐Goutières syndrome: An update and results of interferon‐α studies
Authors: GoutièRes Françoise , Aicardi Jean , Barth Peter G. , Lebon Pierre
Published: January 2005

Twenty‐seven patients with familial encephalopathy with calcification of the basal ganglia and chronic cerebrospinal fluid (CSF) lymphocytosis (Aicardi‐Goutières syndrome) are reviewed. In 19 children, the onset was within the first 4 months of life. Most patients had normal head circumference at birth, but 21 developed microcephaly between 3 and 12 months. Neuroimaging showed severe and progressive brain atrophy in all patients. The extent and intensity of the calcification was variable even in the same sibship. CSF lymphocytosis persisted beyond 12 months of age in 7 children. High levels of interferon‐α were found in serum and CSF in 14 patients. The higher CSF levels suggest intrathecal synthesis. Tubuloreticular inclusions related to the presence of interferon were found in 4 additional children. The 19 patients still alive (6 older than 10 years) are profoundly disabled. However, the syndrome may present with individual variations in severity, rapidity of evolution, and imaging features. Neuropathological examination in 2 patients failed to detect significant inflammatory lesions and showed only foci of necrosis and wide‐spread demyelination. This study supports an autosomal recessive inheritance for this syndrome. The high level of interferon‐α is not explained but may play a role in the pathogenesis of the disorder.
On the finer structure of the synapse of the Mauthner cell with especial consideration of the ‘Golgi‐net’ of bethe, nervous terminal feet and the ‘nervous pericellular terminal net’s of held
Authors: Marui Kiyoyasu
Published: January 2005

In the ‘axone cap’ and on the surface of Mauthner's cell a Golgi net structure was very distinctly demonstrated by means of Levaditi's method; in Heidenhain and other preparations a similar network was brought out. The Golgi net is of glious nature and is in close relation to the nervous elements in the synapse. According to the results of this study, the unmedullated parts of the nerve fibers are also enveloped in a sheath of glious tissue; the finer structure of this glia sheath is as yet unknown.
A contribution to the study of vasomotor reflexes
Authors: Ogata D. , Vincent Swale
Published: January 2005

In dogs under ether or chloroform, stimulation of sensory nerves (saphenous, tibial, peroneal, sciatic, ulnar, and median) causes usually increased respiratory movements when narcosis is not profound or curare is not employed. These increased movements produce a fall of blood‐pressure, and when they are very violent, one cannot obtain any pressor reflex even with a strong stimulation. When much increased respiratory movements are prevented by very deep narcosis or brain compression, fall of blood‐pressure due to this cause does not occur. Mechanical interference with the circulation as a result of the increased movements of the thoracic walls seems to be the main cause of this fall, since it can be eliminated by opening the thorax. When this complication is not taken into careful consideration the results of vasomotor experiments are liable to be misinterpreted.
The development of the motor nuclei of the hindbrain of the chick, Gallus domesticus
Authors: Eckardt Lisgar Bowne , Elliott Rush
Published: January 2005

A study of the development of the motor‐nuclei of the hindbrain of the domestic fowl, Gallus domesticus, during the entire period of incubation has been made, including the time of appearance of the nuclear groups and the subsequent differentiation of each. The nuclear pattern presented by this form is very similar to that of other avian forms as recorded in the literature.
Nerve endings in the human pleura pulmonalis
Authors: Larsell O.
Published: January 2005

Vasomotor fibers are found in the blood vessels of the human pleura pulmonalis. They reach the pleura by way of the inter‐lobular septa and the blood vessels which find their way to the pleura by this route. Ultimately, however, they are derived from the periarterial plexus at the hilnm of the lung.
The blood supply of the lateral geniculate body in the rat
Authors: Tsang Yü‐Chüan
Published: January 2005

The anterior chorioidal artery supples the main part of the ventral nucleus and the anterodorsal pole of the dorsal nucleus.
The hippocampal and parahippocampal cortex of the emu (Dromiceius)
Authors: Craigie E. Horne
Published: January 2005

In the emu, which is considered to be one of the most primitive living birds, the pallium is relatively well developed. A large hyperstriatum accessorium forms the cephalic end of the hemisphere.
Research on the regeneration of the spinal cord in the cat submitted to the action of pyrogenous substances (5 or 3895) of bacterial origin
Authors: Arteta J. L.
Published: January 2005

No Absract.
Intralaminar, interlaminar, callosal, and thalamocortical connections in frontal and parietal areas of the albino rat cerebral cortex
Authors: Jacobson Stanley
Published: January 2005

The present study represents an attempt to relate intracortical connections as seen with the Nauta degeneration method to those seen with the Golgi neuron stain.

Lesions were placed in frontal or parietal cortex of 20 albino rats. Fourteen of the brains were prepared with the Nauta ('57) method or a modification of this method (Jacobson, '63).

Only in parietal areas 3, 1, 2 were lesions limited to layers I through IV. In these brains the Nauta methods revealed only intercortical (callosal) and interlaminar connections (from II through IV to V and VI). Lesions which extended into layer V of forntal and parietal cortex showed the following connections: intercortical, interlaminar (connecting Vb to VIa); intralaminar fibers (spreading tangentially in V); extracortial to striatum and brain stem. A lesion into layer VI of frontal and parietal cortex showed more callosal and projection fibers, and the intracortical portion of the thalamocortical fiber was cut. Thalamocortical fibers ran tangentially and obliquely in VI vertically in V through I, and tangentially in I, and formed pericellular degenaration in layers VI through IIIb and preterminal degeneration in VI through IIIb, and I. The brains prepared with the Marchi or Gudden method did not show these details. In brains stained with the Golgi rapid method, axons of thalamic and cortical neurons were seen whose distribution corresponded to these connections.
A quantitative analysis of the distribution of ganglion cells in the cat's retina
Authors: Stone Jonathan
Published: January 2005

Canglion cell density maps of the cat's retina have been constructed from whole‐mounts of the retina stained with methylene blue. The main features of the maps are first, a great increase in density at the specialized central area, and second, a horizontally orientated “arm” of relatively high density extending from the central area into nasal and temporal retinae. The variation of ganglion cell size with density has been examined in the same preparations. This analysis showed that the cells are smallest at the central area, and increase in size as ganglion cell density decreases. Whole‐mount preparations have also been made of the monkey's retina, allowing comparison between the central areas of cat and monkey retinae.
A degeneration study of the course and extent of the pyramidal tract of the opossum
Authors: Bautista Nestor S. , Matzke Howard A.
Published: January 2005

The pyramidal tract was studied in 12 adult opossums. The method employed consisted of unilateral ablation of the motor cortex followed by selective staining of the resulting fiber degeneration with Nauta's silver technique. The results showed that in the upper part of its course the pyramidal tract followed a course similar to that found in other mammals. It descends in the internal capsule, cerebral peduncle, basal pons and the pyramids of the medulla oblongata where it decussates. It then continues to descend in the dorsal funiculus through the entire cervical cord. In the spinal cord fibers appeared to terminate among neurons at the base of the dorsal gray column. No degeneration could be traced directly into cranial and spinal nerve nuclei.
The retinal structure of (Aotes trivirgatus) the owl monkey
Authors: Jones Arthur E.
Published: January 2005

This study reports the results of a light microscope examination of the eyes from 12 Aotes trivirgatus. Direct comparisons were made with the retinae of Macaca cynomologus and Macaca mulatta treated in the same way and stained at the same time.

Both rods and cones are present in all Macaca retinae examined but never seen in the retinae of Aotes. The fovea of Macaca is described and the distribution of retinal but an increase in the number of ganglion cells in the fundus indicates an area centralis. Myelin staining is found in the optic fiber layer and inner plexiform layer of Aotes but not Macaca.
An experimental study of the fornix and hypothalamo‐tegmental tracts in the cat
Authors: Johnson Thomas N.
Published: January 2005

Single electrolytic lesions were placed at various levels of the hippocampus, septum, and amygdala to elicit fiber degeneration from these structures in order to determine their efferent pathways to the hypothalamus. The Nauta silver Impregnation method for degenerated axons was used in this study. Fornix fibers of hippocampal origin were traced to the preoptic, dorsomedial, and posterior hypothalamic areas. Fornix fibers of septal origin were traced to preoptic, paraventricular, and ventromedial hypothalamic nuclei. Degenerated fibers from the baso‐lateral amygdaloid nuclei were traced to the preoptic and ventromedial hypothalamic nuclei through the stria terminalis. Lesions were also placed in the anterior hypothalamic area, the dorsal hypothalamic area, the dorsomedial nucleus, the ventromedial nucleus, the paraventricular nucleus, and the posterior hypothalamic area. Degenerated fibers were traced from these nuclei to the midbrain tegmentum through the hypothalamo‐tegmental tracts.

The hypothalamus, then can be considered a way station for impulses from the limbic lobe to lower brain stem centers.
Geniculo‐striate connections in the rabbit. I. Retrograde changes in the dorsal lateral geniculate body after destruction of cells in various layers of the striate region
Authors: Rose Jerzy E. , Malis Leonard I.
Published: January 2005

Dorsal lateral geniculate body was examined for retrograde changes which follow radiation lesions placed in various cortical layers of the striate region.

Destruction of cells in a thin cortical strip (less than about 200 μ in stained sections) produces, at most, marginal changes in the dorsal lateral geniculate body regardless of which cortical layer is destroyed.

Lesions which are thicker than those mentioned produce, if located in layers 4, 4 and 5 or 5 and 6, definite degenerations. The thicker the lesion the more pronounced are the changes.

Destruction of layers 1 to 3 produces, at most, marginal changes. However, degenerations caused by destruction of layers 4 and 5 appear augmented if supragranular layers are concomitantly destroyed.

Destructions of layers 1 to 5 produce degenerations of a severity similar to those resulting from destructions of layers 1 to 6.

An effective cortical destruction located in some sector of the striate field produces changes in a restricted geniculate region. It is concluded that the survival of cells in a geniculate locus depends upon the entire vertical column of cortical cells rather than on any layer within this column.

Implications of the findings as regards the distribution of terminals of the geniculate neurons are considered. The significance of radiation damage to fibers and of fibers sprouting in the laminar lesion for interpretation of the findings is discussed.
An electron microscopic study of terminal degeneration in the inferior olive of the cat
Authors: Walberg F.
Published: January 2005

An electron microscopic study has been made of terminal bouton degeneration in the inferior olive of the cat following interruption of afferent fibers to the olive. Bouton degeneration is found already on the second day, when the boutons show an increased density of the matrix and a close packing of synaptic vesicles. On the seventh day many mitochondria show marked changes. They disintegrate, and their fragments usually fill the bouton entirely. The synaptic vesicles in such boutons have lost their individuality. Most of the degenerating boutons found at later stages are similar to those found on the seventh day. Degeneration proceeds slowly in the boutons. Occasionally mitochondria and synaptic vesicles can be recognized even at the 112‐day stage. At this time the degenerating boutons often show vacuolization. Where a degenerating bouton participates in a synaptic complex, thickening of the postsynaptic membrane is found even at the 112‐day stage. The dendritic pits found at the 14‐ and 36‐day stages and described in a previous publication (Walberg, '63b) are even more prominent at the one‐hundred‐twelfth day. Glial activity has not been observed. The relation between degenerating boutons and glial profiles, even at the 112‐day stage, is similar to that found in unoperated animals. A very long period is obviously required for the lysis of the degenerating boutons.
Progressive neuronal differentiation in the submandibular ganglia of a series of human fetuses
Authors: Crouse Gail S. , Cucinotta Anthony J.
Published: January 2005

Serial sections of a series of 20 human fetuses, ranging in age from 4+ weeks (6 mm C.R.) through full term (470 mm C.H.) were studied from the standpoint of progressive development changes in the the submandibular ganglion neuroblasts. The basic staining technique used was the silver nitrate method of Holmes, with luxol fast blue, toluidine blue, gallocyanin and H&E employed as alternate counterstains.

General topography and the formation of roots in relation to the developing ganglion were studied.

Morphological changes in the neuroblasts were related to time of first appearance in the series. Neuroblasts were easily distinguished from non‐nervous ganglion cells at the 79 mm stage. Neuroblastic multipolarity was present at the 79 mm stage also, and the cells were predominantly multipolaar at the 278 mm stage. Neurofibrillae, Nissl bodies, well‐formed capsules and the reduction of the number of nucleoli to one or two were characteristics first observed in the 120 mm fetus.

Preganglionic terminal arborizations were observed as early as the 25 mm stage.

Counts of ganglion neuroblasts of four selected fetuses indicated a progressive reduction in numbers from 12,128 at 17 weeks (120 mm) to 5,988 at full term.

Measurements of ganglion neuroblasts of four selected fetuses revealed a progressive increase in the cytoplasmic/nuclear ratio from 1.38 at 15 weeks (97 mm) to 2.05 at full term.
A fine structural analysis of the ventral nerve cord and associated sheath of Lumbricus terrestris L.
Authors: Coggeshall Richard E.
Published: January 2005

This study is a fine structural analysis of the earthworm nervous system and its sheath. The bulk of the findings consists of the addition of fine structural detail to the extant descriptions of the general organization of this system. These findings were of particular interest, (1) that the nervous system possesses one basic type of supportive glial cell, (2) that the zonula occludens was not found anywhere in the structures studied here, and (3) that the sheath of the ventral giant axon is not myelin but consists of a thick covering of many tiny axons and synaptic terminals.
Ascending connections of the anterior ventral cochlear nucleus in the rat
Authors: Harrison J. M. , Irving R.
Published: January 2005

The purpose of the experiment was to determine the connections of the three regions of the anterior ventral cochlear nucleus in the rat. Lesions were placed in the regions of the nucleus in 11 rats. After 25 days each animal was sacrificed and its brain impregnated by the protargol method of Bodian. The superior olivary complex, the lateral lemniscus and the dorsal acoustic stria were examined for diminution of synaptic endings, thinning of neuropil and debris of degeneration.

Complete destruction of region III of the anterior ventral cochlear nucleus produced degeneration in the ipsilateral lateral superior olive and in the medial superior olive of both sides. Destruction of other regions of the anterior ventral cochlear nucleus, region III remaining intact, had no effect upon the lateral or medial superior olive.

Destruction of region II of the anterior ventral cochlear nucleus produced degeneration in the anterior halves of the ipsilateral lateral nucleus of the trapezoid body and the contralateral medial nucleus of the trapezoid body. Complete destruction of other regions of the anterior ventral cochlear nucleus, region II remaining intact, was without effect upon the lateral and medial nuclei of the trapezoid body.

No degeneration was detected following destruction of region I of the anterior ventral cochlear nucleus.
The lateral geniculate complex of the owl monkey Aotes trivirgatus
Authors: Jones Arthur E.
Published: January 2005

The lateral geniculate complex of 38 normal, one borgne, and one enucleated Aotes trivirgatus were studied to determine the number of laminae present, the laminar organization, and the cell content of the geniculate complex. The optic tract does not display apparent fasciculation. The pregeniculate nucleus consists of two laminae and the lateral geniculate of four, with two magnocellular layers and two parvocellular layers. Layers 1 and 4 receive the terminals of contralateral ganglion cells and layers 2 and 3 receive those of ipsilateral ganglion cells. Approximately, a 4:3 numerical relationship between retinal ganglion cells and lateral geniculate nucleus cells exists in one animal in which cell counts were made.
The cortico‐nigral projection in the cat an experimental study with silver impregnation methods
Authors: Rinvik Eric
Published: January 2005

Following lesions of various parts of the cerebral cortex in cats the terminal distribution of cortico‐nigral fibers has been studied by means of silver impregnation methods.

A modest number of cortico‐nigral fibers have been traced from the gyrus proreus, the “supplementary motor” area, the “primary sensory‐motor” area and the “secondary sensory” area. Lesions of the remaining regions of the cerebral cortex did not give rise to terminal degeneration in the substantia nigra. The number of cortico‐nigral fibers was considerably less than expected from the data available in the literature.

The cortico‐nigral projection system is chiefly homolateral. A few crossed cortico‐nigral fibers were found following lesions of the gyrus proreus or the “supplementary motor” area. The majority of the corticofugal fibers from these two cortical region and the “secondary sensory” area terminate on the somata or the proximal dendrites of the cells in the nigra complex. Following lesions of the “primary sensory‐motor” area of the cerebral cortex, terminal degenerating fibers were only seen in the neuropil of the substantia nigra. The findings are discussed briefly.
Somatic Sensory Representation in the thalamic ventrobasal complex of the virginia opossum
Authors: Pubols Benjamin H. , Pubols Lillian M.
Published: January 2005

The present study was designed to investigate the topographic organization of somatic sensory representation in the thalamic ventrobasal complex (Vb) of the Virginia opossum. Tungsten and stainless steel micro electrodes were used to record the electrical activity of single units or single unit clusters in response to mechanical stimulation of the body surface, and the obtained data were used to construct maps of somatic sensory representation in transverse planes of the ventrobasal complex. The general pattern is similar to that found in eutherian mammals. A relatively large contralateral head and face representation is located throughout the entire anterior‐posterior extent of the ventrobasal complex, while a relatively smaller representation of contralateral trunk and limbs is located laterally only in the middle regions of the ventrobasal complex. Representations of middorsal head and trunk are located dorsally while those of limb apices are located ventrally. Activity was also recorded in the posterior nuclear region of the thalamus (Po) and in the subthalamic zona incerta (ZI), and there is a suggestion of topographic organization in these nuclear regions as well.