bluuebunny/crossref_metadata_2025_split huggingface dataset:
Role of nerve growth factor in the expression of trkA mRNA in cultured embryonic rat basal forebrain cholinergic neurons
Authors: Kojima M. , Ikeuchi T. , Hatanaka H. 
Published: January 2005

AbstractUsing a quantitative reverse transcription‐polymerase chain reaction (RT‐PCR), we studied the regulation of trkA mRNA expression in serum‐free, cultured basal forebrain neurons from 17‐day fetal rats. Besides increasing choline acetyltransferase (ChAT) activities, nerve growth factor (NGF) strikingly induced trkA gene expression in a time‐ and NGF concentrationdependent manner. Therefore, NGF might play a critical role in trkA gene expression during the development of basal forebrain cholinergic neurons. Furthermore, to investigate whether this up‐regulation is connected with the trophic effects on basal forebrain cholinergic neurons, we examined the effects of some other neurotrophic agents (BDNF, NT‐3, bFGF, CNTF, and 40 mM KCl) upon ChAT activity and trkA gene expression. Some neurotrophic factors increased ChAT activities to the same degree as NGF, whereas they did not stimulate trkA mRNA expression so potently. NT‐3 plus K252b promotes the tyrosine phosphorylation of TrkA in PC12 cells and increases ChAT activity in cultured basal forebrain cholinergic neurons like NGF (Knusel et al., J Neurochem 59: 715–722, 1992). W e found that NT‐3 plus K252b upregulated the level of trkA mRNA. These results suggested that the expression of trkA mRNA is regulated directly by its specific ligand NGF, rather than neurotrophic effects upon basal forebrain cholinergic neurons and that the up‐regulation is connected to a molecular event initiated by the binding of NGF to the TrkA receptor. ©1995 Wiley‐Liss, Inc.
Microtubule‐associated proteins in developing oligodendrocytes: Transient expression of a MAP2c isoform in oligodendrocyte precursors
Authors: Vouyiouklis D. A. , Brophy P. J. 
Published: January 2005

AbstractThe morphological differentiation of oligodendrocytes is characterized by the formation of multiple, microtubule‐rich processes which endow these cells with the ability to myelinate many axons simultaneously. Since microtubule‐associated proteins (MAPs) strongly influence the structure and function of microtubules, we have investigated their expression in cultured differentiating oligodendrocytes in order to gain insights into MAP function during process formation and stabilization. MAPIB has been compared with two other structural MAPs: MAP4, which is an ubiquitously expressed protein, and MAP2, which hitherto was thought to be confined to neurons and reactive astrocytes. Immunof uorescence microscopy showed that the colocalization of MAP4 with microtubules in oligodendrocyte processes is not as extensive as found previously for MAP1B (Vouyiouklis and Brophy: J Neurosci Res 35:257–267, 1993). Nevertheless, like MAP1B, the expression of MAP4 increases during oligodendrocyte differentiation. In contrast, the expression of MAP2 is transiently elevated in preoligodendrocytes but declines precipitously at the onset of terminal differentiation. Cells of the oligodendrocyte lineage exclusively express a novel isoform of MAP2c which is primarily localized in the cell bodies of preoligodendrocytes. This suggests that MAP2c assists in the initiation of process extension rather than in the stabilization of microtubules in the cytoplasm‐filled membranous extensions of mature cells. MAP‐tau was not expressed at any developmental stage by oligodendrocytes. The distinct subcellular localizations and patterns of developmental expression of MAP1B, MAP4, and MAP2c suggest that these MAPs have different roles in the regulation of the microtubule network during the differentiation of myelin‐forming oligodendrocytes. ©1995 Wiley‐Liss, Inc.
Expression and subcellular distribution of glutamate receptor subunits 2/3 in the developing cerebellar cortex
Authors: Bergmann M. , Fox P. A. , Grabs D. , Post A. , Schilling K. 
Published: January 2005

AbstractThe expression and subcellular location of glutamate receptor subunits 2&3 was investigated in the developing postnatal cerebellum. Immunoblotting revealed that glutamate receptor subunits 2/3 is expressed in an identical pattern of immunoreactive bands of approximately 108 kDa from postnatal day zero to adult animals. Light microscopy showed that within the cerebellar cortex, GluR 2/3 immunoreactivity was essentially confined to Purkinje neurons. Strong immunostaining could be observed at postnatal days 1–3 within Purkinje cell bodies and primary dendrites. With ongoing development, the cell body and an increasingly elaborate dendritic tree was outlined by immunoreaction product. In adult animals, staining of Purkinje cell dendrites was patchy, and staining intensity of the cell body, in particular, was greatly reduced. Ultrastructural analysis revealed that during early postnatal development, immunoreaction product was localized to the cell membrane, but was not confined to postsynaptic densities. From the second postnatal week, glutamate receptor subunits 2/3 immunoreactivity was largely restricted to postsynaptic densities. These observations reveal a developmentally regulated refinement of the subcellular distribution of defining subunits of the AMPA‐type glutamate receptor. The presence of membrane bound receptors prior to the formation of synapses also provides a rationale for the known transmitter‐mediated modulation of Purkinje cell dendritogenesis. © 1996 Wiley‐Liss, Inc.
Cerebral metabolism of glucose in benign hereditary chorea
Authors: Suchowersky Oksana , Hayden Michael R. , Martin W. R. Wayne , Stoessl A. Jon , Hildebrand Anne M. , Pate Brian D. 
Published: January 2005

AbstractBenign hereditary chorea (BHC) is an autosomal dominant disorder characterized by chorea of early onset with little or no progression. There is marked clinical variability in this disease with some subjects having onset in infancy and others with onset in early adulthood. In contrast to Huntington's disease (HD), there is no dementia. Computed tomography is normal in all subjects with no evidence of caudate nucleus atrophy. We present the results of positron emission tomography using 18F‐2‐fluorodeoxyglucose on three patients with this disorder from two families. Cerebral glucose metabolism in one patient was decreased in the caudate nucleus, as previously reported in HD. The other two persons from a second family showed a relative decrease in metabolic rates of glucose in the caudate when compared with the thalamus. It appears that caudate hypometabolism is not specific for HD. These findings suggest that the caudate nucleus may play a significant role in the pathophysiology of some persons with BHC.
Ipsilateral blepharospasm and contralateral hemidystonia and parkinsonism in a patient with a unilateral rostral brainstem– thalamic lesion: Structural and functional abnormalities studied with CT, MRI, and PET scanning
Authors: Leenders K. L. , Frackowiak R. S. J. , Quinn N. , Brooks D. , Sumner D. , Marsden C. D. 
Published: January 2005

AbstractA patient developed progressive right hemidystonia in childhood. Subsequently, left‐sided blephaospasm, slurred and stuttering speech, and right‐sided rigidity and bradykinesia, responsive to dopamine agonists, appeared. Investigation with computed tomography and magnetic resonance imaging (MRI) at age 43 years revealed a left‐sided calcified rostral brainstem‐thalamic lesion of uncertain aetiology. Although no structural lesion was seen in the striatal regions, L‐[18F]‐fluorodopa uptake was severely diminished in the left striatum but normal on the right. Dopamine receptor binding identified by [11C]‐methylspiperone was in the normal range on both sides.
Ramsay hunt syndrome and coeliac disease: A new association?
Authors: Lu Chin‐Song , Thompson Philip D. , Quinn Niall P. , Parkes J. D. , Marsden C. David 
Published: January 2005

AbstractTwo patients with the syndrome of Ramsay Hunt (dyssynergia cerebellaris myoclonica, DCM), associated with malabsorption due to adult coeliac disease, are reported. Both presented with progressive cerebellar ataxia, action myoclonus, and epilepsy. One had gastrointestinal symptoms (recurrent diarrhea and weight loss which responded satisfactorily to a gluten‐free diet), but the other did not. In both patients, jejunal biopsy revealed subtotal villous atrophy; serum folate and vitamin E level were also reduced. Neither a glutenfree diet nor vitamin supplements improved the neurological picture. However, some symptomatic relief was afforded by treatment with clonazepam, sodium valproate, carbamazepine, and piracetam. It could be argued that the association between these two disorders is coincidental. However, since we have found this combination in 2 of 14 consecutive cases with DCM, a causal relationship seems likely, although the underlying mechanism remains unknown. Patients with the Ramsay Hunt syndrome should be investigated for malabsorption, and also undergo small intestinal biopsy.
Simple and complex movements in a patient with infarction of the right supplementary motor area
Authors: Dick J. P. R. , Benecke R. , Rothwell J. C. , Day B. L. , Marsden C. D. 
Published: January 2005

AbstractThe role played by the supplementary motor area (SMA) in the higher‐level organization of motor behaviour (motor programming) has been highlighted by the study of cerebral blood flow during voluntary movements in normal humans. We present a detailed physiological investigation from a patient with a right SMA lesion and show that the right SMA plays a role in programming simultaneous and sequential movements in both arms, though the contralateral arm was the more severely impaired. In addition, we obtained evidence to suggest that the precentral motor cortex may be more responsive to peripheral perturbations when the modulating influence of the SMA is absent. In view of the similarity of the physiological findings in this subject to those in patients with Parkinson's disease, we suggest that the defect of motor programming in Parkinson's disease is likely to reflect functional deafferentation of the SMA.
Involuntary grasping and groping responses to space‐related visual stimuli
Authors: Magnani G. , Mazzucchi A. , Poletti A. , Scoditti U. , Parma M. 
Published: January 2005

AbstractInvoluntary groping‐grasping responses to visual stimuli were studied in relation to the location of the stimuli in the space surrounding the patient. The following space‐related patterns of stimulus‐response were observed in four patients: (a) responses were evoked only by visual stimuli in the hemispace ipsilateral to the arm involved; (b) responses were evoked only by visual stimuli within the reach of maximal arm extension; (c) responses were evoked only by visual stimuli in the space around the hand; (d) all such patterns were variously combined. These findings are discussed in relation to the general problem of specific correlations between sensorimotor subsystems and peripersonal behavioral space.
Anticholinergic‐induced chorea in the treatment of focal dystonia
Authors: Nomoto M. , Thompson P. D. , Sheehy M. P. , Quinn N. P. , Marsden C. D. 
Published: January 2005

AbstractThe occurrence of chorea, induced by trihexyphenidyl (benzhexol hydrochloride) during the treatment of five adult patients who had focal or segmental dystonia, is described. The dose at which chorea appeared ranged from 15 to 60 mg/day (mean 31.7 mg/day). All but one patient had developed common adverse effects of this drug (dry mouth, blurred vision, and confusion) at lower doses (mean 21.8 mg per day). There was an inverse relationship between the age of the patient and the dose of trihexyphenidyl at which chorea developed.
Blepharospasm and autoimmune diseases
Authors: Jankovic Joseph , Patten Bernard M. 
Published: January 2005

AbstractWe studied two patients, one with systemic lupus erythematosus and the other with myasthenia gravis, both of whom had coexistent blepharospasm. The blepharospasm occurred during exacerbations of the autoimmune diseases and improved with immunosuppressive therapy. These cases illustrate the possible association of blepharospasm and autoimmune disorders.
Regional cerebral glucose metabolism in SLE chorea: Further evidence that striatal hypometabolism is not a correlate of chorea
Authors: Guttman M. , Lang A. E. , Garnett E. S. , Nahmias C. , Firnau G. , Tyndel F. J. , Gordon A. S. 
Published: January 2005

AbstractThe pathophysiology of chorea in systemic lupus erythematosus (SLE) is uncertain. Pathologic examination has not identified a specific localtion for the causative lesions (s) and immunologic mechanisms have been suggested in its etiology. In other choreic disorders, such as Huntigton's disease and benign hereditary chorea, glucose hypometabolism in the striatum has been domonstrated by positron computed tomography (PCT) using [18F] deoxyglucose. With this technique we have studied four patients with chorea secondary to SLE. In these patients the regional distribution of cerebral glucose metabolism was normal. In particular, striatal glucose metabolism was within the normal range, even though the ratio of striatal to cortical glucose metabolism was increased. Our results show that striatal hypometabolism, as seen in other disorders manifesting chorea, is not the PCT correlate of the dyskinesia.
Trauma as an etiology of parkinsonism: A historical review of the concept
Authors: Factor Stewart A. , Sanchez‐Ramos Juan , Weiner William J. 
Published: January 2005

AbstractThe relationship between trauma and the development of Parkinson's disease has been an issue in neurology since James Parkinson's initial 1817 essay. This paper will delineate the historical development of the concept of trauma as an etiologic agent in Parkinson's disease and parkinsonism. The strong influence of socioeconomic societal forces in the 1870s with regard to liability lawas and the subsequent acceptance in the medical community of the role of peripheral trauma in producing Parkinson's disease is presented. We will also review present‐day criteria for assigning head trauma an etiologic role in parkinsonism. The discussion will stress current knowledge regarding trauma and parkinsonism, and it will also review the issues of the possible role of the current litigious society's influence on determining a role for trauma in Parkinson's disease.
Cryptococcal meningoencephalitis with parkinsonian features
Authors: Wszolek Zbigniew , Monsour Howard , Smith Philip , Pfeiffer Ronald 
Published: January 2005

AbstractThe subacute onset of parkinsonism in a patient with cryptococcal meningoencephalitis is described. The prompt resolution of the extrapyramidal dysfunction with antifungal agents suggests direct involvement of basal ganglia structures by the cryptococcal infection. Rapid development of parkinsonism in immunocompromised individuals should prompt investigation for an infectious etiology.
The diverse neurological features of Niemann‐Pick disease type C: A report of two cases
Authors: Coleman R. J. , Robb S. A. , Lake B. D. , Brett E. M. , Harding A. E. 
Published: January 2005

AbstractTwo cases of Niemann‐Pick disease type C are described in order to illustrate the variable neurological features of this rare condition. One presented with a predominantly akinetic‐rigid syndrome at the age of 5 years. The second developed progressive ataxia, accompanied by a vertical gaze palsy, when she was 13. Neither patient had hepatosplenomegaly; the diagnosis of Niemann‐Pick disease type C was based on finding foamy storage cells in bone marrow aspirates.
Systemic toxicity of botulinum toxin by intramuscular injection in the monkey
Authors: Scott Alan B. , Suzuki David 
Published: January 2005

AbstractBotulinum toxin (Oculinum) was injected intramuscularly into eight monkeys. The LD50 dose is estimated to be ∼39 U/kg body weight. The lowest dose that caused systemic toxicity, 33 U/kg, was close to the fatal dose range, 38–42 U/kg.
The evolution and distribution of morphological changes in the nervous system of the common marmoset following the acute administration of 1‐methyl‐4‐phenyl‐1,2,3,6‐tetrahydropyridine
Authors: Gibb W. R. G. , Terruli M. , Lees A. J. , Jenner P. , Marsden C. D. 
Published: January 2005

AbstractSix young adult marmosets received 1‐methyl‐4‐phenyl‐1,2,3,6‐tetrahydropyridine in multiple doses (total 8–16 mg/kg over 2–13 days) sufficient to produce a parkinsonian syndrome and were killed up to 3.5 months later. Cellular changes were found in the substantia nigra, ventral tegmental area, and hypothalamus. The earliest histological abnormalities were axonal swellings in proximal parts of nigrostriatal axons. Subsequent changes in the substantia nigra were reduced Nissl staining, reduced cell volume, and aggregation and loss of melanin granules. Other effects were reduced nuclear and nucleolar volumes, depletion of cells, and hyperplasia of glial cells. Shrunken cytoplasm and nuclei stained uniformaly with eosin, and no cells showed cytoplasmic swellings or inclusions. These cellular alterations, with nuclear chages resembling karyolysis, do not occur in Parkinson's disease, which is characterised by Lewy body inclusions and signs of chromatolysis. The rapid appearance of axonal swellings, disruption of Nissl substance, and cell shrinkage suggest an insult to energy producing mechanisms. In this study, the absence of histological evidence of toxicity in the locus coeruleus and also in the substantia innominata and in serotonergic cell groups is unlike the more wide‐spread degenerative changes of Parkinson's disease.
Bereitschaftspotential in tardive dyskinesia
Authors: Adler Lawrence E. , Pecevich Mark , Nagamoto Herbert 
Published: January 2005

AbstractThe bereitschaftspotential or motor readiness potential is a slow negative electroencephalographic wave occurring 150–1500 ms prior to the onset of a voluntary movement. It was measured in 33 subjects: 11 normal controls, 11 medicated schizophrenics with no tardive dyskinesia or evidence of drug‐induced parkinsonism, and 11 patients with tardive dyskinesia. The bereitschaftspotential amplitude was more than two times larger in patients with tardive dyskinesia than in normal controls or schizophrenic patients without tardive dyskinesia. The increased amplitude correlated with the degree of severity of the tardive dyskinesia as measured on the Abnormal Involuntary Movement Scale (AIMS). The finding of the increased bereitschaftspotential amplitude in tardive dyskinesia, taken together with earlier findings of low amplitude in Parkinson's disease, suggests that this potential may reflect the level of dopaminergic activity in the basal ganglia.
Clinical and CT scan findings in a case of cyanide intoxication
Authors: Grandas F. , Artieda J. , Obeso Joséea A. 
Published: January 2005

AbstractA 39‐year‐old man showed a combination of severe parkinsonism and progressive dystonia following attempted suicide with sodium cyanide. Computed tomography (CT) scan showed bilateral lucencies in the putamen and external globus pallidus. The topography of lesions on CT scan closely correlated with the pathological findings described in a previous report of cyanide‐induced parkinsonism. This is the first reported case of cyanide intoxication with delayed‐onset dystonia.
Familial hemifacial spasm
Authors: Friedman Andrzej , Jamrozik Zygmunt , Bojakowski Jacek 
Published: January 2005

AbstractWe present a family in which hemifacial spasm involving in all cases the left side of the face occurred in five persons in three generations. Blink reflexes recorded in two cases demonstrated an unexpected R1 component on the affected side during stimulation of the contralateral side.
Eyelid movement abnormalities in progressive supranuclear palsy
Authors: Golbe Lawrence I. , Lepore Frederick E. , Davis Patricia H. 
Published: January 2005

AbstractWe systematically videotaped eyelid movements in a community‐based series of 38 patients with progressive supranuclear palsy (PSP). Ten patients (26%) had blepharospasm, “apraxia” of lid opening and/or “apraxia” of lid closing. These patients as a group had more severe upgaze paresis but no greater disease duration than the patients without supranuclear lid dysfunction. Patients used a variety of synkinetic movements to overcome lid‐movement abnormalities. One patient displayed “slow blinks”, a phenomenon not previosly described in PSP. Blink rate in PSP, 3.0/min, was markedly lower than that in patients with Parkinson's disease (PD), 12.5/min, and patients with PSP but not PD increased their blink rate during command versional eye movements.
Modeling the functional organization of the basal ganglia. A parallel distributed processing approach
Authors: Mitchell I. J. , Brotchie J. M. , Brown G. D. A. , Crossman A. R. 
Published: January 2005

AbstractDespite recent advances in the understanding of the pathophysiology of movement disorders, little is known about the precise function the basal ganglia play in the control of movement. We review an approach to studying the function of neural systems that is based on the use of a class of computer models known as parallel distributed processors (PDPs) and indicate its potential range of applications to the study of movement disorders. PDPs can be used to construct computational devices that take into account the anatomical and pharmacological properties of real neural systems. They can also provide computational‐level insights that can lead to novel hypotheses concerning brain function. We discuss both these approaches and outline a scheme of the functional organization of the basal ganglia, which predicts some of the pathophysiological mechanisms that mediate movement disorders and which can be formally modeled on a computer.
Cortical activity preceding self‐initiated and externally triggered voluntary movement
Authors: Papa S. M. , Artieda J. , Obeso José A. 
Published: January 2005

AbstractThe cortical electromyogram (EMG) activity, preceding voluntary movemnts, was recorded in 12 normal subjects in two different situations: first, when movements, were self‐induced by the subjects by their own will; and second, in response to threshold electrical stimulation of the index finger, a brief flash of a light‐emitting diode (LED), and a click. Four types of movements were studied: (a) fast extension of the right wrist, (b) fast supination of the left wrist, (c) either movement depending on the subject's own decision or on which index finger was stimulated, and (d) fast sequential right and left wrist extension. In all subjects, self‐initiated movements were preceded by a typical Bereitschaftspotential (BP) starting 1, 290 ± 208 ms before the EMG discharge. When the same movements were triggered by an external clue, there was no BP. The BP was present, although with a shorter duration, when subjects were asked to wait for a brief period after index finger stimulation, before extending the right wrist. From these results, we conclude that the BP is closely associated with the timing of internally generated movements, and that different cortical areas are probably involved in the generation of self‐induced and externally referenced movements in humans.