bluuebunny/crossref_metadata_2025_split huggingface dataset:
Cerebrospinal fluid cholinesterases in aging and in dementia of the alzheimer type
Authors: Atack John R. , May Conrad , Kaye Jeffrey A. , Kay Arthur D. , Rapoport Stanley I. 
Published: January 2005

AbstractProtein concentration and acetylcholinesterase (AChE) and butyrylcholinesterase (BChE) activities were assayed in the cerebrospinal fluid (CSF) of 26 healthy normal subjects (20–86 years old), 27 patients with dementia of the Alzheimer type (DAT), and 10 patients with dementia of the Alzheimer type with extrapyramidal signs (EDAT). In normal subjects, there was an age‐related increase in CSF protein and AChE activity and a significant correlation (p < 0.001) between CSF protein and BChE activity. In the DAT and EDAT groups, CSF AChE activities (mean ± SD = 17.5 ± 3.6 and 15.3 ± 4.4 nmol/min/ml, respectively) were significantly lower (p < 0.05) than in 13 age‐matched control subjects (21.5 ± 5.6 nmol/min/ml). In contrast, neither CSF protein concentration, BChE activity, nor the ratio of AChE/BChE differed significantly between groups. In patients with DAT, CSF AChE activity was significantly lower (p < 0.05) in subjects with an early onset compared to those with a late onset (16.4 ± 3.4 and 19.7 ± 2.8 nmol/min/ml, respectively), and activity in the latter group did not differ significantly from control values. CSF AChE activity was not related to dementia severity and did not change significantly over an 18‐month period. Although these results confirm a cholinergic deficit in patients with DAT, the considerable overlap of CSF AChE activity between groups and the nonsignificant correlation between AChE activity and dementia severity limit the usefulness of CSF AChE as a diagnostic marker of this disorder.
Fiber pathways of cortical areas mediating smooth pursuit eye movements in monkeys
Authors: Tusa Ronald J. , Ungerleider Leslie G. 
Published: January 2005

AbstractRecent studies in rhesus monkeys and human patients have increased our understanding of the role of various cortical regions in the generation of smooth pursuit eye movements. Little is known, though, about the cerebral white matter pathways mediating smooth pursuit. In this paper, we describe both the corticocortical and corticosubcortical projections from areas in the monkey brain known to be involved in smooth pursuit. The corticocortical pathways within one hemisphere consists of a sequence of arcuate fiber bundles interconnecting (1) striate cortex with an area in occipitoparietal cortex called the middle temporal (MT) area, (2) area MT with two areas in parietal cortex called the medial superior temporal (MST) and posterior parietal (PP) areas, and (3) area MST with area PP. An interhemispheric pathway interconnecting areas MT and MST consists of fibers sequentially passing through the tapetum, major forceps, and the splenium of the corpus callosum. The corticosubcortical pathway from area MST to the pontine nuclei and accessory optic system consists of fibers sequentially running through the internal sagittal stratum, the retrolenticular part of the internal sagittal stratum, and the cerebral peduncle. Based on the effects of lesions on pursuit eye movements, these corticocortical and corticosubcortical projections can be divided into sensory, motor, and attentional/spatial systems. Evidence from clinical studies suggests that homologous systems exist in the human cerebrum.
Deficiency of subunits of complex I and mitochondrial encephalomyopathy
Authors: Ichiki Takashi , Tanaka Masashi , Nishikimi Morimitsu , Suzuki Hiroshi , Ozawa Takayuki , Kobayashi Masanori , Wada Yoshiro 
Published: January 2005

AbstractEnzymic activities of the respiratory chain and content of immunochemically detectable subunits in NADH‐ubiquinone oxidoreductase (Complex I) were measured in mitochondria from the skeletal muscles of 4 patients with mitochondrial myopathy, encephalopathy, lactic acidosis, and strokelike episodes (MELAS). The rotenone‐sensitive NADH‐cytochrome c reductase activity was extremely decreased, ranging from 0% to 27% of the control value. In all patients, the content of subunits of Complex I was also reduced in parallel with the rotenone‐sensitive NADH‐cytochrome c reductase activity. It is suggested that the variation in the degree of deficiency of Complex I subunits could explain the clinical heterogeneity of patients with MELAS.
The peripheral neuropathy of necrotizing arteritis: A clinicopathological study
Authors: Said Gérard , Lacroix‐Ciaudo Catherine , Fujimura Harustoshi , Blas Catherine , Faux Nelly 
Published: January 2005

AbstractWe have reviewed the clinical and morphological data from 100 patients with necrotizing arteritis in muscle and/or in nerve samples taken by biopsy. The neuropathy occurred in the context of a multisystem disorder (Group 1) or in apparent isolation (Group 2). The average age of patients was 59 in Group 1 and 61 in Group 2. Females were more commonly affected than males, especially in the first group. Necrotizing arteritis complicated the course of rheumatoid arthritis in 25 patients. In 3 patients necrotizing arteritis was associated with infection with the human immunodeficiency virus, the agent of AIDS. Tests for hepatitis B surface antigen were positive in 19 patients. Mononeuritis was present in 13, mononeuritis multiplex in 62, and distal symmetrical sensory or sensorimotor neuropathy in 19 patients. In both groups of patients, the muscle biopsy was more frequently diagnostic for arteritis than was the nerve biopsy (80% versus 55%). The average incidence of isolated fibers undergoing axonal degeneration was 64.8%; that of demyelinated/remyelinated fibers was 1.9%. We conclude that the combination of nerve and muscle sampling increases the chance of visualizing characteristic arterial lesions in vasculitic neuropathy.
Lesch‐nyhan syndrome: A study of motor behavior and cerebrospinal fluid neurotransmitters
Authors: Jankovic Joseph , Caskey Thomas C. , Stout J. Timothy , Butler Ian J. 
Published: January 2005

AbstractWe studied 5 boys, 2 to 10 years old, with marked or complete deficiency of hypoxanthine‐guanine phosphoribosyltransferase and Lesch‐Nyhan syndrome with varying degrees of mental retardation, dysarthria, chorea, dystonia, spasticity, and ataxia. Four patients had marked reduction of homovanillic acid in the cerebrospinal fluid (CSF) and all showed low CSF 3‐methoxy‐4‐hydroxy phenylethylene glycol, indicating reduced dopamine and norepinephrine turnover. Three patients showed high CSF 5‐hydroxyindoleacetic acid, suggesting increased serotonin turnover. Some patients improved with carbidopa‐levodopa, but others benefited from tetrabenazine, a monoamine‐depleting agent. This study provides support for the theory of abnormal central monoamine metabolism in Lesch‐Nyhan syndrome.
Peripheral neuropathy in the acquired immunodeficiency syndrome
Authors: de La Monte Suzanne M. , Gabuzda Dana H. , Ho David D. , Brown Robert H. , Hedley‐Whyte E. Tessa , Schooley Robert T. , Hirsch Martin S. , Bhan Atul K. 
Published: January 2005

AbstractThe histopathological and immunopathological features of peripheral neuropathy were investigated in 21 patients with the acquired immunodeficiency syndrome (AIDS) or AIDS‐related complex (ARC). Clinical syndromes observed in the 11 (52%) symptomatic patients included distal symmetrical polyneuropathy (DSPN) and chronic inflammatory demyelinative polyneuropathy (CIDP). Specimens from 19 of 20 patients (95%), both symptomatic and asymptomatic, had histopathological evidence of moderate or severe demyelination (79%), axonal degeneration (36%), and mononuclear cell inflammation (37%). Nerves from patients with CIDP and DSPN showed similar degrees of demyelination and axonal degeneration, but inflammation was more intense in CIDP. Immunohistochemical staining identified the majority of inflammatory cells as T lymphocytes or macrophages, with a predominance of CD8+ cytotoxic/suppressor cells. Diffuse immunostaining for human leukocyte antigen (HLA)‐DR was present on endothelial cells, mononuclear inflammatory cells, and Schwann cells, and variable patchy immunostaining for HLA‐DR was present on nerve fibers. Control nerve specimens showed staining for HLA‐DR limited to endothelial, and a few mononuclear, cells. The patterns of immunostaining were similar for AIDS and ARC patients. Human immunodeficiency virus (HIV) was cultured from peripheral nerve in 3 patients, but HIV antigen was not detected by immunohistochemical staining of 8 specimens. The findings implicate HIV infection in nerve, with T cell‐ and macrophage‐mediated tissue destruction as the pathogenetic mechanism of the AIDS/ARC neuropathy.
Brain injury, handedness, and speech lateralization in a series of amobarbital studies
Authors: Woods Roger P. , Dodrill Carl B. , Ojemann George A. 
Published: January 2005

AbstractData on handedness and speech lateralization in patients selected for amobarbital studies have frequently been extrapolated to the normal population, despite the high frequency of brain injuries which might alter lateralization in these patients. To achieve a better definition of the relationships between brain injury, handedness, and speech lateralization, we reviewed the records of 237 consecutive patients who underwent amobarbital testing. Brain injuries sufficient to cause right hemiparesis were strongly associated with left handedness and atypical (right hemisphere or bilateral) speech representation. Among nonhemiparetic patients, abnormal extratemporal radiological findings were associated with an increased incidence of left handedness and atypical speech lateralization. It was not possible to demonstrate any alteration in handedness or speech representation resulting from abnormalities restricted to the temporal lobes, although such alterations could not be excluded. Handedness and speech lateralization established using amobarbital studies in neurosurgical patients may not be representative of the normal population.
Acute fulminating fatal leukoencephalopathy as the only manifestation of human immunodeficiency virus infection
Authors: Jones H. Royden , Ho David D. , Forgacs Pierre , Adelman Lester S. , Silverman Mark L. , Baker Richard A. , Locuratolo Patricia 
Published: January 2005

AbstractA case of acute human immunodeficiency virus (HIV) infection manifested by a rapidly fulminating, necrotizing, demyelinating encephalopathy that led to brain death in 5 days is reported. Autopsy demonstrated predominant white matter lesions, acute neuronal damage, and scanty cellular response. Cultures of cerebrospinal fluid were positive for HIV, suggesting an acute infection.
Somatostatin and neuropeptide Y concentrations in pathologically graded cases of huntington's disease
Authors: Beal M. Flint , Mazurek Michael F. , Ellison David W. , Swartz Kenton J. , McGarvey Usha , Bird Edward D. , Martin Joseph B. 
Published: January 2005

AbstractSomatostatin and neuropeptide Y concentrations have previously been reported to be increased in the basal ganglia in Huntington's disease (HD). In the present study we have extended these findings by examining both somatostatin‐like immunoreactivity (SLI) and neuropeptide Y–;like immunoreactivity (NPYLI) in cases of HD, which were graded according to the severity of pathological degeneration in the striatum. In addition, we surveyed a large number of subcortical nuclei and cortical regions for alternations. Both SLI and NPYLI were significantly increased about threefold in the caudate, putamen, and the nucleus accumbens. Increases in mild and severe grades were similar, which is consistent with a relative but not absolute sparing of striatal aspiny neurons in which somatostatin and neuropeptide Y are colocalized. Significant increases of NPYLI were also found in the external pallidum, subthalamic nucleus, substantia nigra compacta, claustrum, anterior and dorsomedial thalamus, bed nucleus of the stria terminalis, and locus ceruleus. SLI was significantly increased in the external pallidum, red nucleus, and locus ceruleus. Measurements of both neuropeptides were made in 24 regions of the cerebral cortex. Significant increases in both NPYLI and SLI were found in the frontal cortex (Brodmann areas 6, 8, 9, 10, 11, and 45) and temporal cortex (Brodmann area 21), whereas NPYLI was also increased in Brodmann areas 12, 20–22, 25, and 42. Alterations in the cerebral cortex were as pronounced in cases with mild striatal pathological changes as in those with severe striatal pathological changes. These alterations may occur early in HD and could reflect a selective sparing of somatostatin–neuropeptide Y cortical neurons combined with cortical atrophy. The findings are consistent with a possible role for an excitotoxin in the pathogenesis of HD.
Prospective intellectual testing in children with brain tumors
Authors: Duffner Patricia K. , Cohen Michael E. , Parker Mitchell S. 
Published: January 2005

AbstractSixteen children with brain tumors received prospective intellectual evaluations with a follow‐up of 2 to 5 years. All patients were treated with surgery and radiation and 5 received chemotherapy. Most of the children remained within the normal range of intelligence, but intelligence quotient (IQ) scores declined over time. Learning disabilities were present in 15 of 16 children after radiation, and 11 required special educational services. Risk factors associated with decline in IQ appear to be young age at time of radiation and adjuvant chemotherapy.
Lack of association between essential tremor and Parkinson's disease
Authors: Cleeves Lynn , Findley Leslie J. , Koller William 
Published: January 2005

AbstractFour aspects of a possible association between essential tremor (ET) and Parkinson's disease (PD) were investigated: (1) the frequency of extrapyramidal signs in ET; (2) the frequency of concurrent ET and PD (i.e., monosymptomatic postural tremor for 5 or more years prior to onset of PD); (3) the frequency of ET in the families of PD patients; and (4) the frequency of PD in families of ET patients. Two hundred and thirty‐seven ET patients (137 in London and 100 in Chicago) were evaluated. One hundred patients with PD and 100 normal control subjects were also investigated. Mild extrapyramidal signs occurred in only 4.5% of ET patients and were consistent with those found in normal aging. Only 3% of PD patients gave a history of uncomplicated ET. There was no difference in the frequency of a family history of PD between ET patients, a group of PD patients, and control subjects. Frequency of a family history of ET was higher among PD patients than control subjects, although the difference was not statistically significant. These data indicate that there is no association or genetic link between ET and PD.
Gerstmann‐Sträussler‐Scheinker disease: Immunohistological and experimental studies
Authors: Tateishi June , Kitamoto Tetsuyuki , Hashiguchi Hideyuki , Shii Hirofumi 
Published: January 2005

AbstractThe older brother of the patient from whom the Fukuoka‐l strain was isolated was found to have numerous kuru plaques, the main finding common to both siblings. Other clinicopathological features including spongiform change were absent in the older brother. Immunostaining using anti‐kuru plaque core protein and anti‐β‐protein peptide revealed many kuru plaques and a few senile plaques in the older brother. Experimental transmission of the disease to laboratory animals was successful, using tissues from both siblings, through inoculation of fresh brain homogenates, purified prion protein, and formalin‐fixed brain homogenates. Prion protein fractions from the patient's brain shortened the incubation periods and formalin‐fixed mouse brains did not lengthen the periods. The disease in the two brothers can be classified as Gerstmann‐Sträussler‐Scheinker disease, a familial variant of Creutzfeldt‐Jakob disease. Gerstmann‐Sträussler‐Scheinker disease manifests a variety of clinicopathological features. Immunohistological verification of kuru plaques has major diagnostic value in assessing dementia.
Human immunodeficiency virus—associated myopathy: Analysis of 11 patients
Authors: Simpson David M. , Bender Adam N. 
Published: January 2005

AbstractNeuromuscular disorders reported in association with human immunodeficiency virus (HIV) infection include several forms of peripheral neuropathy and polymyositis. We report 11 patients with HIV‐associated myopathy. Five patients with acquired immunodeficiency syndrome (AIDS), 2 with AIDS‐related complex, and 4 otherwise asymptomatic HIV‐infected patients developed progressive proximal muscle weakness. Serum creatine phosphokinase levels were elevated and electromyography revealed abnormal spontaneous activity and myopathy in most patients. All 8 muscle biopsy specimens showed fiber necrosis. Four had inflammatory infiltrates, and nemaline rod bodies were prominent in 3. Immunosuppressant therapy in 5 patients resulted in improvement. Attempts at viral localization in 4 muscle biopsy specimens were unsuccessful. These findings suggest a distinct association between HIV infection and myopathy with features atypical for polymyositis.
Loss of functional suppression is linked to decreases in circulating suppressor inducer (CD4 + 2H4 +) T Cells in multiple sclerosis
Authors: Chofflon Michel , Weiner Howard L. , Morimoto Chikao , Hafler David A. 
Published: January 2005

AbstractA consistent immunological finding in patients with progressive multiple sclerosis is a loss of functional suppression. We have recently found Decemberreases in suppressor inducer T cells in progressive multiple sclerosis as measured by two‐color immunofluorescence using differentiation Marchkers CD4 and 2H4. In the present study, we examined the relationship between functional suppression and circulating CD4 + 2H4 + T cells using a two‐stage assay. (1) T cells were stimulated for 7 days with irradiated non‐T cells (autologous mixed lymphocyte reaction [AMLR]) and harvested. It has previously been shown that suppressor T cells are generated during the course of the AMLR. (2) The AMLR‐generated suppressor T cells were then incubated with mononuclear cells plus pokeweed mitogen, and immunoglobu‐lin (Ig) synthesis was measured. There was less AMLR‐induced suppression of IgG synthesis in patients with progressive multiple sclerosis as compared with normal subjects and patients with other neurological diseases. More importantly, there were significant correlations between Decemberreases in circulating CD4+2H4+ cells and loss of suppression (p < 0.0001) and between Decemberreases in circulating CD4+2H4+ cells and the AMLR (p = 0.009). Thus, the Decemberreases in functional suppression and the Decemberreases in the AMLR in multiple sclerosis appear tightly linked to CD4+2H4+ cells, and their measurement provides a means to monitor suppressor function phenotypically. Decemberreases in suppressor inducer T cells may in part explain immunoregulatory abnormalities observed in multiple sclerosis.
Cognitive deficits in olivopontocerebellar atrophy: Implications for the cholinergic hypothesis of Alzheimer's dementia
Authors: Kish Stephen J. , El‐Awar Munir , Schut Lawrence , Leach Larry , Oscar‐Berman Marchlene , Freedman Morris 
Published: January 2005

AbstractA cerebral cortical cholinergic reduction in dominantly inherited olivopontocerebellar atrophy (OPCA) was recently described. Although the magnitude of the cholinergic reduction was similar to that observed in Alzheimer's disease (AD), none of the OPCA patients was reported to have been demented. We now describe a comprehensive neuropsychological assessment of 11 patients from one of the OPCA pedigrees which we examined biochemically. Detailed neuropsychological testing disclosed previously unrecognized deficits in verbal and nonverbal intelligence, memory, and frontal system function which were positively correlated with the severity of cerebellar ataxia. However, our OPCA patients appeared to be at most only mildly disabled by their cognitive impairment and scored within or close to the normal range on a simple mental status screening examination. This, as well as an absence of any aphasia, apraxia, or agnosia, contrasts with the profile and severity observed in advanced AD dementia, characterized by a similar cortical cholinergic deficit. This finding also suggests that cholinergic reduction may explain only part of the pathophysiology underlying the dementia of AD.
A crossover trial of bromocriptine in the treatment of vascular dementia
Authors: Nadeau Stephen E. , Malloy Paul F. , Andrew Michael E. 
Published: January 2005

AbstractSeven patients with vascular dementia completed an 8‐month randomized double‐blind crossover trial of bromocriptine in a dosage of up to 30 mg per day. Patients were assessed using a modified UCLA Parkinson Rating Scale of symptoms and signs, and neuropsychological testing including the Wechsler Adult Intelligence Scale‐Revised, Wechsler Memory Scale, modified Thurstone Word Fluency Test, Wisconsin Card Sort, a test of visual vigilance, and a reaction time task. Subjects failed to perform significantly better on any measure while on bromocriptine, and on several measures their performance while on the drug was worse.
Motor dysfunction in olivopontocerebellar atrophy is related to cerebral metabolic rate studied with positron emission tomography
Authors: Rosenthal Guy , Gilman Sid , Koeppe Robert A. , Kluin Karen J. , Markel Dorene S. , Junck Larry , Gebarski Stephen S. 
Published: January 2005

AbstractWe compared the severity of motor dysfunction with local cerebral metabolic rates for glucose (lCMRGlc) and the; degree of tissue atrophy in 30 patients with olivopontocerebellar atrophy (OPCA). We devised a scale to quantitate the degree of ataxia in the neurological examinations. lCMRGlc was measured with18F‐2‐fluoro‐2‐deoxy‐D‐glucose and positron emission tomography (PET). Tissue atrophy was assessed by visual rating of computed tomographic scans. PET studies revealed Marchked hypometabolism in the cerebellar vermis, cerebellar hemispheres, and brainstem of OPCA patients compared with 30 control subjects. A significant correlation was found between severity of motor impairment and lCMRGlc within the cerebellar vermis, both cerebellar hemispheres, and the brainstem. A significant but weaker relationship was noted between the degree of tissue atrophy in these regions and clinical severity. Partial correlation analysis revealed that motor dysfunction in OPCA correlated more strongly with lCMRGlc than with the amount of tissue atrophy. These results suggest that the clinical manifestations of OPCA are more closely related to the metabolic state of the tissue than to the structural changes in the cerebellum.
Paroxysmal unilateral dysosmia: A cured patient
Authors: Kaufman Michael D. , Lassiter Kenneth R. L. , Vittal Shenoy B. 
Published: January 2005

AbstractA 31‐year‐old woman with an 11‐year history of unilateral paroxysmal olfactory hallucinations was cured by resection of the homolateral olfactory bulb, which was believed to be the site of origin of the noxious smell.
Myopathy with unique ultrastructural feature in marchinesco‐sjögren syndrome
Authors: Sewry C. A. , Voit T. , Dubowitz V. 
Published: January 2005

AbstractWe have investigated 3 children aged 6, 3, and 2 years, from 2 families, with the clinical features of Marchinesco‐Sjögren syndrome. Muscle biopsy specimens from all 3 were abnormal and showed srrall vacuoles and slight variation in fiber size. Electron microscopy revealed vacuolation and membranous whorls and, in particular, a unique dense membranous structure associated with nuclei. These cases emphasize the involvement of muscle in Marchinesco‐Sjögren syndrome and the importance of electron microscopy in differentil diagnosis.