bluuebunny/crossref_metadata_2025_split huggingface dataset:
Repetitive involuntary movement associated with episodic cerebral ischemia
Authors: Yanagihara Takehiko , Piepgras David G. , Klass Donald W. 
Published: January 2005

AbstractClinical characteristics of 12 patients with carotid occlusive disease who experienced episodes of repetitive involuntary movement were analyzed and correlated with laboratory findings. The majority of patients had rhythmic involuntary movements of one or both extremities on one side, which were characteristically precipitated by standing, walking, or hyperextension of the neck and were promptly alleviated by assuming a sitting or supine position. Six patients had a history of a prior transient ischemic attack or stroke. Electroencephalograms contained no epileptiform activity at rest or during sleep in any patient or during the involuntary movements in 2 patients. Cerebral angiography revealed either occlusion or high‐grade stenosis of the internal or common carotid artery on the side opposite the involuntary movement in all patients. We conclude that the repetitive involuntary movements result from transient hemodynamic ischemic episodes rather than partial (focal) epilepsy.
Isolated angiitis of the central nervous system
Authors: Craven Robert S. , French John K. 
Published: January 2005

AbstractAngiitis confined to the central nervous system is a rare; disorder in which inflammation and consequent irregular narrowings of medium‐sized cerebral arteries are prominent. Clinical and radiological features in 3 patients with this condition are described. All patients responded to either steroid or steroid plus azathioprine therapy.
Self‐abatement of complex partial seizures
Authors: Prichard Paul B. , Holmstrom Valerie L. , Giacinto Joseph 
Published: January 2005

AbstractSeven of 71 patients with complex partial seizures claimed they were able to abate their seizures, while none of 18 with simple partial seizures were able to do so. Self‐abatement exercises included highly stereotyped cognitive and physical components unique to the individual. Those who could abate their seizures had attained higher educational status, better social and vocational adjustment, and better psychological adjustment than did the control group of patients with epilepsy. The self‐abatement group was also more likely to have right hemispheric electroencephalographic abnormalities. Characterization of the self‐abatement group may be relevant to the selection of candidates for behavioral therapy for epilepsy.
Status epilepticus in well–oxygenated rats causes neuronal necrosis
Authors: Nevander Gunilla , Ingvar Martin , Auer Roland , Siesjö Bo K. 
Published: January 2005

AbstractNeuronal necrosis in the brain resulting from status epilepticus of 15 to 120 minutes duration in ventilated and well‐oxygenated rats was assessed. Seizures were induced by inhalation of the convulsant gas flurothyl, and terminated by withdrawal of flurothyl and a single injection of thiopental. The animals were allowed to recover for one week, and neuronal damage was assessed by cell counts following subserial sectioning of the brain and microscopical examination of the sections. Infarction of the pars reticulata of the substantia nigra occurred in 5 of the 6 animals with seizure duration of 30 minutes, and in all animals with longer seizure durations. There also was a common affectation of the central parts of the globus pallidus. The pars compacta of the substantia nigra was never affected. After 45 to 120 minutes of seizures, moderate neuronal necrosis was observed in the neocortex (layers 3 and 4), and after 60 to 120 minutes was seen in amygdaloid and thalamic nuclei, as well as in CA4 and CA1 hippocampal pyramidal cells. Notably, CA3 neurons were not damaged nor were dentate granule cells affected. After 120 minutes of seizures, damage regularly affected the neocortex and the ventral–posterior nuclei of the thalamus. A conspicuous feature was the localization of neuronal necrosis at sites close to the ventricles.
Clinical history, brain metabolism, and neuropsychological function in Alzheimer's disease
Authors: Cutler Neal R. , Haxby James V. , Duara Ranjan , Grady Cheryl L. , Kay Arthur D. , Kessler Robert M. , Sundaram Magesh , Rapoport Stanley I. 
Published: January 2005

AbstractData concerning 7 patients with a diagnosis of presumptive Alzheimer's disease (mean age, 65.6 years) are presented in detail in relation to the patients' regional cerebral metabolic rates for glucose. Rates were measured by positron emission tomography with fluorine 18–labeled fluoro‐2‐deoxy‐D‐glucose under conditions of reduced visual and auditory stimulation. A relationship was found between severity of dementia and brain metabolism. In patients with mild to moderate Alzheimer's disease, memory and intellectual deficits were evident without major reductions in absolute metabolic rates, while ratios of regional to whole brain metabolism revealed reductions in regions of the parietal lobes. In the late, severe form of the disease, brain metabolic rates were consistently and significantly reduced. The findings suggest that memory and intellectual deficits are reflected in reductions of brain metabolism in some brain regions in mild to moderate forms of Alzheimer's disease and that, in the late, severe form of the disease, reductions occur consistently throughout the brain.
Abnormalities of the nucleus basalis in Down's syndrome
Authors: Casanova Manuel F. , Walker Lary C. , Whitehouse Peter J. , Price Donald L. 
Published: January 2005

AbstractOne of the most striking manifestations of Down's syndrome is profound mental retardation. Furthermore, after 35 years of age, many patients with Down's syndrome develop clinical and pathological features of Alzheimer's disease. Since brains of patients with Alzheimer's disease show significant loss of neurons in the nucleus basalis of Meynert (nbM), we sought to establish normal standards of nbM neurons in persons with Down's syndrome and to determine whether reductions in the number of neurons occur with increasing age. The number and size of neurons in the nbM were measured in selected sagittal sections from 5 patients with Down's syndrome and 5 age‐matched controls. The patients (age range, 16 to 56 years) had 29% fewer nbM neurons than controls, and the oldest patient had the lowest cell count of all subjects. The size of nbM neurons did not differ significantly between the two groups. Our results show that the nbM contains fewer neurons in young persons with Down's syndrome than in normal controls and suggest that the number of these nerve cells may be further reduced in older persons with Down's syndrome.
Cerebral infarction in sickle cell trait
Authors: Greenberg Joel , Massey E. Wayne 
Published: January 2005

AbstractSickle cell disease is known to predispose patients to the risk of cerebral infarction. However, only scattered reports exist of the neurological sequelae of the sickle cell trait. Only 8 cases are reported in the English literature, in some of which the sickle cell trait was not documented by hemoglobin electrophoresis. This report describes 2 men, age 35 and 24 years, who developed acute cerebral infarction. Investigation revealed no apparent cause for the lesion other than the sickle cell trait.
Regional glucose utilization and blood flow following graded forebrain ischemia in the rat: Correlation with neuropathology
Authors: Ginsberg Myron D. , Graham David I. , Busto Raul 
Published: January 2005

AbstractRegional patterns of cerebral glucose utilization (rCMRglc) and blood flow (rCBF) were examined in the early recovery period following transient forebrain ischemia in order to correlate early postischemic physiological events with regionally selective patterns of ischemic neuropathology. Wistar rats were subjected to 30 or 60 minutes of graded forebrain ischemia by a method combining unilateral occlusion of the common carotid artery with moderate elevation of intracranial pressure and mild hypotension; this procedure results in a high‐grade ischemic deficit affecting chiefly the lateral neocortex, striatum, and hippocampus ipsilateral to the carotid occlusion. Simultaneous measurements of rCMRglc and rCBF made in regional tissue samples after 2 and 4 hours of postischemic recirculation using a double‐tracer radioisotopic strategy revealed a disproportionately high level of glucose metabolism relative to blood flow in the early postischemic striatum, owing to the resumption of nearly normal rCMRglc in the face of depressed flow. In contrast, the neocortex, which had been equally ischemic, showed parallel depressions of both metabolism and blood flow during early recovery. Light microscopy at 4 and 8 hours after recovery revealed the striatum to be the predominant locus of ischemic neuronal alterations, whereas neocortical lesions were much less prominent in extent and severity at this time. The resumption of normal levels of metabolism in the setting of a disproportionate depression of rCBF in the early postischemic period may accentuate the process of neuronal injury initiated by ischemia and may contribute to the genesis of neuronal necrosis in “selectively vulnerable” areas of the forebrain.
Hallervorden‐Spatz disease: Cysteine accumulation and cysteine dioxygenase deficiency in the globus pallidus
Authors: Perry Thomas L , Norman Margaret G. , Yong Voon Wee , Whiting Sharon , Crichton John U. , Hansen Shirley , Kish Stephen J. 
Published: January 2005

AbstractWe describe neurochemical abnormalities found in the brains of 2 patients with autopsy‐confirmed Hallervorden‐Spatz (HS) disease. In 1 patient, contents of cystine and of glutathione‐cysteine mixed disulfide in the globus pallidus were markedly elevated above values for appropriate control subjects. Activity of cysteine dioxygenase, which converts cysteine to cysteine sulfinic acid, was reduced in the globus pallidus, but normal in the frontal cortex and putamen of both patients. γ‐Aminobutyric acid content was markedly decreased in the globus pallidus and substantia nigra of both patients. These results suggest that cysteine accumulates locally in the globus pallidus in Hallervorden‐Spatz disease as a result of an enzymatic block in the metabolic pathway from cysteine to taurine. Accumulated cysteine may chelate iron, accounting for the local increase in iron content in Hallervorden‐Spatz disease. The combined excess of cysteine and ferrous iron may generate free radicals that damage neuronal membranes to cause the typical morphological changes observed in this disorder.
Progressive multifocal leukoencephalopathy: Investigation of three cases using in situ hybridization with JC virus biotinylated DNA probe
Authors: Aksamit Allen J. , Mourrain Pascale , Sever John L , Major Eugene O. 
Published: January 2005

AbstractUsing the technique of in situ DNA‐to‐DNA hybridization, a JC virus biotinylated DNA probe was developed and applied to formalin‐fixed, paraffin‐embedded, or fixed, frozen sections of brain tissue from three subjects with progressive multifocal leukoencephalopathy (PML). Light microscopy was carried out to correlate the presence of JC virus DNA with the selective infection of oligodendrocytes and astrocytes in PML. Oligodendrocytes (lytically infected) showed the greatest evidence of viral DNA. More astrocytes showing bizarre morphological changes had evidence of viral DNA than did astrocytes that were simply reactive. Viral DNA was not evident in vascular endothelial cells using this technique. Viral DNA replication may be an important initial step which produces the bizarre “transformed” astrocytes of PML. Findings in this study do not support the hypothesis that vascular endothelial replication is important in the pathogenesis of JC virus–induced PML. In situ hybridization with biotinylated JC virus probe may be useful in the diagnosis of PML on brain biopsy specimens.
Treatment of cerebrotendinous xanthomatosis with chenodeoxycholic acid
Authors: Pedley Timothy A. , Emerson Ronald G. , Warner Carolyn L. , Rowland Lewis P. , Salen Gerald 
Published: January 2005

AbstractWe describe a patient with cerebrotendinous xanthomatosis who was treated for one year with chenodeoxycholic acid. Modest clinical improvement was accompanied by marked improvement in visual and brainstem auditory evoked potentials. Improved central nervous system function coincided with return of plasma and cerebrospinal fluid cholestanol levels to normal.
Dopaminergic and cholinergic lesions in progressive supranuclear palsy
Authors: Ruberg Merle , Javoy‐Agid France , Hirsch Etienne , Scatton Bernard , Lheureux Rémy , Hauw Jean‐Jacques , Duyckaerts Charles , Gray Françloise , Morel‐Maroger Ariel , Rascol André , Serdaru Michel , Agid Yves 
Published: January 2005

AbstractIn 9 patients with progressive supranuclear palsy and in 27 controls, dopamine and homovanillic acid concentrations, choline acetyltransferase (CAT) activity, and the number of [3H]spiperone and [3H]quinuclidinyl benzilate binding sites were measured post mortem in the striatum (caudate nucleus, putamen, and nucleus accumbens), substantia innominata, and frontal cortex. Dopamine and homovanillic acid concentrations were reduced in the caudate nucleus and putamen but not in the nucleus accumbens or frontal cortex, indicating that the nigrostriatal dopaminergic system is lesioned in patients with progressive supranuclear palsy (as in those with Parkinson's disease) but not the mesocortical and mesolimbic dopaminergic systems, which are lesioned in parkinsonian patients. CAT activity and [3H]spiperone binding decreased in parallel fashion in all the structures. In the striatum, this suggests that the cholinergic neurons, which are target cells of the nigrostriatal system, also degenerate in this disease. This might explain the decrease in the number of dopamine receptors as well as the inefficacy of levodopa or anticholinergic therapy in these patients. The decrease in CAT activity in the substantia innominata and the frontal cortex indicates that the innominatocortical cholinergic system is lesioned in patients with progressive supranuclear palsy and may play a role in the intellectual deterioration observed. This lesion is also found in demented patients with Alzheimer's and Parkinson's diseases.
Progressive supranuclear palsy: Relationship between extrapyramidal disturbances, dementia, and brain neurotransmitter markers
Authors: Kish S. J. , Chang L. J. , Mirchandani L , Shannak K. , Hornykiewicz O. 
Published: January 2005

AbstractBiochemical markers for the major neurotransmitter systems were measured in the brains of 5 patients who had died with neuropathologically confirmed progressive supranuclear palsy. A marked nigrostriatal dopamine deficiency, observed in 4 of the 5 patients, was sufficient to explain the parkinsonian features (especially bradykinesia) observed in patients with progressive supranuclear palsy. Dopamine concentrations, however, were normal in the nucleus accumbens, hypothalamus, and temporal cortex. Brain noradrenalin, serotonin, γ‐aminobutyric acid, and aspartic acid levels were generally normal. Normal brain choline acetyltransferase activity (the marker enzyme for cholinergic neurons) in the 2 patients with severe dementia suggests that, at least in some patients, the cognitive impairment in this disorder is likely to be related to noncholinergic neurotransmitter system changes. The glutamic acid concentration was elevated in many brain areas in 3 of the 5 patients studied.
Nonepileptogenic epileptiform electroencephalographic activity
Authors: Klass Donald W. , Westmoreland Barbara F. 
Published: January 2005

AbstractAccumulated experience has delineated numerous distinctive electroencephalographic (EEG) patterns that are morphologically epileptiform but have no relationship to the process responsible for generating epileptic seizures and have little or no practical value for diagnosis. These anomalous patterns need to be distinguished carefully from the highly abnormal types of EEG activity that continue to have important diagnostic value.
Monoclonal IgM in a patient with paraproteinemic polyneuropathy binds to gangliosides containing disialosyl groups
Authors: Ilyas Amjad A. , Quarles Richard H. , Dalakas Marinos C. , Fishman Peter H. , Brady Roscoe O. 
Published: January 2005

AbstractMonoclonal IgM K from a patient with polyneuropathy associated with paraproteinemia was found to bind to several polysialogangliosides. Binding of IgM to gangliosides was shown by enzyme‐linked immunosorbent assay (ELISA) and by overlaying thin‐layer chromatograms of human brain and peripheral nerve gangliosides with the patient's serum followed by radioiodinated goat antihuman IgM. The latter technique showed that the IgM paraprotein reacted with a number of gangliosides. In an ELISA the IgM paraprotein reacted strongly with GD2, GD3, GD1b, and GT1b, but not with GM1, GM3, and GD1a. Thus, the epitope for the patient's IgM paraprotein appears to involve the disialosyl configurations.
Identification of the inflammatory cells in the central nervous system of patients with adrenoleukodystrophy
Authors: Griffin Diane E. , Moser Hugo W. , Mendoza Querubin , Moench Thomas R. , O'Toole Susan , Moser Ann B. 
Published: January 2005

AbstractAdrenoleukodystrophy is a disorder of long‐chain fatty acid metabolism associated with adrenal cortical insufficiency and central nervous system demyelination. The central nervous system disease is unusual in that it is abrupt in onset and accompanied by a considerable infiltration of mononuclear inflammatory cells. To determine the nature of these inflammatory cells, immunocytochemical staining was carried out on the mononuclear cells in the brain and cerebrospinal fluid of patients with adrenoleukodystrophy. Monoclonal antibodies to T lymphocytes (T11), the helper/inducer (T4) and cytotoxic/suppressor (T8) subsets of T lymphocytes, B lymphocytes (B1), and monocyte/macrophages (Ml or esterase) were used. Mononuclear cells in the perivascular cuffs of autopsy material from 4 patients were, on average, 59% T cells, 34% T4 cells, 16% T8 cells, 24% B cells, and 11% monocyte/macrophages. Cerebrospinal fluid from 8 of 10 patients had increased IgG concentrations. Mononuclear cells in the cerebrospinal fluid of 6 patients with active disease were, on average, 61% T cells, 40% T4 cells, 16% T8 cells, 3% B cells, and 18% monocyte/macrophages. This distribution of cells is similar to that found in the central nervous system during a cellular immune response and suggests the possibility that one component of this disease is immunologically mediated.
Acute effects of antidepressants on hippocampal seizures
Authors: Clifford David B. , Rutherford James L. , Hicks Frederick G. , Zorumski Charles F. 
Published: January 2005

AbstractElectrically kindled hippocampal seizures in rats were used to evaluate the acute effects of the antidepressants amitriptyline, imipramine, desipramine, bupropion, maprotiline, and trazodone on behavioral and electrical convulsions. All of the drugs reduced afterdischarge duration significantly. Behavioral seizures were not significantly reduced by bupropion or trazodone, whereas the other drugs did reduce seizure severity. Afterdischarge threshold was not modified by these drugs. In contrast, phenobarbital significantly elevated threshold and reduced seizure severity and afterdischarge duration. Amitriptyline was the most effective antidepressant, attenuating both seizure severity and afterdischarge duration.
Multiple sclerosis: Activated cells in cerebrospinal fluid in acute exacerbations
Authors: Noronha Avertano , Richman David P. , Arnason Barry G. W. 
Published: January 2005

AbstractCerebrospinal fluid (CSF) lymphocytes from three young patients undergoing acute exacerbations of multiple sclerosis were studied by flow cytometry. Using a new method that simultaneously measures cell‐surface antigens and the cell‐cycle phase, we determined that the CSF lymphocytes in these patients were activated. The majority of the activated cells expressed the OKT4 phenotype, while very few expressed the OKT8 phenotype.
Thrombotic cerebral vasculopathy associated with herpes zoster
Authors: Eidelberg David , Sotrel Ana , Horoupian D. S. , Neumann Paul E. , Pumarola‐Sune Tomas , Price Richard W. 
Published: January 2005

AbstractWe describe the clinical, radiographic, and pathological findings in 3 patients with large‐vessel cerebral vasculopathy following herpes zoster. Two of the patients were studied at postmortem examination, and a brain biopsy was performed in the third. Each of the 3 patients suffered thrombotic occlusions of large vessels without notable inflammatory or granulomatous changes following trigeminal or segmental herpes zoster infection. In the 2 autopsied patients, varicella‐zoster virus (VZV) antigens were detected by immunoperoxidase staining within the media of the affected cerebral arteries. Little or no inflammation was associated with the foci of the VZV antigens. These studies provide evidence that the vasculopathy following herpes zoster may result from direct VZV infection of the artery and the in situ thrombosis can develop within the infected vessels in the absence of clear inflammatory vasculitis.
Impaired slow axonal transport in wobbler mouse motor neuron disease
Authors: Mitsumoto Hiroshi , Gambetti Pierluigi 
Published: January 2005

AbstractWe studied slow axonal transport and morphometry of forelimb axons in wobbler mice and controls. In wobbler mice, the total radioactivity migrating with the slow transport was decreased by 50%. The velocity of transport also appeared to be reduced; 15 days following administration of a radioisotope, polypeptides migrating with slow component a of transport did not form a peak and remained mostly 2 mm from the spinal cord, while in controls slow component a was distributed as a peak which was located 4 mm from the cord. The ratios of the 68‐kDa neurofilament subunit to tubulin and actin were significantly decreased (p < 0.01 and p < 0.005, respectively). This finding is consistent with a preferential reduction of the radioactivity migrating with neurofilament proteins in wobbler mice. Moreover, both the size and number of myelinated axons were markedly diminished, but their length was not significantly different, indicating that dying‐back does not take place in axons of wobbler mice up to 12 mm from the spinal cord. The reduction in axonal transport may be due to the reduction in number and caliber of the axons and/or to reduced protein synthesis in cervical lower motor neurons; however, the abnormal distribution of the radioactive substance definitely results from impairment of the slow transport in the axons of the forelimb roots of wobbler mice. The transport impairment is not related to the presence of morphological changes in the perikaryon of wobbler mouse lower motor neurons, as it is much more widespread than would be expected if only altered neurons were involved. Whether the impairment of the slow transport is the primary pathogenetic event in wobbler mouse lower motor neuron disease, as well as in other animals and human motor neuron diseases, remains to be established.
Low CSF protein concentration in idiopathic pseudotumor cerebri
Authors: Chandra Vijay , Bellur Srinath N. , Anderson Robert J. 
Published: January 2005

AbstractAn inverse relationship between the cerebrospinal fluid (CSF) opening pressure and the corresponding protein value was found for patients with idiopathic pseudotumor cerebri who underwent their first lumbar puncture (LP) prior to the initiation of therapy. This relationship provides evidence for a pressure‐dependent bulk flow absorption for CSF and also suggests that patients with high CSF pressures will have low CSF protein concentration.