bluuebunny/crossref_metadata_2025_split huggingface dataset:
Recurrent status epilepticus in children
Authors: Shinnar Shlomo , Maytal Joseph , Krasnoff Lawrence , Moshe Solomon L. 
Published: January 2005

AbstractStatus epilepticus is an uncommon but life‐threatening seizure. Little is known about the risk of recurrent status epilepticus in patients who present with an initial episode. To determine the risk of recurrent status epilepticus in children, we prospectively followed 95 children, identified at the time of their first episode of status epilepticus, for a mean of 29.0 months (range, 4–60 months). The patients' ages ranged from 1 month to 18 years (mean, 4.6 years). The cause of the status epilepticus was classified as idiopathic (n = 24), remote symptomatic (n = 18), febrile (n = 29), acute symptomatic (n = 18), or progressive neurological disorder (n = 6). Sixteen children (17%) had at least 2 episodes of status epilepticus. The risk of recurrent status was 4% (n = 1) in the idiopathic group, 44% (n = 8) in the remote symptomatic group, 3% (n = 1) in the febrile group, 11% (n = 2) in the acute symptomatic group, and 67% (n = 4) in those with progressive neurological disease. Recurrent status epilepticus occurred primarily in neurologically abnormal children. While neurologically abnormal children accounted for 34% (n = 32) of all children with status epilepticus, they comprised 88% (n = 14) of the children with recurrent status epilepticus (p< 0.001) and all 5 of the children with multiple (≥ 3) episodes of status (p< 0.001). Fifteen of 16 children with recurrent status epilepticus were being treated with antiepileptic drugs at the time of recurrence. The morbidity and mortality of status epilepticus were low. No child had a measurable deterioration in neurological function following a subsequent episode of status epilepticus. Recurrent status epilepticus occurs primarily in children with an underlying neurological abnormality. Status epilepticus reoccurs in this group despite conventional antiepileptic drug therapy.
Motion‐defined letter detection and recognition in patients with multiple sclerosis
Authors: Giaschi D. , Regan D. , Kothe A. , Hong X. H. , Sharpe J. A. 
Published: January 2005

AbstractTwo important distinctions in visual perception are (1) between the detection and recognition of shapes (e.g., letters), and (2) between the recognition of shapes defined by a difference in brightness and the recognition of shapes defined by a difference in motion. We report that 6 of 10 patients with multiple sclerosis showed impaired recognition for motion‐defined (Md) letters, although the detection of Md letters was normal as were both detection and recognition of luminance‐defined letters. We have shown that this was not a function of acuity loss or the loss of ability to detect motion or a general failure of recognition per se, but was confined to a loss of ability to recognize Md letters. The neurological implications of these findings are discussed, and it is suggested that the Md letter test be used by others interested in the central pathology of visual disorders.
β‐Adrenergic receptor density and function of peripheral blood mononuclear cells are increased in multiple sclerosis: A regulatory role for cortisol and interleukin‐1
Authors: Zoukos Y. , Leonard J. P. , Thomaides T. , Thompson A. J. , Cuzner M. L. 
Published: January 2005

AbstractAn increased density of β‐adrenergic receptors was demonstrated on peripheral blood mononuclear cells (PMMCs) from patients with progressive or replapsing‐remitting multiple sclerosis (MS). The same observation was made in patients with chronic active rheumatoid arthritis, but not in those with myasthenia gravis. The affinity of the receptors was within the normal range in all tested groups of patients and there was a positive correlation between density and function as determined by intracellular cyclic AMP production after stimulation with isoproterenol. A putative link between inflammatory processes and the functional upregulation of β‐adrenergic receptors on PBMCs was tested by in vitro studies with the soluble mediators interleukin‐1 and hydrocortisone. A functional upregulation of β‐adrenergic receptors was observed when PBMCs from normal control subjects were cultured in the presence of either mediator, whereas the already upregulated receptor density on PBMCs from patients with MS remained unchanged. Whether this represents a recovery mechanism to inflammation in MS or a blunting of homeostatic immunoregulatory mechanisms requires further investigation.
Immunological detection of glutamate receptor subtypes in human central nervous system
Authors: Blackstone Craig D. , Levey Allan I. , Martin Lee J. , Price Donald L. , Huganir Richard L. 
Published: January 2005

AbstractGlutamate receptors are the principal excitatory neurotransmitter receptors in the central nervous system and are involved in a number of normal and pathological neuronal processes. Using subunit‐specific antipeptide antibodies developed against the predicted amino acid sequences of several rat glutamate receptor cDNAs, we have identified these proteins in post‐mortem human central nervous system tissue. Immunoblotting of dissected brain regions demonstrates that these receptor proteins are differentially distributed. The ability to identify these proteins in post‐mortem human tissues should allow examination of the changes in levels of receptor subtypes that occur in a variety of neurological and psychiatric diseases.
Morphometry, histopathology, and tomography of cerebral atrophy in the acquired immunodeficiency syndrome
Authors: Gelman Benjamin B. , Guinto Faustino C. 
Published: January 2005

AbstractPatients with the acquired immunodeficiency syndrome (AIDS) commonly display evidence of gross cerebral atrophy, but its true incidence and pathophysiology in the general AIDS population are unknown. In this study, we measured cerebrospinal fluid (CSF) spaces in 64 consecutively autopsied patients with AIDS, compared them with age‐matched non‐AIDS subjects, and correlated them with the microscopic neuropathology at autopsy. Thirty‐seven of the patients with AIDS (58%) had a CSF space index greater than two standard deviations above the mean of the age‐matched control subjects. CSF spaces were expanded the most in frontal and temporal lobes; ventricular spaces were expanded more than sulcal spaces. Regression analysis between age and the measured CSF indexes showed that practically none of the deviation in patients with AIDS could be explained by age‐related atrophy. Patients with atrophy were much more likely to have human immunodeficiency virus type 1–associated histopathological changes in their brains, but the relationships were too weak to establish the microscopic cause of the atrophy. Measurement of CSF spaces in antemortem computed tomographic (CT) scans from 47 of the patients with AIDS revealed the same regional pattern of brain wasting. CT diagnosis of cerebral atrophy was strongly concordant with the postmortem diagnosis, but its severity was diagnosed slightly more conservatively at autopsy. Thus, quantitative analysis of CSF spaces in a robust sampling of patients with AIDS confirms that cerebral atrophy is a sensitive, common, and frequently subtle indication of central nervous system human immunodeficiency virus type 1 infection that may occur independently from any single specific microscopic abnormality. Its presence, severity, and regional pattern are detected with reasonable consistency using CT scanning.
Recombinant tissue plasminogen activator in acute thrombotic and embolic stroke
Authors: del Zoppo Gregory J. , Poeck Klaus , Pessin Michael S. , Wolpert Samuel M. , Furlan Anthony J. , Ferbert Andreas , Alberts Mark J. , Zivin Justin A. , Wechsler Lawrence , Busse Otto , Greenlee Ralph , Brass Lawrence , Mohr J. P. , Feldmann Edward , Hacke Werner , Kase Carlos S. , Biller Jose , Gress Daryl , Otis Shirley M. 
Published: January 2005

AbstractAn open angiography‐based, dose rate escalation study on the effect of intravenous infusion of recombinant tissue plasminogen activator(rt‐PA) on cerebral arterial recanalization in patients with acute focal cerebral ischemia was performed at 16 centers. Arterial occlusions consistent with acute ischemia in the carotid or vertebrobasilar territory in the absence of detectable intracerebal hemorrhage were prerequisites for treatment. After the 60‐minute rt‐PA infusion, arterial perfusion was assesed by repeat angiography and computed tomography scans were performed at 24 hours to assess hemorrhagic transformation, Of 139 patients with symptoms of focal ischemia, 80.6% (112) had complete occlusion of the primary vessel at a mean of 5.4 ± 1.7 hours after symptom onset. No dose rate response of cerebral arterial recanalization was observed in 93 patients who completed the rt‐PA infusion. Middle cerebral artery division (M2) and branch (M3) occlusions were more likely to undergo recanalization by 60 minutes than were internal carotid artery occlusions. Hemorrhagic infarction occured in 20.2% and parenchymatous hematoma in 10.6% of patients over all dose rates, while neurological worsening accompanied hemorrhagic transformation (hemorrhagic infarction and parenchymatous hematoma) in 9.6% of patients. All findings were within prospective safety guidelines. No dose rate correlation with hemorrhagic infarction, parenchymatous hematoma, or both was seen. Hemorrhagic transformation occured significantly more frequently in patients receiving treatment at least 6 hours after symptom onset. No relationship between hemorrhagic transformation and recanalization was observed. This study indicates that site of occlusion, time to recanalization, and time to treatment are important variables in acute stroke intervention with this agent.
Magnetoencephalographic localization of epileptic cortex—impact on surgical treatment
Authors: Paetau Ritva , Kajola Matti , Karhu Jari , Nousiainen Unto , Partanen Juhani , Tiihonen Jari , Vapalahti Matti , Hari Riitta 
Published: January 2005

AbstractA 24‐channel, Planar, superconducting quantum interference device gradiometer, sampling a fourth of the head surface over brain tissue, was used to determine the site of an epileptic focus in a 36‐year‐old woman with intractable complex partial epilepsy. The other presurgical findings appeared divergent: a large arachnoid cyst over the right parietal convexity, dissimilar interictal electroencephalographic patterns, and several neuropsychological dysfunctions. The equivalent current sources of magnetoencephalographic spikes were in the right posterior temporal region of the cortex, 4 cm apart from the cyst. Surgical exploration of the area pinpointed by magnetoencephalography revealed a pachygyric patch of cortex displaying focal discharges on the electrocorticogram. After resection, a dramatic reduction of seizures occurred. The good agreement between electrocorticography and magnetoencephalography warrants future investigation of multichannel magnetoencephalography as a potential alternative to invasive presurgical recordings.
Computer model of ethosuximide's effect on a thalamic neuron
Authors: Lytton William W. , Sejnowski Terrence J. 
Published: January 2005

AbstractEthosuximide appears to have a specific effect on the low‐threshold calcium current in thalamic cells. This may be related to its efficacy in the treatment of absence epilepsy. We used a computer model of an individual thalamocortical neuron to better understand the alteration in the low‐threshold calcium current under voltage clamp and to predict response to current injection in the presence of ethosuximide. The full model included nine voltage‐sensitive ionic channels and a realistic dendritic morphology. The model reproduced the two major responses seen in tissue slices: repetitive spiking with depolarization and the low‐threshold calcium spike elicited on release from hyperpolarization. The alteration in low‐threshold calcium current with ethosuximide can be explained by a 10‐mV depolariting shift in the steady‐state activation curve for this channel with a 10% reduction in maximum channel permeability. Simulations of current injection showed that ethosuximide diminished the low‐threshold calcium spike while leaving the tonic firing pattern unaffected. Our results support the hypothesis that ethosuximide's effects on low‐threshold calcium current might selectively alter the dynamics of slow bursting in thalamic cells.
Spectral analysis of the rapid eye movement sleep electroencephalogram in right and left temporal regions: A biological marker of Alzheimer's disease
Authors: Petit Dominique , Montplaisir Jacques , Lorrain Dominique , Gauthier Serge 
Published: January 2005

AbstractSpectral analysis of electroencephalograms (EEGs) for both wakefulness and rapid eye movement (REM) sleep was performed over the temporal regions in 8 patients with mild to moderate Alzheimer's disease and in 8 age‐matched control subjects. EEG slowing in Alzheimer patients was found to be much more prominent during REM sleep than during wakefulness. In addition, asymmetry on the awake EEG of Alzheimer patients was found to be even more prominent than on the REM sleep EEG. When EEG values of the most impaired hemisphere during REM sleep were examined, no overlap was found between the two groups either for the ratio of slow to fast frequencies or for percent power of each of the frequency bands. This was not the case for the awake EEG. These results suggest that diagnostically meaningful cutoff values for discriminating patients with mild to moderate Alzheimer's disease from age‐matched control subjects can be derived from the REM sleep EEG of the temporal lobe.
Myelin basic protein–specific T lymphocytes in multiple sclerosis and controls: Precursor frequency, fine specificity, and cytotoxicity
Authors: Jingwu Zhang , Medaer Robert , Hashim George A. , Chin Ying , van den Berg‐Loonen Ella , Raus Jef C. M. 
Published: January 2005

AbstractA panel of 90 myelin basic protein (MBP)‐specific T‐cell lines were derived from peripheral blood of eight patients with multiple sclerosis and four normal subjects. The precursor frequency of MBP‐reactive T cells in peripheral blood mononuclear cells ranged from 10−7 to 9 × 10−7 (mean, 6.7 × 10−7) in the group of patients with multiple sclerosis and from 0.5 × 10−7 to 9.8 × 10−7 (mean, 5.6 × 10−7) in the control subjects. This difference between the two groups was not statistically significant (p > 0.1). These T‐cell lines expressed exclusively CD3+CD4+CD8‐ phenotypes and were restricted predominantly by HLA‐DR molecules. When tested with fragments and synthetic peptides of human MBP, these MBP‐specific T‐cell lines (45 lines for each group) displayed a limited heterogeneous pattern with a biased recognition to peptide 84‐102 and the C‐terminal peptide 149‐171. The reactivity to the 84‐102 region of MBP was associated with the HLA‐DR2, DRw15 (DRwl5,2) haplotype, whereas the recognition to peptide 149‐171 did not correlate with a particular HLA‐DR allele(s). Furthermore, the majority of T‐cell lines (>75%) were found to exhibit substantial cytotoxic activity against MBP‐coated target cells, but showing no significant difference between these two groups. This MBP‐dependent Cytotoxicity was not associated with epitope specificities of the T‐cell lines tested.
Isolated granulomatous angiitis of the spinal cord
Authors: Caccamo Dario V. , Garcia Julio H. , Ho Khang‐L. 
Published: January 2005

AbstractWe describe a 31‐year‐old diabetic man, with granulomatous angiitis confined to the spinal cord, who developed rapidly progressive spastic paraplegia, clinically interpreted as being secondary to a spinal cord infarct. At the time of autopsy, vasculitis was limited to the spinal cord, without involvement of cerebral vessels. The inflammatory cells were predominantly CD4+ T lymphocytes, with few CD8+ T and B lymphocytes. The phenotypical composition of the inflammatory infiltrate is similar to that described in other granulomatous disorders such as sarcoidosis and tuberculin reaction.
Cortical biopsy in Alzheimer's disease: Diagnostic accuracy and neurochemical, neuropathological, and cognitive correlations
Authors: DeKosky Dr Steven T. , Harbaugh Robert E. , Schmitt Frederick A. , Bakay Roy A. E. , Chui Helena Chang , Knopman David S. , Reeder Teddi M. , Shetter Andrew G. , Senter Howard J. , Markesbery William R. ,   The Intraventricular Bethanecol Study Group
Published: January 2005

AbstractNeurochemical assessments were performed on biopsy samples taken from the right frontal lobe of patients diagnosed with Alzheimer's disease (AD), before the implantation of a ventricular catheter and pump assembly for the infusion of bethanechol chloride as an experimental therapy. The pathologically diagnosed patients with AD (n = 35; mean age, 67 ± 1.5 yr) were compared with a group of samples from normal age‐equivalent autopsied controls (n = 22; mean age, 68 ± 2 yr) and autopsied AD brains (n = 11; mean age, 73 ± 2 yr). Samples were assayed for choline acetyltransferase (ChAT), acetylcholinesterase, binding to {3H}quinuclidinyl benzilate as an index of total muscarinic cholinergic binding, and [3H}pirenzepine binding as an index of Ml cholinergic receptor subtype binding. Mean levels of ChAT activity were decreased in the biopsied patients to 36% of age‐matched autopsied controls. The loss of ChAT activity correlated significantly with the Mini‐Mental State Examination, an index of global cognitive function. Mean ChAT activity in autopsied AD cortex was further decreased compared with controls, indicating continuous decline through the course of the disease. Acetylcholinesterase followed a similar, less dramatic decline. No differences were found in {3H}quinuclidinyl benzilate binding or {3H}pirenzepine binding between biopsied and autopsied controls. Neuritic plaque counts did not correlate with either the Mini‐Mental State Examination or ChAT activity in the biopsy specimens. The correlation of cortical ChAT activity with degree of dementia, although considerably weaker than those of cortical synaptic density with dementia, is the first demonstration of such a relationship in biopsied patients, and suggests another reason why the AD brain may be unresponsive to presynaptic cholinergic manipulations or attempts at enhancement.
Serial cranial and spinal cord magnetic resonance imaging in multiple sclerosis
Authors: Wiebe S. , Lee D. H. , Karlik S. J. , Hopkins M. , Vandervoort M. K. , Wong C. J. , Hewitt L. , Rice G. P. A. , Ebers G. C. , Noseworthy J. H. 
Published: January 2005

AbstractTwenty‐nine mildly disabled patients with multiple sclerosis underwent serial clinical and magnetic resonance imaging (MRI) evaluations (pre‐ and postgadolinium cranial and spinal cord MRI) on at least 3 occasions at 13‐week intervals and during periods of suspected relapse. Using clinical judgment of the presence of recent active disease as the gold standard, combined MRI studies confirmed the clinical impression of active disease in 93% of follow‐up visits (sensitivity) and the absence of active MS in 63% of follow‐up visits (specificity). None of the cranial and spinal MRI‐detected abnormalities disappeared. Gadolinium administration particularly increased the yield of spinal MRI. Cranial MRI alone detected 80% of the MRI‐active visits. Clinical and MRI concordance was significantly better for the presence of recent disease activity than for the anatomical localization of the presumed site of activity. MRI evidence of apparent ongoing disease activity was seen more frequently in patients believed to have active multiple sclerosis in the preceding year (13 of 21) than in patients who had been in clinical remission for at least the 2 preceding years (2 of 8). Although clinical evidence of new disease activity was much less common in patients with active, chronic‐progressive disease (1 of 8) than in patients with active, relapsing disease (9 of 13), the proportion of patients with either infrequent relapses, frequent relapses, or slow chronic‐progressive disease in the preceding year in whom MRI activity developed and the pattern of this new MRI activity was similar between these types of active patients. This finding suggests that although there are differences in the clinical expression of disease, there may not be a fundamental difference between mild, active relapsing and mild, active progressive multiple sclerosis as defined by MRI.
Dopamine D1 and D2 receptors in progressive supranuclear palsy: An autoradiographic study
Authors: Pascual J. , Berciano J. , Grijalba B. , del Olmo E , González A. M. , Figols J. , Pazos A. 
Published: January 2005

AbstractDopamine Dl and D2 receptors were studied in brain tissue sections from a typical patient with progressive supranuclear palsy and in 7 age‐matched brains. The sensity of Dl receptors in the caudate‐putamen and fontal cortex of the patient was within control limits, By contrast, the density of nigral Dl receptors and strial D2 receptors was dramatically reduced in the patient as compared to the control brains. This work shows again that the loss of striatal D2 receptors is the most plausible explanation for the poor response to dopaminergic drugs in patients with progressive supranuclear palsy. While the loss of nigral Dl receptors can be explained by the loss of nigral neurons, it seems that neurons bearing striatal Dl receptors are spared in progressive supranuclear palsy. The clinical effects of selective D1 agonists are worth testing in this devastating disorder.
Chronic encephalitis (rasmussen's syndrome) and ipsilateral uveitis
Authors: Harvey A. Simon , Andermann Frederick , Hopkins Ian J. , Kirkham Trevor H. , Berkovic Samuel F. 
Published: January 2005

AbstractTwo children are reported with acute uveitis during the early progressive phase of chronic encephalitis (Rasmussen's syndrome). In both children, the side of the uveitis was ipsilateral to the side of cerebral inflammation, although in 1 child there were milder inflammatory changes in the contralateral eye. This association adds weight to the viral hypothesis of chronic encephalitis and raises the possibility of primary ocular infection and neurotropic spread to the brain.
Neurological involvement in Wegener's granulomatosis: An analysis of 324 consecutive patients at the Mayo Clinic
Authors: Nishino Hiroshi , Rubino Frank A. , DeRemee Richard A. , Swanson Jerry W. , Parisi Joseph E. 
Published: January 2005

AbstractNeurological involvement in Wegener's granulomatosis was studied by reviewing the charts of 324 consecutive patients in whom the diagnosis was made at the Mayo Clinic. One hundred nine patients (33.6%) had neurological involvement. Peripheral neuropathy occured in 53; cranial neuropathy, in 21; external ophthalmoplegia, in 16; cerebrovascular events, in 13; seizures, in 10; cerebritis, in 5; and miscellaneous involvement, in 25. The mean age and sex ratio were similar in the patients with and those without neurological involvement. Among the patients with peripheral neuropathy, 42 had mononeuropathy multiplex; 6, distal symmetrical polyneuropathy; and 5, unclassified peripheral neuropathy. Multiple mononeuropathy was a major presenting symptom in 8 patients. A significantly higher percentage of patients with peripheral neuropathy, compared to those without peripheral neuropathy, had kidney involvement (p < 0.001). The second, sixth, and seventh cranial nerves were most frequently affected. Multiple cranial nerves were affected in 8 patients. Unusual neurological manifestations in the miscellaneous group were spastic paraparesis, temporal arteritis, Horner's syndrome, and papilledema.