bluuebunny/crossref_metadata_2025_split huggingface dataset:
Posterior cerebral artery stenosis
Authors: Pessin Michael S. , Kwan Eddie S. , DeWitt L. Dana , Hedges Thomas R. , Gale Daniel , Caplan Louis R. 
Published: January 2005

AbstractWe analyzed the clinical features of symptomatic posterior cerebral artery (PCA) stenosis in 6 patients selected from 15 patients with angiographically documented PCA atherostenosis occurring during a 7‐year period. Transient ischemic attacks (TIAs) were the major presentation in 5 patients. A homonymous visual field defect was present in 2 patients. TIA symptoms were predominantly visual or sensory, or both. The most common visual symptom was difficulty seeing to one side. One patient saw flashing lights. Sensory spells were always paresthetic, usually involving the arm and hand and occasionally the face and leg. Three patients had visual and sensory spells together. Two patients with a visual field defect had calcarine infarcts found by computed tomography. All patients were treated with warfarin. During followup (4 months to 4 years), no patient had a new stroke in the PCA territory, and only one continued to have TIAs. PCA atherostenosis is rarer than PCA embolic occlusion. In contrast to those with PCA embolism, our patients with PCA atherostenosis had more TIAs and fewer infarcts. The clinical features of PCA stenosis—preponderance of visual and sensory TIAs‐distinguish this vascular lesion from stenosis of the middle cerebral artery.
Endemic tropical spastic paraparesis associated with human T‐lymphotropic virus type I: A clinical and seroepidemiological study of 25 cases
Authors: Vernant J. C. , Maurs L. , Gessain A. , Barin F. , Gout O. , Delaporte J. M. , Sanhadji K. , Buisson G. , De‐Thé G. 
Published: January 2005

AbstractTropical spastic paraparesis (TSP) is a common myeloneuropathy with primary and predominant involvement of the pyramidal tract and minimal sensory loss. The epidemic form of TSP is related to toxic nutritional factors, but the endemic form occurs in clusters in tropical areas, especially in India, Africa, the Seychelles, Colombia, and areas of the Caribbean. We describe the clinical and epidemiological features of 25 TSP patients from Martinique (French West Indies) with serum antibodies to human T‐lymphotropic virus type I (HTLV‐I). Furthermore, all 11 patients who were seropositive for HTLV‐I had specific HTLV‐I antibodies in their CSF. All were women. The age of onset varied from 25 to 60 years (mean, 45 years). The main clinical features are spastic paraparesis or paraplegia with spasticity of the upper limbs, minimal sensory loss, and bladder dysfunction. Minimal estimated incidence and prevalence are 1 per 100,000 inhabitants per year and 8 per 100,000, respectively. Seventeen percent of the relatives of patients with HTLV‐I–associated TSP have HTLV‐I antibodies (1 husband and 7 children). In Martinique, the prevalence of HTLV‐I antibodies in the general population is about 2% and reaches 10% for neurological disorders other than TSP. Since our initial report, the association between spastic paraparesis and HTLV‐I has been confirmed in Jamaica, Colombia, and Japan, suggesting the neurotropism of this lymphotropic human retrovirus.
A correlation between cerebrospinal fluid mylein basic protein and anti–myelin basic protein in multiple sclerosis patients
Authors: Warren Kenneth G. , Catz Ingrid 
Published: January 2005

AbstractFree and bound levels of myelin basic protein (MBP) and anti–myelin basic protein (anti‐MBP) antibodies were measured by radioimmunoassay in the cerebrospinal fluid of patients with multiple sclerosis who were experiencing acute exacerbations or progressing disease. In a cross‐sectional study, free levels of MBP correlated with those of free anti‐MBP, and bound MBP levels correlated with those of bound anti‐MBP in both groups of patients with active disease. However, acute exacerbations of multiple sclerosis were characterized by higher free MBP and anti‐MBP levels with lower levels of bound fractions. Conversely, patients with progressing disease had higher titers of bound than free fractions. Longitudinal studies of individual patients confirmed the association of higher titers of free anti‐MBP with acute exacerbations and higher levels of bound anti‐MBP with chronic progressing disease.
Adrenoleukodystrophy: Dietary oleic acid lowers hexacosanoate levels
Authors: Rizzo William B. , Phillips Mary W. , Dammann Andrea L. , Leshner Robert T. , Jennings Sandra S. , Avigan Joel , Proud Virginia K. 
Published: January 2005

AbstractAdrenoleukodystrophy (ALD) is an X‐linked disorder characterized by demyelination, adrenal insufficiency, and accumulation of saturated very‐long‐chain fatty acids (VLFA), particularly hexacosanoate (C26:0). We treated 5 patients with adrenoleukodystrophy (3 males and 2 symptomatic female carriers) for 6 months with a diet enriched in oleic acid (C18:1) and moderately restricted in C26:0. Elevated plasma and erythrocyte levels of C26:0 decreased in a time‐dependent manner during treatment. Total plasma C26:0 concentration was lowered by 50 9% ± (p < 0.01); it became normal in the female carriers. The total erythrocyte level of C26:0 decreased (44 ± 5%; p < 0.001) into the normal range in all patients. Significant decreases were noted in the saturated VLFA composition of plasma and erythrocyte sphingomyelin and erythrocyte phosphatidylcholine during dietary treatment. In general, decreases in saturated VLFA levels were accompanied by increases in monounsaturated VLFA levels, while total VLFA values did not change. This novel approach to the treatment of adrenoleukodystrophy, in which there is an exchange of monounsaturated VLFA for the more toxic saturated VLFA, may prove clinically beneficial in this disorder.
A new dietary therapy for adrenoleukodystrophy: Biochemical and preliminary clinical results in 36 patients
Authors: Moser Ann B. , Borel Janet , Odone Augusto , Naidu Sakkubai , Cornblath David , Sanders Donald B. , Moser Hugo W. 
Published: January 2005

AbstractA new dietary regimen has been administered for periods ranging from 60 days to 1 1/2 years in 34 patients with various forms of X‐linked adrenoleukodystrophy (ALD), as well as in 1 patient with neonatal ALD and 1 patient with infantile Refsum's disease. The diet combines the administration of a glyceryl trioleate oil (GTO) with the dietary restriction of very‐long‐chain fatty acids (VLFA), particularly hexacosanoic acid (C26:0). Reductions in the levels of plasma C26:0 and other VLFA were achieved in 25 of the 36 patients. Fifteen of these 25 patients were treated for more than 100 days. The mean reduction of the plasma C26:0 level was 53% (range, 22 to 73%) in these 15 patients. While the focus of the study was on biochemical variables, comparison of pre‐ and post‐diet studies of peripheral nerve function showed improvement in 1 patient with adrenomyeloneuropathy (AMN) and 1 heterozygote. In contrast, 2 patients with ALD onset in childhood developed new neurological deficits while on therapy. We conclude that it is possible to lower plasma VLFA levels in ALD patients. A clinical trial is indicated to test whether this approach can alter the neurological progression in patients with AMN or in symptomatic heterozygotes, and to determine whether it can prevent the onset of neurological disability in asymptomatic persons who have the biochemical defect of ALD.
Clinical and magnetic resonance features of primary intracranial arachnoid cysts
Authors: Gandy Samuel E. , Heier Linda A. 
Published: January 2005

AbstractThe clinical features of 33 patients with primary intracranial arachnoid cysts are reviewed. Magnetic resonance imaging was performed in 9 patients and was particularly useful in the multiplanar definition of complex cysts and in differentiating arachnoid cysts from other lesions that are hypodense on computed tomographic scans. Hydrocephalus, cranial nerve palsies, or symptomatic brain compression was considered an indication for surgical intervention in 21 patients. Outcome was uniformly good: mild focal weakness and seizure disorders constituted the sequelae.
False lateralization by surface EEG of seizure onset in patients with temporal lobe epilepsy and gross focal cerebral lesions
Authors: Sammaritano Michele , de Lotbinière Alain , Andermann Frederick , Olivier André , Gloor Pierre , Quesney Luis F. 
Published: January 2005

AbstractMedically intractable temporal lobe seizures developed in 3 patients with radiological and clinical evidence of a gross focal cerebral lesion acquired early in life. All had bilateral independent epileptogenic discharges from the lateral and inferomesial regions of both hemispheres. Scalp and sphenoidal electroencephalographic (EEG) recordings suggested that the seizures originated from the side contralateral to the known cerebral lesion. Because the lateralizing evidence presented by the ictal EEG conflicted with the clinical data, depth electrodes were implanted stereotaxically to determine the side of onset of the seizures. These studies showed that the seizures originated from the limbic structures of the damaged hemisphere in all 3 patients. At operation the mesial temporal lobe structures showed gliotic changes in all. The patients have remained seizure‐free for 3 to 13 years postoperatively. These findings suggest that depth electrode recordings may be required to clarify the lateralization of seizure onset in such cases. Extracranial EEG findings must be interpreted with caution in epileptic patients who have gross focal lesions.
Examination of motor function in lesions of the spinal cord by stimulation of the motor cortex
Authors: Thompson P. D. , Dick J. P. R. , Asselman P. , Griffin G. B. , Day B. L. , Rothwell J. C. , Sheehy M. P. , Marsden C. D. 
Published: January 2005

AbstractElectrical stimulation of the motor cortex was performed in 3 patients with cervical cord trauma and in 3 with cervical myelopathy caused by cervical spondylosis. Abnormal conduction in central motor tracts was demonstrated in 5 of the 6 patients; yet in 4 of these, cortical somatosensory evoked potentials from the same limb were of normal latency. In the remaining patient who had normal motor latencies, the somatosensory evoked potentials were delayed on one side. Electrophysiological examination of the motor pathways by cortical stimulation may therefore be of considerable value in the assessment and management of patients with traumatic and compressive disease of the spinal cord.
Expression of human immunodeficiency virus in cerebrospinal fluid of children with progressive encephalopathy
Authors: Epstein Leon G. , Goudsmit Jaap , Paul Deborah A. , Morrison Susan H. , Connor Edward M. , Oleske James M. , Holland Bart 
Published: January 2005

AbstractThe retrovirus that causes acquired immune deficiency syndrome (AIDS) is now designated the human immunodeficiency virus (HIV). The cerebrospinal fluid (CSF) of 27 children with HIV infection was assayed for intra‐blood‐brain barrier (IBBB) synthesis of HIV‐specific antibodies and for the presence of HIV antigen. In this cohort, 11 children had a progressive encephalopathy (PE), 9 had a static encephalopathy (SE), and 7 had normal neurological findings (N). IBBB synthesis of HIV‐specific antibodies was identified (using matched serum and CSF specimens) in 7 of 11 children with PE, 4 of 9 children with SE, and 2 of 7 children with N. HIV antigen was found (using a highly sensitive solid‐phase enzyme immunoassay) in the CSF of 8 of 11 children with PE, none of the children with SE, and none of the 7 children with N. On the basis of these data, we conclude that: (1) IBBB synthesis of HIV antibodies indicates invasion of the central nervous system but may reflect prior or current infection; and (2) HIV antigen in CSF indicates viral expression and correlates with the occurrence of PE. These findings strongly implicate HIV as the causative agent of PE in these children. The assay for HIV antigen in the CSF may be of value in determining the prognosis of children with HIV infection and for evaluating the efficacy of therapeutic agents against this retrovirus.
Catecholamines predict outcome in traumatic brain injury
Authors: Hamill Robert W. , Woolf Paul D. , McDonald Joseph V. , Lee Louyse A. , Kelly Mary 
Published: January 2005

AbstractActivation of the sympathetic nervous system attends traumatic brain injury, but the association of the severity of neurological impairment and recovery with the extent of sympathetic nervous system stimulation is poorly defined. In this study, plasma norepinephrine (NE), epinephrine (E), and dopamine (DA) levels were measured serially in 33 patients with traumatic brain injury and compared with the Glasgow Coma Score (GSC), which was obtained concurrently. A catecholamine gradient that reflected the extent of brain injury was demonstrated within 48 hours of the injury. In patients with a GCS of 3 to 4, NE and E levels increased four‐ to fivefold and the DA level increased threefold above normal (NE, 1686 ± 416 pg/ml; E, 430 ± 172 pg/ml; DA, 236 ± 110 pg/ml), while patients with mild brain injury (GCS, ≥ 11) had slightly elevated or normal levels. Patients with marked (GCS, 5 to 7) and moderate (GCS, 8 to 10) traumatic brain injuries had intermediate levels. The prognostic value of determining admission levels of NE was shown in patients with an admission GCS of 3 to 4 1 week after injury. Patients with severe and unchanging neurological impairment 1 week after injury had markedly elevated initial NE levels (2,176 ± 531 pg/ml), whereas initial NE levels (544 ± 89 pg/ml) were only mildly elevated in patients who improved to a GCS of greater than 11. These data indicate that markedly elevated NE levels predict outcome in patients with comparable neurological deficits. Thus levels of circulating catecholamines are excellent endogenous and readily quantifiable markers that appear to reflect the extent of brain injury and that may predict the likelihood of recovery.
HIV antigen in the brains of patients with the AIDS dementia complex
Authors: Pumarola‐Sune Tomas , Navia Bradford A. , Price Richard W. , Cordon‐Cardo Carlos , Cho Eun‐Sook 
Published: January 2005

AbstractHuman immunodeficiency virus infection was identified immunohistochemically in the brains of 8 patients with acquired immune deficiency syndrome dementia complex. Using a monoclonal antibody against a structural viral protein (p25), infection was detected in white matter and basal ganglia in a distribution paralleling that of the major neuropathological abnormalities. Viral antigen was identified principally in perivascular and parenchymal macrophages and in multinucleated cells of macrophage origin that were identified morphologically and by immunocytochemical staining for acid phsophatase isozyme. In 4 of the 8 patients, viral antigen was also detected in acid‐phosphatase–negative, process‐bearing neuroglial cells; in 2 patients, antigen was detected in basal ganglion cells that were morphologically consistent with neurons and in alkaline‐phosphatase–positive cells with elongated nuclei that were most likely of endothelial origin.
Familial cavernous malformations of the central nervous system and retina
Authors: Dobyns William B. , Michels Virginia V. , Groover Robert V. , Mokri Bahram , Trautmann James C. , Forbes Glenn S. , Laws Edward R. 
Published: January 2005

AbstractWe studied a family in which 4 persons from three generations had multiple cavernous malformations (“angiomas”) of the central nervous system (CNS) and/or retina and found accounts in the literature of sixteen other families with this condition. In these families with familial cavernous malformation of the CNS and retina, 92% of pathologically documented vascular malformations were cavernous; 50% of those subjects affected had multiple CNS and/or retinal vascular malformations and 68% (excluding probands) were symptomatic. Cutaneous vascular lesions were an inconsistant manifestation. Autosomal dominant inheritance with high penetrance was confirmed.
Diencephalic seizures: Responsiveness to bromocriptine and morphine
Authors: Bullard Dennis E. 
Published: January 2005

AbstractTwo patients with posttraumatic diencephalic seizures, characterized by autonomic dysfunction and extensor posturing, had partial responses to bromocriptine and complete responses to morphine. Probable synergism between the two agents was noted. These 2 cases suggest the potential effectiveness of this regimen for the treatment of diencephalic seizures, raise questions regarding the role of the dopaminergic and opioid systems in this disease entity, and support the hypothesis that diencephalic seizures represent a release phenomenon in the brain.
Factors that influence the occurrence of response variations in Parkinson's disease
Authors: De Jong G. J. , Meerwaldt J. D. , Schmitz P. I. M. 
Published: January 2005

AbstractRetrospective study of 129 patients treated with dopamine substitution showed that the occurrence of response variations is related to the duration of therapy and not to the nature of the predominant symptom (tremor or hypokinesia and rigidity) or the age of onset of the disease. A relationship was found between the occurrence of response variations and the severity of the disease when levodopa therapy was started. Patients fared worst who were in Hoehn and Yahr stages I and II when levodopa therapy was begun.
Age‐related shrinkage of cortically projecting cholinergic neurons: A selective effect
Authors: Mesulam M‐Marchsel , Mufson Elliott J. , Rogers Joseph 
Published: January 2005

AbstractThe number and size of basal forebrain neurons that provide the cholinergic innervation for the cerebral cortex, amygdala, and hippocampus were studied in young and aged mice. The results showed that these neurons became substantially smaller with increasing age. This effect was relatively selective, since the immediately adjacent cholinergic neurons in the striatum did not show a change of similar magnitude. The shrinkage of these basal forebrain neurons may account for the decline of cholinergic innervation that occurs with age. In the material that we examined, aging did not influence the number of cholinergic neurons in the basal forebrain, only their size. It seems, therefore, that the age‐related changes in cholinergic function (and their putative behavioral consequences) are not associated with a substantial component of irreversible cell death.
Aminergic systems in Alzheimer's disease and Parkinson's disease
Authors: D'Amato Robert J. , Zweig Richard M. , Whitehouse Peter J. , Wenk Gary L. , Singer Harvey S. , Mayeux Richard , Price Donald L. , Snyder Solomon H. 
Published: January 2005

AbstractBiochemical markers for serotoninegic and catecholaminergic neurons in frontal and temporal poles were examined post mortem in brains of patients with Alzheimer's disease, Parkinson's disease, and the two combined. Binding of [3H]citalopram to serotoninergic uptake sites and levels of serotonin were Decemberreased by 40 to 50% in brains of persons in each disease category. In contrast, significant reductions of catecholaminergic markers were not detected. In all three disease groups, the choline acetyltransferase activity was reduced by 50 to 60%. Binding sites for adenosine (A1), muscarinic cholinergic, phencyclidine, β‐adrenergic, and calcium antagonist receptors were unchanged. We conclude that substantial damage to serotoninergic neurons occurs in persons with Parkinson's and Alzheimer's diseases.
The concept of coupling blood flow to brain function: Revision required?
Authors: Lou Hans C. , Edvinsson Lars , MacKenzie Eric T. 
Published: January 2005

AbstractA tight coupling exists between brain function and cerebral perfusion in most situations. The Roy and Sherrington hypothesis has been widely accepted to account for the phenomenon: Increased neuronal metabolic activity will give rise to the accumulation of vasoactive catabolites, which decrease vascular resistance and thereby increase blood flow until normal homeostasis is reestablished. However, the hypothesis does not account for the disproportionate increase in flow that occurs in a number of circumstances. There are additional difficulties in reconciling more recent experimental data with the Roy and Sherrington hypothesis. In this review we direct attention toward the rich perivascular nerve supply to all parts of the cerebral circulation as possibly being an alternative control system allowing for rapid parallel changes in flow and neuronal activity.
Risk factors for ischemic stroke: A prospective study in Rochester, Minnesota
Authors: Davis Patricia H. , Dambrosia James M. , Schoenberg Bruce S. , Schoenberg Devera G. , Pritchard David A. , Lilienfeld Abraham M. , Whisnant Jack P. 
Published: January 2005

AbstractA cohort of 1,804 residents of Rochester, Minnesota, who were at least 50 years old, free of stroke, and who underwent examination at the Mayo Clinic in 1960, was followed for 13 years. During this period, there were 110 first ischemic strokes and 616 deaths without stroke. The time of onset, if available, or the time of diagnosis of potential risk factors was determined for all patients during the study and was used to construct a proportional hazards model of time to occurrence of stroke with time‐dependent risk factors. The model included 8 risk factors (2 fixed and 6 time dependent). For these, the individual relative risk are: 1.6 for age (per 10 years), 2.0 for males, 4.0 for definite hypertension, 3.9 for transient ischemic attacks, 2.2 for hypertensive heart disease, 2.2 for coronary heart disease, 1.7 for congestive heart failure, and 1.7 for diabetes mellitus. Atrial fibrillation was not a significant risk factor using time dependent multivariate analysis.
Effect of levodopa treatment on contrast sensitivity in Parkinson's disease
Authors: Bulens C. , Meerwaldt J. D. , Van der Wildt G. J. , Van Deursen J. B. P. 
Published: January 2005

AbstractWe studies contrast sensitivity function in 10 parkinsonian patients before and after levodopa treatment. Pretreatment contrast sensitivity function was abnormal in 16 of the 20 eyes. After treatment, only high‐frequency loss was observed in 6 eyes. All other types of deficit disappeared under treatment. These changes of contrast sensitivity function following treatment suggest that dopamine is functional transmitter in the visual pathways.
Molecular pathogenesis of neurotropic viral infections
Authors: Gonzalez‐Scarano F. , Tyler Kenneth L. 
Published: January 2005

AbstractClassical virologists defined a number of viruses that affect the nervous system and identified tissue tropism, extraneural replication, and viremia as important parameters that determine whether viral infections will affect the central nervous system. Molecular techniques are expanding this knowledge by permitting us to relate specific genes and gene products to two defined phenotypes: neuroinvasion and neurovirulence. Two converging situations make this knowledge particularly useful: (1) the development of antiviral drugs and subunit vaccines, which mandate that pathogenesis be related to specific regions of the viral genome; and (2) the expanding problem of central nervous system infections in immunodeficient states.