bluuebunny/crossref_metadata_2025_split huggingface dataset:
Proton magnetic resonance spectroscopic imaging for discrimination of absence and complex partial seizures
Authors: Cendes Fernando , Stanley Jeff A. , Dubeau FrançOis , Andermann Frederick , Arnold Douglas L. 
Published: January 2005

AbstractWe performed proton magnetic resonance spectroscopic imaging of the temporal lobes between, during, and soon after nonconvulsive seizures in 20 patients with documented temporal lobe epilepsy, 5 patients with primary generalized epilepsy, and 2 patients with secondary generalized epilepsy. Our objective was to determine whether there were metabolic changes observable by magnetic resonance spectroscopic imaging during seizures and whether these changes were specific for focal or generalized nonconvulsive seizures. We found a significant increase in lactate to creatine plus phosphocreatine (lactate/creatine) values, reflecting an imbalance in energy supply and demand or an adaptation in response to ictal neuronal discharges, during and soon after complex partial seizures, but not during or soon after absence seizures associated with generalized epilepsy. In patients with temporal lobe epilepsy, the N‐acetylaspartate resonance relative to creatine plus phosphocreatine was low in one or both temporal lobes, indicating neuronal loss or damage. This was not observed in patients with primary generalized epilepsy. The regions with abnormal lactate/creatine and N‐acetylaspartate/creatine values corresponded to the epileptogenic focus as defined by clinical‐electroencephalographic investigation. There was no change in the N‐acetylaspartate/creatine values in the temporal lobes between the interictal, ictal, or postictal states. We conclude that (1) partial seizures are associated with abnormally high lactate levels, but absence seizures are not, and (2) no short‐term changes of N‐acetylaspartate occur during or soon after complex partial seizures or absence seizures. These findings may be related to the lack of postictal confusion in patients with absence seizures, as well as with the more benign course of primary generalized epilepsy with nonconvulsive attacks.
Stereotactic thalamotomy in tremor‐dominant Parkinson's disease: An H215O PET motor activation study
Authors: Boecker Henning , Wills Adrian J. , Ceballos‐Baumann Andres , Samuel Michael , Thomas David G. T. , Marsden C. David , Brooks David J. 
Published: January 2005

AbstractStereotactic thalamotomy is an effective treatment for severe drug‐resistant tremor. The thalamus, however, facilitates motor activiry, and thalamotomy would be predicted to inhibit movement‐associated cortical activation. Two tremulous parkinsonian patients were studied with H2 15O position emission tomography before and after left ventalis intermedius thalamotomy. Subjects were scanned at rest and during performance of externally paced joystick movements in freely selected direstions with the right hand. Thalamotomy relieved tremor but, as perdicted, led to dercreased activation of the left sensorimotor cortex, lateral premotor cortex, and parietal area 7 on hand movement.
1H‐magnetic resonance spectroscopy—determined cerebral lactate and poor neurological outcomes in children with central nervous system disease
Authors: Ashwal Stephen , Holshouser Barbara A. , Tomasi Lawrence G. , Shu Stanford , Perkin Ronald M. , Nystrom Gerald A. , Hinshaw David B. 
Published: January 2005

AbstractBy using proton magnetic resonance spectroscopy (1H‐MRS), cerebral lactate has been shown to be elevated in a wide variety of pediatric and adult neurological diseases. In this study we compared 36 newborns, infants, and children with elevated lactate peaks on 1H‐MRS with 61 patients witout an identifiable lactate signal. 1H‐MRS was acquired from the occipital gray and parietal white matter (8 cm3 volume, STEAM sequence with echo time = 20 msec, repetition time = 3.0 seconds) and data were expressed as ratios of different metabolite peak areas (N‐acetylaspartate [NA]/creatine [Cr], NA/choline [Ch], and Ch/Cr) and the presence of a characteristic lactate doublet peak at 1.3 ppm. Outcomes (Pediatric Cerebral Performance Category Scale score; PCPCS) were assigned 6 to 12 months after injury. Patients with lactate peaks were more likely to have suffered a cardiac arrest, were more often hyperglycemic, and had lower Glasgow Coma Scale scores on admission. They were also more likely to have abnormal metabolite ratios when compared with agematched controls or with patients without detectable lactate. Of prognostic importance, patients with increased lactate were more likely to be severely disabled (39% vs 10%), survive in a persistent vegetative state (13% vs 2%), or have died (39% vs 7%). In contrast, patients with similar conditions without increased lactate were more likely to have had a good outcome (23% vs 3%) or recovered to a mild (38% vs 6%) or moderate disability (20% vs 0%). Our data suggest that 1H‐MRS is useful in the prediction of long‐term outcomes in children with neurological disorders. Patients with elevated cerebral lactate are more likely to die acutely or are at greater risk for serious long‐term disability.
Atrophy of the corpus callosum, cognitive impairment, and cortical hypometabolism in progressive supranuclear palsy
Authors: Yamauchi Hiroshi , Fukuyama Hidenao , Nagahama Yasuhiro , Katsumi Yukinori , Dong Yun , Konishi Junji , Kimura Jun 
Published: January 2005

AbstractRecent studies disclosed neurofibrillary degeneration in layer 3 of the association cortex in patients with progressive supranuclear palsy. This lesion may be associated with corpus callosum atrophy and may impair the function of cortical regions indispensable for complex cognitive activity. To investigate whether corpus callosum atrophy is associated with cognitive impairment and cerebral cortical hypometabolism, we studied 10 patients with progressive supranuclear palsy using magnetic resonance imaging and positron emission tomography with fluorodeoxyglucose as a tracer. Compared with 23 age‐matched control subjects, the patients had significantly decreased callosal area‐skull area ratios, with anterior predominance of the degree of atrophy. The corpus callosum atrophy was accompanied by a decreased mean cortical glucose metabolic rate, predominantly in the frontal region of the cortex, and poor performance on the picture arrangement subtest of the Wechsler Adult Intelligence Scale and the verbal fluency task. We conclude that corpus callosum atrophy with anterior predominance is present in progressive supranuclear palsy, and that this atrophy is associated with cognitive impairment and cerebral cortical hypometabolism, especially in the frontal cortical region. Corpus callosum atrophy may reflect the pathological changes in the cerebral cortex, accentuated in the frontal region, that contribute to the development of frontal lobe dysfunction in this disease.
Leukodystrophy in patients with ovarian dysgenesis
Authors: Schiffmann Raphael , Tedeschi Gioacchino , Kinkel R. Philip , Trapp Bruce D. , Frank Joseph A. , Kaneski Christine R. , Brady Roscoe O. , Barton Norman W. , Nelson Lawrence , Yanovski Jack A. 
Published: January 2005

AbstractWe describe clinical, biochemical, pathological, and spectroscopic findings in 4 women, aged 15 to 29 years, from three unrelated families who had a unique combination of a central neurvous system white matter disease and primary ovarian failure. All had normal initial development but 3 had borderline low IQ and academic difficulties in primary school. Puberty did not develop in 2 patients and was arrested in a third patient. The fourth patient had premature ovarian failure at the age of 13 years. Head magnetic resonance imaging showed diffuse white matter disease, with frontal cortical atrophy in the most clinically advanced patient. All patients had normal karyotype and normal findings on extensive evaluations for known leukodystrophies, for other metabolic diseases, and for causes of ovarian failure. Proton magnetic resonance spectroscopic imaging showed reduction of choline‐containing compounds in the affected white matter in all patients and reduction of N‐acetylaspartate in the unaffected frontal white matter of 2 patients. All patients had evidence of primary gonadal insufficiency with a normal hypothalamic‐hypophyseal axis. Pathological analysis showed streak ovaries in 1 patient and signs of hypomyelination, and gliosis on brain biopsy in another patient. In conclusion, we present a novel group of patients who have in common leukodystrophy, primary ovarian dysfunction, and magnetic resonance spectroscopic abnormalities.
Periventricular nodular heterotopia and intractable temporal lobe epilepsy: Poor outcome after temporal lobe resection
Authors: Li L. M. , Dubeau F. , Andermann F. , Fish D. R. , Watson C. , Cascino G. D. , Berkovic S. F. , Moran N. , Duncan J. S. , Olivier A. , Leblanc R. , Harkness W. 
Published: January 2005

AbstractWe describe 5 women and 5 men with periventricular nodular heterotopia and electroclinical features suggestive of temporal lobe epilepsy, who were surgically treated for control of medically refractory seizures. Magnetic resonance imaging revealed bilateral periventricular nodular heterotopia in 7 of the 10 patients. Because of the lack of clear localization, 6 patients were studied with intracranial depth electrode recordings. Seizures were of hippocampal onset (3 patients), regional temporal lobe onset (2 patients), or occipital‐temporal onset (1 patient). Anterior temporal lobectomy was performed in 6 patients; selective amygdalohippocampectomy, in 1; and anterior temporal lobectomy plus resection of the heterotopic tissue, in 3. None of the 9 patients followed for more than 12 months postoperatively were seizure free. Two patients were initially seizure free for approximately 18 months, but then seizures recurred. One patient had a major reduction in seizure frequency at a 39‐month follow‐up after most of the unilateral heterotopic tissue was included in the temporal resection. Temporal resection did not lead to a long‐term favorable outcome in this group of patients with periventricular nodular heterotopia and epileptogenic discharges involving the temporal lobe. This sugests a more widespread disorder with epileptogenic activity possibly originating in or near the heterotopic tissue. The clinical and electrographic features of periventricular nodular heterotopia pointing to temporal lobe origin are misleading and temporal resection does not result in long‐term cessation of seizures.
Short‐ and long‐term survival and function of unilateral intrastriatal dopaminergic grafts in Parkinson's disease
Authors: Wenning Gregor K. , Odin Per , Morrish Paul , Rehncrona Stig , Widner Håkan , Brundin Patrik , Rothwell John C. , Brown Richard , Gustavii Björn , Hagell Peter , Jahanshahi Marjan , Sawle Guy , Björklund Anders , Brooks David J. , Marsden C. David , Quinn Niall P. , Lindvall Olle 
Published: January 2005

AbstractSix patients with Parkinson's disease were followed for 10 to 72 months after human embryonic mesencephalic tissue from four to seven donors was grafted unilaterally into the putamen (4 patients) or putamen plus caudate (2 patients). After 8 to 12 months, positron emission tomography showed a 68% increase of 6‐L−[18F]‐fluorodopa uptake in the grafted putamen, no change in the grafted caudate, and minor decreases in nongrafted striatal regions. There was therapeutically valuable improvement in 4 patients, but only modest changes in the other 2, both of whom developed atypical features. Patient 4 was without L−dopa from 32 months and had normal fluorodopa uptake in the grafted putamen at 72 months. Overall, the L−dopa dose was reduced by a mean of 10 and 20%, “off” time was reduced by 34 and 44%, and the “off” phase Unified Parkinson's Disease Rating Scale motor score by 18 and 26%, and the duration of the response to a single L−dopa dose increased by 45 and 58% during the first and second years after surgery, respectively. Rigidity and hypokinesia improved bilaterally, but mainly contralateral to the implant. No consistent changes in dyskinesias were observed. We conclude that transplantation of embryonic mesencephalic tissue leads to highly reproducible survival of dopaminergic neurons, inducing clinically valuable improvements in most recipients.
Sporadic focal dystonia in Northwest Germany: Molecular basis on chromosome 18p
Authors: Leube B. , Hendgen T. , Kessler K. R. , Knapp M. , Benecke R. , Auburger G. 
Published: January 2005

AbstractIdiopathic focal dystonia (IFD) is the most common form of idiopathic torsion dystonia in the Euroamerican population, with a prevalence of about 30 per 100,000. Although most patients claim a negative family history, we recently mapped this syndrome to chromosome 18p as an autosomal dominant trait in Family K from Northwest Germany. We now have investigated sporadic patients with IFD from the same geographic area both clinically and molecularly with chromosome 18p markers. The data indicate that most of these apparently sporadic patients have inherited the same mutation as Family K from a common ancestor and, in fact, owe their disease to autosomal dominant inheritance at low penetrance. The data also indicate that this dystonia mutation (DYT7) is the predominant cause of IFD, at least in this area of Northwest Germany, and that its location can be narrowed from a 30‐ to a 6‐centimorgan region close to marker D18S1098.
Nicotinic receptor stimulation protects neurons against β‐amyloid toxicity
Authors: Kihara T. , Shimohama S. , Sawada H. , Kimura J. , Kume T. , Kochiyama H. , Maeda T. , Akaike A. 
Published: January 2005

Abstractβ‐Amyloid (Aβ), a major constituent of senile plaques in Alzheimer's disease (AD), is thought to contribute to the neurodegeneration. We examined the effects of nicotinic receptor agonists on Aβ cytotoxicity in cultured rat cortical neurons. The number of viable neurons decreased significantly when cultures were exposed to synthetic Aβ peptides (25–35). Concomitant administration of nicotine with Aβ markedly reduced the number of dead cells. This nicotine‐induced neuroprotection was dependent on the concentration. When hexamethonium or mecamylamine, nicotinic antagonist, was added, neuroprotective effect of nicotine was blocked, which indicates that effect of nicotine was mediated by nicotinic receptors. In addition, a selective α7‐receptor antagonist, α‐bungarotoxin (α‐BTX), blocked the neuroprotective effect of nicotine. Furthermore, incubation with 3–(2,4)‐dimethoxybenzylidene anabaseine (DMXB), a selective α7–receptor agonist, protected against Aβ‐induced neuronal death. These results suggest that α7–receptor activation plays an important role in neuroprotection against Aβ cytotoxicity. This study suggests that nicotinic receptor stimulation, especially α‐receptor activation, may be able to protect neurons from degeneration induced by Aβ and may have effects that counter the progress of AD.
Calcium‐permeable α‐amino‐3‐hydroxy‐5‐methyl‐4‐isoxazole propionic acid receptors: A molecular determinant of selective vulnerability in amyotrophic lateral sclerosis
Authors: Williams T. L. , Day N. C. , Ince P. G. , Kamboj R. K. , Shaw P. J. 
Published: January 2005

AbstractThe cause of the selective degeneration of motor neurons in amyotrophic lateral sclerosis (ALS) remains unexplained. One potential pathogenetic mechanism is chronic toxicity due to disturbances of the glutamatergic neurotransmitter system, mediated via α‐amino‐3‐hydroxy‐5‐methyl‐4‐isoxazole propionic acid (AMPA)‐sensitive glutamate receptors. Functional AMPA receptors consist of various combinations of four subunits (designated GluR1–4). The GluR2 subunit is functionally dominant and renders AMPA receptors impermeable to calcium. Most native AMPA receptors in the mammalian central nervous system (CNS) contain the GluR2 subunit and are calcium impermeable. We have investigated the composition of AMPA receptors expressed on normal human spinal motor neurons by in situ hybridization to determine their likely subunit stoichiometry. Highly significant levels of mRNA were detected for the GluR1, GluR3, and GluR4 subunits. However, GluR2 subunit mRNA was not detectable in this cell group. The absence of detectable GluR2 mRNA in normal human spinal motor neurons prdicts that they express calcium‐permeable AMPA receptors unlike most neuronal groups in the human CNS. Expression of atypical calcium‐permeable AMPA receptors by human motor neurons provides a possible mechanism whereby disturbances of glutamate neurotransmission in ALS may selectively injure this cell group.
Autosomal dominant dementia with widespread neurofibrillary tangles
Authors: Reed Lee A. , Schelper Robert L. , Solodkin Ana , Van Hoesen Gary W. , Van Hoesen Gary W. , Solodkin Ana , Morris John C. , Trojanowski John Q. , Grabowski Thomas J. , Talbot Chris J. , Schmidt Marie Luise , Goate Alison , Wragg Michelle A. 
Published: January 2005

AbstractSeveral familial dementing conditions with atypical features have been characterized, but only rarely is the neuropathology dominated solely by neurofibrillary lesions. We present a Midwestern American pedigree spanning four generations in which 15 individuals were affected by early‐onset dementia with long disease duration, with an autosomal dominant inheritance pattern, and with α‐rich neurofibrillary pathology found in the brain post mortem. The average age at presentation was 55 years with gradual onset and progression of memory loss and personality change. After 30 years'l disease duration, the proband's neuropathologic examination demonstrated abudant intraneuronal neurofibrillary tangles (NFTs) involving the hippocampus, pallidum, subthalamic nucleus, substantia nigra, pons, and medulla. Only sparse neocortical tangles were present and amyloid plaque were absent. The tangles were recognized by antibodies specific for phosphorylation‐independent (Tau−2, T46, 133, and Alz‐50) and phosphorylation‐dependent epitopes (AT8, T3P, PHF‐1, 12E8, AT6, AT18, AT30) in proteins. Electron microscopy of NFTs in the dentate gyrus and midbrain demonstrated paired helical filaments. Although the clinical phenotype resembles Alzheimer's disease, and the neuropathologic phenotype resembles progressive supranuclear palsy, an alternative consideration is that this familial disorder may be a new or distinct disease entity.
In vivo skeletal muscle mitochondrial function in Leber's hereditary optic neuropathy assessed by 31P magnetic resonance spectroscopy
Authors: Lodi R. , Taylor D. J. , Tabrizi S. J. , Kumar S. , Sweeney M. , Wood N. W. , Styles P. , Radda G. K. , Schapira A. H. V. 
Published: January 2005

AbstractWe used 31P magnetic resonance spectroscopy (31P‐MRS) to asses in vivo skeletal muscle mitochondrial function in 10 Leber's hereditary optic neuropathy patients/carriers with a mitochondrial DNA (mtDNA) mutation at one of three nucleotide positions, 11,778, 14,484, and 3,460. We studied one affected patient for each mutation and two unaffected carriers with the 11,778 or 3,460 mutation and three carriers with 14,484. All subjects were homoplasmic except the two 3,460 carriers, who showed 80% and 15% of mutated mtDNA. 31P‐MRS at rest disclosed some abnormalities in all subjects. In particular, the phosphorylation potential was below the normal range in all cases. During recovery from exercise, the maximum rate of mitochondrial ATP production (Vmax) was reduced to 27% of normal in the 11,778 mutation and to 53% in the 14,484 mutation patient/carrier groups. Mitochondrial Vmax was within the normal range in all subjects with the 3,460 mutation but correlated inversely with the percentage of mutated mtDNA. This in vivo study shows that the 11,778 mutation causes a mitochondrial impairment more severe than the 14,484 and that the 3,460 mutation results in only a mild depression of muscle mitochondrial function.
Ontogeny of ipsilateral corticospinal projections: A developmental study with transcranial magnetic stimulation
Authors: Müller K. , Kass‐Iliyya F. , Reitz M. 
Published: January 2005

AbstractTranscranial magnetic stimulation (TMS) has been used to describe the maturation of the corticospinal tract in children. Ipsilateral corticospinal connections have been demonstrated with TMS in patients with congenital mirror movements, in patients after hemispherectomy, and in children with hemiplegic cerebral palsy. The goal of the study was to find out whether corticospinal ipsilateral projections in children can be demonstrated during the first decade of life as part of normal ontogeny. For this purpose, we examined 50 normal children (age range, 3–11 years) with focal TMS over the left and right hemispheres to target muscles in proximal and distal parts of the upper extremity (first dorsal interosseus, biceps brachii, and brachioradialis). To lower the stimulation threshold, we stimulated under voluntary preinnervation. In two‐thirds of the children we elicited ipsilateral motor evoked potentials (MEPs). This occurred more often in proximal than in distal muscles. The latency of the ipsilateral MEPs was about 12 to 14 msec longer than the usual contralateral response. From the age of 10, and in adults, ipsilateral MEPs could not be detected. Also considering lesion data from adult patients, the most likely explanation for the disappearance of ipsilateral corticospinal connections after the age of 10 years is an increasing transcallosal inhibitory influence during development. The presence of ipsilateral corticospinal connections appears to be a normal state in ontogeny.
Alterations in opioid receptor binding in Parkinson's disease patients with levodopa‐induced dyskinesias
Authors: Piccini P. , Weeks R. A. , Brooks D. J. 
Published: January 2005

AbstractLevodopa‐induced dyskinesias remain a major challenge in the therapeutic management of Parkinson's disease (PD). Their etiology is unknown although dysfunction of striatal opioid transmission has been implicated in experimental models of PD. To determine whether the opioid system is involved in human dyskinetic PD, we measured in vivo opioid receptor binding in PD patients with and without levodopa‐induced dyskinesias, using positron emission tomography (PET) and the opioid receptor ligand [11C]diprenorphine. Striatal and thalamic/occipital uptake ratios were calculated using a region of interest (ROI) approach. In addition, we used statistical parametric mapping (SPM) and images reflecting the volume of distribution of [11C]diprenorphine to assess changes in cerebral receptor binding on a voxel‐by‐voxel basis. By using the ROI approach, we found significantly reduced striatal and thalamic opioid binding in dyskinetic, but not in nondyskinetic, PD patients. The SPM approach confirmed reduced availability in these areas and, in addition, showed decreased cingulate and increased prefrontal opioid receptor binding in the dyskinetic patients. Our findings confirm that altered opioid transmission is part of the pathophysiology of levodopa‐induced dyskinesias in PD and support further investigation into the role of opioid agents in the management of these involuntary movements.