bluuebunny/crossref_metadata_2025_split huggingface dataset:
Subarachnoid blood and headache: Altered trigeminal tachykinin gene expression
Authors: Linnik Matthew D. , Sakas Damianos E. , Uhl George R. , Moskowitz Michael A. 
Published: January 2005

AbstractSensory axons from the trigeminal ganglion (V) innervate cephalic blood vessels and use the preprotachykinin gene products, substance P (SP) and neurokinin A (NKA), as putative neurotransmitters conveying nociceptive information. Blood in the subarachnoid space is accompanied by severe headache. We now report that this painful stimulus, which should enhance activity in V, specifically alters tachykinin peptide and mRNA levels in V and perivascular axons. Marked reductions in SP levels were observed in basilar artery segments within 4 hours after intracisternal blood injection which persisted for 48 hours and recovered by 7 days. SP peptide levels in V were elevated by 49% two days after blood injection. The changes in SP peptide levels were accompanied by increases in ganglionic content of the preprotachykinin mRNA that codes for the peptide. Blood‐induced peptide depletion in arteries and subsequent increases in peptide and mRNA in V are consistent with increased neuronal activity and enhanced neuropeptide release. These results implicate the tachykinin‐utilizing trigeminovascular neurons in the sequelae of subarachnoid hemorrhage.
Ideational apraxia: A deficit in tool selection and use
Authors: Ochipa C. , Rothi L. J. G. , Heilman K. M. 
Published: January 2005

AbstractWe report a 67‐year‐old left‐handed man who exhibited an ideational apraxia in both clinical and nonclinical natural settings following a right hemisphere infarction. His inability to use tools could not be explained by a motor production deficit (ideomotor apraxia), because he made content errors and could not match tools with objects. His deficit could not be attributed to an agnosia or language comprehension deficit, because he could name tools and point to tools on command. Based on our testing, it appeared that this patient had a loss of knowledge related to tool use.
Nonconvulsive status epilepticus following cerebral angiography
Authors: Vickrey Barbara G. , Bahls Fredrick H. 
Published: January 2005

AbstractA 64‐year‐old man developed lethargy and aphasia immediately following cerebral arteriography with iothalamate meglumine. An electroencephalogram showed continuous epileptiform activity. The patient was treated with intravenous phenytoin with complete resolution of clinical symptoms and electroencephalographic epileptiform abnormalities. The diagnosis of nonconvulsive status epilepticus should be considered in cases of altered consciousness following cerebral arteriography.
Neurological disease associated with antiphospholipid antibodies
Authors: Briley Dennis P. , Coull Bruce M. , Goodnight Scott H. 
Published: January 2005

AbstractThe Hemostasis and Thrombosis Laboratory at the Oregon Health Sciences University identified 80 patients with significantly elevated anticardiolipin antibody (ACLA) levels. We reviewed all of their available medical records and found that 25 of these patients had associated neurological symptoms or disorders. These symptoms and disorders could be grouped into four distinct clinical patterns comprising encephalopathy, multiple cerebral infarctions, migraine‐like headaches, and visual abnormalities including amaurosis fugax and ischemic optic neuropathy. Cerebral ischemia best explained these neurological dysfunctions. There was no correlation between the presence or absence of neurological disease and ACLA levels, but ACLA levels were higher in patients with encephalopathy than in others with neurological involvement (p < 0.05). How neurological dysfunction and the presence of these antiphospholipid antibodies are related remains to be clarified. Nevertheless, in patients with unexplained cerebral ischemia, establishing the presence of ACLA may have prognostic and therapeutic importance. In particular, acute immunosuppressive therapy and plasmapheresis may be useful in patients with acute ischemic encephalopathy.
Amaurosis fugax associated with antiphospholipid antibodies
Authors: Digre Kathleen B. , Durcan F. Jane , Branch D. Ware , Jacobson Dan M. , Varner Michael W. , Baringer J. Richard 
Published: January 2005

AbstractIn more than 50% of amaurosis fugax patients under 45 years of age no cause for the episodes of visual loss is identifiable. We have encountered 6 young adults (4 women and 2 men) with episodes of amaurosis fugax associated with elevated levels of antiphospholipid antibodies. Splinter hemorrhages of the nail beds were present in most patients. Treatment with antiplatelet medications and anticoagulants appeared to reduce the frequency of episodes and might prevent central retinal artery occlusions or stroke.
Diabetic truncal neuropathy: Topography of the sensory deficit
Authors: Stewart John D. 
Published: January 2005

AbstractThe distributions of sensory abnormalities in 17 episodes of diabetic truncal neuropathy among 7 patients with diabetes mellitus are described. The patterns are highly variable: the distribution of adjacent main spinal nerves may be involved, resulting in a complete dermatomal band of dysesthesia, but almost two‐thirds of the episodes were restricted to the distribution of the ventral or dorsal rami of the spinal nerves or branches of these rami or varying combinations of these distributions.
The time course of steroid action on blood‐to‐brain and blood‐to‐tumor transport of 82Rb: A positron emission tomographic study
Authors: Jarden J. O. , Dhawan V. , Moeller J. R. , Strother S. C. , Rottenberg D. A. 
Published: January 2005

AbstractBlood‐to‐brain and blood‐to‐tumor transport rate constants for Rb (K1) and apparent tissue blood volume (Vb) were estimated in 8 patients with primary or metastatic brain tumors before and approximately 6 hours after a 100‐mg intravenous bolus injection of dexamethasone using 82Rb and positron emission tomography. Eight additional patients were studied to evaluate test‐retest variability and repositioning errors. Six hours following dexamethasone administration tumor K1 (but not Vb) was significantly reduced compared with contralateral control brain regions (p < 0.03). These data are consistent with our previously published 24‐hour‐postdexamethasone data and suggest that comparable effects of corticosteroids on brain/tumor capillaries may be observed at 5 to 6 and 24 hours. The time course of dexamethasone‐induced alterations in brain/tumor capillary permeability supports the view that these alterations may be responsible for at least some of the antiedema effects of corticosteroids.
An immunohistochemical investigation of the human neostriatum in huntington's disease
Authors: Goto Satoshi , Hirano Asao , Rojas‐Corona Rogelio R. 
Published: January 2005

AbstractThe neostriatum of 7 autopsied patients with Huntington's disease (HD) was examined immunohistochemically using purified antibody against calcineurin, which may be present only in the medium‐size spinous neurons of the mammalian striatum. This study revealed a marked loss of calcineurin‐positive cells in the caudate nucleus and the putamen in all HD patients, compared with control subjects, and there was some variation among the HD patients. Four HD patients showed significantly lower density of calcineurin‐positive cells in the caudate nucleus than in the putamen. The remaining calcineurin‐positive cells in the caudate nucleus and the putamen had a mosaic‐like pattern, demonstrating a subregional difference in distribution. This finding suggests that there are subregional as well as compartmental differences in the vulnerability of the calcineurin‐positive cells in the striatum of patients with HD.
Juvenile myoclonic epilepsy: An autosomal recessive disease
Authors: Panayiotopoulos Chrysostomos P. , Obeid Tahir 
Published: January 2005

AbstractWe undertook genetic study of patients with juvenile myoclonic epilepsy (JME) from 17 families. There was a mean of 8 children in each sibship. Siblings were affected in 8 sibships, and some families had more than 2 members affected by JME. Half‐siblings and parental involvement were found in only 1 sibship each. The segregation ratio was 0.123 but increased to 0.18 with correction for age of onset. Parental consanguinity was found in 9 (45%) of the sibships. The evidence establishes an autosomal recessive mode of inheritance for JME.
Amyotrophic lateral sclerosis: Abnormalities of the tongue on magnetic resonance imaging
Authors: Cha Choon H. , Patten Bernard M. 
Published: January 2005

AbstractWe compared the magnetic resonance images of the tongues of 16 amyotrophic lateral sclerosis (ALS) patients with those of 20 control patients and found the tongue in ALS patients is more frequently and more severely involved than suspected clinically, with major abnormalities of size, shape, position, and internal structure. The tongue size in ALS, as measured in the sagittal plane, can be reduced by as much as two‐thirds of normal. The shape of the tongue in ALS tends to be rectangular or square rather than curved as is normal. As severity of the disease increases, the position of the tongue changes so that the bulk of the muscle falls away from the incisors and no longer is in contact with the hard or soft palate. The normal radial bands from the anterior floor of the mouth to the mucosal surface are often missing in ALS as are the two curvilinear bands that run parallel to the mucosal surface and intersect the radial bands. Also, there is a mottled disorganization of the internal structure of the tongue with areas of increased and decreased signal intensity.
Antibodies to GM1 and GD1b in patients with motor neuron disease without plasma cell dyscrasia
Authors: Shy Michael E. , Evans Vance A. , Lublin Fred D. , Knobler Robert L. , Heiman‐Patterson Terry , Tahmoush Albert J. , Parry Gareth , Schick Paul , Deryk Theodora G. 
Published: January 2005

AbstractFiftynine percent of 49 patients with motor neuron disease and 25% of 91 control subjects had IgM antibodies to ganglioside GM1 but usually not to GD1b at titers less than 1:80. This suggests that antibodies to GM1 may be part of the normal human antibody repertoire. However, given the higher incidence of antibodies to GM1 in patients with motor neuron disease, there may be specific epitopes important in antiganglioside antibodies associated with motor neuron disease.
Characterization of ethosuximide reduction of low‐threshold calcium current in thalamic neurons
Authors: Coulter Douglas A. , Huguenard John R. , Prince David A. 
Published: January 2005

AbstractThe mechanism by which ethosuximide reduces thalamic low‐threshold calcium current (LTCC) was analyzed using voltage‐clamp techniques in acutely isolated ventrobasal complex neurons from rats and guinea pigs. The ethosuximide‐induced reduction of LTCC was voltage dependent: it was most pronounced at more‐hyperpolarized potentials and did not affect the time course of activation or inactivation of the current. Ethosuximide reduced LTCC without altering the voltage dependence of steady‐state inactivation or the time course of recovery from inactivation. Dimethadione reduced LTCC by a similar mechanism, while valproic acid had no effect on LTCC. We conclude that ethosuximide reduction of LTCC in thalamic neurons is consistent with a reduction in the number of available LTCC channels or in the single LTCC channel conductance, perhaps indicating a direct channel‐blocking action of this drug. Given the importance of LTCC in thalamic oscillatory behavior, a reduction in this current by ethosuximide would be a mechanism of action compatible with the known anticonvulsant effects of this drug in typical absence seizures.
Parkinsonism and neurofibrillary tangle pathology in pigmented nuclei
Authors: Rajput A. H. , Uitti Ryan J. , Sudhakar S. , Rozdilsky B. 
Published: January 2005

AbstractFour parkinsonian patients had neurofibrillary tangles, neuronal loss, and gliosis restricted to the substantia nigra and locus ceruleus. No Lewy body inclusions or other neuropathological changes accounting for parkinsonism were found in any of these patients. Their clinical features were characterized by an early age of onset, absence of dementia, absence of other neurological abnormalities, good response to drug therapy, and a long, slowly progressive course of illness. None of the patients had any history of encephalitis. These patients either represent a forme fruste of postencephalitic parkinsonism or a new entity thus far not described.
Myoclonic epilepsy and ragged‐red fibers with cytochrome oxidase deficiency: Neuropathology, biochemistry, and molecular genetics
Authors: Lombes Anne , Mendell Jerry R. , Nakase Hirofumi , Barohn Richard J. , Bonilla Eduardo , Zeviani Massimo , Yates Allan J. , Omerza Jennifer , Gales Tracy L. , Nakahara Keichi , Rizzuto Rosario , Engel W. King , Dimauro Salvatore 
Published: January 2005

AbstractA 36‐year‐old man with myoclonic epilepsy and ragged‐red fibers (MERRF) died after more than 18 years of follow‐up study. He was 1 of 3 affected siblings and the offspring of an affected mother, suggesting maternal transmission. At autopsy, there was neuronal loss and gliosis in the dentate nucleus of the cerebellum and in the inferior olivary nucleus. Skeletal muscle showed ragged‐red fibers, and paracrystalline inclusions in mitochondria by electron microscopy. Biochemical analysis showed a generalized partial defect of cytochrome c oxidase (COX) in mitochondria isolated from all tissues, including brain, heart, skeletal muscle, kidney, and liver. The Michaelis constant (Km) for cytochrome c was abnormally low, suggesting a defect of the mitochondrially encoded subunit II of COX. Immunological studies (enzyme‐linked immunosorbent assay, dot‐blot, Western blot, and immunohistochemistry) showed that the holoenzyme was decreased but subunit II was decreased more than the holocomplex or the nucleary encoded subunit IV. However, Northern and Southern blots showed that the gene for subunit II, as well as the genes for subunits I, III, IV, and VIII, were of normal size and were normally transcribed. A point mutation or a small deletion of mitochondrial DNA, probably affecting the COX‐II gene, may be responsible for the COX deficiency in this case of MERRF.
The pedunculopontine nucleus in Parkinson's disease
Authors: Zweig R. M. , Jankel W. R. , Hedreen J. C. , Mayeux R. , Price D. L. 
Published: January 2005

AbstractThis study demonstrated a significant loss of neurons within the lateral part of the pedunculoponite nucleus pars compacta in individuals with idiopathic Parkinson's disease and in individuals with combined Parkinson's and Alzheimer's disease. We also examined the extent of neuronal loss within the substantia nigra pars compacta, locus ceruleus, dorsal raphe nucleus, and nucleus basalis of Meynert. The number of pedunculopontine nucleus pars compacta neurons in the patients with Parkinson's or Parkinson's and Alzheimer's disease was reduced (average, 40°) in comparison with the number in control subjects or patients with Alzheimer's disease (p < 0.01). This finding correlated significantly with the extent of loss of substantia nigra pars compacta neurons (p < 0.01).
A prospective study on sudden unexpected death in epilepsy
Authors: Leestma Jan E. , Walczak Thaddeus , Hughes John R. , Kalelkar Mitra B. , Teas Shaku S. 
Published: January 2005

AbstractSudden unexpected death accounts for a substantial portion of deaths among epileptics. The incidence of this phenomenon is probably 1 in 370 to 1 in 1,110 in the general epileptic population but may be even higher in the 20‐to 40‐year age group, and still higher if epileptics with symptomatic epilepsy are selected. Sudden unexpected death in epileptics has been observed at least once weekly by the Office of the Medical Examiner of Cook County (Chicago), Illinois, for many years. A year‐long prospective study revealed that victims of this complication of epilepsy are most commonly black males averaging 35 years of age who have infrequent generalized seizures and usually have some structural lesion in the brain responsible for their seizures. They tend to abuse alcohol and have poor compliance with anticonvulsant medication. The electroencephalograms display considerable variability from record to record. At autopsy the heart, lung, and liver weights were heavier and the brain weights were lighter than expected. The mechanisms involved in sudden unexpected death in epileptics may include autonomically mediated cardiac arrhythmia alone or in combination with sudden neurogenic pulmonary edema and “backward” cardiac failure.
Perceptual‐motor and attentional correlates of developmental dysacalculia
Authors: Rosenberger Peter B. 
Published: January 2005

AbstractFrom the patients population of a learning disorders clinic, a group of 72 relative math underachievers was selected for achievement test performance below grade level on mathematics but at or above grade level on reading, with a difference of at least 1.5 standard deviation between the two. A comparison group of 30 relative reading underachievers met the converse criteria. Neurological examination showed mixed laterality preference (right hand and foot but left eye) to be more prevalent among the math underachievers. The two groups were closely comparable on Wechsler intelligence quotient scores, although the freedom from distractibility quotient tended to be lower for the math group. The latter group also showed a lower mean achievement quotient on the Bender Visuomotor Gestalt Test. On a standard questionnaire derived from DSM‐III criteria for attention deficit disorder, the math group showed higher scores for inattention, but not for hyperactivity, impulsivity, or poor peer relations. Both perceptual‐motor and attentional deficits appear to characterize children who are specifically learning disabled for math.
Mossy fiber synaptic reorganization in the epileptic human temporal lobe
Authors: Sutula Thomas , Cascino Gregory , Cavazos Jose , Parada Isabel , Ramirez Lincoln 
Published: January 2005

AbstractThe distribution of the mossy fiber synaptic terminals was examined using the Timm histochemical method in surgically excised hippocampus and dentate gyrus from patients who underwent lobectomy of the anterior part of the temporal lobe for refractory partial complex epilepsy. The dentate gyrus of epileptic patients demonstrated intense Timm granules and abundant mossy fiber synaptic terminals in the supragranular region and the inner molecular layer. In contrast, the dentate gyrus of presenescent nonepileptic primates demonstrated no Timm granules in the supragranular region. In nonepileptic senescent primates, occasional very sparse supragranuler Timm granules were observed that were easily distinguished from the dense pattern observed in association with human epilepsy. The results are morphological evidence of mossy fiber synaptic reorganization in the temporal lobe of epileptic humans, and suggest the intriguing possibility that mossy fiber sprouting and synaptic reorganization induced by repeated partial complex seizures may play a role in human epilepsy.
Alterations of hippocampal acetylcholinesterase in human temporal lobe epilepsy
Authors: Green Robert C. , Blume Howard W. , Kupferschmid Seth B. , Mesulam M‐Marsel 
Published: January 2005

AbstractHippocampal sclerosis is the most common pathological finding associated with human temporal lobe epilepsy. Histochemical study with acetylcholinesterase (AChE) staining was used to investigate 7 surgically resected temporal lobes with hippocampal sclerosis from patients with temporal lobe epilepsy. In all 7 specimens, an abnormal but consistent pattern of staining was noted. In the hilum of the dentate gyrus, AChE‐rich polymorphic cells were relatively preserved in comparison to the pyramidal neurons. In Ammon's horn, AChE fibers were lost in regions corresponding to the pyramidal cell dropout. AChE fibers wre also lost along the inner portion of the molecular layer of the dentate gyrus, yet they were preserved within the outer portions of the molecular layer. These findings provide additional evidence for the relative selectivity of hippocampal pathology in human temporal lobe epilepsy.
Non‐Alzheimer‐type pattern of brain cholineacetyltransferase reduction in dominantly inherited olivopontocerebellar atrophy
Authors: Kish Stephen J. , Robitaille Yves , El‐Awar Munir , Deck H. N. , Simmons Jerry , Schut Lawrence , Chang Li‐Jan , DiStefano Linda , Freedman Morris 
Published: January 2005

AbstractWe recently reported reduced activity of the cholinergic marker enzyme cholineacetyltransferase (ChAT) in several brain regions of patients with dominantly inherited olivopontocerebellar atrophy (OPCA). To document the regional extent of these changes we performed a comprehensive examination of the behavior of ChAT throughout both cerebral cortical and subcortical brain areas in 5 patients from one large OPCA pedigree. As compared with the controls, mean ChAT activities in OPCA were redced by 39 to 72 in all (n = 27) cerebral cortical areas examined and in several thalamic subdivisions, caudate head, globus pallidus, red nucles, and medical olfacotry area. In contradistinction to findings in Alzheimer's disease (AD), mean ChAT levels in OPCA amygdala and hippocampal subdivisions were either normal or only mildly reduced. The lack of severe disabling dementia in our OPCA patients compared with AD patients having a similar cortical cholinergic reduction could be explained by an absence of either a marked cholinergic loss in amygdala or hippocampus or significant loss of noncholinergic cerebral cortical and limbic neurons as occurs in AD brain. We suggest that this and other OPCA pedigrees having a cortical cholinergic reduction represent a unique model for the study of behavioral consequences of a more selective cerebral cortical cholinergic lesion rather than a limbic cholinergic lesion.
Experimental autoimmune motoneuron disease
Authors: Engelhardt Jozsef I. , Appel Stanley H. , Killian James M. 
Published: January 2005

AbstractAn animal model of disease of the lower motoneurons has been developed by inoculating guinea pigs with bovine motoneurons. Four of 9 immunized female animals and 4 of 5 immunized male animals developed symptoms of neuromuscular degeneration marked by weakness, evidence of denervation by electromyographic and morphological criteria, and a loss of motoneurons within the spinal cord. No inflammatory foci were noted within parenchyma or meninges of the central nervous system. The immunized guinea pigs developed high serum titers of IgG class antibodies to motoneurons. Immunohistochemical studies demonstrated the presence of IgG within spinal cord motoneurons and at the end‐plates of immunized animals. This experimental autoimmune motoneuron disease may provide important insights into the cause and pathogenesis of amyotrophic lateral sclerosis, a human motoneuron disease.
Cortical dihydrophyridine binding sites are unaltered in human alcoholic brain
Authors: Kril Jillian J. , Gundlach Andrew L. , Dodd Peter R. , Johnston Graham A. R. , Harper Clive G. 
Published: January 2005

AbstractThe density and affinity of sites labeled by the 1, 4‐dihydrophyridines, [3H]nitrendipine and (+) [3H]PN 200‐110, were not significantly different in superior frontal and parietal cortical membranes from alcoholic patients and nonalcoholic control patients. This is in contrast to the increased number of dihydropyridine receptors and increased functional activity of calcium channels reported in brain from rats treated chronically with ethanol and in neural cell lines grown in the presence of ethanol. These results indicate that 1, 4‐dihydropyridine‐sensitive calcium channels (L type) in the brain, despite possible acute changes, are unaltered following long‐term ethanol exposure in humans.