bluuebunny/crossref_metadata_2025_split huggingface dataset:
Effects of anterior temporal lobectomy on language function: A controlled study
Authors: Hermann Bruce P. , Wyler Allen R. 
Published: January 2005

AbstractThis study represents the first prospective controlled investigation of preoperative versus postoperative (6 months) language function in patients who underwent partial resection of the dominant (n = 15) or nondominant (n =14) anterior temporal lobe for treatment of medically refractory epilepsy. Language dominance was confirmed by in tracarotid sodium amytal test. Thirteen of the 15 patients undergoing anterior temporal lobectomy of the dominant hemisphere were operated on under local anesthesia in order to map language and memory functions intraoperatively. Using a standardized language/aphasia battery, we found a significant trend of worse preoperative language function in patients with dominant hemisphere temporal lobe foci in comparison to patients with nondominant foci. Following anterior temporal lobectomy, neither group showed any significant losses in language function, whereas the dominant hemisphere temporal lobe group showed significant improvement in receptive language comprehension and associative verbal fluency.
Cerebral blood flow requirement for brain viability in newborn infants is lower than in adults
Authors: Altman Denis I. , Powers William J. , Perlman Jeffrey M. , Herscovitch Peter , Volpe Sara L. , Volpe Joseptemberh J. 
Published: January 2005

AbstractMeasurements of regional cerebral blood flow (CBF) with positron emission tomography in adult humans with cerebrovascular disease have demonstrated consistently that values below 10 ml/(100 gm · min) occur only in infarcted brain. Although experimental data suggest that the newborn brain may be more resistant to ischemic injury than the adult brain, the minimum CBF necessary to sustain neuronal viability in newborn infants is unknown. We have measured CBF with positron emission tomography in 16 preterm and 14 term newborn infants and have determined the relationship between CBF and subsequent brain function as assessed by neurological examination and developmental assessment. The range of mean CBF in the preterm infants was 4.9 to 23 ml/(100 gm · min) and the range of mean CBF in the term infants was 9.0 to 73 ml/(100 gm · min). Five preterm infants and one term infant with mean CBF less than 10 ml/(100 gm · min) survived. Three of these 5 preterm infants, with mean CBF of 4.9, 5.2, and 9.3 ml/ (100 gm · min, respectively, have normal neurological examinations and Bayley Scales of 80 or greater at 6, 6, and 24 months of age, respectively. One (mean CBF 6.9) has normal cognitive development (Bayley 103) and a mild spastic diplegia at age 19 months, and one infant (mean CBF 6.2) has a left hemiparesis and a Binet IQ score of 70 at age 33 months. The term infant, with a mean CBF of 9.0 ml/(100 gm · min), was developing normally when he died of Septembersis at age 5 months. These data indicate that mean CBF as low as 5 ml/(100 gm · min) in newborn infants can be associated with normal subsequent neurological development and therefore with preservation of a substantial number of cortical/ neurons. Since the minimal CBF below this value necessary to maintain neuronal viability in newborn infants remains to be determined, measurements of CBF in the newborn period should not be used in determination of brain death.
An isoelectric focusing study in herpes simplex virus encephalitis
Authors: Grimaldi Luigi M. E. , Roos Raymond P. , Manservigi Roberto , Spear Patricia G. , Lakeman Fred D. , Whitley Richard J. 
Published: January 2005

AbstractTo establish an early reliable diagnostic test for herpes simplex virus (HSV) type 1 encephalitis (HSVE), we used isoelectric focusing (IEF) and an IEF‐overlay technique with radiolabeled HSV glycoprotein B (gB) to study 7 serum and 12 cerebrospinal fluid (CSF) samples from 12 patients with presumed or biopsy‐proved HSVE. Blood‐brain barrier damage and increased intra‐blood‐brain barrier IgG synthesis were detected in 5 of the 7 patients with HSVE. CSF oligoclonal bands were found in 6 of 11 patients. Using an IEF‐overlay technique, we detected anti‐gB antibody in all serum (7 of 7) and in 10 of 12 CSF samples. Anti‐gB antibody was found in 4 of 6 CSF specimens obtained within the first week of disease (days 3 to 5) and in all samples collected later in the disease. The pH range of anti‐gB antibody activity was broad (4.5 to 9.5), indicating a heterogeneous immune response to HSV. A hematogenous origin of the CSF antibody was suggested because anti‐gB antibody appeared in serum before matched CSF and because both serum and matched CSF had a similar antibody IEF pattern. Local production of anti‐gB antibody was suggested in some cases because of a greater prominence of anti‐gB antibody in CSF than in matched sera and because CSF oligoclonal bands had anti‐gB antibody activity. In contrast, only one of 6 CSF samples from patients with multiple sclerosis had gB antibody activity; in this case, anti‐gB antibody activity did not correspond in isoelectric point location to oligoclonal bands. The IEF‐HSV‐gB overlay technique may be a useful diagnostic test for HSVE and a valuable research tool for studying qualitative aspects of the HSV humoral immune response.
The neuropathology of aminergic nuclei in Alzheimer's disease
Authors: Zweig Richard M. , Ross Christopher A. , Hedreen John C. , Steele Cynthia , Cardillo Joseptemberh E. , Whitehouse Peter J. , Folstein Marchshal F. , Price Donald L. 
Published: January 2005

AbstractNeuronal loss and the presence of neurofibrillary tangles (NFTs) within aminergic nuclei were examined in a series of patients with Alzheimer's disease (AD). Neuromelanin‐containing neurons within the locus ceruleus and large nucleolus‐containing neurons within the dorsal raphe nucleus and the central superior (raphe) nucleus were counted in 25 patients with AD and in 12 age‐matched control subjects. Numbers of NFTs were quantified in the same regions. Counts were compared with clinical data, including psychiatric evaluations, available for 21 of the patients with AD. Within the locus ceruleus in the patients with AD, abnormalities were more severe at mid level than at caudal or rostral levels (p < 0.01). Within the dorsal raphe nucleus, neuronal loss was most severe caudally (p < 0.05). NFTs, but not neuronal loss, were demonstrated within the central superior nucleus. Neuronal and NFT counts did not correlate at individual levels; the relative severity of both pathological processes was consistent from level to level within nuclei but was less consistent between nuclei. Neuronal loss correlated inversely with age, particularly within the locus ceruleus. Duration of disease correlated inversely with counts of NFTs, particularly within the dorsal raphe nucleus, implying a correlation between NFT counts and rate of progression of disease as all but 3 patients had severe dementia. Significantly, patients with AD complicated by major depression had fewer neurons at the mid level of the locus ceruleus and at the rostral level of the central superior nucleus in comparison with nondepressed patients. There was a trend suggesting greater loss of neurons at all levels of the locus ceruleus and dorsal raphe nucleus in depressed individuals.
Comparison of rate of annual change of mental status score in four independent studies of patients with Alzheimer's disease
Authors: Katzman Robert , Brown Theodore , Thal Leon J. , Fuld Paula A. , Aronson Miriam , Butters Nelson , Klauber Melville R. , Wiederholt Wigbert , Pay Marchy , Renbing Xiong , Ooi Wee Lock , Hofstetter Richard , Terry Robert D. 
Published: January 2005

AbstractLongitudinal studies of subjects with autopsy‐proven Alzheimer's disease in one skilled nursing home and of clinically diagnosed cases (NINCDS/ADRDA criteria) in three community cohorts are compared with regard to the annual rate of change in the error score of the Blessed information‐memory‐concentration test (IMC) in which the maximum number of errors possible is 33. The four cohorts differed significantly from each other in regard to age, education, sex, and the degree of dementia as measured by the initial IMC score. Subjects spanned the age range of 52 to 96 years and had 2 to 20 years of education. The rate of change in error score per year was similar whether the initial error score was 0 to 7, 8 to 15, or 16 to 23; however, the rate was reduced when the initial error score was 24 or above, due to a ceiling effect of the test. Among subjects with initial IMC scores less than 24, the annual rate of change varied considerably. However, the mean annual rate of change, 4.4 errors (SD 3.6, SEM 0.3) per year, was independent of residence in a nursing home, location of the study site, and of the patient's sex or education. Of particular importance was the finding that the rate of change in mental test score was independent of age. It can be concluded that the rate of cognitive deterioration in patients with Alzheimer's disease is quite variable among individuals and is independent of the patient's age and whether the patient resides in the community or in a nursing home. More importantly, the rate of change of the IMC test provides no evidence for a difference between early‐onset and late‐onset Alzheimer's disease. The average rate of change on the IMC and its variance can be used as the basis for designing studies of drugs or procedures that might alter the course of Alzheimer's disease.
Effects of chronic phenobarbital exposure on cultured mouse spinal cord neurons
Authors: Serrano Elba E. , Kunis David M. , Ransom Bruce R. 
Published: January 2005

AbstractThe anticonvulsant phenobarbital (PB), at concentrations of 20, 40, and 90 μg/ml, was chronically applied to cell cultures of mouse spinal cord from day 2 or day 14 after initial plating, and the effects of this exposure on neuronal density and morphological characteristics were determined. Neuronal morphological characteristics were analyzed quantitatively following intracellular injection of the fluorescent dye Lucifer yellow. Cultures exposed to PB for 6 weeks, from day 14 after plating, showed concentration‐dependent reductions in neuronal density; both large and small neurons were equally affected. PB exposure also reduced dendritic branching frequency, and the length of dendrites, of remaining large neurons. A higher percentage of these neurons had a bipolar branching pattern than was normally the case. Neurons in cultures exposed to PB from day 2 after plating, compared with those exposed from day 14, showed significantly less alteration in terms of density and morphological characteristics. Effects on neuronal morphological characteristics increased with duration of drug exposure. Equimolar concentrations of barbituric acid produced effects similar to those produced by PB. Chronic exposure to PB adversely affects survival and morphological characteristics of mammalian central neurons grown in cell culture. Curiously, exposure from the time of initial plating appears to be less deleterious than exposure initiated 2 weeks later. To the extent that neuronal development in vitro can be compared to the situation in vivo, these results, and those of other investigators, raise concerns about long‐term exposure of the developing human nervous system to PB.
Hexosaminidase a deficiency manifesting as spinal muscular atrophy of late onset
Authors: Karni Avi , Navon Ruth , Sadeh Menachem 
Published: January 2005

AbstractProximal lower limb weakness and fasciculations were the only clinical manifestations of hexosaminidase A (Hex A) deficiency in a 39‐year‐old woman. Hex A activity in serum and leukocytes was 0 to 4% by standard heat inactivation fluorogenic substrate methods, and 5% when Hex A isoenzymes were fractionated by diethyl‐aminoethanol cellulose chromatography. Computed tomography of the brain showed cerebellar atrophy. We suggest that Hex A activity studies be done in patients with typical as well as a typical spinal muscular atrophy.
Successful treatment of cherry red spot—myoclonus syndrome with 5‐hydroxytryptophan
Authors: Gascon Generoso , Wallenberg Bradford , Daif Abdul Khadair , Ozand Pinar 
Published: January 2005

AbstractA 14‐year‐old Saudi boy with cherry red spot‐myo‐clonus syndrome and documented neuraminidase deficiency responded well to titrated doses of 5‐hydroxy‐tryptophan as an add‐on treatment.
Paraneoplastic cerebellar degeneration: Clinical‐immunological correlations
Authors: Anderson Neil E. , Rosenblum Marchc K. , Posner Jerome B. 
Published: January 2005

AbstractFour different antineuronal autoantibodies have been identified in 23 of 47 patients with paraneoplastic cerebellar degeneration (PCD). The most common, an antibody against 34‐ to 38‐kDa and 62‐ to 64‐kDa protein antigens in the cytoplasm of Purkinje cells, was found in 18 patients. It is a highly specific Marchker for a severe stereotypical subacute pancerebellar syndrome of truncal and appendicular ataxia, dysarthria, and nystagmus in women with cancer (usually ovarian or breast carcinoma). Different anti‐Purkinje cell antibodies (APCA) were found in 2 other patients with PCD. With two possible exceptions, an APCA was not found in patients with other neurological diseases, with cancer not associated with neurological symptoms, or in normal subjects. Antibodies reactive with neuronal nucleoproteins were identified in 3 other patients with PCD: an antibody that recognized 35‐ to 40‐kDa neuronal antigens was found in 2 women with small‐cell lung carcinorma, while an antibody in a woman with breast carcinoma identified 53‐ to 61‐kDa and 79‐ to 84‐kDa antigens. Detection of an antineuronal antibody in a patient without known cancer should prompt a careful search for a tumor at a site appropriate to the antibody type.
Neurofibrillary tangles in cholinergic pedunculopontine neurons in Alzheimer's disease
Authors: Mufson Elliott J. , Mash Deborah C. , Hersh Louis B. 
Published: January 2005

AbstractThe cholinergic neurons located within the pedunculopontine nucleus (Ch5) of patients with Alzheimer's disease (AD; n = 15), Parkinson's disease (PD; n = 2), and neurologically normal (n = 6) subjects were visualized immunohistochemically using choline acetyltransferase, pharmacohistochemically using acetylcholinesterase, or by reduced histochemical methods using nicotinamide adenine dinucleotide phosphate diaphorase (NADPH‐d). Each histochemical procedure localized a well‐delineated, compact lateral group and a more diffuse medial group of neurons within the pedunculopontine nucleus. Co‐localization experiments revealed that all three enzymes Marchked the same population of cholinergic neurons. The extent of pathological alterations associated with the cholinergic neurons within the compact lateral sector of the pedunculopontine nucleus was examined in sections that reacted for NADPH‐d, counterstained with thioflavin‐S. The average number of neurofibrillary tangles within this portion of the pedunculopontine nucleus was 25.4 (range 0–70) in patients with AD, 1.5 (range 1–2) in those with PD, and 1.2 (range 0–4) in aged control subjects. Of the total number of neurofibrillary tangles counted in AD cases, 72.7% were end‐stage ghosts and 27.3% were tangle‐bearing neurons. The pathological alteration of cholinergic neurons of the compact lateral aspect of the pedunculopontine nucleus may play a role in some of the behavioral features characteristic of AD.
Kearns‐sayre syndrome: Biochemical studies of mitochondrial metabolism
Authors: Martens M. E. , Peterson P. L. , Lee C. P. , Nigro M. A. , Hart Z. , Glasberg M. , Hatfield J. S. , Chang C. H. 
Published: January 2005

AbstractExamination of oxidative metabolism in mitochondria isolated from quadriceps skeletal muscle biopsy specimens of 4 patients with Kearns‐Sayre syndrome has shown that the mitochondria were tightly coupled, with maximal respiratory rates depending on the presence of adenosine diphosphate (ADP), Ca2+, or uncoupler. The state 3 respiratory rates with nicotinamide adenine dinucleotide (NAD)—linked substrates and succinate were much lower than those of control subjects. The cytochrome oxidase activities (measured with ascorbate + phenazine methosulfate as substrates) were also Decemberreased, but this segment of the respiratory chain was not rate‐limiting for succinate or NAD‐linked substrate oxidation. Analyses of the steady‐state reduction kinetics of the respiratory chain carriers revealed that the rate‐limiting step of the impaired respiration with succinate or NAD‐linked substrates lies between the c cytochromes and cytochrome oxidase. Measurement of the total substrate‐reducible (at anaerobiosis) and chemically reducible levels of the cytochromes in mitochondria from 3 patients showed a severe deficiency of cytochrome a + a3 and an excess of the c cytochromes. To our knowledge, this is the first instance in which a mitochondrial electron transfer defect and cytochrome oxidase deficiency has been shown to be associated with an excess of the c cytochromes.
The effect of postischemic blood glucose levels on ischemic brain damage in the rat
Authors: Voll C. L. , Auer R. N. 
Published: January 2005

AbstractThe effect of insulin‐induced hypoglycemia following 10.5 minutes of forebrain ischemia was studied in the rat. All groups received preischemic glucose loading (2 gm/kg) to promote brain infarction. Following completion of ischemia, rats received either 2 to 3 IU/kg (low‐dose group) or 8 to 20 IU/kg (high‐dose group) insulin. During the survival period, blood glucose concentrations were maintained in the ranges of 1.2 to 2.9 mM and 2.9 to 4.9 mM, respectively, for the low‐dose and high‐dose insulin groups. Control rats were given 2 gm/kg glucose immediately following ischemia. During the recovery period, until perfusion at 7 days, they were given glucose, 2 gm/kg, twice daily by intraperitoneal injection, and their drinking water was supplemented with 25% glucose. Mortality (p < 0.05) and postischemic seizure incidence (p < 0.01) were significantly reduced in the low‐dose insulin group compared to the control group. Mortality was increased in the high‐dose insulin group compared to the control group and was associated with an increased incidence of postischemic seizures. Neuropathological examination revealed no cortical infarction in the low‐dose or high‐dose insulin‐treated rats compared to a 60% incidence of cortical infarction in the control group. In addition, the high‐dose insulin‐treated group showed a significant reduction in striatal and hip‐pocampal CA1 selective neuronal necrosis compared to control rats with comparable survivals (p < 0.05). The findings suggest that postischemic blood glucose concentrations play an important role in modulating both ischemic infarction and selective neuronal necrosis.
Biological differences between ischemia, hypoglycemia, and epilepsy
Authors: Auer Roland N. , Siesjo Bo K. 
Published: January 2005

AbstractIschemia, hypoglycemia, and epilepsy have long been thought to produce similar or identical brain damage. Further more, these insults have been assumed to be additive in their damaging effects. These notions have been based on neuropathological observations in the hippocampus and cerebral cortex, and on the tenet that energy failure (ischemia. hypoglycemia) and increased demand for energy (epilepsy) similarly give rise to selective neuronal neuronal mecrosis Recently, other bases for considering these three insults identical have grown out of observations that loss of calcium homeostasis is common to all and that an excitotoxic mechanism of selective neuronal necrosis exists in all three conditions. Fundamental differences between ischemia, hypoglycemia, and epilepsy include the underlying neurochemical changes induced, the neuronal revival times, the time course of neuronal death, the distribution of selective neuronal necrosis, and the likely excitotoxins released. Lactic acid accumulation, implicated in damage to the neuropil as well as to neuronal cell bodies, also occurs to different degrees and in different distributions in the three conditions. The degree and distribution of pannecrosis is thus also different in ischemia, hypoglycemia, and epilepsy.
Motor vehicle crashes in dementia of the alzheimer type
Authors: Friedland Robert P. , Koss Elisabeth , Kumar Anand , Gaine Sean , Metzler Diane , Haxby James V. , Moore Angela 
Published: January 2005

AbstractTo assess the occurrence and related features of motor vehicle crashes in patients with dementia of the Alzheimer type (DAT) we studied 30 patients who had been followed longitudinally and 20 healthy age‐matched control subjects. Data were gathered from first‐degree relatives who had lived with the subjects for the previous 5 years or more. Fortyseven percent of the DAT patients incurred at least one crash while they were driving, whereas only 10% of the control subjects had had a crash in the previous 5 years. The odds ratio for crashes in the DAT group was 7.9 (p < 0.01). Moreover, in 77% of DAT patients, a deterioration in driving performance was noted, and 63% of the patients had stopped driving. However, only 42% of the DAT patients who stopped driving did so before a crash occurred. Mean illness duration was 4.0 (± 1.8) years, and the mean Mini‐Mental Status Examination score was 19.9 (± 6.3) at the time of the first crash in the DAT group. The occurrence of crashes was not significantly correlated with dementia severity or with disease duration. These data suggest that the occurrence of driving crashes in patients with DAT is an important public health problem.
Phenotypic heterogeneity in familial alzheimer's disease: A study of 24 kindreds
Authors: Bird T. D. , Sumi S. M. , Nemens E. J. , Nochlin D. , Schellenberg G. , Lampe T. H. , Sadovnick A. , Chui H. , Miner G. W. , Tinklenberg J. 
Published: January 2005

AbstractWe report the clinical and neuropathological characteristics occurring in 180 demented individuals from 24 kindreds with familial Alzheimer's disease (FAD). Each family had at least two affected generations, and at least one autopsy or brain biopsy was compatible with the diagnosis of AD. Forty‐nine neuropathological specimens or reports were reviewed. Mean age of onset for the total group was 54.7 years ± 11.5, with a large range of 30 to 84 years. Mean age at death was 63.5 years ± 12.2, with a range of 46 to 85. Mean duration of disease was 8.8 years ± 4.4, with a range of 1 to 23 years. Six findings suggested phenotypic heterogeneity in FAD. (1) Five families represented an early age of onset group with mean onset at 42 years (range 30 to 51 years) and mean disease duration of 6.7 years. (2) Eight families represented a late onset group with mean onset at 68 years (range 59 to 78 years) and a mean duration of 8.5 years. (3) Seven families were of Volga German ancestry, all originating from the same two villages in Russia. Mean age of onset was 55.9 years (range 40 to 72 years), with a mean disease duration of 10 years. This group probably represents the genetic founder effect of an autosomal dominant gene for AD. (4) One family had the unusual characteristics of neurofibrillary tangles and granulovacuolar change but no amyloid plaques, a mean disease duration of more than 11 years, and a “schizophrenia‐like” onset. (5) One family with late onset also had clinical and pathological evidence for anterior horn cell disease. (6) Two autopsies in 1 family both showed remarkable rarefaction of myelin and expansion of perivascular spaces in centrum semiovale (état criblé), with marked leptomeningeal and cortical amyloid angiopathy, distinct from the other FAD brains. It remains to be determined whether the clinical and pathological differences between these families represent genetic heterogeneity at the biochemical or molecular level.