bluuebunny/crossref_metadata_2025_split huggingface dataset:
Ammonia metabolism in Reye syndrome and the effect of citrulline
Authors: Delong G. Robert , Glick Thomas H. 
Published: January 2005

AbstractAmmonia metabolism in Reye syndrome was studied by quantitative analysis of the time course of hyperammonemia and the urinary excretion of ammonia, urea, and total nitrogen. These measures were then utilized to assess the effect of citrulline administration in 8 patients compared to results in 22 patients managed without citrulline. Two indices of the severity and duration of hyperammonemia correlated strongly with mortality: the half‐time for decline of hyperammonemia and the area under the hyperammonemia curve (an index of the total burden of ammonia presented to the brain). These results suggest that the total amount of ammonia delivered to brain may be important to the pathogenesis of encephalopathy. Citrulline‐treated patients had more severe disease at admission and greater abnormalities in indices of nitrogen and ammonia metabolism, though the latter did not reach significance. The urine ammonia/urea nitrogen excretion ratio, an index of the efficiency of ammonia conversion to urea, normalized more rapidly in the citrulline‐treated group, evidence that citrulline may have improved urea cycle function. Overall mortality did not differ in the two groups. The deaths of 2 citrulline‐treated patients in this small group were attributable to factors unrelated to treatment, however, so the possible effect of citrulline on mortality was not definitively tested. No indication was found that citrulline was harmful, nor that it increased ammonia levels.
Evidence for autosomal recessive inheritance in systemic carnitine deficiency
Authors: Di Donato S. , Rimoldi M. , Cornelio F. , Bottacchi E. , Giunta A. 
Published: January 2005

AbstractIdentical twin girls died at age 3 years from acute episodes of metabolic encephalopathy reminiscent of Reye syndrome. Biochemical studies in one of the twins demonstrated low levels of carnitine in muscle, liver, and plasma. Carnitine content was low in the liver and plasma of both parents, implying autosomal recessive transmission.
Childhood multiple sclerosis: Clinical features and demonstration of changes in T cell subsets with disease activity
Authors: Hauser Stephen L. , Bresnan Michael J. , Reinherz Ellis L. , Weiner Howard L. 
Published: January 2005

AbstractUsing monoclonal antibodies that identify T cell subsets in human beings (T4 = inducer cell; T5 = suppressor/cytotoxic cell), we have previously shown that most patients with multiple sclerosis (MS) have decreased T5 cells and an elevated T4: T5 ratio during periods of disease activity. We have recently studied three children with MS beginning at 4 years 11 months, 10 1/2 years, and 2 years 11 months of age. Clinical symptoms included a relapsing brainstem syndrome in the first, Devic syndrome and recurrent optic neuritis in the second, and multiple attacks with accumulating neurological deficits in the third. Two of these children represent the youngest cases of MS yet reported. In each child, circulating T5 cells were reduced or absent during an acute exacerbation of disease; following stabilization, T5 cells returned and the T4: T5 ratio became normal. No changes in T cell subsets were seen in a group of age‐matched healthy children or in children suffering from other neurological diseases. Our finding that young children with active MS have a pattern of T cell abnormalities identical to that of adult MS patients suggests that the same pathophysiological process which causes MS in adults occurs in young children as well.
A new autoradiographic approach for imaging forebrain dopamine distribution
Authors: Wooten G. Frederick , Horne Malcolm K. 
Published: January 2005

AbstractRegional distribution of carbon 14 activity in the forebrain was studied autoradiographically following systemic administration of tracer doses of L‐[14C]‐dopa to rats pretreated with carbidopa. Five minutes after the injection, regional forebrain distribution of carbon 14 varied with regional cerebral blood flow. At 45 minutes, 2 hours, and 4 hours after administration of 14C‐dopa, however, carbon 14 activity had accumulated selectively in forebrain regions rich in dopamine (e.g., the striatum, olfactory tubercle, and nucleus accumbens). A unilateral lesion of the substantia nigra markedly attenuated the accumulation of carbon 14 in the ipsilateral striatum, while reserpine pretreatment reduced by about 50% the accumulation of carbon 14 in the striatum bilaterally. These results outline a new autoradiographic method for mapping forebrain dopamine systems and suggest an approach to imaging brain dopaminergic neurons in humans by positron emission tomography.
Recurrent herpes simplex encephalitis: Recovery of virus after Ara‐A treatment
Authors: Dix Richard D. , Baringer J. Richard , Panitch Hillel S. , Rosenberg Sidney H. , Hagedorn Joel , Whaley Jay 
Published: January 2005

AbstractA 54‐year‐old man developed clinical findings consistent with herpes simplex virus (HSV) encephalitis. These signs included an abrupt onset of focal central nervous system disease, cerebrospinal fluid pleocytosis, localized electroen cephalographic abnormalities, and a computerized tomographic scan showing right temporal lobe involvement. Treatment with adenine arabinoside (Ara‐A) resulted in improvement. Two months later he again became confused, and a left hemiparesis developed. Although biopsy revealed extensive necrosis and inflammatory response, HSV antigens and herpesvirus particles were not detected. Culture of biopsy tissue yielded HSV type 1 only after 18 days. A second course of Ara‐A was administered but the patient failed to improve and died four months later. Extensive inflammatory necrosis of both temporal lobes involving gray and white matter was found. Cultures were negative for HSV. The recovery of virus from our patient during the second encephalitic episode should raise concerns regarding the efficacy of Ara‐A treatment and the role of the virus in recurrent disease. In addition, the importance of maintaining biopsy tissue in culture for prolonged periods is emphasized.
Variable waveforms in downbeat nystagmus imply short‐term gain changes
Authors: Abel L. A. , Traccis S. , Dell'Osso L. F. , Ansevin C. F. 
Published: January 2005

AbstractA patient with downbeat nystagmus subsequent to ankylosing spondylitis was studied. His nystagmus was found to exhibit both increasing‐ and decreasing‐velocity exponential slow phases as well as the linear form more often reported. Alternation between waveforms sometimes occurred on a beat‐to‐beat or even intrabeat basis. Possible expalnations for all three waveforms are presented in terms of short‐term gain changes in cerebellar compensation for leaky brainstem neural integrators. A computer model was developed and its results are discussed.
Changes in regional cerebral blood flow during the course of classic migraine attacks
Authors: Lauritzen Martin , Olsen Tom Skyhøj , Lassen Niels A. , Paulson Olaf B. 
Published: January 2005

AbstractRegional cerebral blood flow (rCBF) following carotid arteriography was studied in thirteen patients with classic migraine. Using the 133xenon intraarterial injection method, rCBF was measured in 254 areas in one hemisphere. Nine patients developed a characteristic attack following arteriography and were examined by a series of rCBF studies, spaced by intervals of 5 to 10 minutes. A wave of reduced blood flow originating in the posterior part of the brain and progressing anteriorly was observed in eight of the nine patients. The oligemia advanced at a speed of 2 mm per minute over the hemisphere, progressing anteriorly but not crossing the rolandic or sylvian sulcus. Typically, the spreading oligemia reached the primary sensorimotor area after symptoms from that area had begun and persisted there long after the focal symptoms had disappeared. The observed time course suggests that the focal symptoms are not secondary to the oligemia. We suggest that focal symptoms and blood flow changes may be secondary to spreading depression of Leao.
Parental age as a risk factor in Alzheimer's disease
Authors: Corkin Suzanne , Growdon John H. , Rasmussen Shelley L. 
Published: January 2005

AbstractBecause of the associations between Alzheimer's disease and Down's syndrome, advanced maternal and paternal age, which are risk factors for Down's syndrome, have been proposed as risk factors for Alzheimer's disease. Parental age data were obtained from a group of patients with Alzheimer's disease and compared with comparable data for a group of nondemented subjects matched for age and socioeconomic level. As a way of detecting a familial causative factor in Alzheimer's disease, life‐span data were also collected for parents of patients and control subjects. The results indicated that patients with Alzheimer's disease and nondemented control subjects did not differ in the mean ages of their parents at the time of the patients' or subjects' births, nor did parental age correlate significantly with age at onset of disease. Moreover, the parents of patients with Alzheimer's disease did not differ in mean age at death from the parents of nondemented control subjects.
Outcome in neonates with convulsions treated in an intensive care unit
Authors: Bergman Ira , Painter Michael J. , Hirsch Robert P. , Crumrine Patricia K. , David Ronald 
Published: January 2005

AbstractNeurological and developmental outcome was assessed in 131 survivors of neonatal seizures aged 1 to 5 years who had been treated in a single intensive care unit from 1976 to 1979. Half the children had been born at less than 37 weeks' gestational age, and 28% at 31 weeks or less. Fifty‐one children were normal on examination, 17 had minor abnormalities, 25 had moderate disabilities, 30 had severe disabilities, 6 had died because of profound neurological deficits, and 2 could not be located. Recurrent nonfebrile seizures had developed in 26 children. Most children with motor handicaps or visual loss were intellectually retarded, but 10 of 15 children with bilateral hearing loss were intellectually normal. Of 77 children whose seizures were caused by a hypoxic‐ischemic insult, 41 developed moderate or severe disabilities. As determined by multivariate analysis, significant neonatal predictors of poor outcome in this group included seizures with late onset, tonic seizures, and seizures lasting for many days. Although seizure freqency and neonatal mortality associated with seizures were greatest in very premature infants, the outcome in premature infants who survived was not significantly different from that of term infants.
Hepatitis a virus immunoglobulin m antibody in acute neurological disease
Authors: Bosch Vivian V. , Dowling Peter C. , Cook Stuart D. 
Published: January 2005

AbstractSera from 167 patients with Guillain‐Barré syndrome, 224 patients with other neurological diseases, and 10 normal subjects were tested for hepatitis A virus– specific immunoglobulin M antibodies. Two patients with classic Guillain‐Barré syndrome and 1 patient with acute myeloradiculopathy had high titers of virusspecific IgM antibody. All three patients had jaundice and laboratory evidence of liver involvement before the onset of the neurological disease. None of the other sera from patients with Guillain‐Barré syndrome or control subjects were positive for hepatitis A virus.
Lack of clinical efficacy of chronic oral physostigmine in Alzheimer's disease
Authors: Jotkowitz Seymour 
Published: January 2005

AbstractTen patients with mild to severe Alzheimer's disease were treated for up to 10 months with 10 to 15 mg oral physostigmine daily or with placebo, according to a single blind, multiple crossover protocol. No clinically meaningful improvement occurred with physostigmine treatment.
Occupational adjustment following neurosurgical treatment of epilepsy
Authors: Augustine Elizabeth A. , Novelly Robert A. , Mattson Richard H. , Glaser Gilbert H. , Williamson Peter D. , Spencer Dennis D. , Spencer Susan S. 
Published: January 2005

AbstractOccupational adjustment was investigated in a group of 32 adult epileptic patients followed for 1 to 10 years after cortical resection for intractable seizues. An employment status rating was assigned according to the following scale: (1) employed (working 75 to 100% of full time); (2) underemployed (25 to 74%); and (3) unemployed (0 to 24%). The number of patients employed increased from 14 to 23, whereas the number of underemployed decreased from 8 to 0. The unemployed group showed little change. An improvement or maintenance of employment status was correlated directly with improved postoperative seizure control. Conversely, poor occupational adjustment was often associated with fair or poor seizure control following operation. Unemployment was also related to the presence of preoperative psychiatric disorders, a history of past unemployment, and cognitive disturbances affecting nondominant hemisphere function. Although improvement in seizure control is important, this investigation suggests it is only one of many influences on the occupational adjustment of patients following surgical treatment of epilepsy.
Neurodevelopmental outcome of survivors with posthemorrhagic hydrocephalus following grade II neonatal intraventricular hemorrhage
Authors: Krishnamoorthy K. S. , Kuehnle K. J. , Todres I. D. , Delong G. R. 
Published: January 2005

AbstractThis report describes the neurodevelopmental outcome in 12 children who developed posthemorrhagic hydrocephalus following Grade II intraventricular hemorrhage. Grade II intraventricular hemorrhage is defined as the partial filling of both lateral ventricles by intraventricular blood, documented by computed tomographic brain scan. Characeristics of the study group included a mean birth weight of 1.8 kg and a mean gestation of 32.3 weeks. Subjects were followed for three to seven years (mean, four and one‐half years). Neurological evalutions were conducted prospectively. Bayley's Scales of Infant Development and McCarthy's Scales of Children's Abilities were used for the psychometric evalutions. All children were ambulatory. Only 1 had moderate spastic diplegia. Nine children (75%) had IQ scores within the normal range. The results of this prospective analysis suggest a favorable outcome for infants surviving with Grade II intraventricular hemorrhage, even with the complication of posthemorrhagic hydrocephalus.
Outcome of neonatal intraventricular hemorrhage with periventricular echodense lesions
Authors: McMenamin Joseph B. , Shcackelford Gray D. , Volpe Joseph J. 
Published: January 2005

AbstractThe incidence of periventricular‐intraventricular hemorrhage (PV‐IVH) in a group of 460 pretern infants with birth weight less than 2,250 gm, studies by cranial ultrasonography, was 39%. Sixty‐four (36%) of the infants with periventricular‐intravenmtricular hemorrhage had, in addition, perventricular intraparenchymal echodensity (IPE) evident on ultrasound scan. Thirty‐three of the 64 infants had large IPE, and 31 had small IPE. Large IPE consisted of globular echodensity, most often onj the side of maximum intraventricular hemorrhage, extending from the external angle of the laterakl ventricle into major portions of the white mater of the frontal and parietal lobes; small IPE, often bilateral, consisted of linear echodensity extending for a few millimeters from the external ange of the lateral ventricle into the periventricular white matter. The outcome for ingfants with large and a mall IPE differed markedly. Mortality was greatest(94%) for infants with large IPE and birth weight less thasn 1,000gm. All survivors with large IPE, regardless of birth weight, had modrate to severe neurological deficits evident on follow‐up. In contrast, infants with small IPE and birth weight, had moderate to severe neurological deficts evident on follow‐up. In contrast, infants with small IPE and birth weight less thasn 1,000 gm had a mortality of38%. Moreover, 70% of all survivors with small IPE were free of neurological deficits on follow‐up. The difference in outsome appeared to relate in largest part to the severity of the parenchymal involvement. Thjese data have major significance for decisions concerning management of infants with periventricular‐hemorrhage and intraparenchymal involvement.
Painful lumbosacral plexopathy with elevated erythrocyte sedimentation rate: A treatable inflammatory syndrome
Authors: Bradley Walter G. , Chad David , Verghese Jacob P. , Liu Hsiu‐Chih , Good Paul , Gabbai Alberto A. , Adelman Lester S. 
Published: January 2005

AbstractSix patients had a syndrome of painful lumbosacral plexopathy and elevated erythrocyte sedimentation rate. Sural nerve biopsy in each case showed axonal degeneration and epineurial arterioles surrounded by mononuclear inflammatory cells. Differential fascicular involvement suggested an ischemic cause in three nerves, but no patient had a necrotizing vasculitis. None of the six patients had vasculitis or cancer. Three of the six were diabetic and were initially thought to have diabetic plexopathy, but deterioration continued despite control of the diabetes. These six patients appeared to have an ischemic neuropathy with an immunological basis. Five were treated with immunosuppressant drugs, and in four the plexopathy improved or was arrested. The pathogenesis is unclear, but postmortem findings in one case suggest that the syndrome does not stem from an underlying vasculitis.
Abnormal neuromuscular transmission in an infantile myasthenic syndrome
Authors: Albers James W. , Faulkner John A. , Dorovini‐Zis Katerina , Barald Kate F. , Must Rachel E. , Ball Richard D. 
Published: January 2005

AbstractA term infant required intubation for respiratory depression. Examination revealed hypotonia and areflexia with intact extraocular movements. Electrodiagnostic studies demonstrated defective neuromuscular transmission characterized by borderline low motor evoked amplitudes, profound decremental responses at all stimulation rates, and moderate facilitation (50 to 740%) 15 seconds after 5 seconds of 50 Hz stimulation. Repetitive muscle action potential responses were not recorded following stimulation of nerves by single shocks. Sensory evoked responses and needle electromyographic findings were normal, as were acetylcholine receptor antibody levels. Results of muscle histochemical analyses, including acetylcholinesterase stains, were normal. End‐plate histometric analyses demonstrated only a slight reduction in mean synaptic vesicle diameter compared with that in an adult control subject. In vitro muscle contractile properties, stimulating the muscle directly, were normal. Anticholinesterase medications were ineffective. Guanidine produced clinical deterioration. The amplitude of motor evoked responses progressively declined, whereas the percentage of decrement and amount of post‐tetanic facilitation increased. Although the nature of the transmission defect was not identified, the data are consistent with abnormal acetylcholine resynthesis, mobilization, or storage without abnormality of release or receptors.
Midbrain paresis of horizontal gaze
Authors: Zackon David H. , Sharpe James A. 
Published: January 2005

AbstractUnilateral paramedian involvement of the midbrain tegmentum causes monocular paralysis of adduction in the ipsilateral eye, paresis of contralateral saccades in the opposite eye, and conjugate paresis of ipsilateral smooth pursuit. The adduction paralysis can be nuclear, or internuclear from a lesion in the medial longitudinal fasciculus. This distinctive midbrain syndrome of horizontal gaze paresis is exemplified by means of quantitative infrared oculographic, radiological, and neuropathological correlation in two patients with predominantly paramedian midbrain tumors involving the mesencephalic reticular formation and the oculomotor nucleus. Binocular paralysis of elevation provided evidence that one human oculomotor nucleus contains axons to both superior rectus muscles, as does the simian oculomotor nucleus. The midbrain tectum was spared. These pathophysiological correlations indicate that the mesencephalic reticular formation contains pathways that control contralateral saccades and ipsilateral smooth pursuit.
Unique aspects of human newborn cerebral metabolism evaluated with phosphorus nuclear magnetic resonance spectroscopy
Authors: Younkin Donald P. , Delivoria‐Papadopoulos Maria , Leonard Joe C. , Harihara V. Subramanian , Eleff Scott , Leigh PhD John S. , Chance Britton 
Published: January 2005

AbstractIn vivo phosphorus nuclear magnetic resonance spectroscopy (31P NMR) was used to evaluate the pattern of phosphate compounds in seven newborn babies (mean gestational age, 32 weeks; birth weight, 1,430 gm; age, 37 days) with a history of perinatal asphyxia. Spectra were collected in a 1.9 Tesla superconductive magnet with surface coil techniques. The spectra had characteristic peaks for phosphorylated monoesters (PME), inorganic phosphate (Pi), phosphodiesters (PD), phosphocreatine (PCr), and ATP. In contrast to cortical spectra from mature animals, these newborn infant 31P NMR spectra were dominated by a large PME peak and had small PCr, Pi, and PD peaks. Intracellular pH, as measured from the chemical shift of the Pi peak relative to the PCr peak, was 7.1 ± 0.1 (SD). We studied one infant postmortem, and a large PME peak was present in his spectrum. The presence of PME 3 hours after death strongly suggests that it is not a sugar phosphate. In NMR spectroscopy, compounds are identified by their chemical shift relative to a known standard (PCr); the chemical shift of the PME peak was 6.5 ppm, suggesting that it is a mixture of phosphoryl ethanolamine and phosphoryl choline. The PCr/Pi ratio (1.3 ± 0.7) and the PCr/ATP ratio (0.7 ± 0.4) were lower in these babies than in mature animals (greater than 2 and greater than 1.4, respectively); the PME/PD ratio (1.2 ± 0.6), however, was much greater in the infants (mature animals, less than 0.2). These findings suggest that there are unique aspects of human newborn cerebral metabolites and bioenergetic reserve.
Adoptive transfer of murine relapsing experimental allergic encephalomyelitis
Authors: Lublin Fred D. 
Published: January 2005

AbstractRelapsing experimental allergic encephalomyelitis (EAE), an autoimmune disorder resembling multiple sclerosis, has been produced by inoculating SJL/J mice with spinal cord or myelin basic protein in appropriate adjuvants. To determine whether initially sensitized lymphocytes or the persistence of antigen depots in the animal were responsible for the relapsing episodes of inflammatory demyelination, adoptive transfer studies were undertaken utilizing lymphocytes from relapsing EAE‐immunized donors transferred directly or after in vitro culture. In direct‐transfer studies donor lymphocytes produced clinical and pathological signs of relapsing EAE in 3 of 7 recipients of lymph node lymphocytes and 1 of 5 recipients of splenic lymphocytes. In vitro culture of lymphocytes in myelin basic protein or T cell growth factor prior to transfer increased both the incidence of disease and the number of animals having relapses, and allowed transfer with fewer lymphocytes. Because all animals had delayed onset of disease, this study demonstrates that the ability to develop relapsing inflammatory demyelination is transferable with lymphocytes and does no require the presence of antigen.