bluuebunny/crossref_metadata_2025_split huggingface dataset:
The effect of thiamine deficiency on local cerebral glucose utilization
Authors: Hakim A. M. , Pappius H. M. 
Published: January 2005

AbstractRats maintained on a thiamine‐free diet for two to seven weeks and control animals were studied by the [14C]deoxyglucose technique prior to the development of histological lesions. This technique permits measurement of local cerebral glucose utilization (LCGU) in discrete nuclei and tracts. Levels of thiamine in brain and blood were also determined. In the 41 central nervous system (CNS) structures in which it was measured, cerebral glucose utilization decreased with diminishing concentration of cerebral thiamine. Thus, the primary metabolic consequence of thiamine deficiency is a widespread reduction in cerebral glucose utilization. Furthermore, with decreasing cerebral thiamine concentrations, glucose utilization declined more rapidly in many of the structures which in humans develop histological lesions with prolonged thiamine deficiency than in structures less susceptible to the development of lesions. One determinant of the specific distribution of histological lesions occurring in human thiamine deficiency may be the variable rate at which the CNS structures lose their metabolic activity with continuing thiamine deficiency.
Neurogenic pulmonary edema in unexpected, unexplained death of epileptic patients
Authors: Terrence Christopher F. , Rao Gutti R. , Perper Joshua A. 
Published: January 2005

AbstractEight cases of unexpected, unexplained death in young ambulatory epileptics were examined postmortem with special attention to the heart and lungs. Lung weights uniformly exceeded the expected value, with gross evidence of hemorrhagic pulmonary edema. Microscopic examination revealed moderate to severe pulmonary edema with protein‐rich fluid as well as alveolar hemorrhage. There was no evidence of recent or old myocardial disease.Although death due to a seizure is usually thought to be almost instantaneous, the neurogenic pulmonary edema exemplified by these cases takes time to develop and may be remediable. The high frequency of absent or nontherapeutic anticonvulsant levels at the time of death in these patients may play in a possible centrally mediated adrenergic cause of neurogenic pulmonary edema and ventricular arrhythmia.
Chronic GM1 gangliosidosis presenting as dystonia: I. Clinical and pathological features
Authors: Goldman James E. , Katz David , Rapin Isabelle , Purpura Dominick P. , Suzuki Kinuko 
Published: January 2005

AbstractClinical and pathological studies are reported from investigation of a 27‐year‐old man with GM1 gangliosidosis who experienced a slowly progressive dystonia that began about age 4, primarily affected the face and limbs, and eventually became almost totally incapacitating. There was only mild intellectual deterioration; myoclonus, seizures, and macular cherry‐red spots were never observed. Postmortem examination revealed intraneuronal storage, localized predominantly to the basal ganglia, in which neurons contained round, multilamellated inclusions. Golgi studies revealed meganeurites arising from medium spiny neurons. Other areas of the central nervous system appeared relatively unaffected, although small basilar dilatations were observed in scattered cortical pyramidal neurons and Purkinje cell dendrites showed focal swellings. Vacuolated cells of the reticuloendothelial system were observed, including Kupffer cells and histiocytes in the spleen, marrow, and intestinal tract. Biochemical analysis revealed a generalized β‐galactosidase deficiency with specific accumulation of GM1 ganglioside in the basal ganglia.
Local cerebral glucose utilization in thermally traumatized rat brain
Authors: Pappius H. M. 
Published: January 2005

AbstractLocal cerebal glucose utilization (LCGU) Was studied using the 14C‐deoxyglucose method in rats with focal thermally induced lesions in the left parietal cortex. A depression of LCGU developed with time after production of the lesion, being most prominent throughout the cortical areas of the lesioned hemisphere: 42 ± 2% (SE) of normal 3 days after the lesion, was induced. Corresponding results in other regions were: contralateral cortical areas, 86 ± 2%; ipsilateral and contralateral subcortical structures, 74 ± 2% and 84 ± 1%, respectively. Brainstem structures were not affected. In white matter, bilateral depression LCGU reached its peak 24 hours after production of the lesion—the reduction ipsilateral to the lesion was 61 ± 4% of normal and the contralateral reduction was 64 ± 4%. LCGU returned to normal within 5 days in all affected areas. No corresponding change in local cerebral blood flow were observed. These results suggest a widespread depression in the functional state of the traumatized brain.
Canine inherited ataxia
Authors: Cork Linda C. , Troncoso Juan C. , Price Donald L. 
Published: January 2005

AbstractA previously unrecognized canine disorder consists of familial cortical cerebellar degeneration with many characteristics that are similar to inherited ataxias in humans. The disease occurs in Gordon setters and appears to be inherited as an autosomal recessive trait. The dogs appear normal during the first 6 months of life, but betwen 6 and 12 months of age they develop a broad‐based gait and dysmetria, followed by truncal ataxia. Nystagmus occurs late. The disorder is slowly and insidiously progressive. Neuropathological examination shows atrophy of the cerebellar cortex, particularly the pars in termedia. There is loss of Purkinje and granule cells, more severe in older dogs. Other parts of the neuraxis appear normal. These pathological changes resemble those of the familial human cerebellar cortical atrophies.
Baclofen in the treatment of staff‐man syndrome
Authors: Miller Frank , Korsvik Holly 
Published: January 2005

AbstractBaclofen, 90 mg daily, was given to a patient with stiff‐man syndrome diagnosed by electromyography and clinical criteria. The patient, bedridden for three years, experienced a decrease in rigidity, cramping, stiffness, and spasm and was able to walk on the thirtieth day of treatment.
A comparison of stapedial reflex fatigue with repetitive stimulation and single‐fiber emg in myasthenia gravis
Authors: Kramer Lynn D. , Ruth Roger A. , Johns Michael E. , Sanders Donald B. 
Published: January 2005

AbstractThe pattern of stapedial reflex fatigue in response to pulsed acoustic stimulation was measured and compared to results of repetitive nerve stimulation and single‐fiber electromyography (EMG) in 89 patients with myasthenia gravis. Studies were also made on 22 patients with other neuromuscular disorders and 40 control subjects with no evidence of neuromuscular impairment. Stapedial reflex fatigue exceeded normal control values in 84% of the patients with myasthenia gravis. Repetitive stimulation and single‐fiber EMG measurements were abnormal in 56% and 91% of this same population, respectively. Stapedial reflex abnormalities were most prevalent in patients with mild forms of myasthenia (predominantly ocular or oropharyngeal weakness). Of 22 nonmyasthenic patients with neuromuscular disease tested, 6 had abnormal stapedial reflex fatigue according to our normal values, indicating that this form of testing also detects other diseases of the motor unit. The measurement of stapedial reflex fatigue is painless, is easy to perform, and requires minimal patient cooperation. Due to the relatively high occurrence of abnormal stapedial reflex fatigue in patients with myasthenia gravis, this procedure appears to have considerable potential value in screening and monitoring patients for the presence of defects in neuromuscular transmission.
Prognosis of optic neuritis with special reference to cerebrospinal fluid immunoglobuhs and measles virus antibodes
Authors: Nikoskelainen Eeva , Frey Harry , Salmi Aimo 
Published: January 2005

AbstractForty‐eight patients with optic neuritis (ON), first seen in 1970 to 1973, were neurologically and neuroophthal mologically reexamined after 7 to 10 years. Twenty‐seven patients (56%) had probable MS, and 9 (19%) had possible MS. During the attack of ON, the cerebrospinal fluid (CSF) samples and serum/CSF measles antibody ratios were studied. Twenty patients had increased relative immunoglobulin G (IgG % of total protein) in their CSF; 19 of these had probable or possible MS. However, 17 of 28 patients with a normal relative IgG value had also developed MS. CSF electrophoresis was abnormal in 20 patients with ON; reexamination showed that 19 had probable or possible MS. Sixteen of 27 patients with normal electrophoresis had also developed MS. Serum/CSF measles antibody ratio had decreased in 19 patients; 13 of these had probable MS and 3 had possible MS. Of 29 patients with a normal measles antibody ratio, 14 had probable MS and 6 had possible MS. The conclusion is that examination of the CSF in ON gives valuable prognostic information because increased relative IgG, abnormal electrophoresis, or a decreased measles antibody ratio implies a high risk of developing MS. A normal CSF does not, however, rule out the possibility of dissemination.
Viral antibody activity of oligodonal and polyclonal immunoglobulins synthesized within the central nervous system in multiple sclerosis
Authors: Rostrom Björn , Link Hans , Laurenzi Maria A. , Kam‐Hansen Slavenka , Norrby Erling , Wahren Britta 
Published: January 2005

AbstractThin‐layer polyacrylamide gel isoelectric focusing (PAG IEF), a very high capacity method for separating immunoglobulins (Ig), was performed on cerebrospinal fluid (CSF) and serum. It was followed by antigen immunofixation with measles, mumps, herpes simplex (HSV), and rubella virus antigens and anti‐human Ig autoradiography in order to demonstrate viral antibodies in separated Ig zones. Two of 11 control patients and 21 of 25 patients with multiple sclerosis (MS) displayed one or more zones of viral antibodies in the CSF without any counterpart, or with distinctly fainter zones, in the serum. Such reaction patterns were taken to indicate the possibility of intrathecal antibody synthesis. Antibody synthesis to measles was found in one to five zones in 76% of the patients with MS; antibody zones were found to HSV in 36% of the patients, to mumps in 12%, and to rubella in 12%. In 36% of the patients, two or three different antibody specificities (of which one was always measles) were found simultaneously in individual autoradiogram zones. For all viral antibodies detected in the CSF autoradiograms, their counterparts in oligoclonal or polyclonal IgG zones (or both) were demonstrable by PAG IEF of the corresponding CSF. The majority of patients with MS also had one or more oligoclonal CSF IgG zones without known antibody specificity. Antigen immunofixation and autoradiography are mainly qualitative. It is not known whether the viral antibodies present in oligoclonal or polyclonal IgG zones in MS CSF reflect a polyclonal B cell activation, a disease‐specific immune reaction, or both.
Adverse effects of phenobarbital on morphological and biochemical development of fetal mouse spinal cord neurons in culture
Authors: Bergey G. K. , Swaiman K. F. , Schrier B. K. , Fitzgerald S. , Nelson P. G. 
Published: January 2005

AbstractThe effects of chronic phenobarbital treatment on neuronal development were assessed in cell cultures of fetal rodent spinal cord. The activity of the principal enzyme involved in acetylcholine synthesis, choline acetyltransferase (CAT), and counts of large spinal cord neurons were used as indicators of neuronal development. Phenobarbital (30 to 120 m̈g/ml) added to cultures for two‐ or six‐week periods produced dose‐dependent decreases in both CAT activity and neuron counts. Barbituric acid at doses equimolar to those at which phenobarbital produced these decreases had no effect.
Prolonged intracerebral infection with poliovirus in asymptomatic mice
Authors: Miller James R. 
Published: January 2005

AbstractA heretofore unrecognized prolonged asymptomatic infection has been identified in mice intracerebrally inoculated with Lansing strain type II poliovirus. Virus was detected by infectivity assay and nucleic acid hybridization and was found in brains as long as 77 days after injection. Viral RNA replication occurred, indicating that persisting virus was not an inactive residuum of the infecting dose. Virus was sometimes found by infectivity even when viral RNA could not be demonstrated by hybridization, but detection by nucleic acid hybridization alone never occurred. Among 56 animals–including mice that died of infection, mice with prolonged infection, and survivors from which virus was not recovered–no animal had a serum neutralization titer above 1:2 in a plaque reduction assay Moreover, brain homogenates of 30 survivors without virus did not have neutralization titers above 1:4 in the plaque reduction assay. These findings were the same as in mock‐infected mice.
Temporospatial effects on orthodromic sensory potential propagation during ischemia
Authors: Nielsen Viggo Kamp , Kardel Troels 
Published: January 2005

AbstractSensory potentials recorded at two to four sites along the median nerve during ischemia in normal subjects were analyzed with respect to the effects of conduction distance and duration of ischemia. Latency increased rectilinearly with the squared duration of ischemia in minutes, and the rate of the latency increase along the nerve was a power of the preischemic latency, a measure of conduction distance. This finding indicates a temporospatial slowing of sensory conduction velocity of ischemic nerves. Calculated latencies fitted with measured values within ±0.2 msec in 95% of 385 recordings. The compound potential amplitude decreased exponentially with the concomitant increase in temporal dispersion, which was the major cause for the decrease in amplitude during the first 20 minutes of ischemia. The nerve conduction pattern during ischemia may serve as a model for biochemical axon membrane dysfunction, a possible factor in the pathogenesis of toxic‐metabolic neuropathies.
Altered muscle polyamine levels in human neuromuscular diseases
Authors: Kaminska Anna M. , Stern Lawrence Z. , Russell Diane Haddock 
Published: January 2005

AbstractIntracellular polyamine levels were measured in 17 patients with neuromuscular diseases and 13 normal controls and were correlated with morphological changes in muscle biopsies. Elevated polyamine levels were found in muscles with marked morphological changes, regardless of the nature of the disease process.
Alpha‐pattern coma in a 2‐month‐old child
Authors: Homan Richard W. , Jones Mary Gene 
Published: January 2005

AbstractA 2‐month‐old infant is described in whom the electroencephalographic finding of alpha‐frequency activity was correlated with a clinical state of unresponsiveness. This condition occurred following a period of hypoxia and was similar to alpha‐pattern coma in adults. The finding of alpha‐pattern coma in a young infant suggests that alpha‐frequency activity in comatose states is abnormally generated since alpha‐frequency activity normally is not found in this age group.
Headache
Authors: Lance James W. 
Published: January 2005

AbstractPresent views on the cause and treatment of temporal arteritis, trigeminal neuralgia, pain arising from the neck, benign intracranial hypertension, and other headaches of intracranial origin are summarized. The clinical components of migraine are correlated with recent studies of cerebral blood flow, monoamine changes, and the platelet release reaction. Psychological, physiological, and pharmacological management is based on the holistic concept of migraine as an uninhibited protective reaction. Cluster headache is subdivided into three varieties which respond preferentially to different medication. Tension headache may depend more on vascular mechanisms than excessive muscle contraction, but treatment is still directed at behavioral management and relaxation training with the aid of antidepressant therapy.
Some morphometric aspects of the brain in senile dementia of the alzheimer type
Authors: Terry Robert D. , Peck Arthur , DeTeresa Richard , Schechter Ruben , Horoupian Dikran S. 
Published: January 2005

AbstractImage analysis apparatus was used to count and measure glial and neuronal perikarya in ten size classes in the midfrontal region and superior temporal gyrus of 18 patients with senile dementia of the Alzheimer type for comparison with 12 age‐matched normal specimens. Brain weight was about 8% less in the dementia group. The major differences had to do with larger neurons, which in dementia were reduced by about 40% in the frontal cortex and 46% in the temporal region. The concentration of neuritic plaques did not correlate significantly with brain weight, cortical thickness, or cell counts.
Newborn brain weight in relation to maturity, sex, and race
Authors: Ho Khang‐Cheng , Roessmann Uros , Hause Lawernce , Monroe Gerald 
Published: January 2005

AbstractFresh brain weight, gestational age, body weight, sex, and race were collected from autopsy records of 782 newborns over a 10‐year period. The brain weight of the mature newborn does not differ between males and females of between white and black infants. For the premature, however, brain weight is heavier in white males than in black males and inwhite females than in black females. These differences are similar to those found for adults in our previous studies, and they correspond to differences in gestational age and body weight in the subgroups. The differences in brain weight between races are best explained on the basis of environmental factors.
Olivopontocerebellar atrophy in children: A report of seven cases in two families
Authors: Colan Richard V. , Snead O. Carter , Ceballos Ricardo 
Published: January 2005

AbstractWe present seven cases of progressive ataxia with onset in childhood along with pathological findings in three patients. One patient showed pure cerebellar degeneration and had no visual changes. His brother had classic changes of olivopontocerebellar atrophy with profound amyotropy but no visual changes. A third family member had similar findings with pathological findings intermediate in severity between the first two. The mother and daughter, who are living, are ataxic and have macular degernation. In a second pedigree, all patients affected in three generations were male, but the disease began during adulthood in the first two generations. Myoclonic seizures occureed in the majority of patients.
Neurological manifestations of accidental hypothermia
Authors: Fischbeck Kenneth H. , Simon Roger P. 
Published: January 2005

AbstractIn a series of 97 patients with accidental hypothermia, alcohol abuse and Wernicke's encephalopathy were prominent causes. Pulse, systolic blood pressure, and respiratory rate were all found to decline with decreasing temperature, and there were significant changes (p < 0.01) in level of consciousness, pupillary response, reflexes, and muscle tone. However, even in the lowest temperature range (20° to 27°C, or 68° to 80°F), 6 of 18 patients remained verbally responsive and 10 had intact reflexes. Neither eye movement abnormalities nor extensor plantar responses correlated directly with the degree of hypothermia.
ACTH‐induced cortisol production in multiple sclerosis
Authors: Snyder Bruce D. , Lakatua David J. , Doe Richard P. 
Published: January 2005

AbstractWe measured the total daily urinary cortisol produced by each of 8 patients with multiple sclerosis during intravenous therapy with adrenocorticotropic hormone (ACTH). Results indicated marked daily variation from patient to patient as well as in the same patient, suggesting that ACTH infusions do not result in reliable, consistent, high‐level production of cortisol. We attempted to express the quantity of cortisol produced as an equivalent amount of oral prednisone. This comparison is difficult to make; but, given certain assumptions, the figures suggest that 40 U or 80 U ACTH infusions do not elicit the quantities of steroid generally considered to be indicated for the therapy of major allergic, autoimmune, or neoplastic diseases.
Central somatosensory conduction after head injury
Authors: Hume Ann L. , Cant B. R. 
Published: January 2005

AbstractIn 94 patients with head injuries, conduction between the lower brainstem and the cerebral cortex was studied by recording evoked potentials from the scalp and neck following stimulation of the median nerves. A conduction time 3 SD or more above the normal mean (5.74 ± 0.46 msec) or the absence of an evoked potential over one or both hemispheres was considered abnormal. During successive periods in the first 35 days after injury, the evoked potentials correlated with the outcome (classified as good or not good) in 75 to 84% of patients. Within 3 1/2 days the outcome was correctly predicted in 38 of 49 patients (78%). Six of the 7 surviving patients with persistent asymmetries of the cerebral evoked potentials remained hemiplegic. Serial studies over one year showed that both conduction time and amplitude recovered exponentially over many months, but differences persisted between the patients who made a good recovery and those who remained disabled.
Biophysical and pathological effects of cryogenic nerve lesion
Authors: Myers Robert R. , Powell Henry C. , Heckman Heidi M. , Costello Michael L. , Katz Jordan 
Published: January 2005

AbstractChanges in endoneurial fluid pressure (EFP) and morphology were studied in rat sciatic nerves frozen for 60 seconds with a cryoprobe designed for human cryoanalgesia. The onset of increased EFP was rapid, and a peak of 23 cm H2O was reached within 90 minutes after injury. EFP levels returned to normal 32 days after freezing. The peak value represents the highest EFP yet recorded in an experimental neuropathy. Microscopic examination revealed severe vascular injury as the probable mechanism of edema, with leakage of horseradish peroxidase tracer at the site of injury and diapedesis of polymorphonuclear cells through vessel walls. Wallerian degeneration was also observed in segments of nerve distal to the site of injury. Analysis of EFP data revealed a biphasic pattern of endoneurial edema: initial marked pressure elevation subsides within hours but is followed by a second peak several days later. We interpret this to suggest superposition of two separate pathological processes following cold injury. At first, extensive vascular damage permits plasma and cellular extravasation, which rapidly increases EFP. Subsequently, nerve fibers undergo wallerian degeneration, a process associated with elevated EFP, which is maximal 6 days after injury.
Myotonic dystrophy: Calcium‐dependent phosphatidic acid synthesis in erythrocytes
Authors: Moore R. B. , Appel S. H. , Plishker G. A. 
Published: January 2005

AbstractRecently it was reported that calcium‐dependent phosphatidic acid synthesis in erythrocytes of patients with myotonic muscular dystrophy (MyD) is markedly impaired when compared to that in control subjects. Using 32P‐loaded erythrocytes, we found no significant difference in the levels of 32P‐phosphatidic acid synthesized after exposure to calcium and its ionophore A23187 between patients with MyD and controls. In a batch experiment typical of the experiments with 32P, a twofold increase of phosphatidic acid in both groups was determined by inorganic phosphate measurements. Thus, the specific activity of the 32P‐phosphatidic acid increased four‐ to five‐fold in response to calcium. Analyses of 32P‐polyphosphoinositide breakdown in ghosts and in adenosine triphosphate‐depleted erythrocytes also appeared normal for patients with myotonic muscular dystrophy. Possible discrepancies between the results presented here and those reported previously are discussed.