bluuebunny/crossref_metadata_2025_split huggingface dataset:
Tomographic measurement of local cerebral glucose metabolic rate in humans with (F‐18)2‐fluoro‐2‐deoxy‐D‐glucose: Validation of method
Authors: Phelps M. E. , Huang S. C. , Hoffman E. J. , Selin C. , Sokoloff L. , Kuhl D. E. 
Published: January 2005

AbstractTracer techniques and quantitative autoradiographic and tissue counting models for measuremnt of metabolic rates were combined with positron computed tomography (PCT) and (F‐18)2‐fluoro‐2‐deoxy‐D‐glucose (FDG) for the measurement of local cerebral metabolic rate for glucose (LCMRGlc) in humans. A three‐compartment model, which incorporates hydrolysis of FDG‐6‐PO4 to FDG, was developed for the measure of kinetic constants and calculation of LCMRGlc. Our model is an extension of that developed by Sokoloff et al. Although small, hydrolysis of FDG‐6‐PO4 was found to be significant. A PCT system, the ECAT, was used to determine the rate constants, lumped constant, and stability of the model in human beings. The data indicate that cerebral FDG‐6‐PO4 in humans increases for about 90 minutes, plateaus, and then slowly decreases. After 10 minutes, cerebral blood FDG activity levels were found to be a minor fraction of tissue activity. Precursor pool turnover rate, distribution volumes, and red blood cell‐plasma concentration ratios were determined. Reproducibility (precision) of LCMRGlc measurements (∼2 cm2 regions) was ± 5.5% over a 5‐hour period. The replacement of arterial blood sampling with venous sampling was validated.
Has myelin basic protein received a fair trial in the treatment of multiple sclerosis?
Authors: Alvord Ellsworth C. , Shaw Cheng‐Mei , Hruby Sarka , Kies Marian W. 
Published: January 2005

AbstractAutosensitization to some central nervous system antigen still remains one of the best hypotheses for the continuing pathogenesis of multiple sclerosis (MS). Enough is now known about the cause, pathogenesis, and treatment of experimental allergic encephalomyelitis (EAE) to test this hypotesis. Reports of therapeutic failure of the encephalitogen myelin basic protein (BP) in the treatment of MS have their counterparts in similar therapeutic failures in EAE. Only highly inbred strain 13 guinea pigs respond much less well to simple BP therapy, and monkeys hardly at all. In both strains of monkeys so far studied, a nonspecific adjunctive factor–an antibiotic in Macaca mulatta and a steroid in Macaca fascicularis—is also required. Accordingly, human trials of the therapeutic efficacy of BP in MS should include its administration in large concentrations together with an adjunctive agent.
Myelin basic protein treatment of experimental allergic encephalomyelitis in monkeys
Authors: Alvord Ellsworth C. , Shaw Cheng‐Mei , Hruby Sarka 
Published: January 2005

AbstractTreatment of experimental allergic encephalomyelitis (EAE) in two strains of monkeys with large amounts of myelin basic protein (BP) fails unless an adjunct is also used. In both strains the adjunct by itself is more effective than BP by itself, but in the one strain which could be investigated sufficiently, the combination can be made almost totally effective in reversing EAE. The adjunct varies with the strain of monkey, an antibiotic in Macaca mulatta and a steroid in Macaca fascicularis. Similar adjunctive treatments should be considered in the management of multiple sclerosis, for which EAE remains one of the best studied models.
Degradation of myelin basic protein by cerebrospinal fluid: Preservation of antigenic determinants under physiological conditions
Authors: Alvord Ellsworth C. , Hruby Sarka , Sires Linda R. 
Published: January 2005

AbstractCerebrospinal fluid contains several proteolytic enzymes that can degrade myelin basic protein (BP) under physiological conditions into peptide fragments of various sizes which still contain antigenic determinants capable of binding antibodies to BP. These enzymes are optimally active in either acid (pH 4) or neutral (pH 7 to 8) conditions and can be characterized by the nature of the BP peptide fragments produced. Proteinases resembling cathepsin D, thrombin, plasmin (fibrinolysin), or kallikrein are present in variable amounts in CSF. No relatinship to any particular disease has yet been established.
Treatment of reye syndrome
Authors: Trauner Doris A. 
Published: January 2005

AbstractAlthough the underlying cause of Reye syndrome is not understood, an effective approach to treatment is based on reversing the known metabolic and pathological abnormalities. A multifaceted therapeutic approach aimed at correcting metabolic derangements and combating intracranial hypertension can result in complete recovery from severe cases of Reye syndrome.
Pituitary adenomas: Clinicopathological and immunohistochemical study
Authors: Martinez A. Julio , Lee Arthur , Moossy John , Maroon Joseph C. 
Published: January 2005

AbstractThe results of conventional histological staining, ultrastructural studies, and immunoperoxidase methods were compared in a study of 50 pituitary adenomas and correlated with clinical features. Final diagnosis was based on immunoperoxidase reactions and ultrastructural characteristics. There were 25 prolactin (lactotrophic) cell adenomas, 4 growth hormone (somatotrophic) cell types, 1 mixed growth hormone‐prolactin cell tumor, 3 adrenocorticotropic cell tumors, and 17 undifferentiated cell adenomas. While serum hormone levels, immunoperoxidase reactions, and electron microscopic features generally correlated well with the clinical features, discrepancies emerged in several cases. Among the responsible factors were: (1) the small sample of tumor removed by transsphenoidal hypophysectomy; (2) uncertainty that the tumor was totally removed or that representative samples were furnished to the pathologist; (3) artifacts produced by electrocoagulation and surgical trauma; (4) inclusion in the biopsy of fragments of altered nonneoplastic adenohypophysis; (5) difficulty in distinguishing hyperplasia from adenoma in small fragments; (6) patchy or scattered immunoreactive cells within an adenoma; (7) the presence of a few cells immunoreactive for hormones other than the predominant one within an adenoma (e.g., cells immunoreactive for growth hormone within a prolactin cell adenoma); and (8) paradoxical serum hormone level abnormalities, especially prolactinemia in patients with undifferentiated cell adenomas and no endocrine symptoms. Biological complexity and technical problems associated with surgery for human adenohypophyseal disease result in continued failure to achieve precise diagnostic correlations with pituitary adenomas.
Gilles de la tourette syndrome: Clinical and family study of 50 cases
Authors: Nee Linda E. , Caine Eric D. , Polinsky Ronald J. , Eldridge Roswll , Ebert Michael H. 
Published: January 2005

AbstractFifty patients with Tourette syndrome were evaluated; data included family history, clinical characteristics, response to haloperidol, and side effects during haloperidol therapy. Sixteen patients had a family history of Tourette syndrome, and another 16 had a family history of tics. Twenty‐four families had more than 2 members with Tourette syndrome or tics. There was no preponderance of families with a Jewish, Eastern European background in this sample. Thirty‐four patients had obsessive‐compulsive behavior. Among the 50 patients there was a high frequency of sleep disturbance, learning disability, self‐destructive behavior, inappropriate sexual activity, and antisocial behavior. Family history was significantly related to the occurrence of sleep distrubance, obsessivecompulsive behavior, haloperidol response, and the frequency of side effects caused by haloperidol. The precise mode of genetic transmission in familial Tourette syndrome remains to be determined.
Startle epilepsy complicating down syndrome during adulthood
Authors: Giménez‐Roldán S. , Martín M. 
Published: January 2005

AbstractA woman with Down syndrome—a trisomy 21 karyotype—developed severe startle epilepsy and spontaneous seizures at the age of 20 years. Triggering factors, zeizure patterns, and ictal electroencephalograms were the same as in startle epilepsy occurring in children with cerebral palsy but differed in that there was no clinical or radiological evidence of a focal brain lesion in the vicinity of the motor supplementary cortex. A therapeutic trial with clonazepam substantially reduced the frequency of reflex‐induced and spontaneous seizures.
Mycoplasma antibody in guillain‐barré syndrome and other neurological disorders
Authors: Goldschmidt Barbra , Menonna Joseph , Fortunato Julei , Dowling Peter , Cook Stuart 
Published: January 2005

AbstractCounterimmunoelectrophoresis (CIEP) was used to determine precipitating antibodies to Mycoplasma pneumoniae retrospectively in sera from 100 patients with Guillain‐Barré syndrome (GBS), 125 medical and neurological controls, and 40 normal individuals. Sera from 7 patients produced precipitin lines. These positive cases included 5 patients with GBS, 1 with acute cerebellar ataxia, and 1 with acute disseminated encephalomyelitis. A complement‐fixation test performed with the same antigen showed titers to M. pneumoniae of 1:512 or greater in these 7 sera. In contrast, sera from other patient controls and normal individuals were negative by CIEP and had only low mycoplasma complement‐fixation antibody titers.No distinguishing clinical features separated the 5 seropositive GBS patients from the whole group except for their young age, which parallels that for human mycoplasma infection in general. Additional laboratory findings consistent with acute mycoplasma infection were demonstrated in 6 of the 7 seropositive patients.
Brainstem auditory evoked responses in 200 patients with multiple sclerosis
Authors: Chiappa Keith H. , Harrison Jane L. , Brooks Elizabeth B. , Young Robert R. 
Published: January 2005

AbstractBrainstem auditory evoked responses (BAERs) were recorded from 202 patients with definite, probable, or possible multiple sclerosis (MS). Definitions of abnormality were based only on interwave separations and the wave I/wave V amplitude ratio. Thirty‐two percent of the patients had abnormal BAERs, and the presence of clinically unsuspected lesions was revealed by BAER abnormalities in 7.4%. Thirty‐five percent of the patients who had nystagmus and 53% of those who had internuclear ophthalmoplegia at the time of testing had BAER abnormalities. Forty‐five percent of the abnormalities were elicited with stimulation of one ear only, stressing the importance of monaural stimulation. Click rates faster than 10 per second did not reveal abnormalities undetected at slower rates. BAERs were normal by these criteria in patients with labyrinthine diseases and amyotrophic lateral sclerosis. Thus, the BAER in MS can (1) confirm the presence of central lesions in pateints with suspected brainstem involvement, (2) document the presence of clinically unsuspected lesions, and (3) be followed over time to provide possible assistance in evaluating the effectiveness of therapeutic measures. The BAER is a useful tool in the diagnosis and management of MS.
Recovery following brainstem hemorrhage
Authors: Burns James , Lisak Robert , Schut Luis , Silberberg Donald 
Published: January 2005

AbstractTwo patients made virtually complete recoveries following hemorrhage within the brainstem. Progressive clinical deterioration in each case was managed surgically by evacuation of the hemorrhagic lesion in 1 case and treatment of secondary hydrocephalus in both. Computerized tomographic scanning proved essential for nonsurgical diagnosis and later management.
Reduced seizure control due to spoiled phenytoin capsules
Authors: Cloyd James C. , Gumnit Robert J. , Lesar Timothy S. 
Published: January 2005

AbstractSubtherapeutic phenytoin serum levels and loss of seizure control occurred in a 31‐year‐old man due to decreased bioavailability of oral drug. During storage at extreme temperatures, physical changes of the phenytion capsule resulted in altered dissolution characteristics so that only 50% dissolution occurred at 180 minutes compared with 95% in 120 minutes for control capsules. Similar changes were produced in fresh capsules within seven days at high temperature and humidity. The affected patient has metabolic responses to phenytoin that produce marked fluctuations in serum levels with changes in dose. Altered phenytoin serum concentrations may occur with minor dose changes in such patients despite good compliance.
Radiation‐induced malignant and atypical peripheral nerve sheath tumors
Authors: Foley Kathleen M. , Woodruff James M. , Ellis Frank T. , Posner Jerome B. 
Published: January 2005

AbstractThe reported peripheral nerve complications of therapeutic irradiation in humans include brachial and lumbar plexus fibrosis and cranial and peripheral nerve atrophy. We have encountered 9 patients with malignant (7) and atypical (2) peripheral nerve tumors occurring in an irradiated site suggesting that such tumors represent another delayed effect of radiation treatment on peripheral nerve. The neoplasms appeared 4 to 41 years following radio‐therapy, at a time when the patients (6 females and 3 males) were 16 to 70 years old and cured of their original tumors. Eight patients had received irradiation to treat malignant disease; the ninth had a benign condition.The sites of peripheral nerve tumor were the brachial plexus in 5, cervical plexus in 1, lumbar plexus in 2, and retroperitoneum in 1. Eight patients presented with painful masses, and in 6 of the 8 there was a progressive neurological deficit. Seven patients died with metastases within two years of diagnosis. In all instances the radiotherapy was within an acceptable radiation dosage, yet 3 patients developed local radiation‐induced skin and bony abnormalities. In 3 patients there were either clinical signs or a family history of von Recklinghausen disease, but the malignant peripheral nerve sheath tumors developed only in the radiation port. Animal studies support the clinical observations that malignant peripheral nerve sheath tumors can occur as a delayed effect of irradiation. Other etiological mechanisms, including an increased risk of second primaries in cancer patients and a genetic predisposition to peripheral nerve tumors, may also play a role.
Serum creatine kinase BB and MM concentrations determined by radioimmunoassay in neuromuscular disorders
Authors: Zweig Mark H. , Adornato Bruce , Van Steirteghem Andre C. , Engel W. King 
Published: January 2005

AbstractWe measured the MM and BB isoenzymes of serum creatine kinase (CK) by radioimmunoassay in 89 patients with neuromuscular disorders and 44 definite or possible carriers of Duchenne muscular dystrophy (DMD). The CK‐MM isoenzyme was closely associated with total CK enzymatic activity. CK‐BB was not as closely correlated with total CK but was usually increased in the inflammatory myopathies and DMD and normal in myasthenia gravis, Guillain‐Barré syndrome, and amyotrophic lateral sclerosis as well as in neuropathies in which CK is usually normal. In myopathies, CK‐BB may be useful for assessing the level of disease activity or the regenerative component. More study is necessary to assess the role of CK‐BB in detection of DMD carriers.
Autosomal dominant cerebrovascular amyloidosis: Properties of peripheral blood lymphocytes
Authors: Stefansson Kari , Antei Jack P. , Oger Joel , Burns James , Noronha AvertanoB. C. , Roos Raymond P. , Arnason Barry G. W. , Gudmundsson G. 
Published: January 2005

AbstractSelected Properties of Peripheral blood lymphocytes (PBLs) from five ambulatory affected individuals of a kindred with autosomal dominant cerebrovascular amyloidosis were studied. The percentage of PBLs bearing surface membrane immunoglobulin (SmIg + cells) was increased in the patient group (30 ± 3% versus 20 ± 2%; p < 0.05). The Percentage of PBLs forming early and total E‐rosettes was comparable in patient and control groups. Mitogenic response to concanavalin A (Con A) was suggestively reduced in the patient group, measured both by total 3Hthymidine incorporation and by comparison of stimulation indices. Mitogenic response to phytohemagglutinin and pokeweed was comparable in the two groups. Capping of Con A by PBLs was significantly reduced in the patient group compared with the controls (13 ± 1% versus 26 ± 2%; p < 0.01). The findings of reduced Con A response and increased SmIg+ cells support the hypothesis that immune dysfunction contributes to the development of amyloidosis. The reduced capping suggests altered membrane properties in this autosomal dominant disorder.
Glycerol kinase deficiency with neuromuscular, skeletal, and adrenal abnormalities
Authors: Guggenheim Mary Anne , McCabe Edward R. B. , Roig Manuel , Goodman Stephen I. , Lum Gary M. , Bullen William W. , Ringel Steven P. 
Published: January 2005

AbstractTwo brothers with a recently described inborn error of metabolism characterized by glyceroluria, hyperglycerolemia, and generalized glycerol kinase deficincy had moderate psychomotor reatradation, spasticity, growth failure, a nonspecific myopathy, osteoporosis, and adrenal insufficiency. Glycerol kinase activity in leukocytes and cultured fibroblasts was less thaan 5% of control values. Heptic and renal tissue obtained at autopsy in one patient had similarly low enzyme activity. Thus the deficiency of glycerol kinase in these patients appears to be generalized and heritable, though the relationship of the clinical phenotype to enzymatic deect is not yet established.
A diagnostic index of active demyelination: Myelin basic protein in cerebrospinal fluid
Authors: Cohen Steven R. , Brooks Benjamin R. , Herndon Robert M. , McKhann Guy M. 
Published: January 2005

AbstractWe have developed a radioimmunoassay to measure a specific neurological component, the basic protein of myelin, and have used this test for assessing this component in spinal fluid. The levels of basic protein in spinal fluid correlate closely with the clinical activity of multiple sclerosis; therefore the test can be used for objective evaluation of disease activity in patients with that disorder. Moreover, it is a useful adjunct in the diagnosis of multiple sclerosis and evaluation of potential therapy. In addition, the test is helpful in diagnosing other diseases in which acute breakdown of myelin occurs, especially leukoencephalopathy resulting from irradiation and chemotherapy for treatment of leukemia in children.
Epilepsia partialis continua associated with nonketotic hyperglycemia: Clinical and biochemical profile of 21 patients
Authors: Singh Brij M. , Strobos Robert J. 
Published: January 2005

AbstractIn 21 patients, epilepsia partialis continua (EPC) was an early symptom of nonketotic hyperglycemia and occurred during an initial phase of hyponatremia and mild hyperosmolality. EPC persisted for an average of 8 days, and its duration correlated predominantly with the degree of hyponatremia. Depression of consciousness and cessation of seizures occurred with increasing severity of hyperglycemia and hyperosmolality. In 9 patients, EPC was the first symptom leading to the diagnosis of diabetes mellitus. Four patients died of serious associated illness.The majority of the patients had evidence of a localized structural cerebral lesion. Metabolic disturbances including hyperglycemia, mild hyperosmolality, hyponatremia, and lack of ketoacidosis contribute to the development of EPC in areas of focal cerebral damage.
Methanol poisoning: A clinical and pathological study
Authors: McLean Donald R. , Jacobs Harold , Mielke Bruce W. 
Published: January 2005

AbstractWe report 2 survivors of severe methanol poisoning who developed, apart from blindness, a Parkinson‐like extrapyramidal syndrome characterized by reduced initiative, poor voice volume, masked facies, mild tremor, rigidity, and bradykinesia. Both patients were mildly demented and 1 had hyperreflexia and bilateral Babinski responses.Computed tomographic scans in both patients demonstrated bilateral symmetrical infarction of the frontocentral white matter and putamen. Electromyography in 1 patient showed extensive denervation, mainly involving the legs, but normal motor conduction velocities. L‐Dopa administered to the more severely affected patient had no effect on the parkinsonian features.Autopsy revealed cystic resorption of the putamen and the frontocentral subcortical white matter in addition to widespread neuronal damage throughout the cerebrum, cerebellum, brainstem, and spinal cord.
Distal neuropathy in experimental diabetes mellitus
Authors: Brown Mark J. , Sumner Austin J. , Greene Douglas A. , Diamond Susan M. , Asbury Arthur K. 
Published: January 2005

AbstractAlthough nerve conduction slowing is a well‐accepted abnormality in rats with acute experimental diabetes, reports of neuropathological changes in diabetic rat nerves have been inconsistent. To examine this further, we studied electrophysiological and morphological features of posterior tibial nerves and their distal branches from four‐week streptozotocin‐induced diabetic rats and matched controls.Diabetic rat posterior tibial motor conduction was slowed (mean ± 1 SD, 34.8 ± 3.1 m/sec; controls, 41.2 ± 2.5 m/sec), and evoked muscle response amplitudes were only half of control values. Using quantitative techniques, we documented a diminution in number of the largest myelinated fibers in otherwise normal mid posterior tibial nerves, with an increase in the smaller sizes, indicating either a degree of axonal atrophy or impaired fiber growth during development. The principal pathological finding was active breakdown of myelinated fibers in the most distal motor twigs of hind foot muscles supplied by posterior tibial branches, with preservation of fibers in more proximal segments of these nerves. This anatomical lesion in diabetic nerves could account for both observed conduction slowing and lowered muscle response amplitudes. A consistent feature in both diabetic and control mid lateral plantar nerves was a zone of demyelination that apparently occurs at this natural site of nerve entrapment in rats. Taken together, the pathological abnormalities of peripheral nerve in acute experimental diabetes are best explained as resulting from a distal axonopathy.
Brainstem tumor presenting with unilateral astereognosis
Authors: Feinsod Moshe , Bentin Shlomo , Moscovitch Morris , Wald Uri 
Published: January 2005

AbstractA patient with a brainstem tumor presented with astereognosis. The results of CT scan, evoked response studies, and neuropsychological tests all were consistent with a noncortical origin for the sensory defect.
Congenital stiff‐man syndrome
Authors: Sander James E. , Layzer Robert B. , Goldsobel Alan B. 
Published: January 2005

AbstractA second large family with a dominantly inherited benign disorder resembling the stiff‐man syndrome is reported. The infants are markedly hypertonic at birth, but their tone becomes almost normal by 3 years of age. Stiffness reappears at adolescence, often precipitated by sudden movement or cold, but remains mild when compared with the sporadic disorder.
Neonatal intracranial abscess: Two cases caused by citrobacter and a literature review
Authors: Curless Richard G. 
Published: January 2005

AbstractIntracranial abscesses are uncommon in the newborn. Observation of 2 infants and a review of the literature reveal that the offending organism was a Citrobacter in 12 of the 16 documented cases. In the Neonatal Meningitis Cooperative Study this organism was responsible for meningitis in only 4%. However, an analysis of all published cases of neonatal Citrobacter meningitis reveals a 42% incidence of intracranial abscess. Accordingly, early identification by computerized tomography is important in facilitating prompt surgical therapy.