bluuebunny/crossref_metadata_2025_split huggingface dataset:
Benign myoclonus of early infancy
Authors: Lombroso C. T. , Fejerman Natalio 
Published: January 2005

AbstractSixteen infants, aged 3 to 81/2 months, were studied close to the onset of a myoclonic syndrome akin to that normally called infantile spasms with hypsarrhythmia. None were found to have the clinical and laboratory characteristics common to the syndrome, however: none showed regression or arrest in their neurological/developmental status, nor did their EEG ever develop a hypsarrhythmic or other abnormal pattern. All were followed for a minimum of 18 months to a maximum of nine years. In all patients the myoclonus stopped early, in none persisting after the age of 2 years, and no other seizure phenomena have so far evolved in any of these infants. This favorable outcome occurred in those who were treated as well as those who were not. The self‐limited course of this condition is emphasized within the context of the more unusual myoclonic syndromes of early and late infancy.
The effect of isoprinosine in subacute sclerosing panencephalitis (SSPE)
Authors: Streletz L. J. , Cracco J. 
Published: January 2005

AbstractAlthough no therapeutic agent has proved to be of benefit in subacute sclerosing panencephalitis (SSPE), the use of Isoprinosine has been associated with clinical improvement in several preliminary reports. Two additional patients with SSPE are presented who had repeated serological and electroencephalographic studies during therapy with Isoprinosine. Their clinical courses are discussed in view of the laboratory findings and the natural history of the disease.
Niemann‐pick disease type D: Lipid analyses and studies on sphingomyelinases
Authors: Rao Barbara Gillis , Spence Matthew W. 
Published: January 2005

AbstractLipids and sphingomyelinase activity were studied in spleen, liver, and brain tissues of a 13‐year‐old boy with Niemann‐Pick disease type D (NPD‐D). The greatest lipid changes occurred in spleen; cholesterol, cholesterol esters, total phospholipids, sphingomyelin, and bis‐(monoacylglyceryl)phosphate were increased above normal range. In liver, striking increases were observed in cholesterol and bis‐(monoacylglyceryl)phosphate. Minor changes in neutral and acidic glycolipid patterns occurred in liver, spleen, and brain. Sphingomyelinase activity (optimal at pH 5.0) was elevated above mean control levels in liver and spleen, but not in brain, kidney, or leukocytes. Enzyme properties were generally normal. Activity of NPD‐D liver crude homogenate, but not that of normal liver homogenates, was inhibited at high protein concentrations. Activity levels of a second sphingomyelinase, optimal at pH 7.4, in NPD‐D brain were apparently normal. These findings are generally consistent with the classification of NPD‐D as a sphingomyelin lipidosis.
Leigh's disease in an adult with evidence of “inhibitor factor” in family members
Authors: Whetsell William O. , Plaitakis Andreas 
Published: January 2005

AbstractA 21‐year‐old man with a longstanding history of impaired visual acuity, strabismus, broad‐based gait, and below‐average intellectual capacity developed respiratory difficulties and intermittent generalized weakness at age 19. He subsequently showed signs of massive brainstem dysfunction and died. Postmortem examination demonstrated changes compatible with subacute necrotizing encephalomyelopathy of Leigh involving portions of the diencephalon, midbrain, pons, and medulla as well as portions of the spinal cord and optic nerves. Evaluation of family members for presence of the urinary inhibitor factor for thiamine diphosphate phosphoryl transferase revealed abnormal levels in a brother, a maternal uncle, and the maternal grandfather of the patient.
Peripheral nerve ischemia: Part 2. Accumulation of organelles
Authors: Korthals Jan K. , Korthals Maria A. , Wisniewski Henryk M. 
Published: January 2005

AbstractFollowing simultaneous ligation of the aorta and femoral artery in the cat, organelles accumulated in distal portions of the sciatic nerve and in lower portions of the tibial and personeal nerves. The accumulations were located in the proximal and distal ends of the nerve infarction and delineated the borders of the necrotic area.Topographical analysis of the necrosis and organelle accumulations following ligation of these main arteries showed the larger nerve fascicles to be more sensitive to ischemia than the smaller ones. The center of a nerve fascicle was more sensitive than the periphery. The organelles accumulated in ischemic nerves during the first hours after arterial ligation, presumably as a result of lack of energy for fast axoplasmic transport. Later accumulations reflected mechanical block of axoplasmic transport due to focal necrosis of the nerve.The content of organelle accumulations was similar to that found in lesions resulting from transection except for the presence of 60 to 70 A filaments at the distal end of the infarction.
Carnitine deficiency: Acute postpartum crisis
Authors: Angelini Corrado , Govoni Edmondo , Bragaglia Mercedes M. , Vergani Ludovica 
Published: January 2005

AbstractA 16‐year‐old girl, previously in good health, developed progressive generalized muscle weakness following her first parturition. The neck and proximal limb muscles were especially weak and painful. Carnitine (4‐trimethylamino‐3‐hydroxybutyrate) was markedly decreased in muscle, plasma, and urine. Dietary carnitine supplementation, 2.0 gm daily, was followed by clinical improvement and decreased lipid droplets in muscle biopsy. Together with previously reported cases, 2 fatal, the patient's illness emphasizes the risk of pregnancy in women with carnitine deficiency.
Retroperitoneal neuroblastoma widely metastatic to the central nervous system
Authors: Dresler Steven , Harvey Danny G. , Levisohn Paul M. 
Published: January 2005

AbstractA girl with congenital extraadrenal neuroblastoma died at 3 months of age with multiple parenchymal metastases in the brain and spinal cord. The bones of the skull, cranial dura, venous sinuses, and orbits were not involved. Autopsy findings suggest that the tumor penetrated the spinal meninges and disseminated through the cerebrospinal fluid. It is possible that hematogenous metastasis occurred as well. The intracerebral metastases were not identified on a CT scan performed several hours before the child's death.
Enzyme treatment of spinal cord transected rats
Authors: Feringa Earl R. , Kowalski Timothy F. , Vahlsing H. Lee , Frye Roy A. 
Published: January 2005

AbstractRussian investigators have recently reported clinical recovery of enzyme treated, spinal cord transected rats. Using the exact protocols of Matinian and Andreasian's two most successful treatment regimens, we repeated their experiments using United States produced pure hyaluronidase and trypsin or trypsin and elastase. Animals were evaluated for return of bladder function, clinical evidence of hind limb motor function, cortical evoked response after sciatic nerve stimulation, and axonal transport of cortically injected tritiated proline by regenerated corticospinal axons.None of the treated animals differed from control animals treated only with the enzyme vehicle. No animals walked, had return of voluntary motor activity, showed cortical evoked response, or had evidence for transport of tritiated proline over regenerated corticospinal axons.
Nerve lipid abnormalities in human diabetic neuropathy: A correlative study
Authors: Brown Mark J. , Iwamori Masao , Kishimoto Yasuo , Rapoport Basil , Moser Hugo W. , Asbury Arthur K. 
Published: January 2005

AbstractTo study endoneurial lipid composition in human diabetic neuropathy, we biopsied sural nerves from 3 middleaged men with adult‐onset diabetes mellitus. Magnitude of electrophysiological abnormalities and myelinated fiber loss paralleled the clinical severity of neuropathy in all cases. Cholesterol ester concentration was elevated to about 800% of normal in diabetic nerves. Reduction in total endoneurial lipid concentration correlated best with decrease in myelin volume as calculated from measured fiber diameters. Cholesterol, cerebroside, and most phospholipids were reduced in keeping with the severity of fiber loss in each nerve. The phosphatidylinositol‐phosphatidylserine fraction was most reduced in the least affected nerves. Cerebroside nonhydroxy fatty acids in diabetic nerves were of shorter chain length and more saturated than normal. It is not yet clear whether the abnormalities of phosphatidylinositol‐phosphatidylserine and cerebroside fatty acids are of pathogenetic importance or whether these changes may be the nonspecific consequence of axonal degeneration.
Transfer factor is ineffective in amyotrophic lateral sclerosis
Authors: Olarte Marcelo R. , Gersten Joanne C. , Zabriskie John , Rowland Lewis P. 
Published: January 2005

AbstractSixty‐six patients with amyotrophic lateral sclerosis (ALS) participated in a double‐blind, placebo‐controlled study of transfer factor. A structured rating score of neurological function was recorded after each monthly administration of transfer factor or placebo. Statistical analysis of mean scores for all patients and for those with specific clinical abnormalities showed no significant difference between the two groups. There was no evidence of therapeutic value for transfer factor. During the observation period of nearly one year, more than two‐thirds of the deterioration occurred in the first six months. Meaningful clinical trials of other potential therapeutic agents could probably be conducted within six months in double‐blind crossover trials.
Hypernatremic hemorrhagic encephalopathy
Authors: Young Richard S. K. , Truax Bradley T. 
Published: January 2005

AbstractA 12‐year‐old juvenile diabetic was inadvertently given 500 ml of hypertonic saline intravenously. He developed hypernatremia, hyperosmolality, metabolic acidosis, and hyperglycemia. Seizures and stupor ensued, followed by coma and death. Computerized cranial tomography revealed numerous small subcortical hemorrhages that were verified postmortem.
Neonatal myasthenia gravis in the infant of a myasthenic mother in remission
Authors: Elias Stanton B. , Butler Ian , Appel Stanley H. 
Published: January 2005

AbstractA 26‐year‐old woman was in spontaneous clinical remission from myasthenia gravis (MG) for six months, yet gave birth to a full‐term infant with typical neonatal MG. It is believed that transplacental transfer of anti‐acetylcholine (ACh) receptor antibodies is responsible for neonatal MG; therefore, neonatal MG represents an in vivo assay of the pathogenic potential of anti‐ACh receptor antibodies in 2 human individuals. Anti‐ACh receptor antibodies were present in both mother and infant (titers 12.3 × 10−9 and 4.4 × 10−9 moles per liter, respectively) at the time of birth, and both mother's and infant's sera accelerated the degradation of ACh receptors in myotube cultures. This case suggests that “host factors” unique to the individual appear to modify or even determine whether the presence of anti‐ACh receptor antibodies will result in clinical myasthenia.
Cerebral embolism from septal fibromuscular dysplasia of the common carotid artery
Authors: So Elson L. , Toole James F. , Moody Dixon M. , Challa Venkata R. 
Published: January 2005

AbstractA rare type of fibromuscular dysplasia of the common carotid artery, in the form of a septum, was observed in a patient suffering from recurrent cerebral infarctions. Angiography demonstrated growth fo the lesion and documented the presence of an intraluminal thrombus attached to it. Comparison with reported cases of the more common “string of beads” lesion of fibromuscular dysplasia suggests that the risk for development of ischemic neurological problems is higher for this type of fibromuscular lesion. Surgical correction is recommended, especially for symptomatic patients.
Cellular immune response to measles, mumps, and vaccinia viruses in multiple sclerosis
Authors: McFarland Henry F. , McFarlin Dale E. 
Published: January 2005

AbstractThe cell‐mediated immune response to measles, mumps, and vaccinia viruses was studied in MS patients, normal controls, and neurological disease controls using a lymphocyte proliferation assay. A small but significant difference was found in the response to measles between the MS and normal control groups but not between the MS and neurological disease control groups. In each of the groups, the response of both peripheral blood leukocytes and purified T‐cells to measles was significantly less than the response to mumps or vaccinia. The lower response to measles was not due to the presence of blocking factors or to lack of antigenicity of the measles virus preparation used in the assay. These findings suggest that differences exist in the normal immune response between these viruses. Precise quantitation of the immunological response to viruses in MS and other disease states will depend on identification of the various functionally reactive cell populations.
Eating epilepsy associated with a deep forebrain glioma
Authors: Robertson W. C. , Fariello R. G. 
Published: January 2005

AbstractSeizures associated with deglutition are rare, and previously reported cases of so‐called eating epilepsy have not identified specific electrophysiological or anatomical foci. We evaluated a 14‐year‐old with a deep hemispheric astrocytoma in whom focal sezures were consistently triggered by eating. Electroencephalograms were consistent with a deeply located seizure focus. Eating epilepsy should be grouped with reflex epilepsies that may originate with the diencephalon.
Huntington's disease: Abnormality of lymphocyte capping
Authors: Noronha Avertano B. C. , Roos Raymond P. , Antel Jack P. , Arnason Barry G. W. 
Published: January 2005

AbstractConcanavalin A‐induced lymphocyte capping was studied in 30 patients with Huntington's disease (HD) and 39 controls. A significant decrease in lymphocyte capping was seen in the 24 patients with HD less than 60 years of age compared to 25 age‐matched controls (p < 0.01); above age 60, differences between patients with HD and controls were not significant. These findings lend support to previous observations that generalized membrane abnormalities are present in patients with HD. Studies of HD lymphocyte capping may permit a detailed analysis of membrane function in cells that are readily obtained from living patients.
Subarachnoid spread of ependymoma: Diagnosis and evaluation of therapy by computerized tomography
Authors: Geehr Robert B. , Dohrmann George J. , Dohrmann George J. , Dohrmann George J. , Kier E. Leon , Wagner Franklin C. , Kapp Daniel S. 
Published: January 2005

AbstractThe potential of ependymomas to spread throughout the intracranial subarachnoid space is well documented, though the incidence is disputed. This relates in part to the time required for such metastases to produce symptoms. The diagnosis of such spread, however, is important for therapeutic management. This report demonstrates the effectiveness of computerized tomography in diagnosing spread of ependy moma throughout the basal cisterns and cerebral fissures.
Barognosis in a patient with hemiaraxia
Authors: Angel Ronald W. 
Published: January 2005

AbstractBarognosis was tested in a patient who had a lesion of the right cerebellar hemisphere. While blindfolded, he used each hand to estimate the mass of given test loads, alternating hands between trials. he moved each load sinusoidally, comparing it with the previous one. The period of oscillation was found to be a linear function of the mass. With eah load, the period of oscillation was longer when he used the ataxic hand. On 23 of 59 comparisons between loads, the patient reported incorrectly. Ninety‐one percent of the errors were in the direction of loads feeling heavier when the ataxic hand was used.To explain these results, it is propsed that (1) the perceived magnitude of a load depends on the corollary discharge, and (2) cerebellar damage may reduce the accuracy of the corollary discharge.
Medullablastoma presenting as neck tumors in 2 infants
Authors: Gordon McComb J. , Davis Richard L. , Isaacs Hart , Landing Benjamin H. 
Published: January 2005

AbstractExtension of neuroepithelial neoplasms outside the neuraxis, though uncommon, is well known and usually follows surgical intervention. More unusual is the brain tumor that appears beyond the bounds of the central nervous system prior to operative manipulation. Two infants presented with soft tissue masses in the neck that proved to be medulloblastomas. Autopsy examination in both infants showed primary medulloblastoma of the cerebellar vermis with extensive spread through the subarachnoid spaces. The neck masses may have been direct extension from the central nervous system tumors, though this was not proved in either case.
The effect of alpha‐adrenergic innervation on caudate blood flow
Authors: Levy Lewis , Wicke J. D. 
Published: January 2005

AbstractA thermal diffusion probe, with cannulas for intracerebral microinfusion of drugs and an electrode to monitor electroencephalographic (EEG) activity, was used to examine the local effect of vasoactive amines in a 4 to 5 mm sphere of caudate nucleus in cats. The results demonstrated that it is possible to alter local cerebral blood flow (CBF) without causing any change in systemic blood pressure or heart rate, or in CBF and the EEG in the opposite caudate. One‐microliter intracerebral injections, containing varying amounts of phenylephrine, increased local CBF in proportion to dose. The effect was blocked by intracerebral infusion of phentolamine. However, local alpha‐adrenergic blockade did not inhibit vascular responses to blood pressure elevation or to metabolic influences on local blood flow.
Comparison of antigenic sources for acetylcholine receptor antibody assays in myasthenia gravis
Authors: McAdams Mildred W. , Roses Allen D. 
Published: January 2005

AbstractAntibody titers to the acetylcholine receptor (AChR) from patients with myasthenia gravis, in identical serum samples, were directly compared using denervated rat, human, and baboon muscle as the source of AChR antigen for radioimmunoassay (RIA). Calculations were standardized by using binding isotherms for each antigen source and calculating the percentage of AChR sites labeled with [125I]α‐bungarotoxin at the concentration used in the RIA. In patients with high AChR antibody titers, the antibody concentration when human muscle was used as the antigen source measured up to tenfold higher than that with denervated rat muscle. In patients who had low antibody titers with human muscle antigen, assays using rat denervated muscle AChR frequently failed to demonstrate diagnostically abnormal titers. The data explain differences among several reported series in the percentages of patients with myasthenia gravis who had elevated serum antibody concentrations. AChR antibody concentrations with baboon muscle as the antigen source were comparable to those in which human muscle was used.
Failure of intravenous and intrathecal cytarabine to modify central nervous system IgG synthesis in multiple sclerosis
Authors: Tourtellotte Wallace W. , Potvin Alfred R. , Mendez Mario , Baumhefner Robert W. , Potvin Janet H. , Ma Booe I. , Syndulko Karl 
Published: January 2005

AbstractTo study the nature of the multiple sclerosis (MS) central nervous system (CNS) immune reaction, cytarabine (ara‐C) was administered intravenously to three patients and intrathecally to another seven. Although intravenous administration severely suppressed circulating leukocytes derived from the bone marrow, there was no change in de novo CNS IgG synthesis (rate or presence of CSF IgG oligoclones). Therefore, the MS CNS immune reaction can persist in the presence of severe granulocytopenia and severe monocytopenia of one to two weeks' duration. In four patients who were given ara‐C intrathecally, cytotoxic levels (>10 μg/ml) were present for at least 24 hours. A brief elevation in CSF albumin indicated transient damage to the blood‐brain barrier. Leukopenia did not result in the seven patients treated intrathecally, and de novo CNS IgG synthesis did not change or was transiently increased. Assuming that cytotoxic levels in CSF diffused to the CNS sites responsible for synthesizing IgG, results indicate that CNS IgG synthesis does not depend on in situ rapid turnover of immune cells. None of the ten patients demonstrated neurological improvement or persistent adverse effects.
Cerebrospinal fluid biochemical markers of central nervous system metastases
Authors: Clifford Schold S. , Wasserstrom William R. , Fleisher Martin , Schwartz Morton K. , Posner Jerome B. 
Published: January 2005

Abstractβ‐Glucuronidase and carcinoembryonic antigen (CEA) were measured in the cerebrospinal fluid of patients with cancer. Both substances were found to reliably detect the presence of leptomeningeal infiltration by carcinoma. Neither substance was reliable in the detection of leptomeningeal infiltration by lymphoma or of metastases to the brain parenchyma or spinal epidural space. β‐Glucuronidase was moderately elevated in chronic infectious meningitis, whereas CEA was not. Both markers approached control levels with favorable treatment of the leptomeningeal metastases, reflecting the effectiveness of treatment. Both β‐glucuronidase and CEA hold promise as indicators of early metastatic involvement of the leptomeninges by carcinoma.
Intensive evaluation of referred unclassified neuropathies yields improved diagnosis
Authors: Dyck Peter James , Oviatt Karen F. , Lambert Edward H. 
Published: January 2005

AbstractIntensive evaluation of 205 cases of undiagnosed neuropathy in a center with special approaches and facilities permittede classification of 76% of the patients. Inherited disorders accounted for 42% of the series, 21% of the patients were shown to have inflammatory‐demyelinating polyradiculoneuropathy, and 13% had neuropathies assocciated with other disorders. A considerable improvement in diagnosis was possible from evaluation of the kin of the patients with undiagnosed neuropathy. Analysis of the frequency and type of various sensory symptoms alos was helpful in distinguishing between acquired and inherited neuropathies.
Familial hemiplegic migraine: EEG and CT scan study of two cases
Authors: Gastaut J. L. , Yermenos E. , Bonnefoy M. , Cros D. 
Published: January 2005

AbstractThe cases of a woman and her son with a long history (24 and 9 years, respectively) of hemiplegic migraine are presented. The mother demonstrated exclusively right‐sided paralysis; in the son, paralysis occurred on each side. Electroencephalograms recorded during the attacks were characterized by pseudoperiodic slow sharp waves over the hemisphere contralateral to the hemiplegia. Between attacks the neurological examination and EEG were normal. CT scans performed during and between attacks showed no abnormalities.