bluuebunny/crossref_metadata_2025_split huggingface dataset:
Neurological complications of infantile osteopetrosis
Authors: Lehman Ralph A. W. , Reeves Jerry D. , Wilson W. Bruce , Wesenberg Richard L. 
Published: December 2004

AbstractSeven cases of infantile osteopetrosis are presented. Five of these were available for detailed clinical examination and 2 for retrospective review, including autopsy slides. Neurological deficits in these patients are reviewed. Involvement of the central nervous system parenchyma was suggested by observations of delayed development, ocular abnormalities, and reflex changes as well as radiographic and autopsy findings. Cerebral atrophy was present in several of our patients as well as some reported in the literature and may account for the ventricular enlargement found in many of these patients. Though hydrocephalus may be present, it is unclear that this is frequent or that it can occur without antecedent intracranial hemorrhage. The large head size is not accounted for by calvarial thickening or by hydrocephalus. Despite our patients' small stature, pituitary function appeared to be normal. Surgical decompression may stabilize cranial nerve function, particularly when the optic nerves are involved.
“Disappearing” spinal cord compression: Oncolytic effect of glucocorticoids (and other chemotherapeutic agents) on epidural metastases
Authors: Posner Jerome B. , Howieson John , Cvitkovic Esteban 
Published: December 2004

AbstractFour patients suffering spinal cord compression resulting from epidural metastases were treated with adrenocorticosteroid hormones. For 2 patients, prompt relief of symptoms followed glucocorticoid therapy alone and was associated with marked shrinkage or disappearance of the metastasis, a direct oncolytic effect of the steroids. For the other 2 patients, glucocorticoids combined with other chemotherapeutic agents caused disappearance of the extradural tumor. For 1 patient, failure to recognize the oncolytic effects of the chemotherapy led to an unnecessary surgical procedure, and for a second patient an unnecessary operation was narrowly averted. Thus, for certain patients, glucocorticoids may occasionally have a marked oncolytic effect on epidural metastatic tumors.
X‐Linked muscular dystrophy
Authors: Furukawa Tetsuo , Peter James B. 
Published: December 2004

AbstractX‐linked muscular dystrophy has been separated into two types that are generally considered to be distinct entities. We have investigated three families with X‐linked muscular dystrophy who demonstrate remarkable intrafamilial variability. In one family 2 brothers with a benign type had a maternal uncle who was affected with the Duchenne type. In another family, 4 members had the benign type but a fifth was much more severely affected than in the classic Duchenne dystrophy; therefore the presence of an “aggressive form” is proposed. A third family also had both benign and severe types. A search of the literature revealed families in which the severe and benign types coexisted.The genetic determinants of this heterogeneity are not yet known. Clinical similarities between bening and severe types of X‐linked muscular dystrophy and the presence of families with both types suggest that the two are intimately related.
“Release hallucinations” as the major symptom of posterior cerebral artery occlusion: A report of 2 cases
Authors: Brust John C. M. , Behrens Myles M. 
Published: December 2004

AbstractVisual hallucinations were the presenting symptoms in 2 patients with probable infarcts in the territory of a posterior cerebral artery. They occurred in areas of paracentral scotomas, right in Patient 1 and left in Patient 2. In Patient 1 they were formed, prolonged, and not apparently related to past experience. In Patient 2 they were at first paroxysmal and unformed, with more prolonged metamorphopsia; later there appeared to be palinoptic formed images, possibly postictal in nature. Such hallucinations appear to be of a “release” type, and may be more common than is generally appreciated in patients with posterior cerebral artery occlusion.
Congenital arachnoid cyst with unusual clinical, radiological, and pathological findings
Authors: Rosich‐Pla Antonio , Smith Bernard H. , Sil Ranajit 
Published: December 2004

AbstractChoroid plexus–like structures were found in the wall of a congenital frontotemporal arachnoid cyst in a 14‐year‐old girl who presented with temporal lobe seizures since the age of 6. The temporal cortex beneath the cyst was maldeveloped. The clinical, radiological, and pathological aspects of congenital arachnoid cysts are reviewed.
The influence of route of delivery and hyaline membranes on the risk of neonatal intracranial hemorrhages
Authors: Leviton Alan , Gilles Floyd , Strassfeld Ruth 
Published: December 2004

AbstractThe relationship of route of delivery and hyaline membranes to eight sites of intracranial hemorrhage was evaluated in 513 autopsied newborn infants. Those delivered by cesarian section appeared to be at less risk for most hemorrhages compared with newborns delivered vaginally. However, the reductions in risk were small and did not achieve nominal levels of statistical significance. Thus, “birth trauma” probably contributes little to the risk of most neonatal intracranial hemorrhages. Newborns who developed hyaline membrances appeared to be at greater risk for most hemorrhages than those who did not, particularly if the infant had been delivered by cesarian section. This was especially marked for subarachnoid and germinal matrix hemorrhage.
Idiopathic regressing arteriopathy
Authors: Mokri Bahram , Houser O. Wayne , Sundt Thoralf M. 
Published: December 2004

AbstractThree patients had nonatheromatous occlusive cervicocephalic arterial disease which on follow‐up showed resolution of the occlusive or stenosing abnormalities. The patients' relatively young age (middle thirties to middle forties) and severe unilateral headache or focal head, neck, or mastoid pain were prominent common clinical features. The dominant arteriographic features were intimal irregularities, multiple vessel involvement, tendency to arterial dissection and aneurysm formation, sparing of intracranial arteries, and tendency to partial or complete resolution of the abnormalities and regression toward normal. We have called this presumed entity “idiopathic regressing arteriopathy” pending appropriate histopathological studies.
Lower motor neuron disease with spinocerebellar degeneration
Authors: Page Robert W. , Moskowitz Roland W. , Nash Robert E. , Roessmann Uros 
Published: December 2004

AbstractA patient with polymyositis responded initially to steroid therapy. A muscle biopsy disclosed features of primary myopathy and group atrophy. The patient became refractory to therapy and died with relentlessly progressive weakness. The autopsy disclosed lower motor neuron involvement and degeneration of the spinocerebellar tracts. There was loss of Purkinje cells, which may have occurred secondary to an anoxic episode prior to death. The case is unique because of the limited involvement of the lower motor and spinocerebellar systems.
Supraclavicular and carotid bruits in hemodialysis patients
Authors: Messert Bernard , Marra Thomas R. , Zerofsky Ronald A. 
Published: December 2004

AbstractAuscultation of the neck vessels in patients receiving chronic hemodialysis reveals a high incidence of subclavian and carotid bruits as well as the expected precordial hemic murmurs. These bruits are associated with the high‐output hyperdynamic circulatory state. In older patients the finding could be misinterpreted as evidence of extracranial cerebrovascular disease, but clinical considerations should obviate unnecessary neuroradiological diagnostic procedures.
Temporal lobe oligodendroglioma developing from infancy into adulthood
Authors: Pelc Sabine , Brihaye Jean , Périer Olivier , Heimann Rodolphe , Balériaux Danièle 
Published: December 2004

AbstractClinical, radiological, surgical, and histological features are reported for a patient with a temporal lobe oligodendroglioma that had developed in infancy and was diagnosed and partially resected at age 10. The patient is presently 24 years old and still well. To the best of our knowledge this is the first report of an oligodendroglioma with probable onset at 5 months of life and satisfactory course over 24 years.
The pathophysiology of focal epilepsy: Neurophysiological considerations
Authors: Pedley Timothy A. 
Published: December 2004

AbstractThe study of epilepsy serves to emphasize the importance of integrating basic research with clinical data. In this selective review, the results of recent experiments using intracellular recording techniques, ion‐specific microelectrodes, and a methodology suitable for studying mammalian cortex in vitro are discussed in terms of cellular phenomena which underlie clinical observations made in epileptic patients. This information has begun to clarify: (1) the changes in neuronal behavior associated with interictal and ictal events; (2) alterations in local ionic microenvironment which occur during focal epileptogenesis; (3) intrinsic control mechanisms which serve to restrict seizure spread; (4) neuronal characteristics which account for the differences in seizure patterns seen in infants and adults; and (5) the possible long‐term consequences of recurrent local neuronal hyperexcitability.
Valproic acid in epilepsy: Clinical and pharmacological effects
Authors: Mattson Richard H. , Cramer Joyce A. , Williamson Peter D. , Novelly Robert A. 
Published: December 2004

AbstractThe antiepileptic drug valproic acid was studied in an open clinical trial as adjunct medication for 23 patients with uncontrolled seizures of a generalized or partial type. Two‐thirds of the patients experienced reduction in seizure frequency ranging from 25 to 100%. Extensive testing revealed no evidence of serious systemic toxicity due to the drug. Minor side effects (e.g., nausea, vomiting, or sedation) were usually transient.Sodium valproate syrup and valproic acid in capsules gave equivalent mean low (23.3 μg/ml) and maximum (42.5 μg/ml) serum concentrations. The drug had a relatively short half‐life of 8.7 hours, necessitating administration in divided daily doses.During initiation of valproate therapy there was evidence of a decline in total serum phenytoin concentration (16.5 to 10.2 μg/ml; p < 0.001) while the percentage of free phenytoin increased (10.9 to 20%). The quantity of unbound phenytoin was relatively stable throughout. This observation was interpreted as a drug interaction: valproic acid competed with phenytoin for access to plasma protein binding sites.
The spectrum of primary blastomycotic meningitis: A review of central nervous system blastomycosis
Authors: Gonyea Edward F. 
Published: December 2004

AbstractThree cases of meningitis with initial and exclusive neurological involvement prompted a review of the clinical, cerebrospinal fluid, and pathological findings in an additional 78 patients with central nervous system blastomycosis. The first patient of the 3 had progressive cerebellar dysfunction as the result of chronic basilar meningitis. The second had a C8‐T1 radiculopathy without other evidence of superior sulcus syndrome, and subsequent acute fatal meningitis. The third had aseptic, benign, self‐limited meningitis followed by clinically obvios systemic blastomycosis.Diagnosis is difficult, and it is likely that other cases have been presumptively treated for tuberculous meningitis. A more aggressive approach to diagnosis is proposed that takes into account the condition of the patient, the likelihood of dissemination at necropsy, and the frequent meningeal infections that are negative on culture of lumbar CSF.
Behavioral toxicity of anesthetic gases
Authors: Synder Bruce D. , Thomas Robert S. , Gyorky Zsuzsanna 
Published: December 2004

AbstractOperating room personnel were studied for evidence of acute reversible or chronic cumulative cognitive dysfunction as measured by psychometric testing. In comparison with matched controls, no acute reversible deficits were noted in a relatively unpolluted operating room environment. There were only 2 marginally significant test differences between the control and operating room groups; however, control subjects did better than operating room personnel on 16 of 18 tests at both the beginning and end of the work week. Analyses of the deficit patterns and correlations with years of operating room exposure only weakly support the hypothesis that the test differences are due to toxic exposure.
Familial multiple sclerosis: Clinical, histocompatibility, and viral serological studies
Authors: Eldridge Roswell , McFarland Henry , Sever John , Sadowsky Doris , Krebs Helen 
Published: December 2004

AbstractEvaluation of presumed “multiple sclerosis families” and comparison with recently reported families has led us to the following observations: (1) Seven of our original fourteen presumptive multiple sclerosis families had to be eliminated after personal clinical evaluation of family members failed to confirm the diagnosis in a second close relative. (2) No segregation of HLA type was noted between affected and unaffected individuals in our seven bona fide multiple sclerosis families, and no consistent segregation was noted in the twenty‐eight families reported elsewhere. This supports other genetic evidence that there is not a single, major gene mapping in the HLA complex which predisposes to multiple sclerosis. (3) The DW 2 antigen was increased in frequency among affected members of our families, and the A3 B7 haplotype was more frequent among affected members of other families reported. But unaffected members also tended to have an increased frequency of these same antigens. (4) No relationship was noted between HLA type and antimeasles antibody titer within our families.
Bilateral oculomotor paralysis due to neurosyphilis
Authors: Jordan Kenneth , Marino Joseph , Damast Melvyn 
Published: December 2004

AbstractBilateral third nerve paresis attributed to neurosyphilis has not been documented in the absence of associated neurological deficits. We describe a patient with isolated bilateral third nerve paresis, positive serological findings in blood and cerebrospinal fluid, CSF pleocytosis, and increased protein content. Resolution of these abnormalities occurred when the patient was treated with penicillin.
Levodopa in Parkinson disease: A long‐term appraisal of mortality
Authors: Joseph Clifford , Chassan Jacob B. , Koch Maria‐Luise 
Published: December 2004

AbstractMortality rates for a group of patients in a collaborative multicenter study of levodopa (Larodopa) in the treatment of parkinsonism were compared with corresponding mortality rates in the general population adjusted for age and sex distribution in accordance with that of the parkinsonian patients under study. The death rate, conservatively adjusted for dropouts among parkinsonian patients, is estimated to be approximately 33% greater than that for the general population. This is in contrast to an earlier study of Hoehn and Yahr, prior to the advent of levodopa treatment, where in the mortality rate for parkinsonian patients was shown to be in excess of that in the general population by a factor of 2.9 or nearly 200%.
Four‐year treatment of patients with parkinsonism using amantadine alone or with levodopa
Authors: Timberlake William H. , Vance Michael A. 
Published: December 2004

AbstractHalf of 94 parkinsonian patients improved on amantadine therapy during acute double‐blind trials. In a four‐year follow‐up, amantadine given alone or added to a stable dose of levodopa had its greatest effect in the first month and helped few patients after six months. Levodopa either alone or added to a stable dose of amantadine had a beneficial effect lasting three years or more.The side‐effects of edema and livido reticularis occurred twice as often in women. Confusion and hallucinations appeared sooner on a regimen of 300 mg of amantadine a day, but the ultimate incidence was the same on 200 mg a day. Withdrawal effects from amantadine are no less frequent or serious than from other antiparkinson medications and are not evidence that amantadine is still helping the patient. Considering the years of exposure, the morbidity and mortality do not indicate any risks peculiar to amantadine. Our mortality in all groups combined was 2.4 times that of the age‐ and sex‐matched United States population.
Ischemia‐induced seizures and cortical monoamine levels
Authors: Welch K. M. A. , Wang T.‐P. F. , Chabi Eva 
Published: December 2004

AbstractSeizure activity as a component of the ischemic process possibly responsible for monoamine changes described in the gerbil stroke model was the subject of this study. Abnormal motor activity suggestive of seizures developed one to three hours after unilateral ligation of the common carotid artery in approximately 50% of gerbils that exhibited signs of stroke. Reduction of cortical levels of dopamine and norepinephrine was observed only when seizures occurred in association with stroke. The levels of 5‐hydroxytryptamine were reduced bilaterally in animals with and without signs of stroke and were reduced further in animals with stroke plus seizures.Further study is needed to establish whether the catecholamine changes associated with ischemia‐induced seizures are primary and causative or secondary to seizure activity itself. In the ischemic brain, 5‐hydroxytryptamine metabolism appears disordered independent of seizure activity. Seizure activity must be taken into account when the mechanisms of disordered monoamine metabolism are being examined in the gerbil stroke model.
Regulation of intracranial pressure in rat, dog, and man
Authors: Mann J. Douglas , Butler Albert B. , Rosenthal Jaime E. , Maffeo Charles J. , Johnson Richard N. , Bass Norman H. 
Published: December 2004

AbstractIntracranial resistance and compliance were assessed in the cerebrospinal fluid system of rat, dog, and man by means of low‐volume, short‐duration infusions of artificial CSF into the subarachnoid space. A family of pressure/time response curves was obtained for each species: at low flow rates, steady‐state pressure elevations were associated with marked neurological dysfunction. From these curves a mathematical model was developed which describes transport and pressure‐dependent valvelike characteristics of the CSF outflow resistance. This resistance. This resistance shows a progressive phylogenetic change in rat, dog, and man which increasingly enhances the species' abilities to vent fluid from the intracranial cavity into the venous circulation. Arachnoid villi appear to be the major structures modulating sustained, nonlethal elevations of intracranial pressure.
Amanita mushroom poisoning with recovery from coma: A case report
Authors: Teutsch Carol , Brennan Robert W. 
Published: December 2004

AbstractThe incidence of Amanita mushroom poisoning seems to be increasing in the United States. Its neurological aspects contribute importantly to morbidity and mortality. In reported series, mortality has ranged from 50 to 90%, and survival following the appearance of coma was exceptional. The cause of nervous system involvement was uncertain. In the patient presented here, clinical and electroencephalographic observations were consistent with severe hepatic encephalopathy and correlated closely with liver function abnormalities. Despite the development of coma, full recovery followed the use of thioctic acid, an experimental therapeutic agent.
Chronic paroxysmal hemicrania: A disabling headache syndrome responding to indomethacin
Authors: Price Richard W. , Posner Jerome B. 
Published: December 2004

AbstractA 57‐year‐old man experienced daily episodes of unilateral headache accompanied by homolateral partial Horner syndrome and rhinorrhea for more than 40 years. Treatment with indomethacin eliminated these headaches. The patient's illness is best classified as “chronic paroxysmal hemicrania” as described by Sjaastad and Dale.
Visual dysfunctions in optic tract lesions
Authors: Bender Morris B. , Bodis‐Wollner Ivan 
Published: December 2004

AbstractCharacteristic visual symptoms and signs in 12 patients with neoplasms or aneurysms involving the optic tract are summarized. Blurred vision was the most common initial manifestation. Optic atrophy became apparent in 7 of the 12 patients. Most patients had uniocular central scotomas with reduced visual acuity, and 2 had homonymous scotomas. Field defects were frequently incomplete and incongruous, combining central scotomas with elements of homonymous and bitemporal amblyopias. Seven patients had endocrine disturbances and memory deficits in addition to their visual symptoms.
Studies of fragmented sarcoplasmic reticulum from human skeletal muscle
Authors: Scarpa A. , Dimauro S. , Bonilla E. , Schotland D. L. 
Published: December 2004

AbstractSarcoplasmic reticulum (SR) vesicles were isolated from muscle biopsies of 4 normal volunteers, a patient with McArdle disease (before and during contracture), and a patient with normokalemic periodic paralysis. Fractions were analyzed for purity by electron microscopy and biochemical analysis of specific marker enzymes. Adenosine triphosphate (ATP)‐dependent calcium ion uptake was measured kinetically by the absorbence changes of murexide, a metallochromic indicator of ionized Ca++ concentrations, in the absence of oxalate or other calcium‐complexing anions. In these experiments, time resolution of the Ca++ transport rate was limited by the manual mixing of reagents, which occurred in 1 to 3 seconds. In 1 case the “true” initial velocity of Ca++ uptake was measured by rapid mixing of ATP in a stopped‐flow apparatus and by following the change in absorbence of murexide in a storage oscilloscope. In SR from normal human muscle the ATP‐dependent Ca++ uptake was 3.5 nmoles per second per milligram of protein for the first 5 seconds after ATP mixing. Fast kinetic experiments showed that Ca++ uptake proceeded linearly for the first 500 msec at a rate of 9 nmoles per second per milligram of protein (at 25°C) and then progressively declined to reach steady‐state levels in 40 to 50 seconds. No abnormality of Ca++ transport was found in SR vesicles from the patient with McArdle disease and the patient with normokalemic periodic paralysis.
The action of phenytoin on a composite electrical‐chemical synapse in the lamprey spinal cord
Authors: Selzer Michael E. 
Published: December 2004

AbstractThe effect of phenytoin (PTN), 20 μg per milliliter, was tested on the composite electrical‐chemical synapse between pairs of giant interneurons in the isolated spinal cords of river lampreys (Ichthiomyzon unicuspis). The main observations were that: (1) PTN reversibly reduced the chemical component of the excitatory postsynaptic potential by up to 70%; (2) PTN greatly reduced or eliminated posttetanic potentiation (PTP) of the chemical component; (3) PTN produced little or no decrease in the size of the electrical component; and (4) PTN did not cause blockage of the electrical component during high‐frequency stimulation of the presynaptic neuron. Since the electrical component reflects the presynaptic spike, the suppression of PTP cannot be explained by blockage of this spike during the tetanus. These four observations are consistent with a current hypothesis concerning the anticonvulsant effect of PTN—that the drug inhibits calcium entry into stimulated presynaptic terminals. This would block PTP by reducing the accumulation of calcium in the terminal during repetitive stimulation.