bluuebunny/crossref_metadata_2025_split huggingface dataset:
Ulnar F‐wave conduction velocity in epidural metastatic root lesions
Authors: De Visser Bram W. Ongerboer , Van Der Sande Jaap J. , Kemp Bob 
Published: January 2005

AbstractIn 40 controls and 19 patients with neoplastic lesions of the eighth cervical and first thoracic nerve roots confirmed by myelography, ulnar F‐wave conduction velocity (FWCV) was estimated using the shortest F‐wave latency (FWL). F‐waves were recorded from needle electrodes inserted in the abductor digiti minimi manus muscle after stimulation of the ulnar nerve at the wrist. Motor nerve conduction velocities of distal ulnar segments were normal. It was found that the ulnar FWCV decreases significantly with increasing age. This is not true for FWL. During the initial examinations ulnar FWCV was delayed in 18 patients. At follow‐up, ulnar signs correlated well with changes in FWCV. Low F‐wave frequency and wide FWL differences are also important features in ulnar root disease.
Hysterical seizures: Suggestion as a provocative EEG test
Authors: Cohen Robert J. , Suter Cary 
Published: January 2005

AbstractWe studied 57 patients with poorly controlled or atypical seizures to identify hysterical attacks with a provocative test consisting of initiation and termination of an attack with suggestion and saline injection during electroencephalographic (EEG) monitoring. Using this method, we diagnosed 48 patients as having hysterical attacks. Three additional patients had spontaneous hysterical attacks during routine recordings. Of the 51 patients with hysterical attacks, 78% were female. Two‐thirds of the patients were between the ages of 20 and 35 years. Thirty‐two patients had taken anticonvulsants before the study. Each patient's hysterical attack, aura, and postictum was stereotyped, and the 6 patients subject to both hysterical and organic seizures described the two types of attacks as different stereotyped behaviors. Tongue laceration, urinary incontinence, and postictal confusion were common. Twelve patients had evidence of past or present neurological disease. Abnormal EEGs were recorded in 37% but only 12% had spike or spike and wave discharges. The provocative EEG test employing saline and suggestion is a useful tool in the diagnosis of hysterical “seizures”.
Distribution of papovavirus, myelin‐associated glycoprotein, and myelin basic protein in progressive multifocal leukoencephalopathy lesions
Authors: Itoyama Yasuto , Webster Henry deF. , Sternberger Nancy H. , Richardson Edward P. , Walker Duard L. , Quarles Richard H. , Padgett Billie L. 
Published: January 2005

AbstractTo study how viruses interact with oligodendroglia and produce demyelination, we immunostained paraffin and epon sections of lesions from patients with progressive multifocal leukoencephalopathy (PML) with antisera to papovaviruses, oligodendroglial myelin‐associated glycoprotein (MAG), and myelin basic protein (MBP) according to the peroxidase‐antiperoxidase method. In paraffin sections from a rapidly progressive case of PML, hyperimmune JC virus antiserum stained single oligodendroglia which were located in white matter that appeared normal histologically and stained normally with MAG and MBP antisera. In zones surrounding areas of demyelination, virus‐containing oligodendroglia were most numerous and MAG staining of periaxonal regions was decreased, but there was little change in MBP staining. In demyelinated regions, both MAG and MBP staining were severely altered; also there was much less JC virus staining. In tissue from three other chronic cases, viral antiserum stained fewer oligodendrocytes and the differences in MAG and MBP staining were much less striking. In epon sections from two biopsies of central nervous system tissue, we studied the electron microscopic appearance of oligodendroglia that also had been stained by JC virus antiserum. Virions were present in all nuclei and in some cytoplasmic regions. The results suggest that changes in MAG distribution are useful indicators of early oligodendroglial abnormalities which can cause myelin breakdown.
A newly recognized congenital myasthenic syndrome attributed to a prolonged open time of the acetylcholine‐induced ion channel
Authors: Engel Andrew G. , Lambert Edward H. , Mulder Donald M. , Torres Carlos F. , Sahashi Ko , Bertorini Tulio E. , Whitaker John N. 
Published: January 2005

AbstractFive familial cases (in two families) and one sporadic case of a new congenital myasthenic syndrome were investigated. Symptoms arise in infancy or later life. Typically, one finds selective involvement of cervical, scapular, and finger extensor muscles, ophthalmoparesis, and variable involvement of other muscles. There is a repetitive muscle action potential to single nerve stimulus in all muscles and a decremental response at 2 to 3 Hz stimulation in clinically affected muscles. Microelectrode studies reveal markedly prolonged end‐plate potential (epp), miniature end‐plate potential (mepp), and miniature end‐plate current; normal quantum content of the epp; and a smaller than normal or low‐normal mepp amplitude. Light microscopy demonstrates predominance of type I fibers, small groups of atrophic fibers, tubular aggregates and vacuoles near end‐plates, abnormal end‐plate configuration, and nonspecific myopathic changes. Abundant acetylcholinesterase activity is present at all end‐plates, and the activity and kinetic properties of this enzyme in muscle are normal. Calcium accumulated at the end‐plate in one patient. Quantitative electron microscopy shows decrease in the size of nerve terminals, increase in the density of synaptic vesicles, and reduction in the length of postsynaptic membranes. There is focal degeneration of junctional folds with corresponding loss of acetylcholine receptor, most marked in cases with the lowest mepp amplitude. There are no immune complexes at the end‐plate. Fiber regions near end‐plates display dilation, proliferation, and degeneration of the sarcoplasmic reticulum; nuclear, mitochondrial, and myofibrillar degeneration; and vacuoles resembling those found in periodic paralysis. A prolonged open time of the acetylcholine‐induced ion channel is considered to be the basic abnormality and may account for the physiological, morphological, and clinical alterations.
Inclusion‐body myositis: Clinicopathological studies and isolation of an adenovirus type 2 from muscle biopsy specimen
Authors: Mikol Jacqueline , Felten‐Papaiconomou Annie , Ferchal Françoise , Perol Yvonne , Gautier Bernard , Haguenau Michel , Pepin Bernard 
Published: January 2005

AbstractA case of inclusion‐body myositis appearing clinically as an atypical distal atrophy lasting for more than 14 years was diagnosed by visualization of characteristic filamentous inclusions in subsarcolemmal nuclei and in the sarcoplasm near vacuoles. A strain of adenovirus type 2 was isolated from two successive muscle biopsy specimens, and the serum neutralizing antibody titer to this strain was 1:64. The data provide objective support for the assumption that inclusion‐body myositis is caused by a virus infection of muscle, although the filamentous inclusions differ from all known viral structures.
Neurotransmitter chemistry in feline GM1 gangliosidosis: A model for human ganglioside storage disease
Authors: Singer Harvey S. , Coyle Joseph T. , Weaver Debbie L. , Kawamura Nariko , Baker Henry J. 
Published: January 2005

AbstractAssays for synaptosomal high‐affinity uptake activity (glutamate, γ‐aminobutyric acid, norepinephrine), neurotransmitter synthesizing enzymes (choline acetyltransferase, glutamate decarboxylase, tyrosine hydroxylase), and endogenous neurotransmitters were performed in cats with advanced inherited GM1 gangliosidosis. A significant reduction in uptake activity, ranging from 24 to 77% of control, was demonstrated in motor, occipital, and cerebellar brain regions. This reduction was unassociated with comparable alterations in neurotransmitter levels or synthesizing enzyme activity. We hypothesize that the defect of neurotransmitter inactivation is part of an overall abnormality of synaptic membrane function that could contribute to the neurological symptoms seen in the hereditary gangliosidoses.
Pathological features of persistent infantile sleep apnea with reference to the pathology of sudden infant death syndrome
Authors: Armstrong Dawna , Sachis Paul , Bryan Charles , Becker Larry 
Published: January 2005

AbstractA child with alveolar hypoventilation secondary to abnormal control of respiration survived for two years with phrenic pacemakers. Autopsy revealed brainstem abnormalities and other tissue changes that also have been reported in sudden infant death syndrome. Quantitative changes in the distribution of myelinated fibers of the vagus nerve also were similar to those described in sudden infant death syndrome.
3‐O‐ methyldopa blocks dopa metabolism in rat corpus striatum
Authors: Reches Avinoam , Fahn Stanley 
Published: January 2005

Abstract3‐O‐Methyldopa (OMD) given to rats inhibits striatal uptake and utilization of L‐dopa. Thus, the accumulation of L‐dopa, dopamine, 3,4‐dihydroxyphenylacetic acid, and homovanillic acid in OMD‐pretreated rats after L‐dopa injection is significantly lower compared with control rats. This effect of OMD is dose dependent. OMD inhibits L‐dopa accuumlation in the striatum after inhibition of aromatic amino acid decarboxylase activity with 3‐hydroxybenzylhydrazine‐HCL. This effect is not mediated through inhibition of firing in dopaminergic neurons, since the accumulation of dopamine in the striatum after γ‐butyrolactone injection was also significantly reduced by OMD. It is suggested that OMD competes with L‐dopa and tyrosine uptake into the brain. These findings are in line with clinical observations which indicate that high plasma levels of OMD in parkinsonian patients are associated with poor response to L‐dopa. The data presented here indicate that use of catechol‐O‐methyltransferase inhibitors with L‐dopa may be of value in the treatment of parkinsonian patients.
Characterization of 5‐hydroxytryptamine receptors in human temporal arteries: Comparison between migraine sufferers and nonsufferers
Authors: Skärby Tor , Tfelt‐Hansen Peer , Gjerris Flemming , Edvinsson Lars , Olesen Jes 
Published: January 2005

AbstractAn increase in the sensitivity of temporal arteries to 5‐hydroxytryptamine (5‐HT) has repeatedly been suggested on indirect evidence as an essential component of a migraine attack. We examined the 5‐HT reactivity of isolated human temporal arteries removed from migraine sufferers and from patients operated on because of cerebral disorders. 5‐HT constricted these vessels in a concentration‐dependent manner, the response being attenuated by 10−8 to 10−6M methysergide. There was no difference in reactivity to 5‐HT or methysergide between vessels from the two patient groups. The pA2 value calculated for methysergide in controls was 7.8 ± 0.1 and in migraine patients, 7.6 ± 0.2, which closely agreed with data obtained graphically using Schild plots: 7.8 and 7.7, respectively. No difference existed between the patient groups due to age or sex. Thus, clear evidence has been obtained demonstrating the presence of 5‐HT receptors in human temporal arteries, although we could observe no difference in the character of the 5‐HT receptros between migraine patients and controls.
Cataplexy in variant forms of Niemann‐Pick disease
Authors: Kandt Raymond S. , Emerson Ronald G. , Singer Harvey S. , Valle David L. , Moser Hugo W. 
Published: January 2005

AbstractA combination of cataplexy and a variant form of Niemann‐Pick disease has occurred in five patients described in the literature as well as in two of our patients. In our Patient 1, cataplexy was differentiated from epilepsy by electroencephalographic telemetry and narcolepsy was confirmed by polysomnography. Cataplexy was abolished in both of our patients by treatment with protriptyline. Four of the seven patients had impairment of vertical eye movements. All seven patients had a disorder best classified as type C or D Niemann‐Pick disease or the Neville, Wenger, or Wiedemann variant. This appears to be the first identified relationship between cataplexy and a specific disease.
The pathophysiology of brain ischemia
Authors: Raichle Marcus E. 
Published: January 2005

AbstractBrain ischemia due to a critical reduction in cerebral blood flow is a well recognized and common cause of irreversible brain damage. The observation that brain cells are more resistant to ischemia than was previously assumed on the basis of clinical experience has stimulated considerable investigative work designed to determine those factors responsible for irreversible ischemic cell damage. At this time, data from these investigations indicate that cellular acidosis and biochemical disturbances initiated by abnormal intracellular ion homeostasis may be especially important in determining the ultimate survival of nerve cells. This review examines the biochemical events initiated by ischemia and their potential role in determining the ultimate survival of brain cells.
Syndrome of opsoclonus‐myoclons caused by coxsackie B3 infection
Authors: Kuban Karl C. , Ephros Moshe A. , Freeman Roy L. , Laffell Lori Burtz , Bresnan Michael J. 
Published: January 2005

AbstractCoxsackie B3 was cultured from 2 children who presented within two weeks of each other from the Cape Cod, Massachusetts, area showing opsoclonus and myoclonus. The organism was recovered from the cerebrospinal fluid of 1 patient and from the stools of both. Both children had cerebrospinal fluid pleocytosis and gradual, spontaneous resolution of their symptoms.
Observations on the efficacy of propranolol for the prophylaxis of migraine
Authors: Rosen James A. 
Published: January 2005

AbstractAmong 1,036 patients treated for migraine headache between 1973 and 1980, 865 were given a daily maximum dose of 320 mg of propranolol, while 171 served as differently treated controls. At least one year of treatment was prescribed, at the end of which time 84% of the propranolol group reported sustained improvement compared with similar improvement in only 32% of controls (p < 0.01). The success rate progressively increased during the entire year.
Edentulous orodyskinesia
Authors: Koller William C. 
Published: January 2005

AbstractTwelve of 75 (16%) edentulous patients (mean age, 62 years) were found to have orofacial dyskinesias. Tooth extraction averaged 12 years before the onset of movements. No dentures were present in 50% of dyskinetic patients and in 9.5% of edentulous patients without dyskinesia. Movements of the lips, tongue, and jaw were marked in 3 patients and mild in 9. Functional disability was minimal. None of 75 elderly patients (mean age, 68 years) without tooth extractions had oral dyskinesias.
Neurological and neuroradiological examination of chronic cannabis smokers
Authors: Hannerz Jan , Hindmarsh Thomas 
Published: January 2005

AbstractTwelve subjects, eight male and four female, who had smoked on average more than 1 gm of cannabis daily for 10 years, were investigated with a clinical neurological examination and computed tomography (CT) of the brain. No subject had a history of major head trauma or cerebral infection, and just one abused alcohol. Only the subject with a history of alcoholism showed any abnormal cerebral function on clinical tests or any abnormality in the CT scan, compared with normal controls.
Neurotransmitter alterations in a model of perinatal hypoxic‐ischemic brain injury
Authors: Johnston Michael V. 
Published: January 2005

AbstractVulnerability of neurotransmitter‐specific neurons to hypoxia‐ischemia was examined in the immature rat corpus striatum. Carotid artery ligation plus 2 hours of 8% oxygen atmosphere at 1 week of age produced ipsilateral striatal injury and reduced hemisphere mass 2 and 6 weeks later. Striatal injury was always more severe than damage to overlying cortex. Over half the animals had status marmoratus, a neuropathological change seen in the basal ganglia and thalamus after hypoxic‐ischemic injury in full‐term human infants. Two weeks after the insult, markers for choinergic, dopaminergic, and γ‐aminobutyric acid–containing neurons were all reduced, but the reduction in cholinergic markers was greater than that for the other two transmitters. Muscarinic cholinergic receptors were relatively preserved, but their distribution was disrupted. In adult animals specific activity of cholinergic neuronal markers was normal, suggesting that the balance of neurotransmitters was restored after the early insult.
Freeze‐fracture analysis of plasma membrane cholesterol in duchenne muscle
Authors: Fischbeck Kenneth H. , Bonilla Eduardo , Schotland Donald L. 
Published: January 2005

AbstractWe estimated cholesterol in Duchenne muscle plasma membrane using the sterol‐specific ligand digitoing. In measuring the extent of digitonin‐cholesterol complex formation with freeze‐fracture electron microscopy, we found a significantly greater proportion of surface area taken up by complexes in Duchenne muscle (60% ± 9%) than in controls (36% ± 4%). This finding suggests that a population of Duchenne muscle fibers has a higher concentration of cholesterol in the plasma membrane. Membrane cholesterol is known to affect membrane deformability and the activity of membrane‐bound enzymes and transport proteins; thus a difference in membrane cholesterol might account for structural and functional abnormalities in Duchenne plasma membrane.
Maturational changes in postictal refractoriness and seizure susceptibility in developing rats
Authors: Moshé Solomon L. , Albala Bruce J. 
Published: January 2005

AbstractThe ability of the central nervous system to suppress recurrent seizures as a function of age was determined in rats. Eight electrical stimulations were delivered to the amygdala at 2‐minute intervals in adult and suckling rats that were previously kindled from the left amygdala. During this 16‐minute period, prolonged and severe convulsions were repeatedly elicited in the 16‐day‐old rat pups, whereas convulsions were not always elicited in adul rats. The results suggest that immature rats are more prone to develop status epilepticus than adult rats. Subsequently, the rat pus and implanted but not stimulated littermates were allowed to grow. At maturity, all the surviving animals were kindled from the contralateral (right) amygdala. The previously kindled rats developed seizures significantly faster than did their littermates. However, their respective abilities to suppress recurrent seizures did not differ at 150 and 210 days of age, being similar to the seizure suppression abilities of naive controls and significantly greater than those evidenced in infancy. We conclude that the propensity to develop single seizures in adulthood is directly related to infant seizure history, whereas the ability to suppress multiple seizures is a dynamic process that is modified by age, being minimal early in life and enhanced with maturation independently of history of infantile convulsions.
Nuclear magnetic resonance imaging in multiple sclerosis
Authors: Lukes Steven A. , Crooks Lawrence E. , Aminoff Michael J. , Kaufman Leon , Panitch Hillel S. , Mills Catherine , Norman David 
Published: January 2005

AbstractTen patients with definite multiple sclerosis underwent hydrogen nuclear magnetic resonance imaging with a 3.5 kilogauss superconducting magnet, using the inversion recovery and spin‐echo techniques of signal acquisition. Results were compared with high‐resolution x‐ray computed tomography. Spin‐echo images demonstrated abnormal regions as areas of variably increased signal intensity. The contrast between abnormal and normal white matter improved as the intervals between sequential radiofrequency pulses and between pulse administration and signal sampling were increased. Inversion recovery images demonstrated abnormal areas as regions of decreased signal intensity but did not visualize lesions as well as spin‐echo imaging. Spin‐echo and inversion recovery imaging each demonstrated more extensive abnormalities than did computed tomography.
Serum demyelinating factors in multiple sclerosis
Authors: Seil Fredrick J. , Westall Fred C. , Romine John S. , Salk Jonas 
Published: January 2005

AbstractSera from 21 patients with multiple sclerosis were applied to myelinated spinal cord cultures and evaluated for demyelinating activity. Samples were collected at various times when patients were on or off therapy with myelin basic protein or prednisone or both, and at various stages of disease. Five of 31 serum samples from 17 patients exhibited demyelinating activity when tested in a 40% concentration in tissue culture nutrient medium. Four of the 10 serum samples taken from patients with active or remitting multiple sclerosis (2 samples from the same patient) were demyelinative; 1 of 21 sera collected during stationary periods of disease was positive. There was no correlation between serum demyelinating activity and the presence or absence of treatment. Sera positive for demyelinating activity failed to inhibit myelin formation in initially unmyelinated cerebellar cultures.
Kearns‐sayre syndrome with reduced plasma and cerebrospinal fluid folate
Authors: Allen Richard J. , DiMauro Salvatore , Coulter David L. , Papadimitriou Alexander , Rothenberg Sheldon P. 
Published: January 2005

AbstractA young woman with Kearns‐Sayre syndrome and progressive central nervous system deterioration over 15 years had decreased plasma and cerebrospinal fluid folate levels while receiving phenytoin for a seizure disorder. A muscle biopsy showed a “ragged red fiber” myopathy with reduced muscle carnitine and mitochondrial enzymes. Computed tomographic brain scans showed cerebral white matter hypodensities and bilateral calcification of the basal ganglia. The mechanism for the folate deficiency and altered ratio of plasma to cerebrospinal fluid folate is unknown, but the deficiency may be responsive to replacement therapy.
The crossed upgoing toe sign: A clinical study
Authors: Willoughby Ernest W. , Eason Robert 
Published: January 2005

AbstractWe compared the crossed upgoing toe sign with the plantar response as an indicator of pyramidal tract dysfunction in 125 normal subjects and 192 patients with neurological disorders. A positive crossed upgoing toe sign was associated significantly with a partial pyramidal tract lesion in the contralateral cerebral hemisphere with a frequency similar to that of Babinski's sign. Unlike Babinski's sign, however, the positive crossed upgoing toe sign was lost when pyramidal weakness was severe enough to produce paralysis of voluntary dorsiflexion of the great toe, and it was found only rarely with pyramidal tract lesions of the spinal cord. The crossed upgoing toe sign has little value as a sensitive indicator of a pyramidal tract lesion. It is potentially of limited value as an aid in determining the level of a pyramidal tract lesion (cerebral hemisphere versus spinal cord), but its usefulness is seriously impaired by the high frequency of false positive signs in normal subjects and patients with other neurological disorders.