bluuebunny/crossref_metadata_2025_split huggingface dataset:
Cerebrospinal fluid findings in asymptomatic patients with reactive serum fluoresecent treponemal antibody absorption tests
Authors: Traviesa Daniel C. , Prystowsky Stephen D. , Nelson Barbara J. , Johnson Kenneth P. , Clavan Barbara S. 
Published: January 2005

AbstractIt is common to examine the cerebrospinal fluid in untreated or inadequately treated asymptomatic patients with a reactive serum fluorescent treponemal antibody absorption (FTA‐ABS) test before initiating antibiotic therapy for syphilis. This prospective study evaluated the usefulness of such examination. Four hundred thirty‐two patients over 40 years old, reporting for annual physical examination, had a serum FTA‐ABS test. Thirty‐seven (8.6%) patients and 2 of 4 spouses were reactive repeatedly. Of the 39 patients with reactive tests, 7 had a history of pencillin therapy for syphilis, 5 had received heavy metal therapy, and 27 had no history of syphilis. These 39 patients had a neurological examination, serum VDRL, Treponema pallidum immobilization (TPI), and repeat FTA‐ABS tests by two other laboratories. The TPI test was reactive in 30 (77%). Four had nonspecific neurological signs. Routine CSF examination (cells, total protein, VDRL, glucose, IgG%) on 30 patients with a history of inadequate treatment had a low diagnostic yield. Two patients had an unexplained total protein elevation (57 and 61 mg/dl) and 1 had a mildly increased IgG% (15%). All cell counts, VDRL tests, and glucose levels were normal. Agarose electrophoresis demonstrated one or more CSF immunoglobulin bands in 10 (36%) of 28 patients, possibly representing an immunological marker of past or latent central nervous system infection.
Juvenile amaurotic idiocy (neuronal ceroid lipofuscinosis) and lymphocyte fingerprint profiles
Authors: Baumann Robert J. , Markesbery William R. 
Published: January 2005

AbstractLymphocytes from 3 children with a form of juvenile amaurotic idiocy (characterized by retinal blindness, progressive dementia, and extrapyramidal motor disturbance) were studied by electron microscopy. Numerous fingerprint profiles (FP) were found in vacuolated lymphocytes from all 3 patients and an asymptomatic younger sibling of 1 patient who subsequently became symptomatic. We propose that the combination of this clinical picture and vacuolated lymphocytes with FP is sufficiently distinctive for clinical and research purposes until the biochemical defect is discovered. Wider utilization of ultrastructural study of lymphocytes should increase the number of children diagnosed and allow detection of asymptomatic patients.
Neurotoxicity of intrathecal gentamicin: A case report and experimental study
Authors: Watanabe Itaru , Hodges Glenn R. , Dworzack David L. , Kepes John J. , Duensing George F. 
Published: January 2005

AbstractDistinctive lesions occurred in the brainstem of a 59‐year‐old patient who had had recent Pseudomonas aeruginosa meningitis treated with parenteral and intrathecal gentamicin sulfate. The lesions were multiple, minute, and discrete, and were characterized by loss of axons, spongiosis, axonal swelling with frequent calcification, loss of astroglia and oligodendroglia, and slight inflammatory response. These lesions were restricted to the myelinated fiber bundles of the pons and mesencephalon.Because similar lesions can occur with other intrathecally administered medications and emboli to the brain, an experimental study in rabbits was done. Similar lesions were produced in normal adult rabbits after a single intracisternal injection of gentamicin sulfate with or without preservative at doses equivalent to 50 and 100 times the human therapeutic dose. Lesions were not seen after injection of normal saline, preservative, or gentamicin sulfate with preservative at doses equivalent to 1 and 10 times the human therapeutic dose. A direct relationship was observed between the cerebrospinal fluid concentrations of gentamicin, brain tissue concentrations of gentamicin, and occurrence of the lesions.
Multiple sclerosis in the faroe islands: I. Clinical and epidemiological features
Authors: Kurtzke John F. , Hyllested Kay 
Published: January 2005

AbstractIntensive search over the past five years for all cases of multiple sclerosis (MS) on the Faroe Islands since about 1920 has revealed 25 cases among native‐born resident Faroese up to 1977. All but 1 patient had clinical onset of MS between 1943 and 1960; 1 case began in 1970. Four cases of MS in Faroese with prolonged foreign residence and 5 among Danish‐born Faroese were excluded.The 24 included cases with onset between 1943 and 1960 meet all critèria for a point‐source epidemic. The median year of onset was 1949. The cumulative risk of MS for Faroese in 1940 was 8.7 per 10,000. All 14 early‐onset cases (1943 to 1949) were in patients 11 to 45 years old in 1940; all but 2 late‐onset cases (1952 to 1960) were in persons aged up to 10 years old in 1940. British troops occupied the Faroes in large numbers for five years beginning in April, 1940. During the war, all but 3 patients resided in locations where the troops were stationed, and these 3 also had direct contact with the British.We conclude that there was an epidemic of MS on the Faroes and that the disease was probably introduced by the British troops (or their baggage). If so, then MS on the Faroes is a transmissible disease, most likely infectious; but only about 1 in 500 of the exposed individuals were clinically affected.
Clinical and biochemical aspects of trichopoliodystrophy
Authors: Grover Warren D. , Johnson Waine C. , Henkin Robert I. 
Published: January 2005

AbstractThe clinical and biochemical evaluation of 6 patients with trichopoliodystrophy indicates that the disease process can begin in utero and is related to a selective abnormality in copper metabolism. Examination of 2 infants on the first day of life revealed abnormal neurological signs, a characteristic hair abnormality, and elevated levels of copper and ceruloplasmin. Decreased hepatic copper levels and increased urinary copper excretion were documented during the first week. The 2 neonates demonstrated a progressive decrease in blood copper levels in the first month of life. Four infants identified at ages 2 to 11 months had low values for blood copper and ceruloplasmin. All infants had progressive neurological dysfunction, and 4 of the 6 died at ages ranging form 2 1/2 months to 5 1/2 years. Parenteral copper therapy achieved normal blood and hepatic copper levels in 1 patient, but the copper values in the cerebral cortex and white matter were significantly decreased compared to control specimens.
Postvaccinial encephalomyelitis without cutaneous vaccination reaction
Authors: Rockoff A. , Spigland I. , Lorenstein B. , Rose A. L. 
Published: January 2005

AbstractA 5‐year‐old girl developed a progressive febrile neurological illness consisting of right focal seizures, right hemiparesis, and stupor evolving over a period of six weeks. During the month preceding the onset of her illness she had received two apparently unsuccessful smallpox vaccinations without a skin lesion. Elevation of cerebrospinal fluid gamma globulin and findings on brain biopsy were consistent with postinfectious encephalitis, and a simultaneous increase in serum vacinia antibody titer suggested that the illness was postvaccinial encephalitis. Clinically, the child developed a severe extrapyramidal movement disorder during the acute phase followed by nine months of stuporous unresponsiveness, yet subsequently made a substantial recovery.
Endoneurial lipid composition of normal human sural nerve
Authors: Brown Mark J. , Iwamori Masao , Kishimoto Yasuo , Ostroff Stephen M. , Moser Hugo W. , Asbury Arthur K. 
Published: January 2005

AbstractThe endoneurial lipid composition was determined in 7 human sural nerves from subjects without known neurological disease. The nerves were morphologically normal, with myelinated fiber density of 7,710 ± 1,210/mm2 (mean ± SD). Endoneurium contained 30.7 ± 1.5 mg total lipid per 100 mg of dry weight. Cholesterol was the predominant lipid (9.1 ± 1.0 mg). Cholesterol ester was present only in very small quantities (0.2 ± 0.1 mg). Cerebroside concentration was 4.5 ± 0.3 mg, and sulfatide was 1.5 ± 0.3 mg. The most abundant phospholipid was sphingomyelin (4.3 ± 0.3 mg), followed by phosphatidylethanolamine (4.1 ± 0.2 mg), phosphatidylcholine (3.8 ± 0.2 mg), and phosphatidylinositol‐phosphatidylserine (1.7 ± 0.2 mg). Eighty‐one percent of cerebroside nonhydroxy fatty acids were long chain (C20 to C26), and 67% were saturated. Technological advances now permit microchemical lipid analysis of human sural nerve biopsies in cases of neuropathy. When compared with our normative data, such studies may further the understanding of peripheral nerve disorders.
Atrial myxoma with intellectual decline and cerebral growths on CT scan
Authors: Frank Robert A. , Shalen Philip R. , Harvey Danny G. , Berg Leonard , Ferguson Thomas B. , Schwartz Henry G. 
Published: January 2005

AbstractA woman with left atrial myxoma had progressive disturbance of intellectual function, headache, and prominent constitutional symptoms. The absence of stroke is noteworthy. Multiple high‐density lesions with contrast enhancement were seen by CT scan, suggesting metastatic neoplasms, hematomas, or hemorrhagic infarcts. Serial study showed progressive enlargement of the lesions. The only cerebral lesion examined histologically proved to be an organizing hematoma, originating most likely from adjacent small arteries found to be plugged by embolic myxoma.
A hypothetical explanation of saccadic oscillations
Authors: Zee David S. , Robinson David A. 
Published: January 2005

AbstractEye movements in a patient with saccadic oscillations (ocular flutter) were recorded and analyzed. Findings were related to recent microelectrode studies in the monkey pontine reticular formation which have identified three types of premotor neurons related to saccadic eye movements: burst, tonic, and pause cells. We incorporated these cell types into a hypothetical circuit that generates saccades by rapidly driving the eye to a designated orbital position rather than preprogramming a distance for movement.Physiological measurements suggest that this neural network is unstable and that the burst neurons must be tonically inhibited to prevent saccadic oscillations during periods of fixation. Pause cells, which discharge tonically except during saccades, when they pause, appear to inhibit burst cells and prevent such saccadic oscillations. Analysis of our patient's behavior indicates that many types of saccadic oscillations can be explained and classified by assuming an abnormality of pause cell control over saccadic burst neurons.
Degeneration of the cerebellar system in X‐chromosome—linked copper malabsorption
Authors: Iwata Makoto , Hirano Asao , French Joseph H. 
Published: January 2005

AbstractExamination of the cerebellar system of 5 autopsied patients with X‐chromosome–linked copper malabsorption led to the discovery that among cerebellar afferent systems, only the dorsal spinocerebellar tract showed consistent degenerative changes. Cerebellar cortical lesions comprised granule cell loss and Purkinje cell abnormalities that included nuclear and cytoplasmic degenerative changes with cell loss and deficient dendritic arborization as well as the presence of somatic sprouts. Perisomatic baskets were often absent in the area of sprouting Purkinje cells. Cerebellar cortical topographical analysis revealed relative sparing of the caudal portion of the cerebellum. The nodulus was intact in all patients. The dentate nucleus and the superior cerebellar peduncle were preserved. The red nucleus was consistently degenerated.
Patterns of incidence of brain tumors in children
Authors: Gold Ellen B. , Gordis Leon 
Published: January 2005

AbstractA population‐based community‐wide study of the incidence of brain tumors in children from 1960 through 1974 was conducted in the Baltimore area. Incidence rates in boys declined from 1960 to 1974 and were higher in whites than in blacks in all age groups except children under 5 years. A peak in incidence rates was observed in white 5‐to 9‐year‐olds and in blacks from 0 to 4 years of age. In addition, incidence rates tended to be higher in boys than in girls at all ages except at puberty—the 10‐ to 14‐year‐old age group—when rates in girls exceeded those in boys. The findings suggest that both host and environmental factors may be involved in the cause of brain tumors in children.
Metals in spinal cord tissue of patients dying of motor neuron disease
Authors: Kurlander Harold M. , Patten Bernard M. 
Published: January 2005

AbstractTo evaluate the role of toxic metals in causing motor neuron disease (MND), we used a photon‐excited, energydispersive x‐ray analytical system to measure the metal content of spinal ventral horn tissue. Specimens were taken from the cervical and lumbar enlargements of 7 patients who died of MND and the results compared with those found in 12 control patients. Anterior horn lead levels were elevated in MND patients compred to controls (mean, 40.7 μg/gm versus 14.6 μg/gm; p <0.05) and lead levels correlate with the duration of illness (r = +0.84, p < 0.05). Only 2 MND patients had detectable manganese levels (72.3 and 132.2 μg/gm) whereas 1 control had detectable manganese (14.3 μg/gm). One MND patient had 244 μg/gm selenium, but 3 controls had levels of 180, 58, and 62. Patients with the histories of greatest environmental exposure to metals during life exhibited the highest tissue levels of metals after death; despite chelation therapy for about a year, high levels remained in their tissue.
Evaluation of the insulin receptor in myotonic dystrophy
Authors: Festoff B. W. , Moore W. V. 
Published: January 2005

AbstractOnly 2 of 6 patients with myotonic dystrophy had glucose intolerance and hyperinsulinemia. All, however, had markedly reduced insulin binding to specific receptors on circulating monocytes. A significant difference in receptor affinity for insulin was observed, but the difference in affinity was not as marked as that in the amount of 125I‐insulin bound per cell, which was sixfold greater in control cells. No evidence for a circulating factor competing with insulin for binding sites could be demonstrated. These alterations in binding did not directly correlate with glucose intolerance or hyperinsulinemia and are consistent with a postulated generalized membrane defect. Study of the insulin receptor in this disorder may uncover regulatory mechanisms in normal and pathological conditions, including myotonic dystrophy.
Failure of chronic dorsal column stimulation in multiple sclerosis
Authors: Rosen James A. , Barsoum Adib H. 
Published: January 2005

AbstractIn view of the favorable claims of Cook and others, an open trial of chronic dorsal column stimulation was undertaken in 9 patients who had moderately advanced multiple sclerosis (MS) but were still ambulatory. No objective improvement was noted by either author during the postoperative observation period of 6 to 37 months; the occasional subjective improvement was ascribed to placebo effect. This procedure can no longer be recommended for MS patients.
Lorazepam in status epilepticus
Authors: Walker Jonathan E. , Homan Richard W. , Vasko Michael R. , Crawford Isaac L. , Bell Rodney D. , Tasker William G. 
Published: January 2005

AbstractLorazepam, a dichloro‐3‐hydroxy‐1,4‐benzodiazepine, has been shown to be a potent anticonvulsant in animal models of epilsepsy and has minimal depressant effects on respiration and circulation in humans. The effects of this compound were studied in status epilepticus. Twenty‐five patients were given intravenous lorazepam during status epilepticus of varying cause. Four or 8 mg of the drug controlled status in 22 of the 25 patients. Although single seizures recurred in 5 of the 22 patients, none experienced recurrence of status during a prolonged follow‐up period. Transient respiratory arrest occurred in 1 patient, but no other significant complications were observed. Studies of plasma drug levels suggest that most patients attain good seizure control at concentrations between 30 and 100 ng per milliliter. Clinical observations indicate that repetitive injections are not required for continuing control of seizures in patients whose seizures are initially controlled. Lorazepam appears to be an effective and safe drug for treatment of status epilepticus, with a duration of control longer than that achieved with diazepam.
Infantile neuroaxonal dystrophy and giant axonal neuropathy: Are they related?
Authors: Begeer J. H. , Houthoff H. J. , Van Weerden T. W. , De Groot C. J. , Blaauw E. H. , Le Coultre R. 
Published: January 2005

AbstractLight and electron microscopic findings from two sural nerve biopsies obtained at a one‐year interval from a patient with the clinical features of Seitelberger's disease are described. Ballooned axons with accumulations of membranous profiles, vesicles, mitochondria, and a homogeneous center were present, and there were masses of 90 A filaments in endothelial, endoneurial, perineurial, and Schwann cells. These pathological alterations were less prominent in the second nerve biopsy, which showed a more pronounced decrease in myelinated fibers. The case shows that a generalized increase of 90 A filaments in structures of the peripheral nervous system is not a phenomenon exclusively occurring in patients with giant axonal neuropathy and, furthermore, that it may be a transitory feature.
Skin fibroblast microtubular network in Alzheimer disease
Authors: Harper C. G. , Buck D. , Gonatas N. K. , Guilbert B. , Avrameas S. 
Published: January 2005

AbstractA preliminary observation from another laboratory recently suggested that a systemic microtubular defect may exist in Alzheimer disease. To investigate this hypothesis, we obtained skin biopsies from 4 patients with Alzheimer disease and 2 age‐matched controls Fibroblast cultures were established and the tubulin networks examined using immunoadsorbent purified antitubulin antibody and the indirect immunoperoxidase technique as well as electron microscopy. The cells were also examined after treatment with vinblastine and at senescence.The microtubular network appeared as delicate fibers radiating From the perinuclear region toward the cytoplasmic margins. No differences were recognized between the controls and the fibroblast cultures from patients with Alzheimer disease. Microtubules were not visualized following incubation with vinblastine, but positively staining intracytoplasmic paracrystalline inclusions were noted. No abnormalities of microtubules were recognized in the electron microscopic examinations. These findings suggest that the neurofibrillary neuronal degeneration of Alzheimer disease is not a manifestation of a systemic disorder of the microtubular network.
Multisystem triglyceride storage disorder with impaired long‐chain fatty acid oxidation
Authors: Angelini Corrado , Philippart Michel , Borrone Carla , Bresolin Nereo , Cantini Marcello , Lucke Sandra 
Published: January 2005

AbstractA five‐year‐old girl presented with congenital ichthyosis, hepatosplenomegaly, vacuolized granulocytes (Jordans' anomaly), and myopathy. Pathological, ultrastructural, and biochemical studies revealed nonlysosomal, multisystemic triglyceride storage. The cultured fibroblasts had increased uptake but decreased oxidation of labeled oleate. The patient failed to produce ketone bodies on fasting. A medium‐chain triglyceride diet reversed the hepatomegaly. These studies are all consistent with a partial defect in the catabolism of long‐chain fatty acids. This newly identified syndrome is presumably transmitted as an autosomal recessive trait.
Familial stroke syndrome associated with mitral valve prolapse
Authors: Rice G. P. A. , Boughner D. R. , Stiller C. , Ebers G. C. 
Published: January 2005

AbstractIn a 27‐member family, 8 individuals had mitral valve prolapse (MVP) by echocardiography. Four of them suffered a total of eight cerebral ischemic events in the first four decades of life. In none of these patients could risk factors for stroke, other than MVP, be found. Stroke did not occur in individuals free of MVP. This report draws attention to a previously unrecognized cause of familial stroke which strengthens the relationship between MVP and premature stroke.
Cold face test in the assessment of trigeminal‐brainstem‐ vagal function in humans
Authors: Khurana Ramesh K. , Watabiki Sadakiyo , Hebel J. R. , Toro Rodrigo , Nelson Erland 
Published: January 2005

AbstractStudy of the reflex heart rate response in humans to apneic facial immersion (simulated diving) and its modifications showed that bradycardia caused by simple application of cold compresses to the face (cold face test) correlated well with that produced by the simulated diving reflex. Bilateral application of cold stimulus to the individual divisions of the trigeminal nerve revealed the ophthalmic division to be the most sensitive pathway for this reflex. The cold face test was standardized in 50 normal individuals and further validated in 10 patients by comparison with the simulated diving reflex, the Valsalva maneuver, and administration of atropine. Patients with diabetes mellitus, brainstem stroke, multiple sclerosis, or Shy‐Drager syndrome developed less than normal bradycardia or minimal tachycardia in response to the cold facial stimulus. The cold face test is a novel, simple, safe, and economical test of the integrity of trigeminal‐brainstem‐vagal reflex pathways, can be utilized practically to assess vagal and brainstem dysfunctions, and has the special advantage of being applicable even in an uncooperative or comatose patient.
The multisensory physiological and pathological vertigo syndromes
Authors: Brandt Thomas , Daroff Robert B. 
Published: January 2005

AbstractVertigo occurs with either physiological stimulation or pathological dysfunction of any of the three stabilizing sensory systems: vestibular, visual, and somatosensory. The physiological syndromes, induced by intersensory or intrasensory mismatches, include motion sickness and space sickness as well as height, visual, somatosensory, auditory, head‐extension, and bending‐over vertigo. This review emphasizes the relationship between these physilological forms of vertigo and the pathological clinical vertigo syndromes.
Pure axonal neuropathy: Nerve xenografts and clinicopathological study of a family with peripheral neuropathy, hereditary ataxia, focal necrotizing encephalopathy, and spongy degeneration of brain
Authors: Appenzeller O. , Kornfeld Mario , Atkinson Ruth 
Published: January 2005

AbstractThree family members had the unusual combination of severe peripheral neuropathy, atypical hereditary ataxia, spongy degeneration of cerebral hemispheres, and cerebellar and brainstem foci of necrotizing encephalopathy, proved pathologically in one autopsied case. A sural nerve from a patient, devoid of myelinated fibers, was transplanted into thymectomized, lethally irradiated, and bone marrow reconstituted adult mice. A normal number of myelinated fibers was present in the grafts eleven weeks later. Eighteen weeks after grafting, mice were reconstituted with syngeneic thymus to return immunocompetence. Schwann cells in the graft were rejected and axons became totally denuded of myelin after thymus reconstitution. The peripheral neuropathy is thus due to axonal disease since human Schwann cells were capable of normally myelinating regenerating mouse axons. A puzzling feature after rejection was the absence of myelin debris containing macrophages in the grafts. It is suggested that part of the rejection process in this model is mediated by antibody rather than by cellular mechanisms.