bluuebunny/crossref_metadata_2025_split huggingface dataset:
Concanavalin a binding of the cell surface of Duchenne muscle in vitro
Authors: Heiman‐Patterson Terry D. , Bonilla Eduardo , Schotland Donald L. 
Published: January 2005

AbstractUltrastructural examination of concanavalin A (Con A) binding sites in cultured skeletal muscle from six patients with Duchenne muscular dystrophy (DMD) revealed no abnormalities at the cell surface when compared to normal cultured skeletal muscle. Reaction product at the cell surface appeared regular and continuous in both normal and dystrophic cultured muscle cells. These findings indicate that the abnormalities of Con A surface binding found in biopsied muscle of DMD are not present in aneurally cultured dystrophic muscle cells.
Entrapment of the suprascapular nerve at the spinoglenoid notch
Authors: Aiello I. , Serra G. , Traina G. C. , Tugnoli V. 
Published: January 2005

AbstractA 58‐Year‐old fencing instructor developed selective denervation of the infraspinatus muscle. It was found to be caused by entrapment of the suprascapular nerve at the spinoglenoid notch due to a hypertrophied inferior transverse scapular ligament.
Electrophysiological and clinical correlation in myasthenia gravis
Authors: Oh Shin J. , Eslami Nasrollah , Nishihira Takeo , Sarala P. K. , Kuba Tadashi , Elmore Robert S. , Sunwoo Il Nam , Ro Young Il 
Published: January 2005

AbstractRepetitive nerve stimulation testing of the ulnar nerve was systematically performed on 21 normal controls and 120 patients with myasthenia (MG). Diagnostic sensitivity increased from 0% in MG in remission and 17.2% in ocular MG to 100% in severe generalized MG. Six types of responses were observed in MG and could be classified into two distinct patterns based upon disease severity: (1) in mild MG, an abnormal decremental response at low rate of stimulation, normal response at high rate of stimulation, and prominent posttetanic facilitation and exhaustion phenomena; and (2) in severe MG, abnormal decremental responses at low as well as high stimulation rates and less common posttetanic facilitation and rare posttetanic exhaustion phenomena. This difference is most likely due to the severity of the neuromuscular block in MG.
Facioscapulohumeral dystrophy associated with hearing loss and coats syndrome
Authors: Taylor Donald A. , Carroll James E. , Smith Morton E. , Johnson Mary O. , Johnston Glen P. , Brooke Michael H. 
Published: January 2005

AbstractTwo unrelated cases of infantile facioscapulohumeral dystrophy (FSHD) with Coats syndrome of the retina and sensorineural hearing loss are described. Children with otherwise unexplained visual or hearing deficit should be examined for somatic muscle weakness, which may present only as facial weakness. Early recognition of Coats syndrome in children with FSHD may lead to preservation of vision with appropriate therapy.
Voluntary alteration of visual evoked potentials
Authors: Bumgartner James , Epstein Charles M. 
Published: January 2005

AbstractFifteen normal adults had pattern‐shift visual evoked potentials (PSVEPs) using monocular checkerboard pattern reversal. Subjects were instructed to concentrate on the pattern in the first set of trals and then to avoid perceiving it in the second set. Direct, continuous visual observation ensured that the eye remained open and fixed on the pattern. Nonetheless, a third of the subjects could spontaneously alter or obliterate the PSVEP using several maneuvers, including meditation, daydreaming, and convergence. Several could produce shifts in apparent PSVEP latency. With explicit instruction, most subjects could learn to alter their PSVEP by maneuvers that are inapparent even to an observant technician.
Diffuse axonal injury due to nonmissile head injury in humans: An analysis of 45 cases
Authors: Adams J. Hume , Graham D. I. , Murray Lilian S. , Scott Grace 
Published: January 2005

AbstractForty‐five cases of diffuse axonal injury (DAI) brought about by nonmissile head injury in humans are analyzed and compared with 132 cases of fatal head injury without DAI. All cases were subjected to a comprehensive neuropathological study. In the patients with DAI a statistically significant lower incidence of lucid interval, fracture of the skull, cerebral contusions, intracranial hematoma, and evidence of high intracranial pressure were found, with a higher incidence of head injury due to road traffic accident. Brain swelling and hypoxic brain damage were not statistically different in the two groups. The features of DAI in humans are compared with the DAI that has been produced in subhuman primates by pure inertial loading brought about by angular acceleration of the head. The available evidence indicates that DAI in human beings occurs at the time of head injury and is not due to complicating factors such as hypoxia, brain swelling, or raised intracranial pressure.
Epidemiological surveillance of amyotrophic lateral sclerosis and parkinsonism‐dementia in the commonwealth of the Northern Mariana Islands
Authors: Yanagihara R. T. , Garruto R. M. , Gajdusek D. C. 
Published: January 2005

AbstractAmyotrophic lateral sclerosis (ALS) and parkinsonism‐dementia (PD), two fatal neurological diseases of unknown cause, occur in high incidence among the Chamorro people of Guam, the largest and southernmost island within the Mariana archipelago. To reassess and extend our present epidemiological knowledge of these degenerative diseases in this focal geographical region, a systematic search for both disorders was conducted on the remaining inhabited islands of Rota, Tinian, Saipan, and the four remote islands of Anatahan, Alamagan, Pagan, and Agrihan within the Marianas chain. One case of ALS (on Saipan), 2 cases of PD (on Rota and Saipan), and 6 cases of parkinsonism without dementia (2 on Rota, 3 on Saipan, 1 on Tinian) were encountered among the approximately 17,000 inhabitants. No cases of either ALS, PD, or parkinsonism were found in the four remote Northern Islands. An additional 22 cases of ALS and 8 cases of PD were identified from reports of previous case‐finding surveys, hospital records, and death certificates. Among Chamorros born on Rota, the average annual age‐adjusted mortality rates of ALS per 100,000 population were 37.7 for the 15‐year period 1956 to 1970 and 22.5 for the past decade, 1971 to 1980. Among Chamorros born on Saipan, the average annual mortality rates were 7.2 and 3.2 per 100,000, respectively, for the two periods. The mortality rates of PD were also significantly lower on Saipan than on Rota. In general, the age‐adjusted mortality rates of ALS and PD on Rota were similar to those currently observed on Guam. Since the origins and current genotypic composition of Chamorros on all the Mariana Islands are indistinguishable, the strikingly lower mortality rates of ALS and PD on Saipan suggest that environmental factors are far more important than genetic factors in the pathogenesis of these diseases.
Effect of a single oral dose of propranolol on essential tremor: A double‐blind controlled study
Authors: Calzetti S. , Findley L. J. , Gresty M. A. , Perucca E. , Richens A. 
Published: January 2005

AbstractThe effect of a single oral dose of propranolol (120 mg) on essential tremor was investigated in a double‐blind, placebocontrolled study in 26 patients. Hand tremor was recorded by means of accelerometers, and its frequency and amplitude calculated by using spectrum analysis. Recordings were made before and 1 1/2 hours after drug or placebo administration. Pretreatment tremor ranged from 4.2 to 9.6 Hz (median, 6.9 Hz) in frequency and from 0.002 to 1.33 cm (median, 0.014 cm) in amplitude. Neither propranolol nor placebo affected the frequency of the underlying tremor. The amplitude of tremor was reduced by 43 ± 11% (SEM) after propranolol (p < 0.01) and by 12 ± 8% after placebo (NS). The reduction observed after propranolol was significantly greater than that observed after placebo. Thre tremor response after propranolol correlated negatively with baseline frequency and positively with pretreatment amplitude, duration of tremor, and age of the patient. No significant relationships could be found between tremor response, serum propranolol levels, and degree of cardiac beta blockade as assessed by the inhibition of standing tachycardia. There was a clear tendency for patients with small tremor amplitude (less than 0.006 cm hand displacement) to show the least satisfactory response to propranolol. These results indicate that a single oral dose of propranolol is effective in producing a rapid and marked reduction of essential tremor. Measurement of pretreatment amplitude and frequency might by useful in predicting the therapeutic outcome in these patients.
No effect from double‐blind trial of physostigmine and lecithin in Alzheimer disease
Authors: Wettstein Albert 
Published: January 2005

AbstractEight patients with clinically diagnosed mild to severe Alzheimer disease were treated during two six‐week periods by a double‐blind, single‐crossover protocol with placebo or a combination of 18 gm of lecithin and 3 to 10 mg of oral physostigmine daily. No improvement in behavior, recent memory, or other neuro psychological functions occurred.
Cerebellar cortex ultrastructure in ataxia‐telangiectasia
Authors: Paula‐Barbosa M. M. , Ruela C. , Tavares M. A. , Pontes C. , Saraiva A. , Cruz C. 
Published: January 2005

AbstractAn ultrastructural study of a biopsy fragment from the cerebellar cortex of a patient with ataxia‐telangiectasia was performed. There was a sharp reduction in the number of Purkinje cells and an increase in the number of Golgi epithelial cells. Despite the degeneration of Purkinje cell dendrites, the volumetric density of parallel fiber varicosities and the number of synapses in the molecular layer were only slightly reduced. No qualitative nor quantitative abnormalities were noted in the granular layer.An analysis is made of the severe changes in cerebellar circuitry of this patient and their probable functional implications.
Dissociation of early SEP components in unilateral traumatic section of the lower medulla
Authors: Mauguière F. , Courjon J. , Schott B. 
Published: January 2005

AbstractSpinal and scalp early SEPs were recorded, using a noncephalic reference electrode, in a patient with a traumatic cervicomedullary lesion causing unilateral loss of position sense. Cervical N11 and N13 and scalp‐recorded far‐field P14 SEPs were clearly dissociated following stimulation of the affected side. The findings suggest that the P14 component is generated above the foramen magnum, whereas the cervical N13 has a spinal generator.
Neurotoxicity of radiological contrast agents
Authors: Junck Larry , Marshall William H. 
Published: January 2005

AbstractThe most important complications of intravascular administration of contrast agents include idiosyncratic (anaphylactoid) reactions, shock, congestive heart failure, cardiac arrhythmias, acute renal failure, and neurotoxic effects. The incidence of serious neurotoxic effects is low. Entry of contrast agents into the central nervous system normally is limited but may be increased by osmotic opening of the blood‐brain barrier with cerebral arteriography or arch aortography. Most neurotoxic effects are thought to represent direct effects of the contrast agent on brain or spinal cord. Adverse effects with arteriography include seizures, transient cortical blindness, brain edema, injury. Most cases of focal brain deficit (other than cortical blindness) are attributed to embolism secondary to the catheter. Seizures may occur with intravenous administration, especially in patients with brain tumors or other processes disrupting the blood‐brain barrier. The most important adverse effects observed with myelographic agents include acute and chronic meningeal reactions with iophendylate, and seizures and transient encephalopathy with metrizamide.
Regional metabolic changes in the spinal cord related to spinal shock and later hyperreflexia in monkeys
Authors: Schwartzman Robert J. , Eidelberg Eduardo , Alexander Guillermo M. , Yu Jen 
Published: January 2005

AbstractThe regional metabolic rate for glucose (rMRglu) was studied in macaque monkeys after spinal cord transection at T10, both during spinal shock and after reflexes had returned. The rMRglu was measured in all Rexed layers in cord segments both caudal and rostral to the level of the transection utilizing the quantitative [14C]deoxyglucose technique. The monkeys in spinal shock exhibited a significant increase in rMRglu in Rexed layer I and a significant reduction in layers VI to IX of the lumbosacral cord. In contrast, after reflexes had returned the rMRglu was increased in all Rexed layers of the lumbosacral cord; this increase was significant in layers I, V, and VI.
“Primary” spinal intramedullary lymphomas: A review
Authors: Hautzer N. W. , Robitaille Y. , Aiyesimoju A. 
Published: January 2005

AbstractPrimary spinal lymphoma carries a low incidence and poor prognosis. The most reliable diagnostic signs have been segmental swelling of the cord on myelography and increased cellularity of cerebrospinal fluid. Biopsies of spinal tumors or their cerebral extensions showed lymphoma of the non‐Hodgkin type only. Peroxidase‐antiperoxidase staining for light chains helps to distinguish well differentiated lymphomas from infectious processes in some instances. A lymphoma in an immunosuppressed patient fulfilling all criteria for primary growth in the spinal cord is described, and the case compared with others in the literature. Combined radiotherapy and chemotherapy provided a 48‐month survival in one patient.
Neurological complications of vasculitis
Authors: Moore Patricia M. , Cupps Thomas R. 
Published: January 2005

AbstractThe vasculitides are a group of disorders that include the polyarteritis nodosa group of systemic necrotizing vasculitides, hypersensitivity vasculitis, Wegeners's granulomatosis, lymphomatoid granulomatosis, giant cell arteritis, Behçet's disease, and isolated angiitis of the central nervous system. Classification is based on clinical, angiographic, and histological features. The frequency and distribution of neurological involvement vary with the underlying disorder and may provide the initial symptoms. Polyarteritis nodosa and Wegener's granulomatosis may affect the central and peripheral nervous systems, whereas isolated angiitis of the central nervous system and Behçet's disease affect the central nervous system alone. Neurological dysfunction occurs in 80% of patients with polyarteritis nodosa and fewer than 10% of patients with hypersensitivity vasculitis.The mechanism of neurological dysfunction in the vasculitides is tissue ischemia. The clinical effects of ischemia vary, and symptoms may be transient or prolonged. Mononeuritis multiplex, polyneuropathy, and stroke are frequent complications, but encephalopathies, cranial neuropathies, and brachial plexopathies are seen as well. The occurrence of symptoms late in the course of a disease suggests ischemia resulting from healed, scarred vessels as well as from those that are acutely inflamed; this is the case in Takayasu's arteritis and possibly also in polyarteritis nodosa. Treatment is based on identifying and removing the sensitizing agent when possible. Wegener's granulomatosis requires therapy with cyclophosphamide; temporal arteritis, with corticosteroids. In other vasculitides a balance must be reached between the progression of the disease and the side effects of immunosuppression.
Cerebellar blood flow and metabolism in cerebral hemisphere infarction
Authors: Wayne Martin W. R. , Raichle Marcus E. 
Published: January 2005

AbstractPositron emission tomography was used to study the effect of supratentorial infarction on cerebellar metabolic rate for oxygen and cerebellar blood flow. In a control group of patients, the mean cerebellar metabolic rate for oxygen was 2.97 ± 0.11 (standard error of the mean [SEM]) ml−1 · min−1 · hg−1 and mean cerebellar blood flow was 41.1 ± 1.5 ml · min−1 · hg−1. No significant right‐left asymmetry in either cerebellar metabolic rate for oxygen or cerebellar blood flow was noted. Patients with frontal lobe infarction showed 16.8 ± 1.8% (cerebellar metabolic rate for oxygen) and 19.6 ± 2.1% (cerebellar blood flow) differences between cerebellar hemispheres, with the hemisphere contralateral to the cerebral infarction having the lower values. These differences were highly significant (p < 0.001). In addition, cerebellar blood flow and cerebellar metabolic rate for oxygen were significantly decreased in the ipsilateral cerebellar hemisphere (metabolism: 2.13 ± 0.19 ml · min−1 · hg−1; p < 0.002; blood flow: 35.2 ± 2.4 ml · min−1 · hg−1; p < 0.05). Patients with parietooccipital infarction also showed a significant bilateral decrease in cerebellar metabolic rate for oxygen (2.43 ± 0.11 ml · min−1 · hg−1) and cerebellar blood flow (34.6 ± 2.5 ml · min−1 · hg−1) relative to control subjects, but no significant cerebellar asymmetry. Our findings demonstrate a general depression of cerebellar blood flow and metabolism from cerebral hemisphere infarction unrelated to the site of infarction as well as a specific depression occurring contralateral to infarction involving the frontal lobe. These are among the first quantitative data concerning regional cerebellar metabolic rates for oxygen and cerebellar blood flow in humans.
Neuropathological features of a lupus‐like disorder in autoimmune mice
Authors: Rudick Richard A. , Eskin Thomas A. 
Published: January 2005

AbstractNeurological syndromes are prominent in systemic lupus erythematosus, but the neuropathological mechanisms resulting in neurological dysfunction are unknown. We report a neuropathological study of the central nervous system in female NZB/W F1 mice, an animal model of systemic lupus erythematosus. NZB/W mice were studied at 3, 5, 8, 12, and 14 months of age, and 36‐month‐old female C57B16N/NIA mice were studied as aged controls. A lymphoproliferative process was identified in the central nervous system of 39% of 8‐ to 12‐month‐old and all 14‐month‐old NZB/W mice. Infiltrates of lymphocytes and plasma cells were seen in subarachnoid, choroid plexus interstitial, and Virchow‐Robin spaces. Lymphoid cells occasionally infiltrated brain parenchyma or accumulated as nodular masses. Concomitant visceral lymphoid infiltration was noted in 14‐month‐old mice. Dense deposits were seen ultrastructurally in the basal lamina of brain parenchymal capillaries of 14‐month‐old NZB/W mice. These dense deposits were similar in appearance to immune complexes described in glomerular basal lamina, and appeared concomitantly with an advanced lupus‐like glomerulopathy. Similar deposits were not observed in choroid plexus. The possible relevance of these neuropathological changes to human central nervous system lupus is discussed.
Natural killer cells activity in patients with multiple sclerosis given α interferon
Authors: Rice George P. A. , Casali Paolo , Merigan Thomas C. , Oldstone Michael B. A. 
Published: January 2005

AbstractThe purpose of this study was to examine the function and regulation of natural killer cells in vitro and in vivo in patients with multiple sclerosis. Of 12 patients who received 5 × 106 international units of human & interferon, 9 demonstrated an increase in natural killer cell activity within 48 hours as defined by the lysis of 51Cr‐labeled K‐562 cells. The activity was normal before treatment, unlike that of tumor‐bearing patients, and reached baseline levels within one week despite continuous interferon administration over the next six months. The same patients given a placebo preparation failed to show this enhanced natural killer cell activity.We also studied K‐562 killing in 36 other patients and age‐ and sex‐matched control subjects and were unable to demonstrate any differences between the two groups or any correlation of natural killer cell function with disease activity. The in vitro augmentation of natural killer cell activity by purified measles virions, which is associated with the release of interferon, and by the isolated glycoproteins of measles virus, which activates natural killer cells without the extracellular release of interferon, was similar in both patients and control subjects. Further, the proportion of cells defined by the monoclonal antibody HNK‐1, which defines both natural killer cells and a small subset of cytotoxic T lymphocytes, was normal. In patients with multiple sclerosis, the normality of natural killer cell function, as defined by these several interrelated assays, speaks against a defect in this nonspecific antiviral defense mechanism in the pathogenesis of the disease.
Schwann cell remyelination of demyelinated axons in spinal cord multiple sclerosis lesions
Authors: Itoyama Yasuto , Webster Henry DeF. , Richardson Edward P. , Trapp Bruce D. 
Published: January 2005

AbstractTo investigate remyelination in multiple sclerosis lesions, we immunostained spinal cord sections from patients with multiple sclerosis and neurologically normal (control) patients with antisera to P0 protein, a major constituent of peripheral nervous system myelin, and myelin basic protein, which is found in both central and peripheral nervous system myelin. In sections from five of the eight patients with no clinical or pathological evidence of neurological disease, P0 immunostaining was confined to peripheral myelin sheaths in dorsal and ventral roots. They were intensely stained, and peripheral‐central nervous system transition zones were clearly demarcated. Sections from the other three control patients contained a few P0‐stained sheaths in the central nervous system near root entry zones or among marginal glia near the dorsal sulcus. Spinal cord sections from six of the ten patients with multiple sclerosis contained clusters of myelin sheaths immunostained by P0 antiserum. These regenerating sheaths of peripheral nervous system origin were most numerous in large lesions and were commonly located in central areas or peripherally near root entry zones. The sheaths were observed frequently in areas of active demyelination and appeared morphologically normal even when surrounded by debris‐filled macrophages. Near margins of small inactive plaques were a few basic protein‐stained oligodendroglia extending processes to thin basic protein‐stained sheaths. These regenerating sheaths of central origin were relatively uncommon and were not observed in areas of active myelin breakdown. Our observations suggest that Schwann cell remyelination is more extensive in spinal multiple sclerosis lesions than previously thought. In very large spinal cord plaques, Schwann cells also apparently have a more important role in myelin sheath regeneration than do oligodendroglia.
Neurological complications of acquired immune deficiency syndrome: Analysis of 50 patients
Authors: Snider William D. , Simpson David M. , Nielsen Surl , Gold W. M. Jonathan , Metroka Craig E. , Posner Jerome B. 
Published: January 2005

AbstractFifty patients with acquired immune deficiency syndrome had complications affecting the central or peripheral nervous systems or both. The patients were either male homosexuals, intravenous drug abusers, or recently arrived Haitian refugees. They ranged in age from 25 to 56. Central nervous system complications were of four kinds: (1) Infections included Toxoplasma gondii abscesses in 5 patients, progressive multifocal leukoencephalopathy in 2, cryptococcal meningitis in 2, Candida albicans in 1, and possible Mycobacterium avium intracellulare in 3. Eighteen patients suffered a subacute encephalitis possibly attributable to cytomegalovirus infection. (2) Tumors consisted of primary lymphoma of the brain in 3 patients and meningeal invasion by systemic lymphoma in 4. (3) Vascular complications included nonbacterial thrombotic endocarditis in 2 patients and cerebral hemorrhages in the setting of thrombocytopenia in 3. (4) Undiagnosed central nervous system problems were evidenced as focal brain lesions in 3 patients and self‐limiting aseptic meningitis in 4. Peripheral neuropathy occurred in 8 patients.