bluuebunny/crossref_metadata_2025_split huggingface dataset:
Alpha‐pattern coma: 24 cases with 9 survivors
Authors: Grindal Alan B. , Suter Cary , Martinez A. Julio 
Published: January 2005

AbstractAlpha‐pattern coma denotes the association of a comatose state with an electroencephalogram consisting predominately of alpha‐frequency activity. Over the past three years we have studied 24 such cases: 14 following cardiopulmonary arrest, 6 following respiratory arrest, and 3 following brainstem infarction. Of the 9 patients who survived, only 1 had a significant neurological deficit. It was found that: (1) alpha‐pattern coma can result from a variety of neurological insults; (2) a significant proportion of patients survive, often with little or no deficit: (3) clinical evidence of intact brainstem function implies a favorable outcome; (4) the electroencephalogram was similar in survivors and nonsurvivors and did not differentiate diffuse cerebral dysfunction from focal brainstem disease; and (5) neuropathological studies demonstrated diffuse cortical as well as brainstem changes.
False diagnosis of orbital apex tumor by CAT scan in thyroid eye disease
Authors: Susac John O. , Martins Albert N. , Robinson Bernard , Corrigan Domenic F. 
Published: January 2005

AbstractComputerized transaxial tomography is now the procedure of choice in diagnosing exophthalmos. Caution must be exercised, however, in interpreting the results. The patient reported here had a swollen inferior rectus muscle that simulated an orbital apex tumor on CAT scan.
Isolated myopathic involvement of the diaphragmatic musculature in a neonate
Authors: Bergen Barry J. , Sangalang Virgilio E. , Aterman Kurt 
Published: January 2005

AbstractA myopathy restricted to the diaphragm was found in an infant who died at 3 months of age of respiratory failure due immobility of the diaphragm. Grossly, the diaphragm was thin and translucent. On microscopical examination few muscle bundles were seen. Most muscle fibers were small, and numerous necrotic fibers with large sarcolemmal nuclei, focal cytoplasmic basophilia, and hyaline or homogeneous eosinophilic degeneration in the center of the fibers were seen.
Ultrastructural lesions of muscle and immunofluorescent deposits in vessels in Reye's syndrome: A preliminary report of serial muscle biopsies
Authors: Hanson P. A. , Urizar R. E. 
Published: January 2005

AbstractThirteen sequential percutaneous skeletal muscle biopsy specimens from 4 patients with Reye's syndrome were studied ultrastructurally and by direct immunofluorescence. Prominent generalized intermyofibrillar edema, mitochondrial disruption, and swelling of vascular endothelium were demonstrated in 2 patients. In all 4 patients there were granular deposits of immunoglobulins G and M in intramuscular vessels, presumably representing antigen‐antibody complexes. We postulate that these changes may be pathogenetically important in this syndrome and suggest that muscle biopsy tissue be utilized for further investigation of Reye's syndrome.
Giant axonal neuropathy: A childhood disorder of microfilaments
Authors: Koch Thomas , Schultz Paul , Williams Roger , Lampert Peter 
Published: January 2005

AbstractA sural nerve biopsy was performed on an 8‐year‐old boy with a chronic, slowly progressive polyneuropathy. Light and electron microscopy as well as teased nerve‐fiber preparations demonstrated numerous giant axons filled with closely packed neurofilaments. Both myelinated and unmyelinated fibers were involved. Segmental demyelination, remyelination, and onion‐bulb formation by multiple Schwann cell processes were observed, suggesting recurrent Schwann cell dysfunction. Abundant aggregates of cytoplasmic microfilaments occurred in Schwann cells, endothelial cells, perineurial cells, endoneurial fibroblasts, and endomysial fibroblasts. These findings support the proposal that giant axonal neuropathy is a generalized disorder of cytoplasmic microfilaments and that segmental demyelination occurs concomitantly with axonal and Schwann cell disease. The pathogenesis of this rare disorder is discussed with reference to experimental toxic neuropathies.
Cerebral blood flow and metabolism in chronically hyperammonemic rats: Effect of an acute ammonia challenge
Authors: Gjedde Albert , Lockwood Alan H. , Duffy Thomas E. , Plum Fred 
Published: January 2005

AbstractThe effects of chronic hyperammonemia on cerebral metabolism were studied in rats four and eight weeks after the construction of a portacaval shunt. Compared to sham‐operated controls, shunted animals had increased arterial concentrations of ammonia and glutamine and decreased glutamate. Cerebral blood flow, measured by xenon 133 washout in animals lightly anesthetized with nitrous oxide, increased from a control of 91 ± 5 (mean ± SEM) to 139 ± 20 ml per 100 gm tissue per minute after shunting for eight weeks; however, the cerebral metabolic rate for oxygen was not different from control four or eight weeks after the shunting procedure. Following intraperitoneal administration of a small ammonium acetate load (2.6 mmol/kg), eight‐week portacaval animals consistently underwent a fall in cerebral blood flow and cerebral oxygen consumption and developed high‐voltage slow waves in the electroencephalogram. Glutamine was produced by the brains of all groups of animals; the cerebral metabolic rate for glutamine was greater than control in eight‐week portacaval rats, the only animals to show a net uptake of ammonia, along with nonspecific effects of chronic portal‐systemic shunting, may lead to uncoupling of cerebral blood flow and oxidative metabolism.
Benign cerebellar hemorrhages
Authors: Heiman Terry D. , Satya‐Murti Saty 
Published: January 2005

AbstractTwo patients with cerebellar hemorrhage and a benign outcome stress the potential for spontaneous resolution and clinical recovery in some cases of this disorder. In each patient, resolution of the hemorrhage was noted on the computed tomographic scan. Computed tomography may detect a cerebellar hemorrhage even though on clinical grounds a brainstem localization is favored.
Prolonged neonatal myasthenia gravis: Electrophysiological studies
Authors: Branch Clinton E. , Swift Thomas R. , Dyken Paul R. 
Published: January 2005

AbstractA female infant with neonatal myasthenia gravis remained weak for an excessively long period compared to the usual situation in this disease process. At 71 days of age, electrophysiological studies revealed evidence of a defect in neuromuscular transmission that improved with intravenous Tensilon therapy. Repeat study at 183 days of age was normal. This case represents an unusually long time for an affected infant to have neonatal myasthenia gravis. Electrophysiological studies are of value in the diagnosis and management of patients with neonatal myasthenia gravis.
Papillophlebitis: Benign retinopathy resembling papilledema or papillitis
Authors: Ellenberger Carl , Messner Kenneth H. 
Published: January 2005

AbstractThree young adult patients are described to illustrate an uncommon syndrome, papillophlebitis. This entity, characterized by mild but protracted monocular visual impairment, edema of the optic disc, retinal venous engorgement, and perivenous retinal hemorrhages, resembles atherosclerotic occlusion of the central retinal vein in older patients. In younger individuals it is benign and self limited, but may be incorrectly diagnosed as optic neuritis or papilledema. These alternatives can usually be excluded if tests of visual function do not indicate an optic nerve conduction defect and if the clinical presentation and examination of the opposite ocular fundus do not disclose evidence of increased intracranial pressure. Because resolution usually occurs spontaneously, immediate neuroroenotenographic tests may be inappropriate. Effective treatment has not been established; local instillation of corticosteroids may hasten recovery.
Sagittal sinus thrombosis as a complication of regional enteritis
Authors: Sigsbee Bruce , Rottenberg David A. 
Published: January 2005

AbstractA young woman with regional enteritis and a history of recurrent thrombophlebitis and pulmonary embolism developed sagittal sinus thrombosis during an acute exacerbation of her illness. Repeated attempts to document a hypercoagulable state were unsuccessful.
Late components of motor unit potentials in a patient with myoglobinuria
Authors: Pickett Jackson B. 
Published: January 2005

AbstractA 45‐year‐old woman had severe weakness, myoglobinuria, and elevated muscle enzyme levels in the serum after a flulike illness. Electromyography some four weeks later showed motor unit potentials with late components; these remained present on subsequent electromyographic examinations performed at approximately monthly intervals for the following year. The persistence of motor unit potentials with late components is taken to imply that the late components were caused either by sprouts that failed to undergo myelination or, more likely, by ectopically innervated muscle fibers.
Treatment of subarachnoid hemorrhage from ruptured intracranial aneurysm with tranexamic acid: A double‐blind clinical trial
Authors: Chandra Benjamin 
Published: January 2005

AbstractA double‐blind clinical trial of tranexamic acid was carried out on 39 patients with fresh subarachnoid hemorrhage from a ruptured aneurysm. Twenty patients received tranexamic acid, 6 gm daily for 14 to 21 days, while 19 patients received conventional therapy of bedrest and dexamethasone when cerebral edema developed, plus isotonic saline. Rebleeding and mortality were reduced by one‐fourth and one‐fifth, respectively (p < 0.001). No side‐effects were observed. Tranexamic acid is valuable in the treatment of subarachnoid hemorrhage caused by ruptured intracranial aneurysms.
Determination of immunoglobulin content of CSF based on light chain characteristics
Authors: Eickhoff K. , Heipertz R. 
Published: January 2005

AbstractThe immunoglobulin light chain (types kappa and lambda) content of normal cerebrospinal fluid is similar to that of normal serum. In inflammatory diseases of the central nervous system a shift in the k/1 ratio in comparison to serum values, usually a relative increase of Igk, can be observed. This increase of the k/1 ratio, though not specific for any disease, is most commonly found in multiple sclerosis. There is no correlation between the IgG content and the k/1 ratio. The methods described here measure bound and free light chains simultaneously. The calculations show that free light chains are present in both normal and inflammatory CSF and that they appear to be of polyclonal origin. The detection of light chain abnormalities in CSF can be taken as an indicator of endogenous immunoglobulin production in the CNS and is of significance for the diagnosis of inflammatory CNS processes, especially when other signs of endogenous immunoglobulin production are absent.
Kearns‐Sayre Syndrome with hypoparathyroidism
Authors: Horwitz Samuel J. , Roessmann Uros 
Published: January 2005

AbstractThe history, clinical findings, and postmortem exainination of a patient with Kearns‐Sayre syndrome are presented. In addition to the usual symptoms, the patient also had hypoparathyroidism. There is increasing evidence that the syndrome is frequently associated with involvement of the endocrine system. Central nervous system involvement consists of a spongy encephalopathy.
Anatomical correlates of electrical and behavioral events related to amygdaloid kindling
Authors: Engel Jerome , Wolfson Leslie , Brown Lucy 
Published: January 2005

AbstractAnatomical structures demonstrating increased glucose uptake during the various stages of amygdaloid kindling in rats were identified by the 14C‐2‐deoxyglucose (DG) autoradiographic technique. Partial (stages 1 and 2) seizures were correlated with increased DG uptake in the ipsilateral amygdala and its direct projection fields. The appearance of generalized motor (stages 3, 4, and 5) seizures was accompanied by less limbic involvement and recruitment of a bilateral system including substantia nigra, specific and nonspecific thalamic nuclei, globus pallidus, and neocortex. Increased hippocampal DG uptake was correlated with prolonged amygdaloid afterdischarge duration but not with the behavioral seizure stage. This study does not reveal which of these structures are responsible for the observed behavioral and electrical events and which are activated by them. It does suggest, however, that three discrete anatomical systems underlie the generation of partial seizures, generalized motor seizures, and local afterdischarge.
Further characterization of bound measles‐specific IgG eluted from SSPE brains
Authors: Mehta P. D. , Kane A. , Thormar H. 
Published: January 2005

AbstractTissue residues from 3 SSPE brains extracted at neutral pH were thoroughly washed with phosphate‐buffered saline and further extracted at acidic pH. The low‐pH extracts had significant measles antibody titers, and their measles‐specific IgG content ranged from 40 to 60% of total IgG. Agarose‐gel electrophoretic analysis of the extracts showed oligoclonal IgG bands, most of which disappeared after suitable absorption with measles virus, demonstrating the presence of restricted measles antibody population. Ouchterlony analysis showed a reaction of identity between bound IgG and IgG extracted at neutral pH. Thus IgG extracted at low and neutral pHs is remarkably similar.
Cytoplasmic filaments in intracerebral cortical vessels
Authors: Nag Sukriti , Robertson David M. , Dinsdale Henry B. 
Published: January 2005

AbstractActin filaments measuring 5 nm in diameter are present in the endothelium of systemic vessels and presumably have a contractile function that may be related to vascular permeability. Little attention has been directed to the presence of similar cytoplasmic filaments in the endothelium of cerebral vessels. This study was undertaken to determine whether filaments are present in the endothelial cells of intracerebral cortical vessels of rats and humans. Ultrastructural studies revealed two populations of endothelial cytoplasmic filaments 5 nm and 10 nm in diameter, respectively. The 5‐nm filaments were abundant in the endothelium of penetrating cortical arterioles, being grouped near the cytoplasmic margins in proximity to cell junctions and along the abluminal side of the endothelial cells. The filaments were scant in capillaries and venules.
Transaqueous diffusion of acetylcholine to denervated iris sphincter muscle: A mechanism for the tonic pupil syndrome (adie syndrome)
Authors: Wirtschafter Jonathan D. , Volk Charles R. , Sawchuk Ronald J. 
Published: January 2005

AbstractThe accepted hypothesis for the pathophysiology of tonic pupil syndrome (Adie syndrome) was rexamined in light of recent developments concerning deneravation supersensitivity of cholinergically innervated smooth muscle. Kinetic analysis suggests that enzymatic hydrolysis is unimportant relative to convective diffusion in the turnover of acetylcholine in the aqueous humor. We postulate that the greater response to near stimuli than to light stimuli and the delay in iris sphincter contraction and relaxation can be explained by release of acetylcholine from the neuromuscular junction of the ciliary muscle followed by transaqueous diffusion to receptor sites on denervated, supersensitive iris sphincter muscle.
Pseudoxanthoma elasticum: A review of neurological complications
Authors: Iqbal Aleem , Alter Milton , Lee Seungho H. 
Published: January 2005

AbstractA case of pseudoxanthoma elasticum with multisystem involvement is described. Neurological complications, as reported in the literature, are reviewed. These include cerebrovascular insufficiency, multiple lacunar infarcts, aneurysms, subarachnoid and intracerebral hemorrhages, progressive intellectual deterioration, and psychic and mental disturbance which may be due to cortical atrophy. Seizures occur more frequently than in the general population. Hypertension and alteration of cerebral vessels are the two basic pathophysiological mechanisms responsible for the neurological complications of this disease.
The morquio syndrome: Neuropathologyxs and biochemistry
Authors: Koto Atsuo , Horwitz Allen L. , Suzuki Kinuko , Tiffany Carol W. , Suzuki Kunihiko 
Published: January 2005

AbstractThe activity of N‐acetyl galactosamine‐6‐sulfate sulfatase was studied for the first time in the liver and brain of a patient with a clinically typical case of Morquio syndrome with keratosulfaturia. As has been demonstrated in the fibroblasts of patients with this syndrome, this enzymatic activity was markedly decreased in both organs.Neuropathological examination revealed moderately swollen neurons containing PAS‐positive, coarse globular inclusions in the cerebral cortex, Ammon's horn, basal ganglia, and thalamic nuclei. Ultrastructurally, the inclusions consisted of stacked, straight or loose, wavy membranes of various lengths, often associated with pale or moderately electron dense homogeneous “lipid droplets.” These ultrastructural features of the inclusions were closely similar to the granulomembranous bodies of Hurler syndrome and the inclusions described in type B of the Sanfilippo syndrome. Unlike those mucopolysaccharidoses, however, no abnormalities were found in the gangliosides in the brain of the patient with Morquio syndrome.
Nemaline (rod) myopathy: Thexs need for histochemical evaluation of affected families
Authors: Bender Adam N. , Willner Judith P. 
Published: January 2005

AbstractHistochemical changes in the mother of a patient with nemaline myopathy were used to identify her as the gene carrier even though rod‐bodies were not present in her muscle biopsy and she was not weak. The patient and her mother both had marked type I fiber predominance with large groups of type I fibers present. Histochemical changes known to occur in nemaline myopathy include smallness and predominance of type I fibers. Such changes support the concept that this disease may result from subtle defects in innervation since fiber types are determined by innervation. Although this disease is thought to be transmitted by autosomal dominant mode, lack of male‐to‐male transmission and a predominance of female cases in the literature suggest that this may be (1) an X‐linked dominant, (2) a sex‐influenced autosomal dominant, or (3) an autosomal dominant which is semilethal in males. The family described here is the first in which a presumably affected parent showed only the histochemical change without rod‐bodies, thus emphasizing the importance of histochemical evaluation of relatives' biopsies for genetic counseling.
Increased sodium plus potassium adenosine triphosphatase activity in erythrocyte membranes in Huntington's disease
Authors: Butterfield D. A. , Oeswein J. Q. , Prunty M. E. , Hisle K. C. , Markesbery W. R. 
Published: January 2005

AbstractDopa‐decarboxylase, acetylcholinesterase, sodium plus potassium stimulated adenosine triphosphatase (Na+ + K+ − ATPase), and membrane‐bound protein kinase were compared in the erythrocytes of patients with Huntington's disease and normal controls. All these enzymes also exist in the basal ganglia. The Na+ + K+‐ATPase level was elevated (p < 0.05) in Huntington's disease, while no significant changes were observed in the other enzymes. This finding is consistent with the concept that Huntington's disease is associated with a general membrane abnormality.
Cerebral hemodynamics and metabolism in pseudotumor cerebri
Authors: Raichle M. E. , Grubb R. L. , Phelps M. E. , Gado M. H. , Caronna J. J. 
Published: January 2005

AbstractA comprehensive analysis of cerebral hemodynamics and metabolism was carried out in 14 patients with pseudotumor cerebri. Tracer techniques were employed to measure cerebral blood flow (CBF) and vascular reactivity to acute changes in arterial carbon dioxide tension and blood pressure, cerebral blood volume (CBV), and the cerebral metabolic rate for oxygen and glucose. There was a small reduction (p < 0.01) in CBF (44 ± 7 ml/100 gm/min; normal, 54 ± 9) with normal vascular reactivity; an increase (p < 0.005) in CBV (4.8 ± 0.8 ml/100 gm; normal, 3.6 ± 0.5), and normal cerebral metabolism. We conclude that an abnormality of the cerebral microvasculature is responsible for an elevation in CBV, but the intracranial hypertension can be explained only by tissue swelling due to an increase in water content. The relationship between the vascular abnormality and the tissue swelling remains to be defined.
Sparing of the onufrowicz nucleus in sacral anterior horn lesions
Authors: Iwata Makoto , Hirano Asao 
Published: January 2005

AbstractSacral anterior horns were examined with special reference to the Onufrowicz nucleus in 3 patients with Werdnig‐Hoffman disease, 2 with healed anterior poliomyelitis, and 1 who had undergone amputation of both legs above the knee. None of these patients showed symptoms of either urethral or anal sphincter disturbances. The Onufrowicz nucleus of the second sacral anterior horn appeared normal in all the patients examined, though the lateral portion of that horn and the medial nuclear group of the third sacral segment were frequently involved. These findings support the view that the Onufrowicz nucleus may be innervating the external sphincter muscle of the urethra and the anus.
Weakness in malignancy: Evidence for a remote effect of tumor on distal axons
Authors: Barron Stephen A. , Heffner Reid R. 
Published: January 2005

AbstractMalignant tumors are known to have a “remote” or nonmetastatic effect on the central and peripheral nervous systems. Eight patients were seen with proximal muscle weakness in association with bronchogenic carcinoma (5), carcinoma of breast (2), and leukemia (1). Electomyography demonstrated small polyphasic motor unit potentials. Muscle biopsy, however, showed evidence of denervation. Electron microscopy revealed morphological abnormalities in the intramuscular segments of axons. This combination of EMG and biopsy findings is presented as evidence for a remote effect of tumor on the intramuscular distal axons.
Huntington'S disease: Treatment with muscimol, A GABA‐mimetic drug
Authors: Shoulson Ira , Goldblatt David , Charlton Maurice , Joynt Robert J. 
Published: January 2005

AbstractMuscimol, a γ‐aminobutyric acid (GABA) analogue that exerts potent and specific agonist effects on GABA receptors, was administered orally to 10 patients with Huntington's disease. In this double‐blind study, muscimol treatment did not result in improvement in these patients' motor or cognitive functions. However, muscimol adminsitration did ameliorate chorea in the most severely hyperkinetic patient, and it was associated with the appearance of dystonic features, electroencephalographic changes, and behavioral alterations in some patients. These latter observations support a functional relationship between GABA‐ergic activity and the genesis of both dystonia and EEG abnormalities in humans. The therapeutic failure of muscimol indicates that the GABA disturbance in Huntington's disease does not alone account for the clinical features of this disorder.This investigation was funded by a grant from the Hereditary Disease Foundation, Beverly Hills, CA. The University of Rochester Clinical Research Center is supported by U.S. Public Health Service Research Grant RR‐00044 from the Division of Research Facilities and Resources, National Institutes of Health. Muscimol was provided by Smith, Kline, & French Laboratories and formulated by the Strong Memorial Hospital Pharmacy.