bluuebunny/crossref_metadata_2025_split huggingface dataset:
Neuropathy and mitochondrial myopathy
Authors: Peyronnard Jean‐Marie , Charron Louise , Bellavance Andrè , Marchand Luc 
Published: January 2005

AbstractIt has been stated that peripheral neuropathy can be a feature of so‐called ophthalmoplegia‐ plus syndrome, but to date one case of hypertrophic neuropathy has been reported. This study is concerned with the clinical, electrophysiology, and pathological observations made in a 37‐year‐old man with progressive extyernal ophthalmcmetric studi9ees of the sural nerve revealed a marked loss of large myelinated fibers and an occasional degenerating axon. Residual fibers had disproportionately thin myelin sheaths in reelation to axon calibedrs. In contrast to the muscle biopsy findings, no mitochondrial paracrystaline inclusions were observed in the nerve. However, the number of mitochondria per square micron of Schwann cell cytoplasm was wlevated when compared with values obtained from normal subjects and a patient with a chronic neuropathy. These findings may indicate an alteration of mitochondrial function common to muscle fibers and Schwann cels which, in nerves, could lead to axon loss and abnormality of myelination.
Parkinson disease, dementia, and alzheimer disease: Clinicopathological correlations
Authors: Boller Fraņois , Mizutani Tomohiko , Roessmann Uros , Gambetti Pierluigi 
Published: January 2005

AbstractClinical records and neuropathological specimens from 36 patients with autopsy‐demonstrated idiopathic Parkinson disease (PD) were reviewed independently and the results compared. Nine (31%) of the 29 patients with adequate clinical data had severe dementia and 7 (24%) had mild dementia. The cerebral cortex showed senile plaques and fibrillary tangles in 15 of the 36 patients (42%). These changes were found in all 9 patients with severe dementia, in 3 of the 7 with mild dementia, and in 3 of the 13 patients with normal mental status.The prevalence of pathologically established Alzheimer changes and dementia among the patients with PD (33%) was over six times that found in an age‐matched population (5.1%). Survival after the onset of PD with Alzheimer disease was shorter than in PD without Alzheimer disease.
Acute parotitis and hyperamylasemia following whole‐brain radiation therapy
Authors: Cairncross J. Gregory , Salmon Jane , Kim Jae‐Ho , Posner Jerome B. 
Published: January 2005

AbstractParotitis, an infrequent, previously unreported complication of whole‐brain radiation therapy, was observed in 4 patients. The acute symptoms, which include fever, dry mouth, pain, swelling, and tenderness, are accompanied by hyperamylasemia. Among 10 patients receiving whole‐brain irradiation, 8 hac serum amylase elevations without symptoms. Both acute parotitis and asymptomatic hyperamylasemia result from irradiation of the parotid glands.
Heart rate changes in convulsive and nonconvulsive neonatal apnea
Authors: Fenichel Gerald M. , Olson Barbara J. , Fitzpatrick Jeri E. 
Published: January 2005

AbstractOne hundred twelve apneic spells of at least 4 seconds' duration were monitored in 15 neonates; 77 episodes were associated with convulsive discharges on the electroencephalogram and 35 were not. Convulsive apnea did not occur in conjunction with bradycardia. Nonconvulsive apnea of less than 20 seconds' duration occurred in the presence of variable heart rates, but nonconvulsive apnea of 20 seconds or longer was nearly always associated with bradycardia.
Lymphocyte responsiveness to acetylcholine receptor in rats with experimental autoimmune myasthenia gravis
Authors: Noguchi Sadako A. , Drachman Daniel B. , Adams Robert N. , Hirsch Robert L. 
Published: January 2005

AbstractWe examined the time course of lymphocyte responsiveness to acetylcholine receptor (AChR) in rats with experimental autoimmune myasthenia gravis (EAMG). Rats were immunized with purified torpedo AChR. At intervals of one to eight weeks later, lymphocytes from the lymph nodes and spleen were cultured with purified torpedo AChR and rat muscle extract containing AChR. Lymphocyte responsiveness (stimulation index) was determined from uptake of 3H‐labeled thymidine by the cultured cells. The response of lymphocytes to torpedo antigen began earlier and rose more rapidly than that to the homologous (rat) antigen. Lymph node cells responded more promptly than spleen lymphocytes. The stimulation indexes peaked at four to six weeks while antibodies to both antigens continued to rise. Delineation of this pattern of lymphocyte responsiveness sheds further light on the pathogenesis of the autoimmune response in EAMG and will be useful in the future design of immunotherapeutic strategies.
Progressive central nervous system deterioration: A complication of advanced chronic lung disease of prematurity
Authors: Ellison Patricia H. , Farina Matthew A. 
Published: January 2005

AbstractFifteen premature infants with severe chronic lung disease of prematurity were treated with ventilatory assistance and continuous positive airway pressure for at least 28 days. Fourteen developed progressive central nervous system deterioration following right heart failure and pulmonary hypertension. Two patterns of deterioration were observed: (1) cerebral, with loss of developmental milestones, increasing hypotonia, and eventual EEG deterioration and death; and (2) brainstem, with progressive apnea, bradycardia, or both, and sudden death or irreversible apnea.
Acne fulminans with inflammatory myopathy
Authors: Noseworthy J. H. , Heffernan L. P. , Ross J. B. , Sangalang V. E. 
Published: January 2005

AbstractAn 18‐year‐old man with acne fulminans developed an acute inflammatory myositis, an association not previously reported. The condition resolved with treatment using systemic corticosteroids.
Progressive white matter destruction following irradiation of an extracranial neoplasm
Authors: Rottenberg David A. , Horten Bruce , Kim Jae‐Ho , Posner Jerome B. 
Published: January 2005

AbstractAlthough numerous “cures” have been reported following surgical extirpation of symptomatic foci of cerebral radiation necrosis, delayed progressive white matter destruction and neurological deterioration may occur in some patients who survive for prolonged periods after operation. The postoperative appearance on CT scans of hypodensity within heavily irradiated white matter structures at a distance from the initial radionecrotic focus or operative site suggests continuing radiation‐induced tissue injury and a poor prognosis. Anticipated survival as well as administered radiation dose must be taken into account when “safe” radiation thresholds are calculated.
C‐6 Glioma Growth in Rats: Suppression with a β‐Adrenergic Agonist and a Phosphodiesterase Inhibitor
Authors: Chelmicka‐Schorr Ewa , Arnason Barry G. W. , Holshouser Sidney J. 
Published: January 2005

AbstractRat C‐6 glioma possesses a β‐adrenergic receptor which, when activated, raises intracellular levels of cyclic adenosine monophosphate (cAMP) in cultured C‐6 glioma cells. The present study shows that growth of C‐6 glioma is suppressed in rats treated with the β‐adrenergic agonist isoproterenol. Addition of papaverine, a cAMP phosphodiesterase inhibitor, to the treatment schedule augments this effect. Pharmacological agents that elevate intracellular cAMP levels may retard the growth of neural tumors in vivo.
Altered protein composition of isolated human cortical neurons in alzheimer disease
Authors: Selkoe Dennis J. 
Published: January 2005

AbstractTo characterize the protein composition of degenerating neurons in Alzheimer disease, enriched fractions of isolated cortical neurons from postmortem Alzheimer brain were compared by ultrastructural and biochemical techniques to neuronal isolates from aged normal controls and from patients with the nonfibrillary degenerative dementia, Huntington disease. Electron microscopy of isolated neurons showed well‐preserved organelles, including nuclear membranes, mitochondria, endoplasmic reticulum, ribosomes, and lipofuscin. Abundant paired helical filaments (PHF) were preserved in affected Alzheimer perikarya. In both sporadic and familial Alzheimer disease, sodium dodecylsulfate‐polyacrylamide gel electrophoresis revealed marked augmentation of a 20,000 mw protein (P20) in neuronal fractions from affected cortex compared to Huntington disease patients and controls. P20 was commonly increased in hippocampal, temporal, and frontal neurons but not in cortex free of PHF. The augmentation of P20 in Alzheimer disease samples appeared to correlate with the presence of PHF‐bearing neurons in the fractions. The increase in P20 in Alzheimer disease was observed in the presence of proteolysis inhibitors, in reverse‐sieved samples, and in whole cortical homogenates. Neurofilament proteins, tubulins, and a 50,000 mw protein showed no changes. In Huntington disease, marked augmentation of a 49,000 mw protein was found in striatal but not cortical homogenates. The relationship of these findings to the biochemistry of brain fibrous proteins and to the molecular pathology of Alzheimer disease is discussed.
Time modulation of spike‐and‐wave activity in generalized epilepsy
Authors: Kellaway Peter , Frost James D. , Crawley James W. 
Published: January 2005

AbstractNineteen patients, aged 4 to 44 years, with generalized epilepsy and generalized 3 Hz spike‐and‐wave activity, were monitored for 24 or 36 hours on one to nine occasions to determine the time distribution of ictal and interictal electroencephalographic discharges and the consistency of the time patterns observed. Two patients with a low incidence of spike‐and‐wave activity had a random pattern of occurrence; the other 17 showed one of three types of time modulation. The latter can be explained by the interaction of two modulative processes, one with a period of about 100 minutes and the other of approximately 24 hours. The pattern observed depends on the phase angle of the two cyclic processes. Treatment with ethosuximide altered the pattern of time modulation, and eventually, when the spike‐and‐wave activity was reduced to a low level, the time distribution became random.
Seasonal (circannual) periodicity of spontaneous intracerebral hemorrhage in minnesota
Authors: Ramirez‐Lassepas Manuel , Haus Erhard , Lakatua David J. , Sackett Linda , Swoyer Jackie 
Published: January 2005

AbstractSeasonal periodicity in the onset of spontaneous intracerebral hemorrhage was studied in 118 consecutive cases occurring during a six‐year span. The patients were urban residents of eastern Minnesota, a region characterized by wide seasonal fluctuations in daylight and temperature. The greatest number of cases consistently occurred each year during January and February. Circsianual (about one year) periodicity was demonstrated by statistical analysis using rhythmometric techniques. This periodicity coincided with that reported for mortality from cerebrovascular and cardiovascular diseases in the United States and elsewhere. Available information suggests that in populations at high risk for vascular diseases, climatic conditions might act as synchronizers to endogenous rhythms influencing the periodic occurrence of pathological vascular events.
Spasms of the iris sphincter
Authors: Stanley Thompson H. , Corbett James J. 
Published: January 2005

AbstractA patient is described who suffered from intermittent spasms of the pupillary sphincter and ciliary muscle. Both intraocular muscles contracted several times a minute, producing sporadic darkening of vision in the affected eye. The spasms continued for eleven days, so it was possible to document them pupillographically. This curious phenomenon seems to have been the result of a transient “irritability” of the ciliary ganglion.
Nonaminergic striatal neurons convert exogenous l‐dopa to dopamine in parkinsonism
Authors: Melamed Eldad , Hefti Franz , Wurtman Richard J. 
Published: January 2005

AbstractIn intact striatum, the enzyme dopa decarboxylase is localized predominantly in dopaminergic nerve terminals. In Parkinson disease, loss of dopaminergic neurons is associated with massive depletion of striatal decarboxylase activity. Nevertheless, efficacy of exogenous L‐dopa in parkinsonism is generally believed to result from its enzymatic decarboxylation to dopamine in the corpus striatum. It has previously been suggested that, after degeneration of nigrostriatal pathways, decarboxylation of administered L‐dopa may occur mainly at such striatal sites as surviving dopaminergic terminals, serotonergic neurons, or capillaries; but currently available data do not favor these hypotheses.Recent experimental studies indicate that a substantial amount of decarboxylase activity is localized in striatal interneurons or efferent neurons that may not normally synthesize monoamines. We propose that after depletion of dopaminergic terminals, these nonaminergic striatal neurons may contain a large fraction of residual dopa decarboxylase activity and may represent an important locus for conversion of administered dopa to functional dopamine in the parkinsonian corpus striatum.
Amyotrophic lateral sclerosis among chamorro migrants from guam
Authors: Garruto Ralph M. , Gajdusek D. Carleton , Chen Kwang‐Ming 
Published: January 2005

AbstractAmyotrophic lateral sclerosis (ALS), which occurs in unusually high incidence among the Chamorro people on the island of Guam, has developed in 28 Chamorro migrants‐24 of whom had onset in the United States, Japan, Germany, or Korea‐‐after periods of absence from Guam of 1 to 34 years. Thus, the latency period for the disease, if caused by environmental factors on Guam, may be over three decades. Four further patients developed ALS within 1 to 14 years of their return to Guam after long‐term residence in the continental United States. The minimum exposure time to environmental variables on Guam, based on age at migration, was 18 years, and all patients had spent their childhood and adolescence on Guam. Estimates of crude mortality rates for ALS from these data are considerably higher than for the United States population, and lower than the ALS mortality rates for nonmigrant Chamorros on Guam.
Mollaret meningitis: Report of a case with recovery after colchicine
Authors: Mora Jesus S. , Gimeno Alberto 
Published: January 2005

AbstractA 13‐year‐old boy developed closely spaced episodes of recurrent febrile meningitis that persisted for 22 months despite antibiotic therapy. During exacerbations the cerebrospinal fluid contained up to 2,500 leukocytes per cubic millimeter, including up to 20% activated monocytoid cells, with high IgM and IgG levels. No cause for the disorder was found, but steroids improved the condition and colchicine therapy apparently cured it.
Normal pyruvate dehydrogenase complex activity in patients with Friedreich's ataxia
Authors: Constantopoulos George , Chang Charles S. C. , Barranger John A. 
Published: January 2005

AbstractNormal activity of the pyruvate dehydrogenase complex was found in platelet‐enriched preparations from two siblings with Friedreich's ataxia who had been reported previously as being deficient in this enzyme.
Brainstem auditory evoked responses in spinocerebellar engeneration and wilson disease
Authors: Fujita Masashi , Hosoki Masako , Miyazaki Motoji 
Published: January 2005

AbstractBrainstem auditory evoked responses (BAERs) were studied in 30 healthy subjects, 20 patients with spinocerebellar ENgeneration (SCD), and 6 patients with Wilson disease. In addition to the standard 8 click per second stimulation rate, increased stimulation frequency was applied which identified wave V for its stability under different stimulation rates. No prolongation of wave latencies was noted in SCD patients, irrespective of clinical features, except in 1 patient who had a latency slightly above the normal range. Wilson disease with neurological symptoms produced prolongation of wave latencies in every case, whereas the patients without neurological involvement showed a normal BAER pattern.
Lisuride in parkinsonism
Authors: Parkes J. D. , Schachter M. , Marsden C. D. , Smith B. , Wilson A. 
Published: January 2005

AbstractLisuride is a soluble ergolene derivative with endocrine effects similar to but more potent than those of bromocriptine. In nine subjects with idiopathic, postencephalitic, or drug‐induced parkinsonism, lisuride at a dosage of 0.05 to 0.15 mg intravenously caused an immediate improvement in tremor, rigidity, akinesia, and postural deformity, but also caused chorea and orofacial dyskinesia. Improvement lasted 2 to 3 hours. Lisuride had little or no effect in a single patient with progressive supranuclear palsy. Oral lisuride therapy, 0.8 to 4.8 mg daily, had similar effects but occasionally caused reduced awareness and hallucinations.
Nerve fiber conduction velocity distributions: Studies of normal and diabetic human nerves
Authors: Cummins Kenneth L. , Dorfman Leslie J. 
Published: January 2005

AbstractWe have assessed the clinical applicability of a previously ENscribed new method for analyzing the distribution of conduction velocities (DCV) in peripheral nerve. Surface‐recorded median sensorimotor compound action potentials (CAPs) were found to contain contributions from classes of fibers conducting between 25 and 80 m/sec in the elbow‐to‐axilla segment. Test‐retest correlation coefficients ranged from 0.91 to 0.98. Increasing stimulus intensity recruited additional fiber activity into the CAP with relative preferential activation of faster‐conducting fibers at lower stimulus levels. Changes in limb temperatur altered the conduction velocity of all fiber classes 4% per ENgree Celsius. A comparison of fiber diameter distribution and DCVs in a normal sural nerve gave a correlation of 0.70 over the range of 7 to 12 μ. Diabetic patients with minimal or no clinical polyneuropathy showed varying ENgrees of DCV shift toward the slower conduction velocities, sometimes even in the face of normal conventional nerve conduction studies. These findings indicate that the DCV may be consiENred the electrophysiological counterpart—not equivalent—of the fiber diameter distribution, with the capacity to distinguish subtle differences in conduction properties of normal and diseased human nerves.
Postictal pleocytosis
Authors: Schmidley James W. , Simon Roger P. 
Published: January 2005

AbstractSix patients showed a transient and otherwise unexplained cerebrospinal fluid (CSF) pleocytosis following a flurry of generalized convulsions. Each had an obvious cause for repeated seizures. No evidence was found for an infectious, inflammatory, neoplastic, or other cause for the pleocytosis. All CSF specimens were clear and colorless, under normal pressure, and bacteriologically sterile. The maximal leukocyte count ranged from 9 to 80 per cubic millimeter and reached a maximum on the day after cessation of convulsions. No specimen contained more than 650 erythrocytes. Two patients initially had a mildly increased CSF protein; glucose values were unremarkable.We propose that the pleocytosis in these patients was a result of frequently repeated generalized convulsions. The mechanism of postictal pleocytosis is uncertain. It may result from transient breakdown of the blood‐brain barrier, which has been demonstrated after seizures in experimental animals. Although infectious causes must first be considered and rigorously searched for, it appears that seizures alone may cause a transient CSF pleocytosis.
Vestibular testing in basilar artery migraine
Authors: Eviatar Lydia 
Published: January 2005

AbstractVestibular function studies were performed in 20 children with symptoms suggesting basilar artery migraine. Sixteen children had abnormalities on bithermal caloric testing. Labyrinthine preponderance was found in 1 child with labyrinthine concussion and in 6 children with symptoms suggesting ischemia of the territory supplied by the basilar artery and the occipital branches of the posterior cerebral arteries. Directional preponderance was found in 4 children with symptoms suggesting ischemia of the posterior temporal branches of the posterior cerebral arteries. Five children had both labyrinthine and directional preponderance. Normal electronystagmographic findings were present in 4 children and correlated clinically with a milder illness. Head trauma was the triggering factor for the migraine in 5 children. Five children developed complex partial seizures and had evidence of directional preponderance (2 children) and combined labyrinthine and directional preponderance (2 children).The presence of abnormalities on electronystagmography in children with basilar artery migraine correlates with the severity of the disease. Directional preponderance or labyrinthine and directional preponderance are more frequently correlated with ischemia in the posterior temporal branches of the posterior cerebral arteries and may suggest a higher risk of developing vertiginous seizures when ischemia is prolonged.
Vascular adrenergic receptor responses in skeletal muscle in myotonic dystrophy
Authors: Mechler F. , Mastaglia F. L. 
Published: January 2005

AbstractThe pharmacological responses of vascular adrenergic receptors to intravenously administered epinephrine, phentolamine, and propranolol were assessed by measuring muscle blood flow (MBF) changes in the tibialis anterior muscle using the xenon 133 clearance technique and were compared in 8 normal subjects and 11 patients with myotonic dystrophy. In cases with advanced involvement of the muscle, the resting MBF was reduced and was not significantly altered by epinephrine before or after α‐ or β‐receptor blockade. In patients in whom the tibialis anterior muscle was normal or only minimally affected clinically, a paradoxical reduction in the epinephrine‐induced increase in MBF was found after a blockade by phentolamine, and the epinephrine‐induced MBF increase was not completely blocked by propranolol as in the normal subjects. These findings point to a functional alteration in the properties of vascular adrenergic receptors in muscle in myotonic dystrophy. While this may be another manifestation of a widespread cell membrane defect in the disease, the possibility that the changes are secondary to the myotonic state cannot be excluded.
Progressive sensory neuropathy in patients without carcinoma: A disorder with distinctive clinical and electrophysiological findings
Authors: Kaufman Michael D. , Hopkins Linton C. , Hurwitz Barrie J. 
Published: January 2005

AbstractSeven patients with severe progressive impairment of kinesthetic sense, mild dysfunction of cutaneous sense, and sparing of motor function were examined during a 3‐year period. The clinical and electrophysiological findings are described in detail. None of these seven has had evidence of cancer despite a thorough investigation and a 3‐ to 16‐year (average, 7 years) period of symptoms. These patients' symptoms were indistinguishable from those of patients with sensory neuropathy and coexistent carcinoma, suggesting that progressive sensory neuropathy is not invariably associated with carcinoma.