Melricflash/CW_MedAbstractsAlt huggingface dataset:
Visually evoked potentials and electroretinography in neurologic evaluation. Electrophysiologic testing of the visual system requires primarily the ERG and the VEP. The flash electroretinogram provides information about the outer retina only. The pattern electroretinogram is derived from both the outer retina and the innermost retinal layers including the ganglion cell layer. The VEP is based on electrical information recorded from the visual cortex in response to stimulation of the retina. Thus, the integrity of the entire visual pathway can be tested. Localizing ability of the VEP is limited. Since the visual cortex is heavily weighted by representation of the central retina, peripheral lesions, including those producing peripheral visual field abnormalities that do not impinge upon central fixation, may produce relatively little disturbance of the VEP. As with most tests, electrophysiologic studies of the visual system must be placed in context of the entire examination, including the patient's history and neurologic and especially neuro-ophthalmologic evaluation. Electrophysiologic testing has three main uses in neurology. Pattern-reversal VEPs may be useful in detecting hidden visual loss in multiple sclerosis; VEPs and ERGs can distinguish function from organic visual loss; and VEPs and ERGs can be useful in the diagnosis of visual loss in nonverbal patients, especially in children. 

Category: nervous system diseases
Alexia without agraphia associated with spleniogeniculate infarction. Pure alexia, following an infarction in the distribution of the left posterior cerebral artery, is attributed to damage of the left occipital lobe and the splenium of the corpus callosum. We describe a case of pure alexia in a 57-year-old woman with infarction of the left lateral geniculate body and the splenium of the corpus callosum, a variation on this classic disconnection syndrome. 

Category: general pathological conditions
Myasthenia gravis. Myasthenia Gravis is a disorder of neuromuscular function resulting from an immunologically based premature destruction of acetylcholine receptors. The disease is characterized clinically by variable weakness accentuated by repetitive muscular activity and usually responding to the administration of acetylcholinesterase inhibitors. Myasthenia Gravis is a complex disease and requires understanding of the many facets of its natural history and immunological basis to ensure optimal individual patient management. The long-term goal is control of the immunological imbalance; treatment regimens include thymectomy, corticosteroids, azathioprine, and plasmapheresis. The common use of acetylcholinesterase inhibitors provides symptomatic relief during variable daily muscular activity. Disability due to myasthenia gravis is to a large extent reversible and death is preventable. Early recognition of myasthenia gravis and appropriate treatment are often rewarded by remission that may be permanent. 

Category: nervous system diseases
Correction of truncus arteriosus with truncal valvar stenosis or insufficiency using two homografts. Surgical correction of truncus arteriosus requires the creation of right ventricular to pulmonary artery continuity and closure of the ventricular septal defect. A variety of conduits have been used including valved and nonvalved. Despite a significant incidence of truncal valvar stenosis and insufficiency, this valve has seldom been replaced. We present 4 cases of truncus arteriosus with truncal valvar stenosis or insufficiency that were repaired using two valved homografts: one to create the pulmonary outflow tract and the other to replace the abnormal truncal valve. Two of these patients are doing well after 4 months. Another child survived the operation and did well for 2 months when she died suddenly. The last child died 14 hours postoperatively from low cardiac output syndrome secondary to diabetic hypertrophic cardiomyopathy. When truncal valvar abnormalities are present, the primary repair of truncus arteriosus in an infant should include replacement of the truncal valve. Total correction can be successfully achieved using two valved homografts, resulting in long-term palliation and freedom from thromboembolic events and the use of anticoagulants. 

Category: cardiovascular diseases
The rat glucagon gene is regulated by a protein kinase A-dependent pathway in pancreatic islet cells. A cAMP response element (CRE) has been identified in the proximal 5'-flanking region of the rat glucagon gene, and activation of the cAMP-dependent pathway in fetal rat intestinal cells leads to an increase in the levels of glucagon mRNA transcripts. In contrast, the human glucagon gene does not contain a similar CRE, and the results of studies using immortalized rat and hamster islet cell lines have suggested that glucagon gene expression may not be regulated by cAMP. To reconcile these observations, we have studied the control of glucagon gene expression. Incubation of primary rat islet cell cultures with forskolin in the presence of low (0.5 g/liter) or high (2.0 g/L) glucose resulted in a 2- to 3-fold increase in the levels of glucagon mRNA transcripts. Forskolin also stimulated the secretion and synthesis of immunoreactive glucagon. The importance of the protein kinase-A-dependent pathway in the regulation of glucagon gene expression was also examined in hamster islet InR1-G9 cells. Cotransfection of a glucagon-chloramphenicol acetyltransferase (CAT) fusion gene containing the glucagon CRE and a cDNA encoding the catalytic subunit of protein kinase-A resulted in stimulation of glucagon-CAT activity in hamster islet cells. Catalytic subunit cotransfection also activated somatostatin-CAT, but no activation of RSVCAT was detected. The results of these experiments suggest that the rat glucagon gene is regulated by a protein kinase-A-dependent pathway in the endocrine pancreas. 

Category: general pathological conditions
Reduction in incidence of inducible ventricular tachycardia after myocardial infarction by treatment with streptokinase during infarct evolution. The aim of this study was to determine whether intravenous streptokinase administered with or without oral aspirin to patients with evolving myocardial infarction reduces the inducibility of ventricular tachycardia at electrophysiologic study and thus the risk of sudden death in infarct survivors. Of 159 patients randomized at Westmead Hospital to the multicenter Second International Study of Infarct Survival (ISIS-2) after streptokinase and aspirin in acute myocardial infarction, 87 underwent electrophysiologic testing 6 to 28 days after infarction to determine their risk of subsequent ventricular arrhythmias (streptokinase 20 patients; aspirin 25 patients; streptokinase and aspirin 21 patients; both placebos 21 patients). Patients who underwent electrophysiologic testing had similar clinical characteristics to those of patients who did not. The stimulation protocol comprised up to and including four extrastimuli applied to the right ventricular apex at twice diastolic threshold. An abnormal result was defined as ventricular tachycardia with a cycle length greater than or equal to 230 ms lasting greater than or equal to 10 s. Ventricular tachycardia was inducible at electrophysiologic study in 8 patients who received placebo streptokinase, but in no patient who received active streptokinase (8 of 46 versus 0 of 41; p = 0.005, Fischer's exact test). Ventricular tachycardia was inducible in 4 patients who received aspirin therapy and 4 who did not (4 of 41 versus 4 of 46; p = NS). During a mean follow-up period of 39 +/- 9 months, there were no spontaneous episodes of ventricular tachycardia, ventricular fibrillation or witnessed sudden death in the streptokinase-treated group compared with three such events in the placebo-treated group (p = 0.13). When compared with placebo therapy, intravenous streptokinase substantially reduced the incidence of inducible ventricular tachycardia in infarct survivors. No similar benefit was attributable to aspirin therapy. 

Category: cardiovascular diseases
Post-traumatic intracerebral pneumatocele: case report. Pneumocephalus occurs in 0.5 to 1.0% of head trauma, but may also occur after neurologic surgery, or as a result of eroding infection or neoplasm. The pathophysiology involves the presence of craniodural fistula allowing ingress of air. A ball-valve mechanism may allow air to enter but not exit the cranium, or CSF leak permits air entrance as fluid leaves the intracranial space. While a "succession splash" is considered diagnostic of pneumocephalus, most patients have nonspecific signs and symptoms such as headache. Therefore, a high index of suspicion in a patient with recent head trauma is necessary. The diagnosis is made radiographically by CT scan. This is generally performed to rule out intracranial hematoma or cerebral contusion in head trauma, but will reveal even very small quantities of air to the unsuspecting physician. Therapy is often noninvasive, allowing the craniodural defect to heal spontaneously. Selected situations require immediate operative repair of the fistula. 

Category: nervous system diseases
Imaging the fetal brain in the second and third trimesters using transvaginal sonography. High-frequency transvaginal probes were used at 20-40 weeks' gestation to develop a systematic examination of the fetal brain. Modeling the procedure after the standard neonatal neurosonographic examination, we attempted to obtain three coronal sections (anterior, midline, posterior) and two sagittal sections (midsagittal, right or left parasagittal). In 70 normal patients, all planes were imaged with a similar frequency (74-76%) except for the posterior coronal plane, which was imaged 59% of the time. Among the first 35 cases, 17% had a complete study, compared with 71% of the second 35 cases. Transvaginal sonography established or changed the diagnosis in five of the 13 cases with central nervous system or other abnormalities. We recommend that a complete fetal neurosonographic examination include transvaginal sonography to complement and enhance the transabdominal examination, especially for cases in which a fetal abnormality is suspected. 

Category: nervous system diseases
Syndromes of transient amnesia: towards a classification. A study of 153 cases. Of 153 patients presenting with acute transient amnesia, 114 fulfilled the proposed strict diagnostic criteria for transient global amnesia (TGA). The prognosis of this group was excellent with the exception of a small subgroup (7%), largely identifiable because of atypically brief or recurrent attacks, who developed epilepsy of temporal lobe type on follow up. Computerised tomography (CT) scans performed on 95 patients were normal, evidence for covert alcoholism was lacking and there was a familial incidence of approximately 2%. By contrast, the group of 39 patients who did not meet the criteria for TGA had a significantly worse prognosis with a high incidence of major vascular events. The groups could not be distinguished on the basis of behavioural characteristics during the attack. The following classification was proposed: 1) pure TGA--attacks fulfilling the strict criteria, and of more than one hour in duration which do not require detailed investigation, 2) probable epileptic amnesia--attacks of less than an hour or rapidly recurrent, 3) probable transient ischaemic amnesia, a minority of cases with additional focal neurological deficits during the attack. 

Category: cardiovascular diseases
When is a prognostic factor useful? A guide for the perplexed. Traditionally, a number of variables have been used to predict outcome in patients with early-stage breast cancer. These tests are simple to perform and relatively inexpensive. Recently, a number of new factors, eg, tumor proliferative index, nuclear DNA content, and amplification or overexpression of growth-promoting genes or oncogenes have been identified as potential predictors of outcome in patients with breast cancer. There is now increasing pressure to introduce such tests into routine clinical practice. How does a clinical practitioner identify which test, or group of tests, best predicts adverse outcome and whether any more clinically useful information is provided than with the use of more traditional factors alone? The aim of a prognostic test in breast cancer is to predict which patients are destined to develop a recurrence of cancer and those who are not. The prognostic usefulness of a test can be expressed in terms of relative risk (RR), which is the ratio of the risk of breast cancer recurrence in patients who test positive to the risk in those who test negative. Methodologic guidelines that should be satisfied by a study evaluating the predictive ability of a test include the following: (1) Was an inception cohort assembled? (2) Was the referral pattern described? (3) Were laboratory and clinical outcomes assessed in a blinded fashion? (4) Was complete follow-up achieved? (5) Was adjustment for extraneous prognostic factors carried out? (6) Were appropriate statistical methods used? An approach is suggested to help the clinician choose the test, or combination of tests, likely to discriminate between "high-" and "low-risk" patients in his/her own practice. The decision regarding what particular threshold value (risk) defined by a prognostic test (or series of tests) warrants adjuvant therapy for an individual patient is a complex one but should be based on a clear presentation of the risks and benefits to the patient. 

Category: neoplasms
Usefulness of d, I sotalol for suppression of chronic ventricular arrhythmias. Sotalol is a unique beta-blocking drug, possessing significant class III antiarrhythmic activity. The efficacy and safety of 2 doses of sotalol (320 and 640 mg/day, divided in 2 doses) were compared to placebo in a 6-week randomized, double-blind, multicenter study of 114 patients with chronic ventricular premature complexes (VPCs) at frequencies of greater than or equal to 30/hour. Sotalol significantly reduced VPCs in patients receiving both low (n = 38) and high (n = 39) doses, compared with patients (n = 37) receiving placebo (by 75 and 88%, respectively, vs 10%; p less than 0.001, sotalol vs placebo; p less than 0.05, high vs low dose). The individual efficacy criterion (greater than or equal to 75% VPC reduction) was achieved in 34% of low-dose and 71% of high-dose sotalol versus 6% of placebo-treated patients (p less than 0.003, sotalol vs placebo; p = 0.007, high vs low dose). Repetitive beats were suppressed 25% by placebo (difference not significant), 80% by low-dose (p less than 0.003) and 78% by high-dose sotalol (p less than 0.005). Sotalol decreased heart rate (by 24 to 25%, p less than 0.001) and increased PR (by 4 to 6%, p less than 0.001) and corrected JT intervals (by 12 to 13%, p less than 0.001), but did not change ejection fraction. Proarrhythmia (nonfatal) occurred in 3 sotalol and in 2 placebo patients. Nine discontinued therapy because of adverse effects (1 low dose and 8 high dose, p less than 0.02). In summary, sotalol is an efficacious antiarrhythmic drug for VPC suppression; in lower doses, it is somewhat less effective but better tolerated. 

Category: general pathological conditions
Endovascular management of arterial intimal defects: an experimental comparison by arteriography, angioscopy, and intravascular ultrasonography. To determine the feasibility of the endovascular management of intimal defects while comparing the accuracy of arteriography with angioscopy and intravascular ultrasonography, we developed an in vivo model of arterial intimal flaps. In 10 superficial femoral arteries of five anesthetized mongrel dogs, intimal flaps were constructed and then imaged by arteriography, angioscopy, and intravascular ultrasound. A flexible microbiopsy forceps was used to remove each intimal flap under angioscopic guidance. Arteriographic lumen diameters were measured and cross-sectional areas calculated. Corresponding measurements by angioscopy and intravascular ultrasound with reduction in luminal area at the flap were obtained by use of computerized planimetry. Uniplanar arteriography identified 60% (6/10) of the intimal flaps, whereas angioscopy and intravascular ultrasound demonstrated 100%. Lumen diameter (in millimeters) measured by arteriography (3.4 +/- 0.6) correlated significantly with measurements by angioscopy (3.5 +/- 0.5, r = 0.77) and intravascular ultrasound (3.5 +/- 0.6, r = 0.96). Similarly, lumen area (square millimeters) by arteriography (9.2 +/- 2.9) correlated with measurements by angioscopy (8.9 +/- 2.2, r = 0.82) and intravascular ultrasound (8.6 +/- 2.7, r = 0.91). Reduction in lumen area by the flap by angioscopy (37 +/- 7%) and intravascular ultrasound (33 +/- 8%) also correlated significantly (r = 0.72). The intimal flaps were removed successfully in all 10 arteries as confirmed by arteriography, angioscopy, and intravascular ultrasound. We conclude that the endovascular management of intimal defects is possible. Additionally, angioscopy and intravascular ultrasound accurately evaluate lumen diameter and area while providing direct assessment of intimal defects. 

Category: cardiovascular diseases
Intravenous immunoglobulin treatment in patients with chronic inflammatory demyelinating polyneuropathy. Clinical and laboratory characteristics associated with improvement. Of 52 patients fulfilling the criteria of chronic inflammatory demyelinating polyneuropathy, 20 (38%) did not improve after intravenous immunoglobulin treatment, two (4%) had a short-lasting improvement and subsequent infusions had no effect, nine (17%) reached a spontaneously or therapeutically induced complete remission, and 21 patients (40%) needed intermittent infusions to maintain improvement. All patients who improved initially had symptoms that significantly interfered with life-style. After treatment, 90% of these patients were independent in their daily activities. Significantly associated with improvement were disease duration of less than 1 year, progression of weakness until treatment, absence of discrepancy in weakness between arms and legs, areflexia of the arms, and slowed nerve conduction velocity of the motor median nerve. The probability of improvement if all these features are present in 93%. 

Category: nervous system diseases
Precertification for percutaneous transluminal coronary angioplasty in Medicare beneficiaries: a melting pot or a need for better national standards? The Health Care Financing Administration has contracted with state peer review organizations (PROs) in its effort to assure the quality of services and eliminate unreasonable and inappropriate care provided Medicare beneficiaries. By law, each state PRO must select 10 procedures for precertification. Coronary angioplasty has been chosen by 45 PROs for precertification and criteria in each state were developed with the advice of local physicians. This report describes the findings of a survey of these precertification criteria in an effort to determine their variability and to compare the PRO criteria to published national criteria created by expert panels. Current precertification criteria of Medicare beneficiaries show significant variability in the priorities and the clinical practice of cardiologists in performing coronary angioplasty, despite established and published guidelines for its safe and efficacious use. It is likely that the establishment of locally based criteria for coronary angioplasty will be geographically uneven and probably have a less than expected impact on the care provided to Medicare beneficiaries. 

Category: cardiovascular diseases
Glucose disposal, beta-cell secretion, and hepatic insulin extraction in cirrhosis: a minimal model assessment. Factors controlling glucose metabolism after IV load were studied in nine patients with compensated cirrhosis and in six age-matched controls. The time courses of glucose, insulin, and C peptide were analyzed by means of the minimal model technique. In cirrhosis, insulin sensitivity was reduced by approximately 70% and glucose-dependent glucose uptake (glucose effectiveness) by 45%. Decreased glucose effectiveness explained 65% of the variance of glucose disappearance and correlated with the ratio of urinary creatinine to height, an independent measure of muscle mass (r = 0.839). beta-cell responsiveness to glucose, measured on C-peptide kinetics, was variable and increased on average by 170% and 107% (first-phase and second-phase, respectively). The total amount of insulin secreted by beta-cells in the course of the study was nearly doubled, whereas the basal insulin secretion rate was in the normal range. The time courses of hepatic extraction of insulin did not differ between groups, and basal extraction was on average 58% in controls and 56% in patients with cirrhosis. It was reduced to 30% in a single patient who had severe hepatocellular failure and large spontaneous portosystemic shunting. We conclude that the alterations in glucose metabolism of cirrhosis include a decreased insulin sensitivity, a reduced glucose effectiveness, and an increased pancreatic responsiveness to glucose, leading to hyperinsulinemia. The hepatic extraction of insulin is reduced only in the very advanced stages of the disease, possibly because of a large reserve capacity of the hepatic parenchyma. 

Category: digestive system diseases
A follow-up study of intractable seizures in childhood. One hundred forty-five children with seizures that were refractory to medical therapy for at least 2 years were followed 5 to 20 years after onset. The majority of children with uncontrollable seizures (61%) were mentally retarded, and most of these (73%) had onset of seizures at younger than 2 years of age. Age of onset was significantly later (mean 5.0 +/- 0.5 yr [SEM]) in the group of children with borderline to normal intelligence. Follow-up data showed remission of seizures in a significant proportion of children with borderline or normal intelligence, with a linear decrease of the percentage with persistent seizures at a rate of about 4% per year. Remission of seizures was much less frequent (1.5%/yr) in the group with mental retardation. Seizure type had some effects on outcome. Children with focal atrophic brain lesions did no worse than those without definable pathology on brain-imaging studies. 

Category: nervous system diseases
Thoracoscopic treatment of spontaneous pneumothorax using carbon dioxide laser. In an effort to improve the success rate of the previously described thoracoscopic electrocautery ablation technique of spontaneous pneumothorax, the carbon dioxide laser was evaluated in 12 patients. The recurrent (5 patients) or persistent (7 patients) spontaneous pneumothoraces were caused by rupture of (1) blebs in 6 patients, (2) intrapulmonary apical type II bullae in 3 patients, and (3) diffuse bullous emphysema, type III, in 3. The air leaks were successfully sealed in all but 1 patient with ruptured type II bulla. Surgical specimen from this single failure suggested that the entire inner lining of the bullae must be thermocoagulated. This technical modification led to successful outcome in 2 subsequent cases. With the use of carbon dioxide laser, it was possible to treat not only small blebs but all types of bullae causing spontaneous pneumothorax. Laser thoracoscopy is effective and safe in treating spontaneous pneumothorax. 

Category: neoplasms