Use of the polymerase chain reaction for detecting Trypanosoma cruzi in triatomine vectors

Two patients with squamous cell carcinoma arising within an epiphrenic diverticulum are reported bringing the total reported in the English-language literature to fifteen. The majority of patients with carcinoma arising in an epiphrenic diverticulum are elderly males who have been symptomatic for years with a known diverticulum. This is the first report of multiple cases of carcinoma within an epiphrenic diverticulum from a single institution and the first report in a female patient. Similar to other reports, these two patients presented with several months of symptoms related to their esophageal diverticulum. It is important to entertain the diagnosis of esophageal carcinoma early in the evaluation of patients with signs and symptoms suggestive of epiphrenic diverticula as early intervention may be the only chance of cure.     

Endoscopic treatment of Zenker's diverticulum using CO2 laser. 17 cases

OBJECTIVES: Various treatment regimens have been used for the management of Zenker's diverticulum. These include surgery (transcervical diverticulectomy, myotomy) and endoscopic treatment with rigid instruments or flexible endoscope. The aim of this study is to report the results of the treatment of Zenker's diverticulum with rigid endoscope applied CO2 laser and to compare them with other types of treatment. PATIENTS AND METHODS: Between May 1991 and November 1996, 17 patients (8 males, 9 females) with symptomatic Zenker's diverticulum underwent endoscopic treatment under short general anesthesia applied CO2 laser. The mean age of the patients was 73.2 years (range: 54 to 97 yrs). All patients had significant symptoms such as dysphagia (15 patients out of 17). All patients were clinically evaluated after the procedure. RESULTS: The endoscopic incision was performed in one session per patient. Symptoms and dysphagia disappeared in all patients except one (dysphagia). Two complications (one cervical emphysema, one fistula) were managed clinically. Mortality was 0%. Mean follow-up was 17 months. There was no recurrence of dysphagia, all patients remained asymptomatic. CONCLUSIONS: Rigid endoscopic treatment of symptomatic Zenker's diverticulum applied with laser CO2 is an efficient and safe method. It should be considered as an alternative therapy for Zenker's diverticulum, especially for patients at a high surgical risk.     

Endoscopic staple-assisted esophagodiverticulostomy for Zenker's diverticulum

We have used a new technique, endoscopic staple-assisted esophagodiverticulostomy (ESED), for the treatment of Zenker's diverticulum. This technique is a modification of the endoscopic approach first described by Mosher in 1917 and popularized by Dohlman and Mattsson in which the common wall between the esophagus and diverticulum was divided without mucosal closure. ESED differs in that an endosurgical stapler is used to create an esophagodiverticulostomy by dividing the common wall between the esophagus and diverticulum, with the mucosal and muscular edges sealed by the staples. We have performed this procedure in six patients, with no perioperative morbidity in any patient. All patients resumed oral intake on either the first or second postoperative day, with no evidence of fistula formation or mediastinitis. Hospital stay has averaged 2 days (range, 1 to 3 days). Patient follow-up after ESED averages 6 months and is available for five of the six patients treated. There has been complete resolution of pretreatment symptoms in these five patients, with resumption of regular diet between postoperative day 3 and day 14 (average, day 9). Our results demonstrate that ESED is a safe and effective treatment for Zenker's diverticulum. This endoscopic approach offers a number of advantages over previously used treatments for Zenker's diverticulum, including reduced morbidity, rapid convalescence, short hospitalization, brief operative time, and predictable resolution of symptoms.     

Neuronal nicotinic ACh receptor antibody in subacute autonomic neuropathy and cancer-related syndromes

BACKGROUND: Autoantibodies specific for the acetylcholine receptor (AChR) of skeletal muscle (containing the alpha1 subunit) impair neuromuscular transmission in myasthenia gravis (MG). AChRs mediating fast synaptic transmission through autonomic ganglia are structurally similar to muscle AChR, but contain the alpha3 subunit. We propose that ganglionic AChR autoimmunity may cause dysautonomia. OBJECTIVE: To test serum of patients with autonomic neuropathy for autoantibodies of neuronal ganglionic AChR specificity. METHODS: We developed an immunoprecipitation radioassay by complexing epibatidine (125I-labeled high affinity agonist) to a Triton X-100-solubilized AChR antigen from peripheral neuroblastoma membranes. Monoclonal rat immunoglobulins (IgG) specific for muscle or neuronal AChRs validated the assay's specificity. We tested serum from 52 healthy subjects, 12 patients with subacute autonomic neuropathy, and 248 patients with other neurologic disorders. RESULTS: Twelve patients had antibodies that bound unequivocally to ganglionic AChR. Five had subacute autonomic neuropathy, and three (of six tested) had Isaacs' syndrome; four of these eight had a carcinoma (lung, bladder, rectum, thyroid). The remaining four seropositive patients (two Lambert-Eaton syndrome, one dementia, one sensory neuronopathy) all had Ca2+ channel antibodies and three had small cell lung carcinoma. No healthy subject had ganglionic AChR antibodies, nor did 62 patients with MG and muscle AChR antibodies. CONCLUSION: Neuronal AChR antibodies are a novel serologic marker of neurologic autoimmunity. The pathogenicity of neuronal AChR autoantibodies in autonomic neuropathy, Isaacs' syndrome, or other neurologic disorders remains to be shown, as has been demonstrated for muscle AChR antibodies in MG. An autoimmune and potentially paraneoplastic etiology is implicated in seropositive patients.     

Leiomyomata of the esophagus. An analysis of 838 cases

Review of the world literature to the end of 1971 has provided data on 838 cases of esophageal leiomyomata, including our own 19 surgically removed lesions. Although esophageal leiomyoma is the most common of the benign tumors of the esophagus, it is still rare compared with carcinoma. It occurs in more men than women, by a ratio of 1.9 to 1. Over 50% of the patients with leiomyoma of the esophagus are asymptomatic. Dysphagia and vague pain are the most frequent symptoms. Pyrosis is mentioned in the literature as present in 40% of the cases, but it is considered mainly as symptom of coexistent hiatal hernia. Diagnostic problems often arise, as the smooth muscle tumors may mimic mediastinal neoplasms, cysts, or even aneurysms, or complicate coexisting hiatal hernia and esophageal diverticulum. Operative management by transthoracic enucleation is the procedure of choice, although resection of the esophagaus may be required in few cases. Postoperative morbidity is minimal and results are excellent.     

Application of genetically engineered animals in pharmacology: the use of green fluorescent protein for selective production of transgenic animals

The myelodysplastic syndromes (MDS) are a heterogeneous group of disorders characterized by peripheral blood cytopenias with a hypercellular bone marrow exhibiting dyspoiesis. The predominant in elderly patients are associated with a high risk of progression to acute myelogenous leukemia. The etiology of MDS is unknown in most cases. About 10% of MDSs are secondary. MDS are classified by the French American British (FAB) classification into five subgroups. The incidence of the disorders is difficult to estimate but it seems to be increasing. Clonal cytogenetic aberrations are found in 30 to 50% of de novo MDS. The only currative treatment for MDS is allogeneic bone marrow transplantation.     

Motility disorders of the esophagus: diagnosis with barium-rice administration

PURPOSE: To evaluate the role of barium-rice administration for a standardized diagnosis of dysphagia and esophageal motility disorders. MATERIALS AND METHODS: Sixty healthy volunteers and 218 patients with various esophageal disorders (achalasia, scleroderma and other connective tissue diseases, neurologic diseases, esophagitis and others) were examined both by a conventional barium study and by a barium-rice study. The barium-rice meal consisted of barium sulfate and boiled rice, mixed half and half. The time required for esophageal clearance of one sip was measured. RESULTS: Normal esophageal transit time in healthy controls was between 5 and 15 s for both methods. In patients, the conventional barium study revealed a prolonged transit time in only 16.5% (36 of the 218 cases). The barium-rice study was abnormal in 51.8% (113 of 218 cases), independent of the underlying disease. The barium-rice study was pathological in 77 of those 182 patients (42.3%) who had normal barium transit time. In 24 patients the radiologic results was confirmed by endoscopy and manometric measurements. CONCLUSIONS: Esophageal motility abnormalities are detected by a barium-rice study with a high sensitivity. With this simple and low-cost method, quantitative and reproducible results can be obtained. Barium-rice administration is a suitable tool for screening and follow-up of patients with dysphagia and esophageal motility disorders.     

Pseudoneurologic syndromes: recognition and diagnosis

Physicians may encounter patients with a collection of psychologic disorders that present with neurologic symptoms or signs, yet have no identifiable structural or functional etiology within the nervous system. These disorders comprise the so-called pseudoneurologic syndromes, which can mimic almost any organic disease. A careful history and physical examination often can identify the psychologic origin of the symptoms. Presenting syndromes can include pseudoparalysis, pseudosensory syndromes, pseudoseizures, pseudocoma, psychogenic movement disorders and pseudoneuro-ophthalmologic syndromes. These presentations may be distinguished from organic disease by observing signs and symptoms or eliciting test responses that are nonphysiologic and incompatible with organic disease. Once a pseudoneurologic syndrome is identified, patients require compassionate and understanding care to resolve underlying emotional problems.     

Bilateral loss of vestibular function: clinical findings in 53 patients

The clinical presentations and aetiologies of a series of 53 cases of bilateral vestibular failure (BVF) seen by the authors over a decade were evaluated by retrospective review of the medical records. Thirty-nine per cent of patients had associated neurological disease; 13% had a progressive cerebellar syndrome with disabling gait ataxia, abnormal eye movements and cerebellar atrophy on neuro-imaging. BVF was usually unsuspected. Nine per cent had cranial or peripheral neuropathies and in this group there was no abnormality of brain stem/cerebellar oculomotor function, but hearing loss was common. Eleven per cent revealed BVF and hearing loss secondary to meningitis, and 6% had other neurological disorders. Idiopathic BVF was found in 21% of cases, characterised by paroxysmal vertigo and/or oscillopsia, but no abnormal clinical signs. Gentamicin ototoxicity accounted for a further 17%, while autoimmune disease was present in 9% of patients. Otological or neoplastic disease was diagnosed in the remaining 13% of patients. It was concluded that neurological, audiological and ocular motor assessments allow the probable cause of BVF to be defined in approximately 80% of cases. A group of BVF related to autoimmune pathologies is reported for the first time, indicating the need for immunological screening. Idiopathic BVF may present with only minor visual or vestibular symptoms, while in patients with cerebellar degeneration, BVF may be unsuspected and, thus, underdiagnosed.     

Globus sensation: pharyngoesophageal function, psychometric and psychiatric findings, and follow-up in 88 patients

Background: The globus sensation has been widely regarded as psychogenic, but organic disorders were found to be etiologically significant. OBJECTIVE: To investigate the structural, functional, psychological, and psychiatric factors possibly eliciting the globus sensation and influencing its course. METHODS: Eighty-eight patients, 67 women and 21 men (aged 22-71 years), referred to 2 tertiary care centers underwent history taking, otolaryngological examination, pharyngoesophageal videofluoroscopy and manometry, psychosocial evaluation, psychometric tests, psychiatric interview, and when indicated, esophagogastroduodenoscopy, esophageal bolus transport, gastroesophageal reflux, and gastric emptying studies. According to revealed disorders, therapy was initiated, and the outcome was studied. RESULTS: Only 15 patients had normal pharyngoesophageal function; of these 15, 6 had chronic tonsillitis or pharyngitis, 3 had thyroid adenomata, 4 had cervical spondylosis, and 1 each had dry oropharyngeal mucosa and chronic bronchitis. Of the other 73 patients, 2 had pharyngeal dysfunction, 24 had achalasia, 1 had diffuse esophageal spasms, 3 had "nutcracker esophagus," 30 had nonspecific esophageal motor disorders, and 13 had gastroesophageal reflux. Psychometry revealed no more anxiety and depression than in general medical outpatients. Of 58 patients interviewed, 37 met criteria for psychiatric disorders. Psychometric scores and psychiatric characteristics were unrelated to the sensation's course. Therapy was recommended, but only 26 patients were treated accordingly; 22 received nonspecific treatment. Follow-up 3 to 59 months later revealed that the sensation had vanished in 13 patients who had received specific treatment, 5 who had received nonspecific treatment, and 6 who had received no treatment; it was alleviated in 10 who had received specific treatment, 13 who had received nonspecific treatment, and 9 who had received no treatment; and it was unchanged in 3 who had received specific treatment, 5 who had received nonspecific treatment, and 23 patients who had received no treatment. CONCLUSIONS: Pharyngoesophageal disorders may be sensed only vaguely, inducing the globus sensation. Psychological and psychiatric characteristics could be relevant to the discomfort experienced but are unlikely to be etiologically significant.     

An increase in basal glucocorticoid concentration with age induces suppressor macrophages with high-density Fc gamma RII/III

BACKGROUND: The diagnosis of Tourette syndrome may be overlooked in patients with severe psychopathologic disorder but mild motor manifestations of Tourette syndrome. OBJECTIVE: To describe 4 patients with long-lasting general psychopathologic disorder and previously unrecognized mild motor and phonic tics exacerbated during adulthood by the onset of tremor; all of the patients had been referred for the evaluation of psychogenic tremor. SUBJECTS: Four adult patients, with previous psychiatric diagnoses of depression (2 cases), generalized anxiety disorder (3 cases), malingering (1 case), and conversion disorder (3 cases). METHODS: Single case studies. RESULTS: Clinical interviews disclosed that the 4 patients had positive family histories of Tourette syndrome, and all had mild motor and phonic tics that had started before the age of 18 years. On neurologic examination, 2 patients had bilateral postural tremor of the hands that varied in frequency, rhythmicity, and amplitude, and the other 2 had resting tremor mimicking parkinsonism. All 4 patients described involuntary somatic sensations of the affected limbs, which they attempted to alleviate by executing movements. No consistent positive placebo response was observed, but in all patients tremoric movements improved with haloperidol. CONCLUSIONS: These cases illustrate an unusual movement disorder (tremor as a "tic equivalent") in adults with Tourette syndrome and emphasize that cases of the syndrome with mild tics often go unrecognized, precluding adequate treatment.     

Progressive degenerative aphasia: clinical and neuroradiological observations in 18 cases

INTRODUCTION: In some types of degenerative dementia aphasia is the main disorder. In primary progressive aphasia. (PPA) atrophy is limited to the dominant peri-sylvan region. We present 18 cases of progressive aphasia of degenerative origin, with or without dementia. MATERIAL AND METHODS: We describe the clinical and neuro-radiological findings in 3 patients with 'aphasic dementia and motor neuron disease (ADMND)', 7 with 'semantic dementia' (DS), and 4 with 'fronto-temporal dementia' with 'marked non-fluent aphasia' (AFTD). Criteria published in recent years were used. RESULTS: In patients with ADMND non-fluent aphasia progressed to global aphasia, with dementia occurring after 2-9 months, and death after an average of 17 months. In cases with SD, initial anomic aphasia progressed to transcortical sensory or global aphasia, and in patients with AFTD, Broca's aphasia or motor transcortical aphasia progressed to global aphasia. Seven of these patients had been initially diagnosed as having PPA and became demented after two years or more. In most of the cases the cognitive disorder had the characteristics of fronto-temporal dementia. All cases had cortical atrophy or asymmetrical cortical or cortico-subcortical atrophy. The 4 cases of non-fluent PPA were not demented after 21 months-6 years of illness, and showed perisylvan and left fronto-temporopolar atrophy. CONCLUSIONS: The PPA may correspond to the initial form of at least three varieties of dementia, usually the fronto-temporal type. Dementia occurs after two years or more, except in patients with motor neurone disease, when there is a latent period of less than one year.     

Charcot-Marie-Tooth disease. Clinical study in 45 patients

Charcot-Marie-Tooth (CMT) disease is the commonest inherited peripheral neuropathy. The clinical study of 45 patients with CMT is presented. They were derived from Antonio Pedro Hospital of Universidade Federal Fluminense in Niteroi, RJ, Brazil. Such patients could be divided by the motor conduction velocity in two types: a demyelinating form or type I (11 cases) and an axonal form or type II (34 cases). The disease was inherited as an autosomal dominant trait in 23 patients and as an autosomal recessive trait in 7 cases. In 15 patients the disorder was sporadic. The age of onset was in most of our cases before the 20 years. All of them had distal weakness in lower limbs. 38.2% had also distal weakness in upper limbs. 80% had distal wasting of the lower limbs and 50% had distal wasting of upper limbs. The tendon reflexes were absent in 64% in lower limbs and in 28% in upper limbs. The sensitive impairment in the distal regions of the extremities was mild in most patients. We found enlargement of peripheral nerves in 7 patients of type I. Pes cavus was present in 21 cases and scoliosis in 7. We found postural tremor of hands in 6 patients. In 9 cases there were rare features as mental retardation, trigeminal nevralgia, optic atrophy, deafness and calf enlargement. In most of our cases the clinical course was very slow progressive. A greater severity was seen in our sporadic cases.     

Acute benign edematous polyarthritis in the elderly (or RA3PE syndrome). Clinical course apropos of 13 cases

OBJECTIVES: The position of remitting seronegative symmetrical synovitis with pitting edema (RS3PE syndrome) among inflammatory rheumatic diseases. Is it a distinct syndrome or a clinical feature? PATIENTS AND METHODS: To answer this question a retrospective study was conducted in 13 elderly patients (mean age: 72 years). The clinical course and laboratory findings were followed for 3 to 172 months after disease onset. RESULTS: In two patients, another disease was diagnosed: polymyalgia rheumatica, late onset peripheral spondylarthropathy. Two relapses of RS3PE were noted. There was no clinical difference between initial RS3PE and relapsing RS3PE. In four cases, RS2PE syndrome revealed another disease: dermatopolymyositis, AL amyloidosis, polymyalgia rheumatica, late onset peripheral spondylarthropathy. No rheumatoid arthritis appeared, but one patient was positive for rheumatoid factors. A benign course was observed in all patients, without paraneoplastic syndrome and without death. CONCLUSION: Benign edematous polyarthritis in the elderly is a syndrome that may reveal connective tissue disease or inflammatory rheumatic disease.     

An analysis of the outcomes of treatment of small cell lung cancer in the elderly

BACKGROUND: Many cases of small cell lung cancer will occur in the elderly population but optimal management of the disease in this age group remains uncertain. AIMS: To evaluate treatment of small cell lung cancer in the elderly in Australia and to compare treatment received and outcomes with those of younger patients. To draw insights from these observations into the optimal management of small cell lung cancer in the elderly. METHODS: A retrospective review of treatment charts and case notes for 51 elderly patients and 102 younger patients was undertaken. RESULTS: Elderly patients had similar baseline parameters with respect to disease stage and performance status. Elderly patients were mostly treated uniformly with combination chemotherapy, but suffered more dose reductions than younger patients. Benefits of chemotherapy were seen even in patients with poor performance status. Despite the dose reductions, response rates and survival times for elderly patients were usually similar to younger patients. CONCLUSIONS: Combination chemotherapy is beneficial to elderly patients with small cell lung cancer. Optimal therapy for the elderly may be different from that for younger patients and should be defined through prospective randomised clinical trials.     

Meckel's diverticulum: angiographic findings in 16 patients

OBJECTIVE: The purpose of this study was to assess the sensitivity of angiography in revealing the vitellointestinal artery or other arteriographic abnormalities in patients in whom surgery subsequently proved Meckel's diverticulum. MATERIALS AND METHODS: From the 36 patients who had undergone a Meckel's diverticulectomy between 1980 and 1997 at Hammersmith Hospital or a referring hospital, we selected 18 who had undergone preoperative angiography at our institution. Case notes and angiograms of these 18 patients were reviewed for the presence of a persistent vitellointestinal artery or other angiographic evidence of a Meckel's diverticulum. RESULTS: Angiograms of 16 of 18 patients were available for review. A striking male preponderance existed (male:female = 13:3). Mean age was 28 years (range, 12-65 years). In 11 (69%) of the 16 patients, a persistent vitellointestinal artery was seen that had been noted at the time of the study and reported before surgery for nine patients. Other angiographic abnormalities at the site of the Meckel's diverticulum were present in four patients and included a vascular blush, early venous return, and arterial irregularity. CONCLUSION: Angiography will show a persistent vitellointestinal artery in most individuals with a Meckel's diverticulum who present with chronic gastrointestinal bleeding. However, the recognition of a persistent vitellointestinal artery may be difficult because of overlying vessels, and superselective catheterization of distal ileal arteries may be necessary.     

Diagnosing syncope. Part 1: Value of history, physical examination, and electrocardiography. Clinical Efficacy Assessment Project of the American College of Physicians

PURPOSE: To review the literature on diagnostic testing in syncope and provide recommendations for a comprehensive, cost-effective approach to establishing its cause. DATA SOURCES: Studies were identified through a MEDLINE search (1980 to present) and a manual review of bibliographies of identified articles. STUDY SELECTION: Papers were eligible if they addressed diagnostic testing in syncope or near syncope and reported results for at least 10 patients. DATA EXTRACTION: The usefulness of tests was assessed by calculating diagnostic yield: the number of patients with diagnostically positive test results divided by the number of patients tested or, in the case of monitoring studies, the sum of true-positive and true-negative test results divided by the number of patients tested. DATA SYNTHESIS: Despite the absence of a diagnostic gold standard and the paucity of data from randomized trials, several points emerge. First, history, physical examination, and electrocardiography are the core of the syncope workup (combined diagnostic yield, 50%). Second, neurologic testing is rarely helpful unless additional neurologic signs or symptoms are present (diagnostic yield of electroencephalography, computed tomography, and Doppler ultrasonography, 2% to 6%). Third, patients in whom heart disease is known or suspected or those with exertional syncope are at higher risk for adverse outcomes and should have cardiac testing, including echocardiography, stress testing. Holter monitoring, or intracardiac electrophysiologic studies, alone or in combination (diagnostic yields, 5% to 35%). Fourth, syncope in the elderly often results from polypharmacy and abnormal physiologic responses to daily events. Fifth, long-term loop electrocardiography (diagnostic yield, 25% to 35%) and tilt testing (diagnostic yield < or = 60%) are most useful in patients with recurrent syncope in whom heart disease is not suspected. Sixth, psychiatric evaluation can detect mental disorders associated with syncope in up to 25% of cases. Seventh, hospitalization may be indicated for patients at high risk for cardiac syncope (those with an abnormal electrocardiogram, organic heart disease, chest pain, history of arrhythmia, age > 70 years) or with acute neurologic signs. CONCLUSIONS: Many tests for syncope have a low diagnostic yield. A careful history, physical examination, and electrocardiography will provide a diagnosis or determine whether diagnostic testing is necessary in most patients.     

Spastic disorders of the esophagus

Spastic disorders of the esophagus are found in up to 50% of patients referred for manometry, therapy representing the most prevalent motility disorders in clinical practice. They share in common their manifestations of hypermotility, one of two principal types of esophageal motor dysfunction. Diffuse esophageal spasm is segregated from the nonspecific spastic disorders because of its demonstrated interference with bolus transit. However, the overlap among the spastic disorders in manifestation, course, and management is great; segregation of any disorder within this group is not of paramount importance. Spastic disorders, pain reproduction with provocative testing, and psychological abnormalities are coprevalent in patients with unexplained symptoms, but a cause-effect relationship of the motor abnormalities with the other findings is not established. The physician's charge in determining the relevance of a spastic disorder to the clinical presentation and for creating a treatment plan is to establish a direct relationship of motor dysfunction with symptoms-a task that may require correlation of transit abnormalities with symptoms using tests other than manometry. A variety of treatment options, invasive and noninvasive, are available today for patients who have spastic disorders, and each is effective in appropriately selected candidates.     

Merkel cell carcinoma of the auricle

Esophageal intramural pseudodiverticulosis is a very rare disease with unknown etiology, which especially affects male patients between 45 and 65 years. This disease is characterized by dilatation of the esophageal submucosal glands and their outlets. Stenosis caused by esophagitis due to intramural pseudodiverticulosis is found in most of the known patients. All patients presented with dysphagia, usually of long duration. The characteristic radiographic appearance is numerous intramural esophageal contrast-filled diverticulosis-like pouches--4 mm in depth.