The correct answer is the fifth one. The cause of Chagas disease is Trypanosoma cruzi.

The [HIDDEN] The cause of Chagas disease is Trypanosoma cruzi.

47-year-old woman. Native of Cochabamba (Bolivia), she has been living in Spain for 8 years. In mid 2009 she began to experience palpitations, dyspnea and progressive edema in the lower extremities. She was diagnosed with heart failure secondary to cardiomyopathy due to Chagas disease. What is the etiological agent of this disease endemic in several Latin American countries?

[Stroke of cardioembolic origin in Chagas disease].

American trypanosomiasis, Chagas disease is caused by Trypanosoma cruzi. Between 10% and 30% of infected persons develop the chronic form, with predominance of the cardiac and gastrointestinal forms. Chagas myocardiopathy leads to congestive heart failure, dysrhythmias and thromboembolic phenomena, and may cause strokes. We report two patients, a 57 year old woman and a 52 year old man, carriers of the chronic cardiac form of Chagas disease, with cardioembolic strokes. In both persons, serology was positive for Chagas disease (indirect hemagglutination and indirect immunofluorescence). The causes of atherothrombotic stroke were ruled out on carotid and transcranial Doppler studies. The woman had previously had an infarct of the left middle cerebral artery. She was admitted with a stroke involving the vertebrobasilar territory. On Holter studies there was second degree atrioventricular block and the electrocardiogram showed severe dilated myocardiopathy. On magnetic resonance studies there was an old left temporoparietal infarct and recent ischaemia of the pons and cerebral peduncle. She was anticoagulated and a pacemaker implanted. The man had a right middle cerebral artery infarct. His electrocardiogram showed atrial fibrillation and left anterosuperior block. The echocardiogram showed left ventricular dysfunction and concentric ventricular hypertrophy. The chronic cardiac form of Chagas disease should be included in the differential diagnosis of stroke of cardioembolic origin both in endemic areas and in countries to which persons exposed to infection during the early years of life emigrate.

Chagas disease as a cause of heart failure and ventricular arrhythmias in patients long removed from endemic areas: an emerging problem in Europe.

Chagas disease is a parasitic disease caused by the protozoan Trypanosoma cruzi. In endemic areas (South and Central America), Chagas disease represents a relevant public health issue, and is the most frequent cause of cardiomyopathy. In nonendemic areas, such as Europe, Chagas disease represents an emerging problem following the establishment of sizeable communities from Brazil and Bolivia. Chagas cardiomyopathy represents the most frequent and serious complication of chronic Chagas disease, affecting about 20-30% of patients, potentially leading to heart failure, arrhythmias, thromboembolism, stroke and sudden death. Because late complications of Chagas disease may develop several years or even decades after the acute infection, it may be extremely challenging to reach the correct diagnosis in patients long removed from the countries of origin. We report two examples of Chagas cardiomyopathy in South American women permanently residing in Italy for more than 20 years, presenting with cardiac manifestations ranging from left ventricular dysfunction and heart failure to isolated ventricular arrhythmias. The present review emphasizes that Chagas disease should be considered as a potential diagnosis in patients from endemic areas presenting with 'idiopathic' cardiac manifestations, even when long removed from their country of origin, with potential implications for treatment and control of Chagas disease transmission.

A highly suspected case of chronic Chagas' heart disease diagnosed in Japan.

A 50-year-old South American Indian woman, a native of Brazil and now a resident of Shiga Prefecture, was admitted to our hospital because of dyspnea on exertion. We initially suspected dilated cardiomyopathy due to an enlarged and diffusely hypokinetic left ventricle (LV) on echocardiogram. Coronary arteriograms were normal, and histological examination of right ventricular endomyocardial biopsy specimens showed findings compatible with chronic myocarditis. Magnetic resonance imaging revealed localized thinning and a small apical aneurysm at the LV. Since she had previously lived in a high-risk region for Chagas' disease, two immunological examinations for Trypanosoma cruzi were performed. The results of both tests were compatible with the disease. Recently, an increasing number of patients with Chagas' disease have been found in the United States among immigrants from South American countries, and the risk of transmission of the disease through contaminated blood transfusion is becoming a national problem. We report this case with reference to the present state of the problem in the United States and the potential problems it presents in Japan because of the marked increase in the number of immigrants from the affected area.

Chagas disease: don't forget it in Latin American patients with heart block!

Chagas disease is caused by the parasite Trypanosoma cruzi, and mostly affects poor rural populations of central and south America. It is mainly acquired by bugs (triatoma) but also by ingestion of the parasite (fresh fruit juices) or by foetal-maternal blood passing. Despite an important decrease in transmission during the last decades in several countries, millions of patients are still chronically infected and most of them are asymptomatic. In 2012-2013, two cases were admitted in our cardiac intensive care unit (ICU) with heart block due to Chagas cardiomyopathy. Diagnosis was established by echocardiography and positive serological results for Trypanosoma cruzi. This report underlines that in cases of heart failure and conduction abnormalities of unclear aetiology, Chagas disease should be taken into consideration, even in patients originating from non-endemic countries.

Acute chagasic myocardiopathy after orthotopic liver transplantation with donor and recipient serologically negative for Trypanosoma cruzi: a case report.

Chagas disease (American trypanosomiasis) is caused by the protozoan parasite Trypanosoma cruzi. Chagas disease following solid-organ transplantation has occurred in Latin America. This report presents the occurrence of Chagas disease despite negative serological tests in both the donor and the recipient, as well as the effectiveness of treatment. A 21-year-old woman from the state of Sao Paulo (Brazil) underwent cadaveric donor liver transplantation in November 2005, due to cirrhosis of autoimmune etiology. Ten months after liver transplantation, she developed signs and symptoms of congestive heart failure (New York Heart Association functional class IV). The echocardiogram, which was normal preoperatively, showed dilated cardiac chambers, depressed left ventricular systolic function (ejection fraction = 35%) and moderate pulmonary hypertension. Clinical investigation discarded ischemic heart disease and autoimmune and other causes for heart failure. Immuno fluorescence (immunoglobulin M and immunoglobulin G) and hemagglutination tests for T cruzi were positive, and abundant T cruzi amastigotes were readily identified in myocardial biopsy specimens. Treatment with benznidazole for 2 months yielded an excellent clinical response. At the moment of submission, the patient remains in functional class I. This case highlighted that more appropriate screening for T cruzi infection is mandatory in potential donors and recipients of solid-organ transplants in regions where Chagas disease is prevalent. Moreover, it stressed that this diagnosis should always be considered in recipients who develop cardiac complications, since negative serological tests do not completely discard the possibility of disease transmission and since good results can be achieved with prompt trypanocidal therapy.

Chronic Chagas disease with advanced cardiac complications in Japan: Case report and literature review.

Due to the unprecedented recent increases in global migration, Chagas disease has become a global health threat and its epidemiology has drastically changed. Here we describe the first case in Japan of benznidazole treatment for chronic Chagas disease characterized by advanced cardiac complications. A 55-year-old Japanese-Brazilian woman who had previously presented with chronic heart failure was diagnosed as having Chagas disease and treated with benznidazole to prevent aggravation of her cardiac complications. However, benznidazole administration was stopped on day 56 due to severe drug-induced peripheral neuritis. Sixteen months later, her serologic test for Trypanosoma cruzi is still positive and she is being followed regularly by cardiology. Despite an estimated prevalence of over 4000 cases in Japan, only a few cases of Chagas disease have been reported. A Medline search revealed only 7 cases identified between 1995 and 2014 in Japan: in 6 cases, complications of chronic Chagas disease were apparent at the time of presentation, and sudden death occurred in 2 of these cases due to cardiac complications. This clinical case and literature review re-emphasize the urgent need to establish a surveillance network and improve the diagnostic methods and treatment framework for Chagas disease in Japan.

Chagas heart disease: report on recent developments.

Chagas disease, caused by the parasite Trypanosoma cruzi, is an important cause of cardiac disease in endemic areas of Latin America. It is now being diagnosed in nonendemic areas because of immigration. Typical cardiac manifestations of Chagas disease include dilated cardiomyopathy, congestive heart failure, arrhythmias, cardioembolism, and stroke. Clinical and laboratory-based research to define the pathology resulting from T. cruzi infection has shed light on many of the cellular and molecular mechanisms leading to these manifestations. Antiparasitic treatment may not be appropriate for patients with advanced cardiac disease. Clinical management of Chagas heart disease is similar to that used for cardiomyopathies caused by other processes. Cardiac transplantation has been successfully performed in a small number of patients with Chagas heart disease.

Chagas disease

The most common manifestation is heart disease, which occurs in 14–45% of people with chronic Chagas disease. People with Chagas heart disease often experience heart palpitations, and sometimes fainting, due to irregular heart function. By electrocardiogram, people with Chagas heart disease most frequently have arrhythmias. As the disease progresses, the heart's ventricles become enlarged (dilated cardiomyopathy), which reduces its ability to pump blood. In many cases the first sign of Chagas heart disease is heart failure, thromboembolism, or chest pain associated with abnormalities in the microvasculature.

InternalMed_Harrison

The first signs of acute Chagas disease develop at least 1 week after invasion by the parasites. When the organisms enter through a break in the skin, an indurated area of erythema and swelling (the chagoma), accompanied by local lymphadenopathy, may appear. Romaña sign— the classic finding in acute Chagas disease, which consists of unilateral painless edema of the palpebrae and periocular tissues—can result when the conjunctiva is the portal of entry. These initial local signs may be followed by malaise, fever, anorexia, and edema of the face and lower extremities. Generalized lymphadenopathy and hepatosplenomegaly may develop. Severe myocarditis develops rarely; most deaths in acute Chagas disease are due to heart failure. Neurologic signs are not common, but meningoencephalitis occurs occasionally, especially in children <2 years old. Usually within 4–8 weeks, acute signs and symptoms resolve spontaneously in virtually all patients, with commencement of the asymptomatic or

Veterinary parasitology

Divisions of veterinary parasitology Veterinary protozoology Veterinary protozoology is focused on protozoas with veterinary relevance. Examples of protozoan parasites: Babesia divergens Balantidium coli Besnoitia besnoiti Cryptosporidium parvum Eimeria acervulina Eimeria tenella Giardia lamblia (also known as Giardia duodenalis) Hammondia hammondi Histomonas meleagridis Isospora canis Leishmania donovani Leishmania infantum Neospora caninum Toxoplasma gondii Trichomonas gallinae Tritrichomonas foetus Trypanosoma brucei Trypanosoma equiperdum

[Apical lesion of the left ventricle in clinical evolution of chronic Chagas cardiopathy].

Study of clinical evolutionary aspect in patients with apical lesion of chronic Chagas' heart disease diagnosed by left cineventriculography. 55 patients, 32 female, aged 22 to 69 years with epidemiologic data and positive serological tests, all presenting cardiovascular symptoms, 20 in cardiac failure (10 with left ventricular failure and 10 in congestive heart failure). Heart size was normal in 35 patients and 20 showed cardiomegaly on chest X-ray examinations (slight in 8, moderate in 6 and marked in 6), EKG showed premature beats in all patients and conduction defects in 38 instances with a predominance of right bundle branch block associated with left anterior divisional block in 19 cases. The following morphological aspects of the apical lesions were observed: mammillary--23 (41.82%); digital--18 (32.73%) and semilunar--14 (25.45%). In 8 cases (14.54%) intraventricular thrombi were detected and in 10 (18.18%) there were associated dyskinetic areas. The clinical course of 36 patients (65%) has been followed in the out patient department for 35 to 192 (97.83) months. Seven patients (12.73%) did not return for consultation after having been studied for 22 to 56 (42.83) months, and 12 (22%) died after a follow-up period of 19 to 176 (68.58) months. Detection of 17 thromboembolic episodes occurred in the 14 (25%) patients, 9 pulmonary encephalic and 1 peripheric, very frequent in patients with cardiac failure (11/14-78%). Cardiac pacemakers were implanted in 18 cases due to complete A-V block in 11, to sinus node dysfunction in 6, and to sinus arrest in 1. Among the survivors 29 (80%) maintained a normal heart size. The most frequent cause of death (12) was heart failure in 8, followed by cerebrovascular attacks in 3 and malignancy in 1. The estimated survival in 192 months was of 75.8%, males (74.5%) and females (76.6%). A presence of heart failure however was a limiting factor, with estimates of 96.8% survival in patients without cardiac decompensation, as compared to 36.5% in cases with heart failure. The apical lesion is an additional factor of mortality in Chagas' heart disease after the development of congestive heart failure.

Charles Darwin

Death and funeral In 1882 he was diagnosed with what was called "angina pectoris" which then meant coronary thrombosis and disease of the heart. At the time of his death, the physicians diagnosed "anginal attacks", and "heart-failure". It has been speculated that Darwin may have had chronic Chagas disease. This speculation is based on a journal entry written by Darwin, describing he was bitten by the "Kissing Bug" in Mendoza, Argentina, in 1835; and based on the constellation of clinical symptoms he exhibited, including cardiac disease which is a hallmark of chronic Chagas disease. Exhuming Darwin's body would probably be necessary to definitively determine his state of infection by detecting DNA of infecting parasite, T. cruzi, that causes Chagas disease.

[Chronic Right Ventricular Failure Revealing a Large Compressive Hydatid Cyst at the Cardiology Department of the Aristide le Dantec Hospital (Dakar, Senegal)].

Hydatidosis, an anthropozoonosis caused by the development of the <iEchinococcus granulosus</i larva in human, is a parasitic infection that is endemic in many countries. Pericardial localization in the absence of other cardiac involvement is extremely rare. Its spontaneous evolution is serious because of the risk of rupture. We report the case of a 65-year-old patient, who received limb edema that had been going on for 4 months, and in whom the physical examination noted a 3/6 tricuspid regurgitation and a right heart failure syndrome. Transthoracic echocardiography revealed an intra-pericardial mass whose contents appeared fluid with hyperechogenic area. It measured 86x61 mm and significantly compressed the straight cavities. Thoracic computed tomography showed an appearance in favor of an aged intrapericardic hydatid cyst (GHARBI V) with mass effect on the right cavities. The hydatid serology was negative. The patient did not accept the surgical cure. She had been put on albendazole and diuretic treatment. Fatal outcome was reported after two-month follow-up.

Myocarditis

Infections The most common causes of myocarditis are infectious organisms. Viral infections are the most common cause in developed countries, with a majority of cases being caused by those with single-stranded RNA genetic information such as Coxsackie viruses (especially Coxsackie B3 and B5). Globally, Chagas disease is the leading cause of myocarditis, which results from infection with the protozoan Trypanosoma cruzi. Bacteria can also result in myocarditis, although it is rare in patients with normal heart function and without a preexisting immunodeficiency. A list of the most relevant infectious organisms is below. Viral: adenovirus, parvovirus B19, coxsackie virus, rubella virus, polio virus, Epstein-Barr virus, hepatitis C virus, and severe acute respiratory syndrome coronavirus 2 (SARS‑CoV‑2, causing COVID-19) Protozoan: Trypanosoma cruzi and Toxoplasma gondii (causing Chagas disease and toxoplasmosis, respectively)

Life expectancy analysis in patients with Chagas' disease: prognosis after one decade (1973-1983).

We studied the evolution of chronic Chagas' disease in 107 patients with a positive Guerreiro-Machado reaction and 22 non-chagasic, non-heart disease control subjects for a follow-up period of 3 to 10 years (mean follow-up of 4.9 years). After completion of invasive and non-invasive studies, chagasic patients were classified into four groups: IA (normal ECG, without heart disease; 18 patients); IB (normal ECG, early left ventricular segmental abnormalities; 13 patients); II (abnormal ECG, advanced myocardial damage, no signs of heart failure; 42 patients); and III (abnormal ECG, end-stage, congestive heart failure; 34 patients). One out of five group IA patients re-studied with invasive methods evolved to group IB (20%); 4 group IB patients evolved to group II (33%) and 6 group II patients evolved to group III (15%). The life expectancy of patients in groups IA and IB (normal ECG) was similar to that of our control group, whereas in groups II and III it was significantly decreased (P less than 0.001). Nine group II patients (23%) and 28 group III patients (82%) died during the follow-up period. Main terminal events were refractory congestive heart failure, sudden death and systemic thromboembolism. Our findings suggest that chronic Chagas' disease follows an evolutionary course from asymptomatic, normal ECG group I stage to arrhythmic (II) and congestive (III) stages. Subjects with a positive Guerreiro-Machado reaction showed a significantly lower life expectancy than our control group, but only when clinical and/or ECG abnormalities were identified.

Prevalence of Trypanosoma cruzi infection in pregnant Latin American women and congenital transmission rate in a non-endemic area: the experience of the Valencian Health Programme (Spain).

This study describes the results of the health programme implemented in the Valencian Community (Spain) to achieve an early diagnosis of Chagas disease in pregnant Latin American women and their newborns. During 2009 and 2010, 1975 women living in the health districts of three university hospitals were enrolled via midwives or at the time of delivery. Diagnosis of disease was performed using two serological tests with different antigens. Congenital infection was diagnosed by parasitological, molecular or serological methods from blood samples obtained at birth or in subsequent controls. The overall seroprevalence of Chagas infection in pregnant women from 16 different endemic countries was 11·4%. Infection was higher in those from countries in the Gran Chaco Region (Bolivia, 34·1%; Paraguay, 7·4%; Argentina, 5·3%). Eight newborn infants from Bolivian mothers had congenital Chagas which represents a vertical transmission rate of 3·7%. In conclusion, this work supports the benefits of offering an early diagnosis to pregnant women and newborns during routine prenatal healthcare.

[American trypanosomiasis and chronic Chagas cardiopathy at the "Ignacio Chaves" National Institute of Cardiology].

The cumulated experience at the Instituto Nacional de Cardiolog ía Ignacio Chavez during the period from 1977 to 1988 in regard of American Trypanosomiasis (AT) and its sequelae, chronic Chagasic Cardiopathy (CHC) was reviewed. There are 39 cases. One was an acute myocarditis in a child 16 months old. Three, subacute diseases among young patients and 35 cases of chronic disease with severe heart compromise. Heart failure was the most important manifestation in 19 patients. Arrhythmia was the dominant one in 8 cases. Both conditions did occur in 8, 2 had Stokes Adams syndrome as presenting complaint and one had pulmonary embolism. The surface EKG was always abnormal and heart enlargement was noted in 36 cases on chest X rays. All patients had serologic evidence of antitrypanosoma antibodies, 28 had heart reactive antibodies in their serum, 24 had polyclonal hypergammaglobulinemia and the same proportion had rheumatoid factor. Only the acute case showed another non-organ specific autoantibody. Echocardiography was useful to show dilatation of all cavities, left ones were more severely affected, abnormal movement was also detected with lower frequency, apical aneurysm and left ventricular hypertrophy were rarely detected. Fifteen out of 16 cases studied by angiogram did show normal coronary arteries, abnormal movement was demonstrated in 62% and apical aneurysm was present in 4 cases. Studies with EKG monitoring (Holter) and electrophysiology confirmed electrical abnormalities. Although once considered an exotic disease, CHC is probably underdiagnosed because the lack of methodic studies looking for epidemiologic, clinical and seroimmunologic features in patients with dilated myocardiopathy. Cardiologist practicing in south and southeast states in Mexico should be aware of this heart ailment.(ABSTRACT TRUNCATED AT 250 WORDS)

Validation of a rapid immunochromatographic assay for diagnosis of Trypanosoma cruzi infection among Latin-American Migrants in Geneva, Switzerland.

Chagas' disease is a global public health problem due to the recent exchange of population between Latin America and other regions, including Europe. The recent development of rapid diagnostic tests (RDTs) for Trypanosoma cruzi infection may improve patient access to diagnosis and care worldwide. We evaluated the diagnostic accuracy of the Chagas Stat-Pak RDT in a cohort of undocumented Latin-American migrants living in Geneva, Switzerland. Study participants were enrolled in a primary health care center. The Chagas Stat-Pak test was performed independently on blood and serum samples. A combination of two commercialized enzyme-linked immunosorbent assay (ELISA)-based serological tests was used for comparison (reference standard). A total of 999 adults (median age, 36 years) were included in the study; the majority were women (83%) and originally from Bolivia (47%) or Brazil (25%). A total of 125 participants (12.5%) were diagnosed with T. cruzi infection; with the exception of three individuals, all individuals diagnosed with T. cruzi were originally from Bolivia. The sensitivity and specificity of the Chagas Stat-Pak test on blood samples were 95.2% (95% confidence interval [95% CI], 89.2% to 97.9%) and 99.9% (95% CI, 99.3% to 100%), respectively. When the test was performed on serum samples, the sensitivity was 96% (95% CI, 91% to 98.3%), and the specificity was 99.8% (95% CI, 99.2% to 99.9%). The concordance of test results for blood and serum samples was 99.7%. Both negative and positive predictive values were above 98%. The Chagas Stat-Pak is an accurate diagnostic test for T. cruzi infection among Latin-American migrants living in Europe. The mild deficit in sensitivity should be interpreted in light of its ease of use and capacity to provide immediate results, which allow more people at risk to have access to diagnosis and care both in countries where Chagas' disease is endemic and in countries where this disease is not endemic.

Papain-like protease

Parasites A number of parasites, including helminths (parasitic worms), use papain-like proteases as mechanisms for invasion of their hosts. Examples include Toxoplasma gondii and Giardia lamblia. In many flatworms, there are very high levels of expression of cysteine cathepsins; in the liver fluke Fasciola hepatica, gene duplications have produced over 20 paralogs of a cathepsin L-like enzyme. Cysteine cathepsins are also part of the normal life cycle of the unicellular parasite Leishmania, where they function as virulence factors. The enzyme and potential drug target cruzipain is important for the life cycle of the parasite Trypanosoma cruzi, which causes Chagas' disease. Plants

[Myocardial disease, heart failure and reactional tuberculoid leprosy].

A 60-year-old woman was admitted with congestive heart failure, essential hypertension and abdominal distension. Her son reported that she appeared with red spots in the body and that she was under dapsone therapy. Seven months ago there was sudden increase of the skin lesions. In the 11th day after admission she underwent a stroke that progressed to decerebration and she expired on the fourth day. Autopsy confirmed CHF due to chronic myocarditis related to Chagas' disease. Aneurysm of the apical region of the left ventricular chamber was also observed leading to thrombosis and systemic embolism with brain and spleen hemorrhagic infarct. In the encephalous there was edema, uncus herniation and hemorrhagic infarct of the brain stem. The skin lesions were due to reactional tuberculoid hanseniasis (RHT) with focal lesions in axillary lymphnodes, nasopharyngeal mucosa and in the posterior tibial nerve. The pathogenesis of RHT is discussed as well as its differentiation with the BT group of Ridley and Jopling and its probably relationship with the secondary tuberculoid hanseniasis reported by Ridley. The focal lesions are also discussed with END to the involvement of a peripheral nerve trunk what is said to be uncommon in this form of Hansen's disease.

Chagas disease

Chagas disease, also known as American trypanosomiasis, is a tropical parasitic disease caused by Trypanosoma cruzi. It is spread mostly by insects in the subfamily Triatominae, known as "kissing bugs". The symptoms change over the course of the infection. In the early stage, symptoms are typically either not present or mild, and may include fever, swollen lymph nodes, headaches, or swelling at the site of the bite. After four to eight weeks, untreated individuals enter the chronic phase of disease, which in most cases does not result in further symptoms. Up to 45% of people with chronic infection develop heart disease 10–30 years after the initial illness, which can lead to heart failure. Digestive complications, including an enlarged esophagus or an enlarged colon, may also occur in up to 21% of people, and up to 10% of people may experience nerve damage.

Massive right-sided cardiac thrombosis in Chagas' heart disease without left ventricular dysfunction.

A 63-year-old woman with the diagnosis of mega-oesophagus secondary to chronic Chagas' disease and no past cardiac history was referred for cardiac evaluation. The resting ECG showed right bundle-branch block, whereas a 2-D echocardiogram revealed marked right ventricular dilatation with hypokinesia, right atrial dilatation, normal pulmonary artery pressure, and normal left ventricular ejection fraction. A large, irregularly shaped mass, arising from the right atrium and protruding into the right ventricle through the tricuspid valve, with several different bizarre forms inside the right atrium during systole and/or diastole was seen on 2-D echocardiogram. Therefore, massive right-sided thrombosis can be detected in Chagas' disease patients with no overt right- and left-sided ventricular failure.

Trypanosoma cruzi infection in Elche (Spain): comparison of the seroprevalence in immigrants from Paraguay and Bolivia.

Chagas disease is a global public health problem due to the recent emigration of people from Latin America to other regions, including Europe. The aim of this study is to determine the prevalence of Trypanosoma cruzi infection among Paraguayans and Bolivians living in Elche (Spain),  a city located in the Mediterranean Coast of Spain. A cross-sectional study was conducted. Capillary blood samples were obtained through a finger prick, and collected on filter paper. An enzyme-linked immunosorbent assay and indirect immunofluorescence tests were performed to search for anti-T.  cruzi IgG antibodies in the filter papers. Thirteen out of 201 participants were infected with T. cruzi in this study, seven out of 73 Bolivians and six out of 128 Paraguayans, corresponding to seroprevalences of 9·59% (95%CI, 4·72-18·5%) and 4·69%  (95%CI, 2·17-9·85%), respectively. Palpitation, chest pain, and migration from rural endemic areas were the most common clinical and epidemiological risk factors associated with T. cruzi infection detected in the Paraguayan group. This study highlights that Chagas  disease is no longer limited to the Bolivian population living in Spain. It is important to note this wider prevalence and, therefore, not discount Paraguayans in the screening for Chagas disease in Spain. Indeed, this should be considered for all immigrants from Latin America.

Chagasic cardiomyopathy and Pompe disease: case report.

Pompe disease is a lysosomal storage disease with an autosomal recessive inheritance characterized by an insufficient activity of the acid alpha-glucosidase enzyme. The incidence varies from 1:40000 to 1:200000 live births and cardiac involvement in adults is rare. Chagas disease is an infection caused by the protozoan Trypanosoma cruzi, in which one-third of the cases progress to the chronic form, and may lead to cardiac involvement, usually from the fifth decade of life onwards. We report a case of a patient with Chagas and Pompe diseases who had early cardiac involvement and rapid evolution to heart failure. A 43-year-old male patient with a history of ischemic stroke at 28 years with gait ataxia sequelae. A few years after the episode, he experienced gait impairment and difficulty climbing stairs, attributed to stroke. A family screening for Pompe disease was carried out years later, and thus the diagnosis was made. As for Chagas disease, the investigation was performed because the patient lives in an endemic area. The cardiovascular physical examination did not show significant changes. The electrocardiogram showed sinus rhythm with left bundle branch block and first-degree atrioventricular block; the transthoracic echocardiogram demonstrated left ventricular systolic dysfunction; the Holter monitoring showed several episodes of ventricular tachycardia. The patient is undergoing optimized treatment for heart failure and enzyme replacement therapy for Pompe disease. Cardiomyopathy with early onset and with rapid evolution suggests overlap of the two diseases.

Chagas disease in Latin American pregnant immigrants: experience in a non-endemic country.

Chagas disease is a systemic chronic parasitic infection by Trypanosoma cruzi endemic in Latin America. Migration of women of childbearing age from Latin America to developed countries may spread the disease to non-endemic areas through vertical transmission. Prospective study of seroprevalence of T. cruzi infection in immigrant Latin American pregnant women during a 5-year period (from 2006 to 2010) in Spain. Seven out of 545 participants were seropositive for T. cruzi [prevalence 1.28%, 95% confidence interval (CI) 0.06-2.56]. Four (57%) were from Bolivia and three (43.%) from Paraguay. The seroprevalence in pregnant women from Bolivia was 10.26% (95% CI 4.06-23.58) and in participants from Paraguay was 6.52% (95% CI 2.24-17.5). No congenital transmission occurred. Seroprevalence of T. cruzi infection in Latin American pregnant women coming from Bolivia and Paraguay is high. Those women should be screened for T. cruzi to control mother-to-child transmission in non-endemic areas.

[Acute phase of Chagas disease in the Brazilian Amazon region: study of 233 cases from Pará, Amapá and Maranhão observed between 1988 and 2005].

Two hundred and thirty-three cases of the acute phase of Chagas disease, from Pará, Amapá and Maranhão, were observed between 1988 and 2005. One hundred and sixty were studied retrospectively from 1988 to 2002 and seventy-three were prospectively followed up from 2003 to 2005. Among the cases studied, 78.5% (183/233) formed part of outbreaks, probably due to oral transmission (affecting a mean of 4 individuals), and 21.5% (50/233) were isolated cases. Cases were taken to be acute if they presented positive direct parasitological tests (fresh blood, thick drop or Quantitative Buffy Coat, QBC) and/or positive anti Trypanosoma cruzi IgM. Xenodiagnosis was also performed on 224 patients and blood culturing on 213. All the patients had clinical and epidemiological evaluations. The most frequent clinical manifestations were fever (100%), headache (92.3%), myalgia (84.1%), pallor (67%), dyspnea (58.4%), swelling of the legs (57.9%), facial edema (57.5%), abdominal pain (44.3%), myocarditis (39.9%) and exanthema (27%). The electrocardiogram showed abnormalities of ventricular repolarization in 38.5%, low QRS voltage in 15.4%, left-axis deviation in 11.5%, ventricular ectopic beats in 5.8%, bradycardia in 5.8%, tachycardia in 5.8%, right branch block in 4.8% and atrial fibrillation in 4.8%. The most frequently observed abnormality on the echocardiogram was pericardial effusion, in 46.2% of the cases. Thirteen (5.6%) patients died: ten (76.9%) of them due to cardiovascular involvement, two due to digestive complications and one due to indeterminate causes.

Community-based screening of Chagas disease among Latin American migrants in a non-endemic country: an observational study.

Chagas disease is a parasitic disease endemic to Latin America, but it has become a disease of global concern due to migration flows. Asymptomatic carriers may host the parasite for years, without knowing they are infected. The aim of this study is to assess prevalence of Chagas disease and evaluate the participants' level of knowledge between Latin American migrants attending a community-based screening campaign. Three community-based campaigns were performed in Alicante (Spain) in 2016, 2017 and 2018, including educational chats and blood tests for Trypanosoma cruzi serology. Participants completed a questionnaire assessing knowledge about the mechanisms of transmission, disease presentation, diagnosis, and treatment. People seropositive for T. cruzi underwent diagnostic confirmation by two different tests. Results were analyzed by multivariable logistic regression and expressed as adjusted odds ratios (aORs), adjusting for age, sex, and time in Spain. A total of 596 participants were included in the study; 17% were aged under 18 years. Prevalence in adults was 11% [54/496; 95% confidence interval (CI): 8.3-14.5%] versus 0% among children. All but one case were in Bolivians. Diagnosis was independently associated with having been born in Bolivia (aOR: 102, 95% CI: 13-781) and a primary school-level education (aOR: 2.40, 95% CI: 1.14-5.06). Of 54 people diagnosed with Chagas disease (most of whom were asymptomatic), 42 (77.7%) returned to the clinic at least once, and 24 (44.4%) received treatment. Multivariable analysis showed that coming from Argentina (aOR: 13, 95% CI: 1.61-1188) or Bolivia (aOR: 1.90, 95% CI: 1.19-3.39) and having received information about Chagas disease in Spain (aOR: 4.63, 95% CI: 2.54-8.97) were associated with a good level of knowledge on the disease. Having primary level studies (aOR: 0.59, 95% CI: 0.34-0.98) and coming from Ecuador (aOR: 4.63, 95% CI: 2.52-847) were independently associated with a lower level of knowledge. Community-based interventions are a good strategy for diagnosing neglected diseases such as Chagas disease in non-endemic countries and for identifying and treating infected, asymptomatic individuals.

Clinical, electrocardiographic and echocardiographic abnormalities in Latin American migrants with newly diagnosed Chagas disease 2005-2009, Barcelona, Spain.

Following Latin American migration, Chagas disease has inevitably appeared in non-endemic countries in Europe and elsewhere. New policies are necessary to prevent transmission in those countries but the long, often undetected chronic period of the early stages of the disease also renders epidemiological studies important. The main objective of our study was to determine the presence of clinical, electrocardiogram (ECG) and echocardiographic abnormalities in a population of Latin American migrants infected with Trypanosoma cruzi at the moment of diagnosis. We performed a hospital-based observational study of 100 adult patients with newly diagnosed Chagas infection between January 2005 and December 2009. Thirty-seven patients were classified within the Brazilian Consensus on Chagas cardiomyopathy early cardiac stages (A or B1) and 49 presented pathological findings (stage B2) according to the Panamerican Health Organization Classification. Overall, 49 patients showed ECG and/or echocardiographic alterations. The presence of ECG and ecocardiographic alterations were significantly associated (p=0.038). The most frequent ECG and echocardiographic findings were right bundle branch block (12 cases) and impaired left ventricular wall relaxation (24 cases), respectively. In conclusion, ECG and echocardiographic alterations coherent with Chagas cardiomyopathy were found in a large proportion of newly diagnosed Latin American migrants infected with T. cruzi. In the mid-term, Chagas disease might become an important cause of chronic cadiomyopathy in our attendance area.

Transfusion-acquired Trypanosoma cruzi infection.

A 3(1/2)-year-old girl with Stage 4 neuroblastoma received multiple blood components and was subsequently diagnosed with Chagas disease, which is caused by Trypanosoma cruzi. All blood donors of the units that were transfused were requested to return to the collection facility for a blood sample to be tested for antibodies to T. cruzi. One first-time donor was found to be positive for the presence of T. cruzi antibodies. This donor was originally from Bolivia and immigrated to the United States 17 years previously. She had not returned to her native country since her emigration. This is the seventh reported case of Chagas disease transmission by blood transfusion in the United States and Canada. Although this would not be expected to occur in New England, it did, and this case demonstrates the significance of the immune status of patients as it relates to transfusion-acquired infections, the impact of geographic mobility in disease transmission, and the need for a licensed screening test for Chagas disease for the US blood supply.

[Cardiac complications of American trypanosomiasis (Chagas disease). Various case reports and general observations].

Three young female patients with heart problems and positive serologic tests or characteristic histologic lesions for Chagas' disease (American trypanosomiasis) are reported, one of them having presented with acute anterior myocardial infarction with only minor coronary lesions. The most prominent features of the disease are discussed with emphasis on late cardiac complications. Chagas' cardiomyopathy is the leading cause of sudden death in Latin America, where an estimated 10 to 12 million people are infected in endemic areas from southern Mexico to southern Argentina. It has also been termed "emboligenic cardiomyopathy" since arterial embolism is a very frequent complication. Embolic obstruction of a coronary artery may therefore well be the most probable cause of myocardial infarction in young people with Chagas' disease, although other mechanisms cannot be excluded.

Human and sylvatic Trypanosoma cruzi infection in California.

In August 1982, a 56-year-old woman from Lake Don Pedro, California, developed acute Chagas' disease (American trypanosomiasis). She had not traveled to areas outside the United States with endemic Chagas' disease, she had never received blood transfusions, and she did not use intravenous drugs. Trypanosoma cruzi cultured from the patient's blood had isoenzyme patterns and growth characteristics similar to T. cruzi belonging to zymodeme Z1. Triatoma protracta (a vector of Trypanosoma cruzi) infected with T. cruzi were found near the patient's home, a trypanosome resembling T. cruzi was cultured from the blood of two of 19 ground squirrels (Spermophilus beecheyi), and six of 10 dogs had antibody to T. cruzi. A serosurvey of three groups of California residents revealed antibody to T. cruzi by complement fixation in six of 237 (2.5 per cent) individuals living near the patient and in 12 of 1,706 (0.7 per cent) individuals living in a community 20 miles northeast of the patient's home, but in only one of 637 (0.2 per cent) blood donors from the San Francisco Bay area. This is the first case of indigenously acquired Chagas' disease reported from California and the first case recognized in the United States since 1955. This investigation suggests that transmission of sylvatic Trypanosoma cruzi infection to humans occurs in California but that Chagas' disease in humans is rare.

Emerging acute Chagas disease in Amazonian Brazil: case reports with serious cardiac involvement.

Four cases of serious cardiac attacks by autochthonous Trypanosoma cruzi infection from the Brazilian Amazon are reported; three of them occurred in micro-epidemic episodes. The manifestations included sudden fever, myalgia, dyspnea and signs of heart failure. Diagnosis was confirmed by specific exams, especially QBC (Quantitative Buffy Coat) and natural xenodiagnosis. Despite treatment with benznidazol, three patients died with serious myocarditis, renal failure and cardiac tamponade. The authors call attention to the emergence of this disease and reveal a previously unknown pathogenicity of T. cruzi strains in this area, added to a non-usual transmission form.


Again we are presented with a case to make a diagnosis and then, in this case, perform a confirmatory test. Now the guiding symptom is bilateral papilledema which is indicative of intracranial hypertension. Although he does not comment that a space-occupying lesion has been ruled out, given the normal neurological examination and the other symptoms, it is deduced that it is idiopathic intracranial hypertension or pseudotumor cerebri. Other data suggestive of this entity include: women of reproductive age, obesity, and diplopia (due to involvement of the VI cranial nerve). It is characteristic that the headache and visual blurriness suddenly worsen in the mornings when getting up or changing posture, producing transitory blindness (Neurology, Zarranz). The lumbar puncture invariably shows an increase in cerebrospinal fluid pressure, so the correct answer is 4.

Again we are presented with a case to make a diagnosis and then, in this case, perform a confirmatory test. Now the guiding symptom is bilateral papilledema which is indicative of intracranial hypertension. Although he does not comment that a space-occupying lesion has been ruled out, given the normal neurological examination and the other symptoms, it is deduced that it is idiopathic intracranial hypertension or pseudotumor cerebri. Other data suggestive of this entity include: women of reproductive age, obesity, and diplopia (due to involvement of the VI cranial nerve). It is characteristic that the headache and visual blurriness suddenly worsen in the mornings when getting up or changing posture, producing transitory blindness (Neurology, Zarranz). The lumbar puncture invariably shows an increase in cerebrospinal fluid pressure, so [HIDDEN].

A 29-year-old obese woman, with no past history of interest, consults for a month of intense, bilateral, pulsating headache, accompanied by horizontal diplopia and episodes of monocular amaurosis lasting seconds. Examination is normal, except for the presence of bilateral papilledema. Which of these tests do you think will allow you to fully confirm the diagnosis?

COINCIDENCE OF IDIOPATHIC INTRACRANIAL HYPERTENSION AND LEBER HEREDITARY OPTIC NEUROPATHY. A CASE REPORT.

This paper describes a case of a long-term monitoring of a patient with optic nerve swelling on the ocular background (papilledema), accompanied by symptoms of intracranial hypertension, on whom a genetic examination was performed as part of differential diagnosis, confirming Leber Hereditary Optic Neuropathy with the m.3460G&gt;A mutation. Casuistry: During the examination of a 5-year-old patient after an alleged head injury at a bouncy castle, an optic nerve papilla with unclear boundaries was described on the ocular background of both eyes. Neurological examination, including brain Magnetic Resonance Imaging, was indicated to rule out possible intracranial hypertension. Both examinations yielded a finding within the norm. After eight years of regular follow-up, the patient attended to our clinic with acute problems in terms of sudden visual impairment during baseball training. The performed eye examination revealed a deterioration of the vision of the right eye on counting fingers to 50 cm, vision of the left eye to 0.4 naturally, a slowed photoreaction of the right pupil, prominent optic nerve papilla with unclear boundaries on both eyes, dilated and more coiled vessels with a crossing phenomenon, the retinal periphery shows no focal changes. Due to the swelling of the papilla, acute deterioration of the vision and the suspected intracranial hypertension, the patient was immediately referred for neurological examination and subsequent hospitalization. There, the patient underwent computer tomography of the brain, venography of the dural venous sinuses and an initial laboratory examination that showed no pathology. There was increasing headache, nausea and vomiting throughout the period. A lumbar puncture was performed. The cerebrospinal fluid pressure before sampling was 285 mmH2O and 100 mmH2O after sampling. The biochemistry of the fluid was normal with negative microbiology. Evoked visual potentials had bilaterally prolonged latencies, which corresponds to optic nerve compression. An ophthalmological examination ruled out a drusen papilla. Using Optical Coherence Tomography, a 600 &#956;m edema was detected. The patient underwent two relieving lumbar punctures, which led to a subjective improvement without objective improvement. Finally, the neurosurgeon referred the patient for ventriculoperitoneal drainage. Due to the impaired vision and lack of response to the therapy induced, a genetic test was performed, which confirmed Leber Hereditary Optic Neuropathy with the mutation of m.3460G&gt;A. Despite the substantially improved identification of the Leber Hereditary Optic Neuropathy, the diagnosis may still be significantly delayed. The variability of initial findings, the rare incidence of the disease and few well-defined symptoms of the disease lead to significant diagnostic difficulties and late commencement of treatment. It is not possible to say whether there was a coincidence of IIH and LHON or whether the signs of IIH are a possible concomitant of the acute phase of LHON.

[Benign intracranial hypertension. History, clinical features and treatment in a series of 41 patients].

Benign intracranial hypertension (BIH) is a syndrome characterized by the abnormal elevation of the intracranial pressure with a normal composition of the cerebrospinal fluid (CSF) and in absence of ventriculomegaly or some intracranial expansive lesion. The present work seeks, by means of the analysis of diagnosed BIH patients to evaluate its epidemic, clinical and therapeutic features. 87 histories from intracranial hypertension diagnosed patients with normal cerebral CT were reviewed, between 1999 and 2002. 41 BIH patients were selected. The reached results allow us to draw the following profile, a woman (> 70%) of between 21 and 30 years (29%), smoker, obese (59%) with an recent increase of weight (37%) that consults after spending more than three months with headache (89%), alterations of the visual acuity (> 50%) and nauseas with some vomiting (> 40%). In the exploration, it presents with bilateral papilledema (100%), a CSF pressure bigger than 20 cmH2O (40,78 15,55 cmH2O) with normal composition, without alterations in the neuroradiological study results. The treatment with acetazolamide was favourable (51,2%), being definitive (70%) the lumbar peritoneal shunt when it is specified (30,7%), being improved these figures in those patients with a smaller pressure of the CSF in the moment of the diagnosis (p<0,035).

[A case of spontaneous intracranial hypotension without any history of positional headache].

A 52-year-old woman was admitted to our hospital with sudden diplopia, right ophthalmalgia, and occipital pain. The nature of her headache was continuous, and changing her position provided no relief. Three weeks before her admission, she experienced continuous cough and rhinorrhea during a few days. On admission, her neurological examination revealed right abducens paralysis, and right lateral gaze aggravated diplopia. Brain magnetic resonance imaging scan demonstrated diffuse pachymeningeal enhancement by gadolinium. Radioisotope cisternography showed a leak of cerebrospinal fluid from near the third lumbar vertebra. The first lumbar puncture revealed an initial pressure of 0 mm H2O. She was given a diagnosis of spontaneous intracranial hypotension due to a leak of the cerebrospinal fluid. During her hospitalization, she complained of vertigo and nausea. We found horizontal gaze nystagmus to left side and sensorineural deafness in her left ear, which improved in a few days. After treatment by a lumbar epidural continuous infusion of saline and a lumbar epidural blood patch, her headache and abnormal MRI findings had improved. The continuous cough before her admission may be the cause of the leak of cerebrospinal fluid. We had difficulty in diagnosis only by the history and clinical examination, because she had no history of orthostatic headache.

[The Tolosa-Hunt syndrome: report of a case with recurrent (9 times) painful ophthalmoplegia (author's transl)].

A 48-year-old woman was referred to the First Dept. of Int. Med., Nagasaki Univ. Sch. Med., in August, 1979, with a six-month history of recurrent episodes of right-sided painful ophthalmoplegia and diplopia. An epidode affected the right eye, lasted one to two weeks, and relapsed every month. On examination she had a complete ptosis on the right side and pain on the right eye. All extraocular muscle supplied by the 3rd nerve were paralysed. The pupils were equal in size both sides, reacting to light completely. Visual acuity was normal except myopia. All the other cranial nerves and the remainder of central nervous system was normal. Results of thyroid function tests and of lumbar puncture were normal. The glucose tolerance test showed a mild diabetic pattern. Blood and CSF cultures for bacteria, fungi, and acid-fast bacillus were negative. The skull, brain CT scan, and carotid angiogram were within normal limits. A tentative diagnosis of Tolosa-Hunt syndrome was made after an unproductive search for a cause for this woman's painful ophthalmoplegia and unsuccessful treatment of ophthalmoplegia with antibiotics or diet therapy for mild hyperglycemia. The patient was given prednisolone 30 mg daily orally when she had the 9th attack of painful ophthalmoplegia Pain, ptosis, and diplopia disappeared in 5 days and she did not show any recurrence of symptoms over the next 7 months.

Idiopathic Intracranial Hypertension: A Case Report.

Idiopathic Intracranial Hypertension is a rare occurrence in young, physically fit male and a diagnosis of exclusion among most patients presenting with signs and symptoms of raised intracranial pressure. Here we describe a case of a young male in the ideal weight range with no previous exposure to offending chemicals presented with a history of headache, obscuration of vision, and photopsia. On examination, there were no positive neurological findings. Increased opening pressure was found on the lumbar puncture. Ophthalmological examination revealed bilateral papilledema. Humphreyâ€™s Visual field test showed peripheral field loss. Magnetic resonance imaging scan of the brain and orbits were normal. The patient was diagnosed and managed in primary care setting after neurosurgical consultation. Though rare, we should suspect idiopathic intracranial hypertension in ideal body weighted male if the headache is persistent after other causes of headache have been ruled out.

Headache and Ophthalmoparesis: Case Report of an "Atypical" Incomplete Miller-Fisher Syndrome.

To expand the differential diagnosis of headache and ophthalmoparesis by describing a case report in which anti-GQ1b was demonstrated to be the cause. Anti-GQ1b antibody syndrome refers to a clinical spectrum of conditions that share common mechanisms and overlapping manifestations, including the Miller-Fisher syndrome, pharyngeal-cervical-brachial weakness, and Bickerstaff brainstem encephalitis. Rare atypical cases presenting as acute ophthalmoparesis (AO) without ataxia or areflexia have been described. Headache is a rare condition in these disorders. A 49-year-old woman with no history of headaches began experiencing an acute severe bilateral throbbing headache associated with nausea and photophobia. Five days later, she developed constant binocular horizontal diplopia. Bilateral paresis of both sixth nerves was noted. Her ocular fundi, tendon reflexes, and other findings of the physical exam were normal. In addition, both a brain MRI performed with gadolinium and a lumbar puncture yielded normal results. Serum anti-GQ1b IgG was found to be positive. Her symptoms resolved completely following treatment with immunoglobulins (0.4 g/kg/day for 5 days). This is the first reported case of AO related to anti-GQ1b antibodies presenting with headache as its initial symptom. The presence of anti-GQ1b antibodies should be determined in patients with headache and AO of unknown origin. Immunoglobulins could hasten the resolution of symptoms in these patients.

Pseudotumor cerebri induced by minocycline therapy for acne vulgaris.

We report a case of a young girl who developed pseudotumor cerebri while taking minocycline for acne vulgaris. A 16-year-old girl without a history of menstrual irregularity, weighing 60 kg (body mass index: 26.0%) presented with a history of 1 week of headache and sudden onset of a horizontal diplopia. Examination revealed bilateral papilledema and an abduction deficit in her right eye. Her cerebrospinal fluid had an opening pressure of 400 mm H(2)O and a normal composition. Following normal findings on computed tomography and magnetic resonance imaging, a diagnosis of pseudotumor cerebri was made. She reported receiving minocycline to treat acne vulgaris during the previous 3 weeks. The headache resolved with withdrawal of minocycline. The diplopia and papilledema resolved after two lumbar punctures, although the visual field defects persisted. The minocycline concentrations in the serum and cerebrospinal fluid taken after cessation of the drug were below the detectable level. The role of minocycline should be considered and routine ophthalmologic examination during minocycline treatment should be performed when pseudotumor cerebri occurs in patients treated for acne vulgaris.

[Video-Head impulse test with little diagnostic impact in vertigo-patients].

Video-head impulse test (V-HIT) is more and more becoming a routine test in patients with vertigo, contributing information about the vestibulo-ocular reflex (VOR). According to Ewald's second law, the unilateral pathological test points to this side's peripheral organ as being diseased. The value in clinical routine is still unclear. 171 consecutive patients with vertigo that had received a V-HIT and caloric testing at presentation in an academic ENT-department were included. By chart-review, they were categorized in different groups with unilateral peripheral, central and other etiology of vertigo, irrespective of their V-HIT result. Then the latter was analyzed within the different groups with respect to Gain, Gain-Asymmetry (GA) and Catch-up Saccades (CS). Canal Paresis (CP) from caloric testing was compared to GA. In patients with unilateral peripheral disease, 31 % showed a pathological gain (< 0.8), the mean GA was 4.53 % (± 16.72 %) and 60 % had CSs. In patients with presumed or assured central etiology, these data were 28 %, -1,56 % (± 17,89 %) and 45 %. Isolated CS occurred only sporadically. CP was not correlated with GA in all groups (p = 0,114). In this study V-HIT showed little diagnostic use, especially in separating peripheral from central disease. The lacking correlation between asymmetry in caloric testing and asymmetry of V-HIT gain challenges current pathophysiological concepts of impaired VOR.

Pseudotumor cerebri and ciprofloxacin: a case report.

We present a case of ciprofloxacin-associated pseudotumor cerebri in a 22-year-old African American woman. Withdrawal of ciprofloxacin in our patient resulted in complete resolution of ciprofloxacin-associated pseudotumor, as evidenced by a normal neuro-ophthalmic examination and a cerebrospinal fluid opening pressure of 140 mmH20. A 22-year-old African American woman presented with a headache of two weeks duration, visual blurring and horizontal diplopia after starting ciprofloxacin for pyelonephritis. An ophthalmic examination revealed that she had left eye esotropia, and a picture of the fundus demonstrated bilateral disc swelling without spontaneous venous pulsations. Magnetic resonance imaging of the brain and a magnetic resonance venogram were normal. A diagnostic lumbar puncture demonstrated an elevated opening pressure of 380mmH2O in a supine position. Laboratory examinations, including a cerebrospinal fluid exam, were unremarkable. ciprofloxacin-associated pseudotumor can cause chronic disabling headache and visual complications. Therapy is sub-optimal, often symptomatic, insufficient and complicated by side effects. When ciprofloxacin-associated pseudotumor presents in an atypical population, an inciting agent must be suspected because prompt withdrawal of the agent may lead to complete resolution of symptoms and prevent recurrence of similar episodes.

Evaluation of the Dizzy and Unbalanced Patient -- History and Physical

HINTS exam The HINTS exam is a bedside test that can help distinguish between central and peripheral causes of vertigo and dizziness. Notably, it should only be performed on patients who are currently symptomatic (acutely vertiginous). It consists of 3 parts: Head impulse: the patient should be able to maintain fixation with rapid movement of their head from side to side. Inability to do so (a positive test) is evidenced by horizontal nystagmus followed by a rapid corrective saccade. Nystagmus: Direction-changing or untestable nystagmus is considered a positive test and should prompt a workup for a central etiology. Test of skew: eyes are alternately covered. Upward deviation of the uncovered eye is a positive test and should prompt a workup for a central etiology.

Fulminant idiopathic intracranial hypertension and venous stasis retinopathy resulting in severe bilateral visual impairment.

To report a complicated case of fulminant idiopathic intracranial hypertension and concomitant venous stasis retinopathy leading to postpapilledema optic atrophy. Case report. A 34-year-old morbidly obese woman with a history of idiopathic intracranial hypertension (IIH) presented with a 1-month history of bilateral vision loss, diplopia, and left eye pain after being lost to follow-up for 6 years. Fundus examination revealed florid papilledema with venous tortuosity bilaterally. Brain and orbit magnetic resonance imaging showed bilateral globe flattening, intraocular optic nerve swelling in both eyes, and no abnormality on magnetic resonance venography. After additional workup including lumbar puncture with an opening pressure of 55 cm H2O, a diagnosis of IIH was confirmed. Medical treatment with oral carbonic anhydrase inhibitors was initiated, followed by same-day bilateral optic nerve sheath decompression and ventriculoperitoneal shunt placement the following week. Fundus examination 2 months later revealed a persistent blood and thunder fundus suggestive of bilateral central retinal vein occlusions. Over the course of 6 months, both eyes displayed postpapilledema optic atrophy with light perception and hand motion vision in the right and left eyes, respectively. On Goldmann perimetry, the patient had vague limited isolated responses in both eyes to the largest target. Fulminant IIH can present with profoundly blinding complications recalcitrant to aggressive medical and surgical intervention. Central retinal vein occlusion is an uncommon blinding complication of IIH.

The importance of visual field testing in idiopathic intracranial hypertension.

Idiopathic intracranial hypertension (IIH) is a disease of unknown cause typically affecting obese women in the childbearing years. Although headache is the most common symptom, the major morbidity of IIH is visual loss, with 5% to 10% of patients progressing to blindness. While about 95% of patients with IIH have visual loss documented by perimetry, only about one-third notice their visual loss because most loss occurs in the peripheral visual field. Since treatment decisions in IIH are made primarily by changes in visual field function, serial perimetry is the most critical test to obtain when following patients with IIH. This article describes the role of visual field testing in the monitoring of IIH patients in clinical practice, including its importance in communication among providers.

Chronic Headaches After a Concussion in an Obese 16-Year-Old Girl.

Jennifer is a 16-year-old Latina girl who is new to your practice. During her first well visit, she mentions that she has had daily headaches for 2 years. They began after sustaining a concussion in a car accident. Typically, her headaches are bilateral and "squeezing"; they occur in the afternoons and last for a few hours. Her concussion also resulted in depressed mood, which has improved over time.When you ask if her headaches have changed recently, she says that they have been worse for the last few days. The quality and severity are unchanged; however, they now occur first thing in the morning, are worse when supine, and no longer remit. In the last 2 days, she has developed new-onset blurry vision, nausea, dizziness, photophobia, and sonophobia. Although she previously experienced sadness with her concussion, she now feels irritable. She has never used tobacco, alcohol, or drugs, and she takes no medications.On examination, her body mass index is above the 99th percentile. You note mild papilledema bilaterally. She has no focal neurological deficits. The remainder of her examination is normal.You send her to an emergency department. Her head computed tomography is normal. A lumbar puncture demonstrates an opening pressure of 32 cm H2O; she feels relief after the procedure. She is admitted with a diagnosis of benign intracranial hypertension and is started on acetazolamide. What is the differential diagnosis of chronic headaches in an obese adolescent? How should a busy community pediatrician manage Jennifer acutely? What follow-up care should Jennifer receive?

Papilledema

In intracranial hypertension, the optic disc swelling most commonly occurs bilaterally. When papilledema is found on fundoscopy, further evaluation is warranted because vision loss can result if the underlying condition is not treated. Further evaluation with a CT or MRI of the brain and/or spine is usually performed. Recent research has shown that point-of-care ultrasound can be used to measure optic nerve sheath diameter for detection of increased intracranial pressure and shows good diagnostic test accuracy compared to CT. Thus, if there is a question of papilledema on fundoscopic examination or if the optic disc cannot be adequately visualized, ultrasound can be used to rapidly assess for increased intracranial pressure and help direct further evaluation and intervention. Unilateral papilledema can suggest a disease in the eye itself, such as an optic nerve glioma. Signs and symptoms

Neurology_Adams

This has proven difficult and a variety of methods have been proposed. The main concern, as alluded to earlier, is the prevention of visual loss but headache may become disabling enough to require special attention. Most patients with idiopathic intracranial hypertension will be found initially to have no, or minor visual changes aside from the papilledema; the headache and lumbar puncture pressure then guide treatment. Besides symptomatic relief of headaches, the progression of visual loss is the main concern. At least one-fourth of patients have recovered within 6 months after treatment by repeated lumbar punctures and drainage of sufficient CSF to maintain the pressure at normal or near-normal levels (<200 mm H2O). The lumbar punctures have been performed daily or on alternate days at first, and then at longer intervals, according to the level of pressure. While cumbersome and sometimes impractical, evidently, this is sometimes sufficient to restore the balance between CSF

Uncommon Presentation of Idiopathic Intracranial Hypertension in a Patient with Polycystic Ovary Syndrome: A Case Report.

Idiopathic intracranial hypertension is a rare condition characterized by increased intracranial pressure without clinical, laboratory, or radiological evidence of intracranial pathology. Early management could prevent irreversible outcomes. A 17-year-old single Arabian female of Arab origin presented with a 2-day complaint of horizontal diplopia and transient visual obscurations. She denied any history of headache or decreased vision. The patient was diagnosed with polycystic ovary syndrome a year prior to presentation. Examination revealed bilateral moderate papilledema and limited left eye abduction. However, visual acuity and fields were normal. Increased intracranial pressure was confirmed by lumbar puncture opening pressure (550 mm H2O). The cerebrospinal fluid composition and imaging of brain and cerebral venous system were normal. The diagnosis of idiopathic intracranial hypertension was confirmed and the patient was treated with acetazolamide 500 mg twice per day. The symptoms totally resolved within 3 days and the papilledema disappeared after 2 months. Awareness of such uncommon presentation of idiopathic intracranial hypertension emphasizes the critical importance of detailed ophthalmic examination and shows the good prognosis of early management.

[Ophthalmological tests value in detection of the optic neuropathy associated with pituitary tumours].

To evaluate the value of ophthalmological tests in detection of the optic neuropathy associated with pituitary tumours. 38 patients with pituitary tumours in 21-73 years old, were examined. Routine ophthalmological examination, kinetic and static perimetry (screening test, "white-on-white" and "blue-on-yellow") and PVEP were performed. In patients with recurrent tumours (group II) more changes was observed in routine ophthalmological examination, perimetry and PVEP compared to patients with early detected pituitary tumours (group I). No patient of group I present changes in routine ophthalmological examination, but they had visual field defects and/or abnormal PVEP. In group I in Ps B-Y more visual field defects was detected than in kinetic perimetry (p<0.025). In the same group hemifield PVEP was more often abnormal than full-field PVEP (p<0.01). In group II similar differences were not observed. In patients with pituitary tumours the most useful test in the optic neuropathy diagnosis are "blue-on-yellow" static perimetry and hemifield PVEP.

Severe papilledema identified 3 weeks after head injury.

A 62-year-old woman presented with rapidly developed visual disturbance without associated headache or nausea 3 weeks after head injury. Ophthalmologic examination revealed bilateral severe papilledema with retinal hemorrhage, and intracranial pressure (ICP) was 17.5 cmH2O estimated by lumbar puncture. Computed tomography and magnetic resonance (MR) imaging showed no evidence of increased ICP, except dilation of the subarachnoid space around the optic nerves with distortion of the nerves. Her visual acuity remarkably improved after steroid and glycerol treatment, and optic fundus examination revealed bilateral clear optic papillae without atrophic changes. Follow-up MR imaging demonstrated that the bilateral optic nerves had regained the normal appearance. These results indicate that the bilateral papilledema was caused by increased subarachnoid pressure around the bilateral optic nerves. We conclude that papilledema can occur with a mildly increased ICP and trapped subarachnoid cerebrospinal fluid around the optic nerves, and papilledema may progress after the ICP is normalized. Papilledema is a warning sign for increased ICP, associated with future visual loss from retinal hemorrhage. Therefore, repeated funduscopic investigation is necessary for the early diagnosis and treatment of papilledema.

Pseudotumor Cerebri with Blindness.

Pseudotumor cerebri, or idiopathic intracranial hypertension (IIH), is a syndrome of elevated intracranial pressure (ICP) of unknown etiology that occurs predominantly in obese women of childbearing age. Pseudotumor cerebri literally means "false brain tumor". It is a "diagnosis of exclusion" therefore a complete work-up to rule out life-threatening causes for increased ICP must be performed through a comprehensive history, complete physical examination, diagnostic imaging, and cerebrospinal fluid (CSF) analysis before the diagnosis can be made. The authors present the case of a young woman with headache, and near blindness due to pseudotumor cerebri. The presentation, diagnosis, and treatment options are discussed.

Demyelinating disease masquerading as a surgical problem: a case series.

We report three cases of demyelinating disease with tumor-like presentation. This information is particularly important to both neurosurgeons and neurologists who should be aware that inflammatory demyelinating diseases can present as a mass lesion, which is indistinguishable from a tumor, both clinically and radiologically, especially when there is no evidence of temporal dissemination of this disease. The first patient was a 42-year-old Malay woman who developed subacute onset of progressive quadriparesis with urinary incontinence. Magnetic resonance imaging of her spine showed an intramedullary lesion at the C5-C7 level. She was operated on and biopsy was suggestive of a demyelinating disease. Retrospective history discovered two episodes of acute onset of neurological deficits with partial recovery and magnetic resonance imaging of her brain revealed demyelinating plaques in the centrum semiovale. The second patient was a 16-year-old Malay boy who presented with symptoms of raised intracranial pressure. A computed tomography brain scan revealed obstructive hydrocephalus with a lesion adjacent to the fourth ventricle. An external ventricular drainage was inserted. Subsequently, a stereotactic biopsy was taken and histopathology was reported as demyelination. Retrospective history revealed similar episodes with full recovery in between episodes. The third case was a 28-year-old Malay man who presented with acute bilateral visual loss and confusion. Magnetic resonance imaging of his brain showed a large mass lesion in the right temporoparietal region. Biopsy was consistent with demyelinating disease. Reexamination of the patient revealed bilateral papillitis and not papilledema. Visual evoked potential was prolonged bilaterally. In all three cases, lumbar puncture for cerebrospinal fluid study was not carried out due to lack of patient consent. These cases illustrate the importance of considering a demyelinating disease in the differential diagnosis of a mass lesion. Critical analyses of clinical presentations coupled with good physical examination are vital in assisting clinicians to reach the correct diagnosis.

[Simple method of differential diagnosis of peripheral and central vertigo--development of diagnostic method and studies of 178 cases].

In patients who complain of vertigo or who have equilibrium disorders, it is often difficult to determine the etiology of the disorder, that is to determine whether it is dependent on a peripheral or central vestibular disorder. To attempt to determine the etiology in these cases, we divised a new method, the caloric eye tracking pattern test (CETP-Test). Seventeen normal subjects and 161 patients were tested. The latter group included 33 with peripheral disorders such as Meniere's disease, benign paroxysmal positional nystagmus, and others, and 128 with central disorders such as vertebral basilar artery insufficiency, cervical vertigo, and others, were tested. The cases of central disorders were limited to those patients whose eye tracking pattern before the caloric stimulation was normal. In normal subjects and in patients with peripheral disorders, it is well known that caloric nystagmus has little influence on the eye tracking pattern. In contrast, in patients with central vestibular disorders, caloric nystagmus evokes abnormalities on the eye tracking pattern, either superimposed or saccades, despite the fact that the eye tracking pattern before the caloric stimulation is normal. First we administer the eye tracking stimulation test using a target which moves horizontally at 0.3 cycle per second. Next, we perform the caloric test on the right ear, using 20 c.c. of ice water for 10 seconds. During the evoked caloric nystagmus we administer the eye tracking test once again. The eye tracking pattern is recorded for 20 seconds beginning 50 seconds after the start of the ice water injection. The procedure repeated on the left ear. The results on each case are presented as three patterns of ENG-recording. We may stat that in normal subjects and in patients with peripheral vestibular disorders, visual suppression of caloric nystagmus remains functional. Caloric induced nystagmus does not affect the CETP. In patients with central vestibular disorders, visual suppression of caloric nystagmus does not function properly because of defects in the visual suppression mechanism. Therefore, caloric nystagmus greatly influences the CETP. Consequently, the CETP may not be smooth when CETP test is administered to patients with central vestibular disorders. We may say also that the visual suppression to the vestibular nystagmus is evoked more strongly by pursuing a moving visual stimulus than by gazing a stational target. These results allow for a differential diagnosis between peripheral and central disorders.

Ocular symptoms secondary to meningeal carcinomatosis in a patient with lung adenocarcinoma: a case report.

Meningeal carcinomatosis (MC) is a rare complication associated with hematologic and solid tumors. MC develops when malignant cells gain access to the leptomeningeal space, producing several clinical symptoms. Loss of vision and ocular motility deficit are the most frequent ocular symptoms reported. Fundus examination usually appears normal, although optic nerve alterations like optic atrophy or papilledema have been described. MC diagnosis is usually completed by magnetic resonance imaging (MRI) and cerebrospinal fluid (CSF) analysis. Indicated treatment for MC usually involves intrathecal chemotherapy combined with radiotherapy, although survival rate is extremely low. A 66-year old man with stage IV metastatic lung adenocarcinoma, presented to the Ophthalmology Department with a two-month history of double vision, soft headaches and dizziness episodes. The patient presented a best visual corrected acuity of 0.7 in his right eye and 0.8 in his left eye. Diplopia was corrected with 6-prism diopters base-out prism in right eye. Funduscopy showed a bilateral papilledema, juxtapapillary exudates and splinter hemorrhages. Brain MRI showed a diffuse leptomeningeal enhancement in cortical sulcus. Lumbar puncture was performed and cerebrospinal fluid (CSF) cytology revealed malignant cells compatible with a diagnosis of MC. Intrathecal chemotherapy was administered. MC is a serious complication of systemic cancer patients, involving a poor prognosis. Early diagnosis is extremely important, although treatment is frequently aimed to reduce the symptoms and extend survival. Eye symptoms may be the chief complaint, so MC should be considered in any patient with vision loss or diplopia accompanied by neurologic symptoms and in the absence of an intraocular cause, especially in the context of systemic cancer.

[Diagnosing bilateral papilledema].

Bilateral optic disc swelling requires following a number of steps from discovery to causal diagnosis. First, it is necessary to differentiate between true optic disc swelling and disc elevation without true swelling. Then fundus examination, visual acuity and visual field, fluorescein angiography, and optical coherence tomography are performed in order to differentiate papilledema secondary to increased intracranial pressure from optic disc swelling secondary to optic neuropathy. Even if the most frequent etiology is idiopathic intracranial hypertension, the clinician must check for the absence of any signs or symptoms related to hypertension secondary to a cerebral tumor or to cerebral venous thrombosis. Fortunately, modern imaging techniques have facilitated the differential diagnoses of optic disc swelling, and the combination of magnetic resonance imaging (MRI) and magnetic resonance venography appears to be necessary each time the diagnosis of idiopathic hypertension is suggested.

Bilateral papilledema associated with retinal hemorrhagic appearance as earliest sign of spinal cord tumor.

To report a case of a 57-years-old female patient who presented with prominent bilateral proptosis and swollen disks. A 57-year-old obese patient admitted to our ophthalmic department complaining of bilateral sense of eye swelling and mild blurring vision. The anterior segment and pupillary reflex were normal on ophthalmological testing. Likewise, eye movements were preserved and she did not report diplopia in any position of gaze. Dilated fundus examination revealed bilateral hemorrhagic papilledema, pre-retinal hemorrhages in both eyes, dot and blot hemorrhages in the peripheral retina. Visual acuity was 20/20 in both eyes. Routine hematological investigations revealed increased both Von Willebrand factor and ristocetin factor and LAC positivity. The lumbar puncture (LP) showed elevated cerebrospinal fluid (CSF) proteins. Magnetic resonance imaging (MRI) with contrast of brain and spinal cord showed signs of intracranial hypertension and the presence of two lesions in continuity with each other respectively located at T12-L1 and L1-L2. Radiologic features were compatible with the diagnosis of ependymoma. A surgical procedure was conducted and confirmed the diagnosis after anatomical pathology analysis. Although rare, bilateral hemorrhagic swollen disks could be an early sign of spinal cord tumors, in particular ependymomas.

[Clinical and diagnostic criteria and differential diagnosis in the first and second stages of the development of Ménière's disease - A modern view of the problem].

The results of a comprehensive examination of 82 patients with clinical signs of definite Ménière's disease, unilateral lesion and confirmed by extratympanic electrocochleography endolymphatic hydrops are presented. The results of the study showed that only 38% of patients had cochleovestibular syndrome due to Ménière's disease. In 45% of patients, Ménière's disease was combined with other diseases: benign paroxysmal positional vertigo, vestibular migraine, persistent postural-perceptual dizziness, superior semicircular canal dehiscence. In 17% of patients cochleovestibular syndrome was due to other reasons: vestibular migraine, tumor of the posterior cranial fossa, superior canal dehiscence syndrome, Cogan's syndrome, enlarged vestibular aqueduct, otosclerosis. Complaints, medical history of the disease and life, brain MRI, temporal bone CT and pure tone audiometry in dynamics are important in suspected Ménière's disease. For 1 and 2 Ménière's disease stages the most important characteristic are: progressive unilateral sensorineural hearing loss and reduced slow faze velocity values of caloric nystagmus in dynamics, mainly in warm response if attacks of vertigo is maintain; normal head impulse test between vertigo attacks and pathological result of this test in attack of vertigo with the normalization of gain during the first day.

InternalMed_Harrison

Papilledema This connotes bilateral optic disc swelling from raised intracranial pressure (Fig. 39-12). Headache is a common but not invariable accompaniment. All other forms of optic disc swelling (e.g., 203 from optic neuritis or ischemic optic neuropathy) should be called “optic disc edema”. This convention is arbitrary but serves to avoid confusion. Often it is difficult to differentiate papilledema from other forms of optic disc edema by fundus examination alone. Transient visual obscurations are a classic symptom of papilledema. They can occur in only one eye or simultaneously in both eyes. They usually last seconds but can persist longer. Obscurations follow abrupt shifts in posture or happen spontaneously. When obscurations are prolonged or spontaneous, the papilledema is more threatening. Visual acuity is not affected by papilledema unless the papilledema is severe, longstanding, or accompanied by macular edema and hemorrhage. Visual field testing shows enlarged blind spots

Transient Visual Obscurations as the Presenting Symptom of Papilledema from COVID-19-Related Cerebral Venous Sinus Thrombosis.

Coronavirus disease-19 (COVID-19) patients are at an increased risk of cerebral venous sinus thrombosis (CVST). Rapid diagnosis and treatment are vital to ensure a favorable outcome for CVST, so clinicians need to be aware of all its potential presentations. We describe a unique case where transient visual obscurations (TVOs) from papilledema were the presenting symptoms of COVID-19-related CVST. A 43-year-old woman, who had tested positive for severe acute respiratory syndrome coronavirus-2 1 month earlier, developed holocephalic headache, TVOs, and bilateral disc edema. She did not seek medical attention until she developed TVOs. Visual acuity was 20/20 and Humphrey visual field testing showed enlarged blind spots in both eyes. She was diagnosed with papilledema and underwent magnetic resonance imaging and magnetic resonance venography of the brain, which revealed right transverse sinus thrombosis. Lumbar puncture was performed, showing elevated opening pressure and normal cerebrospinal fluid contents. Her optic disc edema resolved and visual function remained normal 6 weeks following warfarin and topiramate therapy. Recanalization of the right transverse sinus occurred after 3 months. Although rare, TVOs are important presenting symptoms of COVID-19-related CVST. Ophthalmologists, who may be the first physicians to assess patients with this presentation, should be aware of TVOs as potential presenting symptoms of CVST, so diagnoses can be made in a timely manner.

Acute Headache -- History and Physical

What is the patient's medical history? Does the patient take new medications, or have they recently changed them? Does the patient take "blood thinners"? Is this the worst headache the patient has ever experienced? Was the pain maximal at the onset? Has the patient had any difficulty moving or speaking normally? Did the patient have nausea or vomiting? Does the patient have a fever? Does the patient have any changes in vision or hearing? Does the patient have eye pain? Does the patient have any neck or facial pain? Did the patient have a seizure? Does the patient have dizziness? Does the patient have any sensitivity to light? Does the patient feel generally weak? Is there a weakness in a specific area of their body? Has the patient traveled recently? Has the patient been around sick contacts? Is the patient less than six weeks postpartum? Does the patient have a history of immunosuppression or take immunosuppressive medication?

The twists and turns of optic disk swelling in a teenager.

A 17-year-old girl developed headaches, transient visual obscuration, pulsatile tinnitus, and hearing loss during an episode of pneumonia. Neuro-ophthalmologic examination disclosed severe bilateral optic disk swelling and macular exudate in the right eye. Lumbar puncture showed an opening pressure of 55 cm H2O without neurochemical abnormalities. Subsequent magnetic resonance imaging showed no mass lesion or signs of venous sinus thrombosis, confirming the diagnosis of idiopathic intracranial hypertension. Following the lumbar puncture, the papilledema resolved but visual acuity decreased to 20/40 in the right eye despite normal color vision. Perimetric signs of psychogenic visual loss developed. This case underscores that hyperacute papilledema can simulate bilateral optic neuritis and illustrates the importance of color vision testing in distinguishing macular visual loss from optic neuropathy. It also illustrates the confounding effect of psychogenic visual loss in judging the clinical course of idiopathic intracranial hypertension.

[The Barrés test and Mingazzini test -Importance of the original paper by Giovanni Mingazzini].

In order to find a subtle hemiparesis of the arms and legs, so called "Barré's test" has been routinely used in clinical practice. This eponym has been questioned by several neurologists. To clarify this, I searched and found the original paper by Giovanni Mingazzini, reported in Revue Neurologique in 1913. He showed arm drift test with his original photo, as asking the patient to stretch his arms in front, hands in the same horizontal plane with the manner of swearing and the fingers spread. The eyes are closed. The examiner observes downward drift of the hand after one half to a minute. He described a similar test for the legs in this article. The patient in supine position raises the legs in a 45 degree angle from the bed. If the leg drops downward too early, an organic hemiparesis could be present. Barré described a new leg drift test in 1919 with a patient lying on the abdomen. He also presented the Mingazzini's arm and leg tests with photos as carried by his patient-models in his article of 1937. He did not quote the original article of Mingazzini as a reference. These brought us incorrect information to consider the presence of Barré's arm test.

Idiopathic intracranial hypertension and hemophilia A.

A patient with hemophilia A and long-standing recurrent symptoms of idiopathic intracranial hypertension is described. During his relapses, he experienced headache, and attention and language disturbance, but no visual symptoms. Hemophilia A is a rare inherited coagulation disorder secondary to factor VIII deficiency. Idiopathic intracranial hypertension has been reported in association with prothrombotic conditions and iron deficiency anemia, but not in patients with hemophilia A. Recurrent or chronic headache is not a typical symptom of hemophilia, but headache is a presenting sign of intracranial bleed in persons with hemophilia. Medical history review, clinical neurologic examination, brain magnetic resonance imaging, computed head tomography, and electroencephalogram were performed. Neurologic examination revealed bilateral papilledema during relapses of idiopathic intracranial hypertension. Multiple lumbar punctures preceded by the intravenous administration of factor VIII early in the course of the illness confirmed the presence of elevated cerebrospinal fluid pressures and absence of subarachnoid blood. He had no complications from lumbar punctures. Initial electroencephalograms showed background slowing but later normalized. Magnetic resonance imaging of the brain and computerized tomography of the head were normal. Relapses of idiopathic intracranial hypertension were eventually controlled with the administration of acetazolamide. Idiopathic intracranial hypertension may develop in patients with hemophilia A in the absence of visual symptoms. Therapeutic and diagnostic lumbar punctures were safe to perform on this patient, following the administration of factor VIII.

Alfred Bielschowsky's 1940 legacy for neuro-ophthalmology.

The author was stimulated to write this article by a 1996 visit to the University where Professor Alfred Bielschowsky was Chairman of Ophthalmology in the 1930s. Dr. Bielschowsky was one of the founders of neuro-ophthalmology. This review, with biographical notes, is presented in his honor. Dr. Bielschowsky and the author had similar disruptive experiences, of historic interest, during the Hitler regime in Nazi Germany. Professor Bielschowsky's legacy begins with his contributions to ocular physiology. For instance, his after-image test establishes the presence of retinal correspondence, important for stereoscopic vision. Alfred Bielschowsky taught how an ocular examination is critical for neuro-ophthalmologic diagnosis, localization, prognostication, and treatment. Much of our knowledge is linked with his name. Examples include "Bielschowsky's Phenomenon", explaining dissociated vertical movements, and "Bielschowsky's Doll's Head Phenomenon" (Doll's Eyes), describing proprioceptive reflexes important for localizing intracranial lesions. Dr. Bielschowsky emphasized many pitfalls in the differential diagnosis of oculomotor anomalies. For example, he cautioned against mistaking the compensatory head position in congenital fourth cranial nerve paresis for neck muscle disease. Dr. Bielschowsky's emphasis on the clinical examination remains critical despite today's advanced diagnostic equipment. His legacy is the application of physiology to patient care.