Neurological manifestations of cardiac myxoma: experience in a referral hospital.

INTRODUCTION: Cardiac myxoma is an important but uncommon cause of stroke in younger patients. Few published case series analyse the frequency and clinical presentation of neurological complications in patients with myxoma. OBJECTIVE: To list all neurological complications from cardiac myxoma recorded in our hospital in the past 28 years. PATIENTS AND METHODS: We retrospectively reviewed the neurological manifestations of cardiac myxoma in patients treated in our hospital between December 1983 and March 2012. RESULTS: Of the 36 patients with cardiac myxoma, 8 (22%) presented neurological manifestations. Half were women and mean age of patients was 52.4 ± 11.6 years. Sudden-onset hemiparesis was the most frequent neurological symptom (63%). Established ischaemic stroke was the most common clinical manifestation (75%), followed by transient ischemic attack. The most commonly affected territory corresponded to the middle cerebral artery. Myxoma was diagnosed by echocardiography in all cases. Mean myxoma size was 4.1cm and most of the tumours (63%) had a polypoid surface. All tumours were successfully removed by surgery. There were no in-hospital deaths. CONCLUSIONS: Cardiac myxomas frequently present with neurological symptoms, especially ischaemic events (established stroke or transient ischaemic attack), in younger patients with no cardiovascular risk factors. The anterior circulation is more frequently affected, especially the middle cerebral artery. Echocardiography can facilitate prompt diagnosis and early treatment of the lesion.

Incidence and etiological mechanism of stroke in cardiac surgery. / Incidencia y mecanismo etiológico de ictus en cirugía cardiaca.

OBJECTIVE: We studied patients who had experienced a stroke in the postoperative period of cardiac surgery, aiming to analyse their progression and determine the factors that may influence prognosis and treatment. METHODS: We established a protocol for early detection of stroke after cardiac surgery and collected data on stroke onset and a number of clinical, surgical, and prognostic variables in order to perform a descriptive analysis. RESULTS: Over the 15-month study period we recorded 16 strokes, which represent 2.5% of the patients who underwent cardiac surgery. Mean age in our sample was 69 ± 8 years; 63% of patients were men. The incidence of stroke in patients aged 80 and older was 5.1%. Five patients (31%) underwent emergency surgery. By type of cardiac surgery, 7% of patients underwent mitral valve surgery, 6.5% combined surgery, 3% aortic valve surgery, and 2.24% coronary surgery. Most cases of stroke (44%) were due to embolism, followed by hypoperfusion (25%). Stroke occurred within 2 days of surgery in 69% of cases. The mean NIHSS score in our sample of stroke patients was 9; code stroke was activated in 10 cases (62%); one patient (14%) underwent thrombectomy. Most patients progressed favourably: 13 (80%) scored≤2 on the modified Rankin Scale at 3 months. None of the patients died during the postoperative hospital stay. CONCLUSION: In our setting, strokes occurring after cardiac surgery are usually small and have a good long-term prognosis. Most of them occur within 2 days, and they are mostly embolic in origin. The incidence of stroke in patients aged 80 and older and undergoing cardiac surgery is twice as high as that of the general population.

[Uncommon localization of osteomyelitis]. / Osteomielitis de localización inusual.

Group A beta-hemolytic streptococcus (GAS) causes almost 10% of acute hematogenous osteomyelitis (AHOM). These entities are frequently located in long bones (femur, tibia and humerus), and only 6-8% of all AHOM are located in the pelvis. This uncommon localization delays diagnosis and can lead to inappropriate management, with negative effects on outcomes. We present the case of a 6-year-old boy with high fever, pain in the right hip and difficulty in walking due to pelvic AHOM and septic shock caused by GAS. We also provide a review of the literature.

[Mitochondrial acetoacetyl-CoA thiolase deficiency: neonatal onset]. / Déficit de acetoacetil-CoA tiolasa mitocondrial: inicio en el período neonatal.

We present the case of a 4-day-old newborn with serious dehydration, polypnea, hypertonus and lethargy. Blood analysis showed severe metabolic acidosis with ketonemia, ketonuria and elevation of the GAP anion. Urine analysis revealed increased excretion of 2-methyl-3-hydroxybutyrate acid, tiglycine, and 2-methylacetoacetate acid. Neonatal onset of mitochondrial acetoacetyl-CoA thiolase (T2) deficiency is exceptional. Most patients have no clinical symptoms in the neonatal period. This entity should be considered in patients with acute metabolic acidosis and ketosis with normal glycemia and aciduria. The urine contains large amounts of 2-methylacetoacetate and its decarboxylation products. In the neonatal period, this inherited disorder of metabolism can produce severe hydroelectrolyte disorders in the form of a gradual process or acute episodes, which can occasionally be fatal.

Acute-subacute lead poisoning. Clinical findings and comparative study of diagnostic tests.

A prospective study of an epidemic outbreak of acute lead poisoning characterized by unusual clinical and analytic manifestations was carried out. Its source was traced to lead-contaminated flour. Thirty-two adult patients presented with classic clinical symptoms of saturnism; however, 13 severely poisoned patients simultaneously manifested unusual clinical features such as hemolytic anemia and multiorgan dysfunction. We compared the diagnostic value of the different toxicity markers in 68 of 136 adults exposed to lead. Porphyrin precursors, urine levels of lead, and results from an edetic acid-provocative test gave poor diagnostic information and low correlation values with lead levels in blood. However, erythrocyte delta-aminolevulinic acid dehydratase activity showed a closer correlation with lead levels in blood, although we observed a frequent overlap of results in severe cases. The test for levels of lead in blood was the most reliable, suitable, and simplest test performed, and gave the best correlation with the external dose of lead, the clinical situation, and the toxicologic effects described by 12 different analytic data.

[Progressive multifocal leukoencephalopathy associated with human immunodeficiency virus infection: the clinical, neuroimaging, virological and evolutive characteristics in 35 patients]. / Leucoencefalopatía multifocal progresiva asociada a la infección por el virus de la inmunodeficiencia humana: características clínicas, de neuroimagen, virológicas y evolutivas de 35 pacientes.

BACKGROUND: The clinical, neuroimaging, virologic and evolutive characteristics of progressive multifocal leukoencephalopathy (PML) in 35 AIDS patients are studied. PATIENTS AND METHODS: PML was diagnosed by clinical and neuroimaging criteria in 32 patients and by autopsy in other three. The detection of JC virus (JCV) was done by PCR and further hybridization of the amplified DNA in peripheral blood lymphocytes, urine and CSF. RESULTS: 127 of 930 HIV positive patients were admitted by neuropsychiatric symptoms and of them 35 (SD 27.6%) by PML. The PML patients had a mean CD4 lymphocytes count of 75.3 (82.0)/x 10(6)/l and a HIV viral load of 330,698 (538,971) copies of RNA/ml. Thirty patients did not receive any anti-retroviral therapy or only transcriptase inhibitors monotherapy and five triple anti-retroviral therapy, including a proteases inhibitor. Multiple hypodense lesions on CT (53.1%) and T2 hyperintense lesions on MRI (58.3%) were the most frequent neuroimaging findings. JCV was detected in 20/21 (95.2%) LMP patients: 18/19 detections in lymphocytes, 6/8 in CSF and 4/6 in urine. The mean survival without and with antiretroviral therapy were 3.0 (0.47) and 21.4 (4.4) months (p < 0.001) in 34 patients followed. PML progressed to death in 31/34 patients (91.2%), and remained stable in 3/34 (8.8%). A patient was lost for follow-up. CONCLUSIONS: The application of clinical and neuroimaging criteria and the detection of JCV in CSF are useful for high presumption diagnosis of PML without brain biopsies. JCV detection in lymphocytes and in urine have a much lower predictive value. The evolution and survival of this disease can improve with triple anti-retroviral therapy including a protease inhibitor.

[Infectious endocarditis of the native valve: its epidemiological profile and an analysis of its mortality between the years 1984 and 1993]. / Endocarditis infecciosa sobre válvula natural: perfil epidemiológico y análisis de la mortalidad entre los años 1984 y 1993.

BACKGROUND: The description of the epidemiologic profile and analysis of the mortality of infectious endocarditis (IE) observed from 1984-1993. METHODS: One hundred thirty episodes of IE in a native valve (30 in drug addicts [IVDA] and 20 cases of nosocomial acquisition) were analyzed with right/left/bilateral localization (42/84/4, respectively), infection of the mitral/aortic/tricuspid valve (52/47/34, respectively) and the etiology was determined as Staphylococcus aureus in 52 cases, 41 Streptococcus, 13 negative coagulase Staphylococcus, and 11 Enterococcus. High risk IE were identified by uni and multivariate analysis (MVA). RESULTS: The incidence of IE ranged from 0.36 and 0.70 cases x 1.000 admitted adults/year (mean: 0.50). Transthoracic echocardiography detected bacterial vegetations in 67% of the cases with the validity to predict the development of embolisms being 55%. MVA showed the embolic episodes (present in 45% of the IE) to be associated with the IVDA patients and prolonged fever. The latter complication, being defined as > or = 10 days of fever under appropriate treatment, was observed in 32% of the cases and was due to mild (n = 15) and severe causes (n = 27). Postembolic septic complications were associated to fever with MVA. Twenty three patients died (18%), 2 IVDA and 5 nosocomial IE, mainly due to heart failure (n = 13). The independent risk factor predictors for death (p < 0.05) were: age > or = 60 years (mortality 34%), cerebral embolisms (55%), severe heart failure (37%), and the exclusion of the patient as a candidate for surgery (73%). To the contrary, right IE (mortality 0%) and cardiac surgery (5%) favoured survival. CONCLUSIONS: To improve the prognosis of infectious endocarditis in high risk patients more opportune cardiac surgery accepting greater risks should be performed.

[The clinical significance of culturing Toxoplasma gondii on blood and other organic media]. / Significado clínico del cultivo de Toxoplasma gondii en sangre y en otros medios orgánicos.

BACKGROUND: The aim of this study was to determine the value of the Toxoplasma gondii culture in blood and in other organic fluids in HIV positive and negative patients. METHODS: Retrospective analysis (October 1990-May 1992) was carried out including all patients with positive cultures for T. gondii admitted to the Hospital Central of Asturias. The parasite was identified by monoclonal antibodies against the tachyzoite membrane. All patients with positive cultures were treated with pyrimethamine and sulphadiazine. RESULTS: Three hundred two samples from 256 patients, seropositive and seronegative for HIV, were analyzed. Of the seropositive group 8/45 (18%) had positive cultures for T. gondii versus 9/211 (4.3%) of the seronegative group (p = 0.002). Of the 19 positive samples, 15 were from blood, 3 from bronchoalveolar lavage and one from the vitreous fluid. Four out of 9 patients (44%) with AIDS and encephalic toxoplasmosis (ET) had blood cultures positive for T. gondii. Another 4 patients with AIDS presented toxoplasmenia without visceral involvement. Of the 9 HIV seronegative patients (3 immunodepressed patients), 4 had pulmonary toxoplasmosis, one ocular toxoplasmosis, and other clinical forms of toxoplasmosis were seen in the remaining 4. All the patients evolved to cure except 2 cases coinfected by cytomegalovirus who died. CONCLUSIONS: The identification of Toxoplasma gondii may be performed by blood cultures in half of the patients with AIDS and encephalic toxoplasmosis and in an undetermined percentage of the other clinical forms both in immunocompetent and immunodepressed subjects. In addition, toxoplasmemia has been registered in AIDS patients preceding any other organic seating of the parasite. Early antitoxoplasma therapy may, therefore, be effective.

[Muscle tumor in a girl with HIV infection: extranodal non-Hodgkin's lymphoma]. / Tumoración muscular en una niña con infección por VIH: linfoma no hodgkiniano extranodal.

Non-Hodgkin's lymphomas are the most common cancers in children with human immunodeficiency virus (HIV) infection. Extranodal disease is the most common form of clinical presentation. Treatment with aggressive chemotherapy and highly active antiretroviral therapy has improved the prognosis of these children. We present a 10-year-old severely immunocompromised girl with HIV infection and non-Hodgkin's lymphoma manifested by a muscle mass.

[Leigh syndrome resulting from a de novo mitochondrial DNA mutation (T8993G)]. / Síndrome de Leigh producido por una mutación de novo T8993G en el ADN mitocondrial.

INTRODUCTION: Several degenerative neurological diseases are caused by mutations in the mitochondrial gene coding for subunit 6 of the ATPase. Thus, NARP (neurogenic weakness, ataxia, and retinitis pigmentosa) and Leigh syndromes are associated to a T8993G mutation when the percentage of mutant mitochondrial DNA is low (60 90%) or high (>90%), respectively. Leigh syndrome is also caused by a second mutation in the same position T8993C. CASE REPORT: The patient, a boy that died at 6 months, had generalized hypotonia, psychomotor delay, hepatomegaly, choreic movements and hyporreflexia. MRI showed hypodensities in the basal ganglia and brain stem as well as hyperlactacidemia. Molecular genetic analysis of the mitochondrial DNA showed that the patient had the T8993G mutation in a percentage higher than 95%. No mutated DNA was detected in blood of the proband s mother, maternal aunt and grandmother. CONCLUSIONS: The point mutation T8993G may occur de novo, at high levels, causing neurodegenerative diseases.

[Anaplastic large cell lymphoma. Report of an unusual case]. / A propósito de un linfoma anaplásico de células grandes con manifiesta peculiaridad de la relación clínica-patológica.

An anaplastic large cell lymphoma which fulfills all the criteria required for its inclusion among the soft tissue forms is reported. We point out in this case, in addition to its low frequency in adults, the severe systemic involvement of the disorder with out generalization, the lack of expression of the ALK1 protein, frequently positive in the generalized forms and the absence of response to polichiomiotherapy. We think that this case emphasizes the heterogenicity if the disorder on the clinical pathologycal relation ship of this kind of lymphomas.

[Castleman's disease: a histological concept for a wide clinical spectrum]. / Enfermedad de Castleman: un concepto histológico para un amplio abanico clínico.

A literature review about Castleman's disease after a clinical case with different manifestations of this entity was done. Special emphasis on the histological and clinical aspects of the sickness was given. The different pathological patterns and their broader spread do not match necessarily with the different clinical presentations described. Further conclusions regarding their evolution, prognosis and treatment response can not be easily obtained from these data.

[Epidemiological study of prisoners at risk for AIDS in a Spanish prison]. / Estudio epidemiológico en reclusos con riesgo de SIDA en una prisión española.

Different proportions of the several AIDS risk practices in a Spanish prison during 1988 were studied. Also a epidemiologic study of the infectious diseases of the prisoners with some risk of this type was carried out. Of 135 prisoners with risk of AIDS, it was found that 91.2% of them were drug addicts. The index of positive HIV in all the population study was of 65.2%. 81.5% of the sample had at least one B hepatitis marker and 11.1% had HBs-Ag. 15.4% of the last group also showed HBe-Ag positive. Histories of non-A, non-B hepatitis were not evaluated in 9.6% of all the patients studied. 53.3% had sexually transmitted diseases. It is important to mention the high index of active tuberculosis found (12.6% of the sample), which is higher than the maximum values found in various similar papers. We did not find any differences in the grade of affectation between HIV+ and HIV-.

[Incidence of primary gastric lymphoma and H. pylori infection in the central zone of Asturias]. / Incidencia de linfomas gástricos primarios y de afectación por H. pylori en la zona central de Asturias.

OBJECTIVE: We review our experience with primary gastric lymphomas type B of low (MALT) and high grade in a population of 366,635 people during 4 years and after performing 9,268 gastroscopies. RESULTS: The incidence of these lymphomas in our medium is similar to that found in the world population. Possible disagreements among the biopsy histology and that of the subsequent gastrectomy samples is pointed out. CONCLUSIONS: The frequency of H. pylori involvement in our primary gastric lymphomas is similar to that reported in the literature for the age of our patients, for the MALT lymphomas an well as for the type B high grade.

[A case of uterine leiomyoma with pulmonary metastasis]. / Un caso de leiomioma uterino con metástasis pulmonares.

In a 34-years-old patient complaining about persistent dry cough, multiple bilateral pulmonary nodules were observed. The extremely good condition of the patient and the history of resection four years before of an uterine myoma suggested a pulmonary leiomyomatosis. The hystological study after a pulmonary segmentomy confirmed this diagnostic suspicion. The presence of benign pulmonary metastasis in an uterine leiomyoma is a very rare complication. We review the literature therapeutic aspects of this interesting entity.

[A case of pulmonary sarcoidosis induced by interferon alfa treatment in a female patient with hepatitis C]. / A propósito de un caso de sarcoidosis pulmonar inducido por tratamiento con interferón alfa en una paciente con hepatitis C.

In spite of the generalized use of alpha interferon to treat hepatitis C there are very few cases in the literature on pulmonary sarcoidosis due to this therapy. A new case with this diagnosis is reported and the possible pathogenic mechanisms driving alpha interferon to sarcoid histologic changes are discussed.

[Simultaneous acute infection with human immunodeficiency virus and cytomegalovirus]. / Infección aguda simultánea por virus de inmunodeficiencia humana y citomegalovirus.

2 cases of simultaneous acute infection caused by HIV and CV that showed clinical features of infectious mononucleosis with spontaneous resolution and good evolution is presented. After 12 months following them up, both are symptom free carriers of HIV and without clinical features of CV infection. These cases had a good evolution, which is contrary to what is described in other medical literature.

[Necrotic leukoencephalitis, a new infrequent form of presentation of HIV encephalopathy]. / Leucoencefalitis necrotizante, una forma infrecuente de presentación de la encefalopatía por VIH.

A case of a female infected by HIV whose first manifestation was a focal defect of the motor function caused by a brain lesion shown in the CT scan and NMR as a hemispheric mass with discreet expansive effect and central necrosis. The histologic study showed a necrotic leukoencephalitis related to HIV as etiologic agent. We point out the difficulties in diagnosing and treating this disease.