[Klüver-Bucy syndrome as the initial symptom of adult-type ceroid lipofuscinosis (Kufs' disease)]. / Síndrome de Klüver-Bucy como manifestación inicial de ceroidolipofuscinosis del adulto (enfermedad de Kufs).

AIMS: The purpose of this paper is to report the case of a patient with Kluver-Bucy syndrome caused by adult-type ceroid lipofuscinosis (Kufs' disease) and to review the literature dealing with the causes of this syndrome. CASE REPORT: A 38-year-old male examined because of behavioural changes and cognitive impairment. Brain biopsy findings were characteristic of adult-type ceroid lipofuscinosis. This patient fulfilled the criteria of Kufs' disease, since he had mixed clinical features belonging to both type A (neuropsychiatric disorders) and B (aphasia-apraxia-agnosia syndrome) of the disease. The initial symptoms included several clinical features of Klüver-Bucy syndrome (probable visual agnosia, apathy, increased sexual activity, lack of sexual inhibition, hypermetamorphopsia, increased oral behaviour and changes in dietary habits). CONCLUSIONS: Adult-type ceroid lipofuscinosis is an infrequent clinical entity that is difficult to diagnose owing to the absence of peripheral biological markers and the need to confirm such a diagnosis by means of a histopathological study.

Cytomegalovirus mononucleosis as a cause of prolonged fever and prominent weight loss in immunocompetent adults.

Four immunocompetent adults presented with protracted fever lasting > 6 weeks and severe weight loss, associated with primary cytomegalovirus (CMV) infection. Each patient had spleen enlargement, lymphocytosis and hypertriglyceridaemia, but recovered spontaneously. A further 20 immunocompetent patients with primary CMV infection were also reviewed, and all presented the usual clinical picture of CMV mononucleosis. It was concluded that CMV mononucleosis should be considered in the differential diagnosis in patients with prolonged fever and weight loss if lymphocytosis is present.

[Nifedipine in the treatment of moderate and severe arterial hypertension. Long-term effect on arterial pressure and on the left ventricle]. / Tratamiento de la hipertensión arterial moderada y severa con nifedipino de liberación osmótica. Efectos a largo plazo sobre la presión arterial y la masa ventricular izquierda.

INTRODUCTION AND OBJECTIVES: Moderate-to-severe hypertension is less prevalent than mild hypertension, but it is responsible for more incidences of complications. Its complex treatment requires several drugs, and is often inadequate. This study assessed the efficacy and safety of nifedipine GITS (oral release osmotic system) as monotherapy, in addition to the effects on left ventricular hypertrophy, after a long term follow-up (one year). PATIENTS AND METHODS: Thirty patients with diastolic blood pressure above 105 mmHg were studied after a short placebo phase. They received nifedipine GITS as monotherapy in a single daily dose of 30 mg; dose titration was made the first three months according to response and until they reached figures equal to or below 95 mmHg. By M-Mode echocardiogram, left ventricular mass index and systolic function were calculated at the end of the placebo phase and at 3, 6, 9 and 12 months. Hematological parameters, lipid profile, electrolytes and liver enzymes were assessed at the same periods. RESULTS: In 70% of the patients the blood pressure reached values of 140-90 mmHg. In 16 patients with adequate M-mode recordings, a 12% reduction in left ventricular mass was observed without modification in systolic function. Five patients were retired: two due to adverse events and three due to different reasons (drop out, evidence for secondary hypertension). There were no changes of clinical significance in the hematological or biochemical parameters and no hypertensive crisis occurred. CONCLUSION: The monotherapy with nifedipine GITS was effective in reducing high blood pressure, induced regression in ventricular hypertrophy and showed good tolerance in one year follow-up.

[Myoglandular inflammatory polyp located in the distal end of the rectum]. / Pólipo inflamatorio mioglandular localizado en el extremo distal del recto.

Recently, a new class of colorectal polyps has been described. Because of their histological characteristics, they have been called inflammatory myoglandular polyps. We report the case of a forty years old male, in haemodialysis for six years, who noticed the growth of an anal tumour. It is the first description of one of them placed in the distal part of the rectum.

[Vesical malacoplakia]. / Malacoplasia vesical.

We present a new case of vesical malacoplakia in a 55-year-old male with acute clinical cystitis accompanied by hematuria. We underline the favorable response to treatment with cholinergics and sulfonamides. We comment on the different etiopathogenic and endoscopic points of view. We review the different therapeutic concepts of this singular affection.

[An asymptomatic splenic hamartoma treated by conservative surgery]. / Hamartoma esplénico asintomático tratado con cirugía conservadora.

We present the case of an 8-year-old boy accidentally diagnosed of a splenic tumor by ultrasound and computed tomography scan of the abdomen. The patient was treated surgically with a conservative surgical technique (wedge resection and splenorraphy). The histologic study revealed a hamartoma and by immunohistologic study a clear differentiation with the splenic capillary hemangioma could be made.

[A severe rhinological mycosis: mucormycosis. A report of 3 cases]. / Una micosis rinológica grave: la mucormicosis. Presentación de tres casos.

Mucormycosis is a fungal infection, the most frequent form begins in the nose and the paranasal sinus and can rich the brain. It is a fulminant and often fatal disease, not well known by many specialists. The authors present three cases with positive cultures, histology and postmortem studies, drawing attention to the importance of its prompt diagnosis and aggressive treatment.

Doxazosin in the treatment of mild or moderate essential hypertension: an echocardiographic study.

Sixteen patients with mild or moderate essential hypertension received 1 to 8 mg/day of doxazosin (mean daily dose, 2.7 mg). Blood pressure reduction (supine and standing) was highly significant (p less than 0.001), and no significant changes in heart rate were observed. A significant reduction (p less than 0.01) in left ventricular mass was seen without a change in left ventricular systolic function. All side effects were mild, and only one patient withdrew from the study.

[Streptococcal gangrene and so-called "flesh-eating bacteria disease". A rare and devastating disease]. / La gangrena estreptocócica y la así llamada "enfermedad de las bacterias devoradoras de carne". Una infección rara y devastadora.

Streptococcal gangrene, an unusual form of necrotizing fasciitis with fatal outcome, has been recently rediscovered and has gained popularity with the name "disease of flesh eating bacteria". The incidence of this and other severe diseases caused by Streptococcus pneumoniae has been suggested to be increasing. Only three patients with this disease have been studied at our institution in the last 12 years and in a review of a bacteremic infections caused by beta-hemolytic streptococci a significant increase of these infections was not observed. We report here the clinical and pathological characteristics of streptococcal gangrene as well as a review of the more recent literature.

[Granulomatous angiitis of the basilar artery related to herpes zoster of the 7th cranial nerve]. / Angeítis granulomatosa de la arteria basilar en relación con herpes zoster del VII par.

Granulomatous angiitis of the central nervous system is an uncommon condition characterized by vascular wall necrosis, inflammatory exudate and development of giant cells in medium and small size vessels. The pathogenesis of this disease remains unknown, but it has been associated with immune complexes, mechanical factors and infection by the varicella-zoster virus. We report a young patient who presented with herpes zoster involving the VII cranial nerve and contralateral hemiplegia. Subsequently, pontine infarct and fatal subarachnoid hemorrhage developed. The pathological study showed granulomatous angiitis of basilar artery.

[Beta-hemolytic streptococci in tonsil hypertrophy and recurrent tonsillitis]. / Estreptococos betahemolíticos en la hipertrofia amigdalar y amigdalitis recidivantes.

BACKGROUND: The recurrence of streptococci acute tonsillitis is a complication that often motivates the tonsillectomy. We studied the colonisation of tonsils and adenoids by S. pyogenes and other beta haemolytic streptococci in both surgical indications, recurrent tonsillitis and tonsillar hypertrophy. METHODS: We made for aerobic culture the following specimens, throat swabs, tonsils and adenoids tissue corresponding to 47 children referred for tonsillectomy. RESULTS: S. pyogenes was isolated in 11 cases (23.4%) of tonsils and other beta haemolytic non A streptococci was isolated in 11 cases, of them, group C streptococci was the most frequent with six cases. However in the recurrent tonsillitis group, S. pyogenes was isolated more significantly (47%) that other beta haemolytic streptococci (5.8%). Otherwise in the tonsilar hypertrophy group, S. pyogenes was isolated in the 10% while that other streptococci was isolated in the 33.3%. The culture of 38 adenoids yielded S. pyogenes and beta haemolytic group C streptococci in 6 cases each one (15.7%). CONCLUSIONS: S. pyogenes was isolated more frequently in recurrent tonsillitis that other micro-organisms while that in the tonsilar hypertrophy group predominated streptococci beta haemolytic non A, S. aureus and H. influenzae. Is of interest that the adenoids in our study showed an important reservoir of beta haemolytic streptococci. The throats swabs yielded less of the half of all beta haemolytic streptococci isolated in tonsilar tissue cultured.

[Bladder paraganglioma. Apropos of a case]. / Paraganglioma vesical. A propósito de un caso.

A case of non-secreting bladder paraganglioma whose clinical manifestation was that of painless hematuria is described herein. Treatment was by partial cystectomy.

Nitrendipine and atenolol in essential hypertension in young and middle-aged patients: effect on serum lipids and left ventricular mass.

This study was designed to compare the antihypertensive efficacy of nitrendipine and atenolol in young and middle-aged patients with mild or moderate essential hypertension and to assess treatment effects on plasma lipids and potential changes in left ventricular mass (LVM). After 2 weeks off medication and a 4-week placebo phase, patients who met the inclusion criteria [sitting diastolic blood pressure (DBP) 95 to 114 mm Hg, age below 50 years] entered a 12-week dose-adjustment and maintenance period with nitrendipine or atenolol. Serum lipids were determined before and after therapy. At the same time, LVM was evaluated echocardiographically (M mode). Twenty-two patients completed the double-blind, randomized study. After 12 weeks on nitrendipine, the systolic blood pressure (SBP) and DBP were reduced (p less than 0.005 and p less than 0.001, respectively). No significant changes in heart rate were observed. There were no changes in the lipid profile, and LVM was reduced from 93.7 to 23.4 to 82.4 +/- 22.6 g/m2 of body surface (p less than 0.05). On atenolol the SBP and DBP were reduced (p less than 0.001 and p less than 0.001, respectively). The expected reduction in heart rate was significant (p less than 0.05). Total cholesterol and LDL cholesterol increased by 11% (p less than 0.05) and 12.3% (p less than 0.01), respectively. HDL cholesterol showed a small reduction. Tryglycerides increased by 22% (n.s.). LVM did not change. In conclusion, nitrendipine and atenolol showed comparable antihypertensive efficacy.(ABSTRACT TRUNCATED AT 250 WORDS)

[Neurocysticercosis in a tertiary hospital. New advances in the diagnosis and treatment]. / Neurocisticercosis en un hospital terciario. Nuevos avances en el diagnóstico y tratamiento.

Cerebral cysticercosis is an endemic parasitosis in many developing countries with a great variability in its presentation and prognosis. In the last 37 years (1960-1996) we have studied 18 patients at Fundación Jiménez Díaz. Sixteen were spanish; ten of them (62.5%) came from rural areas and they all were seen before 1988. Ten patients (56%) were males and seven (44%) females. The mean age was 49 years (range: 22-80). The most common symptoms at diagnosis were: seizures (61%), headache (55%), visual disturbances (39%), symptoms of intracranial hypertension (33%), mental disturbances (33%) and other focal symptoms (33%). CT and NMR showed changes in all cases, and hydrocephalia, presence of brain calcifications and ventricular or subarachnoidal parenchymal cysts were the most common findings. Three patients received antiparasitic therapy (praziquantel and albendazol), two of them associated with surgery. The most common surgical procedure was cyst exeresis and ventriculo-peritoneal shunts. The evolution was favorable in 90% of cases.

[Nocardiosis: clinical observations apropos of 9 cases]. / Nocardiosis: observaciones clínicas a propósito de nueve casos.

We have retrospectively analyzed 9 cases of nocardiosis that were studied in our hospital over the past 10 years. 66% of the patients had an associated disease and half of them were on steroid treatment. The clinical manifestations of the infection were pulmonary and cutaneous and in 44% of cases, it disseminated to the central nervous system. The diagnosis was made in all cases isolating the microorganism from pulmonary secretions, puss, spinal fluid and blood. Nocardia asteroides was isolated in 8 cases, and nocardia caviae in one case. There was a 55% mortality rate. The different clinical presentation and evolution are reviewed, as well as prognostic factors and antimicrobial treatment of nocardiosis.

Histological examination of the rat after long-term exposure to subtoxic automotive exhaust gas.

Regarding the potential impact of traffic-born air pollutants on public health, in recent years attention has increasingly been focused on the possible effects on the cardiovascular system. In order to investigate this problem further, the influence of long-term exhaust gas exposure on rats has been studied. One hundred Wistar rats of either sex were exposed 5 X 8 h/week up to 28 months to an atmosphere polluted by the emissions of an idling Otto engine, CO concentrations held constant at 90 ppm. A second group (50 rats) was exposed to 250 ppm for 6 months. Blood parameters and body weight were controlled. Specimens of CNS, heart, vessels, kidney etc. were investigated light microscopically. Focal necroses of the myocardium with inflammatory reactions as well as interstitial fibrosis were found in the heart muscle of the 90 ppm group. In the 250 ppm group endothelial proliferations, edema of the intima and deposits of proteoglycanes in the media were observed. We conclude that subtoxic concentrations of CO which only lead to slight morphologic changes may aggravate preexisting lesions caused by high risk conditions, e.g., hypertension or hypercholesteremia.