[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.

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

Objetivos. Presentar un paciente con síndrome de Klüver-Bucy causado por ceroidolipo fuscinosis del adulto (enfermedad de Kufs) y revisar la literatura sobre a las causas de dicho síndrome. Caso clínico. Se trata de un varón de 38 años de edad valorado por cambios conductuales y deterioro cognitivo. La biopsia cerebral fue característica de ceroidolipo fuscinosis del adulto. Este paciente cumplía los criterios de la enfermedad de Kufs definida, ya que presentaba características clínicas mixtas de los tipos A (alteraciones neuropsiquiátricas) y B (síndrome afaso-apractoagnósico) de dicha enfermedad. Los síntomas iniciales incluyeron varias características clínicas del síndrome de Klüver-Bucy (probable agnosia visual, apatía, aumento de la actividad sexual, falta de inhibición sexual, hiper-metamorfopsia, aumento de la conducta oral y cambios en los hábitos dietéticos). Conclusiones. La ceroidolipo fuscinosis del adulto es una entidad infrecuente y de difícil diagnóstico dada la ausencia de marcadores biológicos periféricos y la necesidad de la confirmación de dicho diagnóstico mediante un estudio histopatológico

Aims. The purpose of this paper is to report the case of a patient with Klüver-Bucy syndrome caused by adult-type ceroid lipofuscinosis (Kufs’ disease) and to review the literature dealing with the causes of this syndrome. Case report. A38-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 mixedclinical 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 dietaryhabits). 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

[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.

[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.

[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.

[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.

[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.

[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.

[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.

[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.

[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.

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.