Prevalence of Toxocara-induced liver granulomas, detected by immunohistochemistry, in a series of autopsies at a Children's Reference Hospital in Vitoria, ES, Brazil.

The aim of this investigation was to study the frequency of visceral larva migrans (VLM) granulomas in autopsies at a Children's Reference Hospital in Vitoria, ES Brazil, where anti-Toxocara antibodies are frequently detected in the serum of children admitted at the hospital. Two liver fragments from 310 autopsies of children aged between 1 and 15 years were paraffin embedded, and sections were stained with hematoxylin and eosin and submitted to detection of Toxocara antigens using a rabbit anti-Toxocara serum. Among the 24 cases with granulomatous lesions, ten had eosinophil-rich granulomas positively stained with the anti-Toxocara serum. Some were typical epithelioid granulomas, with a positive reaction in multinucleated giant cells, epithelioid cells, or necrotic debris. The results showed that VLM granulomas are the most frequent granulomatous hepatitis in children in our county. This agrees with the high prevalence of anti-Toxocara antibodies in the serum of children admitted to the Children's Reference Hospital. The 3.2% frequency of liver VLM granulomas in autopsies is less than the 30-39% frequency of positive serology in these children, probably reflecting the low larval burden in infected children.

p53 Protein expression in meningiomas - a clinicopathologic study of 55 patients.

Formalin-fixed, paraffin-embedded surgical specimens from 55 meningiomas were immunostained with monoclonal antibody against p53 (Immunotech, Marseilles, France). There were 38 women and 17 men ranging between 9 and 82 years of age. The patients were divided into two groups: group I (47 patients) - mildly symptomatic and group II (eight patients) - severe initial symptoms. There were 43 WHO grade I, 11 WHO grade II, and one WHO grade III meningiomas according to the 1999 WHO classification of brain tumors. In group A (n=49), the tumors were attached to the dura mater of the skull base, and in group B (n=6) they were not. Tumor volume ranged from 1.23 cm(3) to 303.37 cm(3). Peritumoral edema was classified into three grades: grade 0 (n=28) no or minimum edema, grade I (n=19) edema reaches at least half of one cerebral hemisphere, and grade II (n=8) holohemispheric edema. Immunostaining for p53 was found in the neuronal nuclei of 18 meningiomas. It was not correlated with gender, age, anatomical location, tumor volume, degree of peritumoral edema, or Simpson's resection grade. Labeling for p53 was observed in 9/12 meningiomas of WHO grades II or III and was correlated with severity of symptoms (P=0.033) and histological grade (P=0.0009). Immunolocalization of p53 was not correlated with the degree of malignancy.

Failure of Partial Hypophysectomy as Definitive Treatment in Cushing's Disease Owing to Nodular Corticotrope Hyperplasia: Report of Four Cases.

Nodular corticotrope hyperplasia is a rare pathology causing Cushing's syndrome owing to a primary pituitary disease or ectopic CRH production. In this study, we evaluated the laboratory and pathological findings and results of transsphenoidal pituitary surgery in four patients with Cushing's disease. Dynamic tests of pituitary-adrenal function (dexamethasone suppression, metyrapone, CRH, and DDAVP tests) were done before and after transsphenoidal pituitary surgery. Plasma and total urinary cortisol, serum 11-deoxycortisol, and plasma ACTH were determined by RIA. Hormonal dynamic tests and radiologic studies were compatible with a pituitary ACTH source. The transsphenoidal surgery revealed the presence of corticotrope hyperplasia confirmed by immunoperoxidase stain and a preserved reticulum framework in the removed pituitary tissue of these four patients. The pituitary surgery led to a short period of improvement in two of the patients (1 and 4), a 3-yr remission in one patient (patient 2), and no improvement in one (patient 3). We conclude that although our patients appear to have inadequate suppression with high-dose dexamethasone, there is no way to diagnose this pathology presurgically, and that total hypophysectomy, bilateral adrenalectomy, and irradiation are the only alternatives for definitive treatment. A CRH-secreting ectopic tumor could not be found in our patients either before or after surgery in the follow-up period.

Non-epileptic myclonus and mitochondrial encephalomyopathy

Foram examinados dois irmäos portadores de uma síndrome miclónica progressiva comn discretos sintomas cerebelares. O exame neurológico mostrava sinais cerebelares moderados e papilas pálidas; mioclonais assíncronas, arrítmicas e assimétricas, um déficit artrestésico e ausência de fraqueza muscular. A atividade de base do EEG era moderadamente lenta e sem atividade irritativa. A TC era normal em ambos os casos. A estimulaçäo fótica intemitente aumentava a freqüência dos abalos mioclônicos que se tornavam bilaterais e sincronos, progredindo para uma crise tônico-clônica generalizada. Potenciais evocados e RMN em um caso foram normais . Drogas anticonvulsivantes foram ineficazes no controle das mioclonias. O diagníostico de encefalomiopatia mitocondrial foi realizado através do achado em espécimes musculares de membranas basais espessadas, com degeneraçäo miofibrilar e um número elevado de mitocondrias distribuídos perifericamente e com uma matriz densa, granular e com alguns vacúolos. Os achados clínicos e eletrográficos sugerem uma origem subcortical para esta síndrome mioclônica

Mitochondrial dysfunction in myasthenia gravis: report of a case

É relatado o caso de um paciente de 11 anos de idade com oftalmoparesia extrínseca, ptose palpebral bilateral e tetraparesia desde os 7 anos de idade. A concentraçäo de anticorpos contra receptor de acetilcolina por radioimunoensaio foi 0,6 nM/1; a pesquisa de anticorpos contra músculo estriado foi negativa. Exame eletromiográfico revelou decremento de 26,1%. Foi tratado com brometo de piridostigmina na dose de 60 mg/d. Submetidos a biópsia de tecido muscular estriado (biceps braquial esquerdo) com avaliaçöes por métodos histoquímicos e microscopia eletrônica, que revelaram: acúmulos de mitocôndrias na regiäo subsarcolemal na coloraçäo pela SDH; aumento da concentraçäo de mitocôndrias e corpúsculos eletrodensos a microscopia eletrônica; esses achados säo sugestivos de miopatia mitocondrial. Em conseqüência da interrupçäo das drogas anticolinesterásicas ocorreu piora das manifestaçöes deficitárias, disfagia e dispnéia. Reintroduzidos os anticolinesterásicos, associados a imunossupressäo com corticosteróides, houve melhora e retomada pelo paciente de suas atividades habituais. Destarte, é discutido o caráter inespecífico das alteraçöes morfogenéticas e disfunçöes de mitocôndrias em outras patologias neuromusuclares, incluindo a miastenia grave, como neste casos em particular