[Lafora's disease: a possible diagnosis of juvenile dementia]. / Doença de lafora: uma possibilidade diagnóstica das demências juvenis.

Five patients aged 12 to 16 years old were admitted between 1987 and 1994 at the neurological unit because of uncontrolled epilepsy. They had had a normal development until the adolescence, when a history of poor school performance and memory difficulty started. It is emphasized the clinical sequence of dementia followed by tonic-clonic seizures and myoclonus, the positive family history and the difficult therapeutic management, in spite of multiple anticonvulsant combinations, including sodium valproate and clonazepam. The clinical and laboratory differential diagnosis were discussed to show that similar cases should be submitted to skin biopsy looking for Lafora bodies in apocrine and eccrine glands. However, the liver is considered as the most reliable site for the biopsy, which in our study showed positivity in four out of the five cases.

[Congenital left sinus of Valsalva aneurysm]. / Aneurisma congênito do seio de Valsalva esquerdo.

Two cases of left sinus of Valsalva congenital aneurysm (SVCA), incidentally found are described. The authors call attention on rarity of them, and present new concepts about their morphogenesis and incidence. They also suggested a higher incidence of asymptomatic and undiagnosed cases of SVCA should be considered.

Alterações do sistema de gênese e condução do estimulo cardíaco na fase aguda da doença de Chagas humana / Changes of the genesis and cardiac stimulation conduction systems in human acute Chagas' disease

O estudo histológico do sistema de gênese e condução do estimulo cardíaco em 4 portadores da forma aguda da doença de Chagas revelou alterações inflamatórias e degenerativas, por vezes com necrose lítica e de coagulação, envolvendo o nódulo sinusal, as paredes atriais direitas (conexões intermodais), o nódulo AV, o feixe de His e seus ramos e fibras de Purkinje. As lesões mais importantes foram observadas nas paredes atriais, nódulo sinusal e na porção intramiocardica do ramo direito. Estes achados oferecem boa correlação com os dados eletrocardiográficos, onde predominam as arritmias sinusais nesta fase da doença. Eles também são sugestivos de que as lesões da fase aguda da doença de Chagas são capazes de deixar sequelas cicatriciais nos que passam para a fase indeterminada da doença

Hemangioendothelioma of the lung and pleura: report of three cases.

Three patients with hemangioendothelioma of the lung and pleura are reported. Two patients underwent removal of the pulmonary lesion: both recovered and were alive one year and eight years later. The patient with pleural involvement had a radical excision of the pleura with the vascular tumor. He died almost two years later. The main histologic feature of this lesion is the presence of neoplastic cells within the endothelium, evidenced by reticulin silver stain.

Aneurisma dissecante de arteria pulmonar associado a esquistossomose pulmonar.Relato de um caso. / Dissecting aneurysm of the pulmonary artery, associated with pulmonary schistosomiasis. Report of a case

Os autores relatam um caso de aneurisma de arteria pulmonar, em pacientes com 26 anos, portadora de esquistossomose pulmonar. O eletrocardiograma, radiografia de torax e cateterismo cardiaco mostraram sobrecarga ventricular direita e hiperrtensao pulmonar. A necropsia revelou tratar-se de aneurisma dissecante de arteria pulmonar com necrose cistica de media e lesoes angiomatoide e plexiforme na vasculatura pulmonar. A evolucao clinica e etiologia do aneurisma sao discutidos, concluindo os autores que a hipertensao e o fator descisivo para a formacao do aneurisma, sendo que a necrose cistica da media aparece secundariamente