"Estudos histoquímicos, bioquímicos e ultraestruturais permitiram recentemente distinguir dois tipos de entidades nosológicas dentre as doençãs conhecidas como Idiotia amaurótica familiar (IAF). O primeiro, que inclui a doença doença de Tay-Sachs, ou tipo infantil (IAF), é constituída por doenças de armazenamento de gangliósides. Sua patogenia é conhecida, baseando-se em deficiências de enzimas lisossômicos. O segundo grupo, cuja patogenia é desconhecida, foi recentemente designado "Ceróide-lipofuscinose neuronal" (CLN) por Zeman e colaboradores. Corresponde aos tipos infantil tardio, juvenil e adulto IAF, caracterizando-se histopatologicamente pelo acúmulo intraneuronal de lipopigmentos... Os autores descrevem quatro casos de Ceróide-lipofuscinose neuronal, sendo dois irmãos do tipo infantil tardio e um casal de irmãos do tipo juvenil. Um autópsia e três biópsias corticais revelaram distensão de moderada a intensa dos neurõnios por lipopigmentos, assim como glicose e atrofia ..."

Resumo do Artigo: em 361 natimortos necropsiados no período de 1966 a 1971, a incidência de sífilis congênita, baseada na pesquisa de osteocondrite sifilítica foi de 9,4%. É possível que a queda de incidência da sífilis em natimortos, nos anos de 1970 e 1971, se deva às medidas profiláticas executadas no Serviço de Pré-Natal da Faculdade. No grupo de fetos sifilíticos, houve maior número de mortes na fase de prematuridade viável que no grupo de fetos não sifilíticos. Tal diferença, porém, não estatisticamente significante.

Em 177necrópsias de pacientes portadores de insuficiência cardíaca crônica, a causa da insuficiência foi: hipertensão arterial essencial e/ou asteroscleose (39,5%), cor pulmonale (14,1%), doença reumática do coração (12,2%), doenças renais hipertensivas (10%), cardiomegalia idiopática (10%), miocardite chagásica (6,1%), cardiopatias congênitas (3,9%), aortite sifilítica (1,1%), endocardite bacteriana subaguda (0,5%), miocardite relacionada com a dematomiosite (0,5%) e pericardite constritiva (0,5%). A alta frequência de cardiomegalia idiopática justifica um estudo clínicopatológico mais acurado dessa patologia, assim como sua relação com a miocardite chagásica, com a qual se pode confundir-se. Neste estudo admite-se que a principal causa da cardiomegalia idiopática seja a desnutrição prolongada dos pacientes. Em cerca da metade dos pacientes com hipertensão essencial a asterosclerose coronária foi leve ou raramente não ocorreu. Nesses pacientes a insuficiência cardíaca ...

A family is reported, in which a Portuguese father and four Brazilian siblings are afected by Andrade´s familial amyloid polyneuropathy (FAP). The father was studied in 1940 and was the first case of FAP reported in the literature, though not identified as such at that time. Three siblings died as consequence of FAP. A comparative study of the clinical profiles is correlated to the pathological findings obtained in for autopsies. Besides the classical neurologic and digestive disturbances, the following are worth mentioning: severe thickening of the leptomeninges due to amyloidosis (cases 1 and 2)with contrast retention in myelography (case 2), unusual pathologic evolution of an elbow fracture (case3), intense renal involvelment ( case3) and Argyll Robertson sign (case 2 and 3).

A case of glioblastoma multiforme of the medulla oblongata in a 13-year-old girl is reported. Clinical features included two-month evolution, involvement of cranial nerves, tetraplegia and sudden respiratory arrest. Anatomically, a necrotizing glioblastoma multiforme involving the lower half of the medulla and the neighborring cervical cord was found. 18 examples of primary glioblastoma of the oblongata were collected from the literature, but it is suggested that several others are probably included, though not emphasized, in the numerous published seres of brain stem gliomas.

Human ependymoblastomas are a recently identified type of primitive malignant glioma with ependymal differentiation (Rubinstein, 1970). Two infantile examples were first described in detail by Rubinstein (1970), who later examined another two (Rubinstein,1972b). The tumour cells were highly inmature and uniform, with plentiful mitoses and extensive leptomeningeal dissemination. They were often arranged in true rosettes, a feature considered diagnostic of ependymomas (Willis, 1968; Russell and Rubinstein, 1971). Similar or identical cases were reported by Zulch and Kleinsasser (1957), Glasauer and Yuan (1963), Wentworth and Birdsell (1966) and Hirano, Ghatak and Zimmer-mann (1973). We would also include the case pictured in figure 17-C of Zulch´s atlas of brain tumors (1971) and at least a few of the " Primitive Neuroectodermal Tumors" reviewed by Hart and Earle (1973) . To the best of our ...

a 38 year-old woman complained of headache, visual impairment, and diabetes isnispidus for 18 months prior to rapid onset of Crushing syndrome and amenorrhea. An autopsy disclosed a pituitary carcinoma invading the right cavernous sinus and two liver metastases. The tumor cells were chromophobic and agranular, with scerse polymorphism and rare mitoses. A review of the literature showed that the literature showed that the association of Cushing syndrome to pituitary carcinomas increases in direct proportion to the occurrence of metastases. Such associationwas npoted in almost half of the reported cases of pituitary carcinomas with extracranial dissemination.

É raro o acometimento do sistema nervoso central e suas meninges por actinomicetos (Actinomyces e Nocardia) 9,11,13,22. Em recente revisão de 181 casos de actinomicose humana 6, apenas cinco apresentaram lesões cerebral, sendo três com envolvimento extenso. A bibliografia nacional sobre actinomicose revela somente seis casos de comprometimento neurológico 2,16,19,27, todos publicados entre 1934 e 1946, não tendo o assunto recebido atenção ulteriormente.

The origin of smooth muscle cells in the aortic intima of rabbits following medial necrosis due to orthostatic collapse (stress) was investigated. eigtheen test animals and six controlswere used. Regeneration of smooth muscle cells in the necrotic media inconspicious or did not occur, and was prominent in the adjacent inner layer. The latter presented muscle cells in changing developmental stages. Immature muscle cells within the intima suggest the origin of the muscle cells in the intima itself. Possibly these elements originated from fibroblast-like undifferentiated subendothelial cells. The latter were rare in the normal aortic intima and more numerous following the colapse. Regenaration of the muscle cells in the intima itself. Possibly these elements originated fro fibroblast-like undifferentiated subendothelial cells. The latter were rare in the normal aortic intima and more numerous following the collapse. Regenaration of muscle cells was inconspicuous or n ot observed ..