Acta neuropathologica. Supplementum最新文献

Sural or superficial peroneal nerve biopsies of patients with clinical diagnosis of Friedreich's ataxia were studied. Patients were divided in two groups, typical and abortive forms: loss of fibers accompanied by axonal atrophy and segmental demyelination are the basic changes in both groups, although the decrease in number of myelinated fibers was most severe in typical FA. In the cases with slower progression there is a tendency to form onion bulb complexes.

On autopsy of a 70-year-old hypertensive male a large arteriovenous angioma in the rostral part of the left cingular gyrus, surrounded by remains of old hemorrhages, and a multilobular saccular aneurysm at the junction of the left anterior cerebral and the communicating arteries were found. The vessels of the angioma and its feeding artery as well as the aneurysm were thrombosed. More recent thrombosis obliterated the venous drainage of the angioma and the oral part of the superior sagittal sinus. Both congenital weakness of the arterial wall and increased circulatory demand might have been responsible for this combined condition.

The demyelinating lesion in MS being characterised by an evolutive polymorphism, led to different opinions about the identification of the hypercellularity. The present work deals with data concerning the abundance and the early appearance of macrophages (M) within the lesion, studied with lipid histochemistry methods. The disposition of 3 various types of previously described M, with different lipid content, demonstrates the progressive centrifugal evolution of the recent lesion. At the edge the M are filled exclusively with intensely Baker (phospholipids) stained myelinic granules whereas inside they have a mixed myelinic and sudano-osmiophylic (esterocholesterolic) composition as revealed by Sudan red, Oil rd, Otan and Schultz methods. Surrounding vessels, in the centre of the lesion, M contain exclusively sudano-osmiophylic (esterocholesterolic) granules and crystals. This arrangement of M as to AGE is due to the progressive disintegrative cycle of phagocyted myelin: the youngest at the edge and those undergoing myelin disintegration towards the centre. It is demonstrative for the progressive and centrifugally oil-spot-like extension of the lesion and might explain the round-shape of the small lesions which stop their extension without any morphological correlations.

Six cases of papillary meningioma are described. Five patients are still alive 2 months, and 2, 7, 8 and 14 years after the operation respectively. No metastases or recurrences have been noted. One patient died from an intercurrent disease 2 years after operation. In all cases the papillary pattern was associated with one of the typical well known forms of meningioma. One case showed histologic features of anaplasia, i.e.: local high cellularity accompanied by mitotic activity and pleiomorphism. Only one or two features suggestive of a higher growth dynamism were noted in the other cases. The mechanism of the papillary structures is discussed. Vasotropism and a feeble cohesion between the cellular perivascular "crowns" are thought to be responsible for the papillary pattern which should be interpreted as a secondary phenomenon. It appears likely that the papillary pattern of meningioma is not always an expression of anaplasia when no other clinical and histologic signs of malignancy are present.

A case of diffuse meningocerebral angiodysplasia is described in which type 4 adenovirus was isolated from the brain biopsy. The pathological features were similar to those previously described by various authors (1,3,4,5) and were not indicative of an infective process. Status epilepticus in the presence of extensive arteriovenous shunting in this dysgenetic cerebromeningeal vasculature is probably responsible for the extensive necrosis observed in the cortex and white matter.

The outcome of viral infection appears to be the result of a complex interplay of both viral and host factors. The virus-host cell relationship as well as the host's immune response are the major factors determining the type of infection and the expression of the disease. Demyelination disease appears to be rare complication of a systemic viral infection. The mayor factor in its expression is an altered response to the virus favoring the establishment of a noncytocidal abortive infection of the glial cells, which in turn induce a complex of potentially pathogenic immune mechanisms.

A microcytotoxicity assay to prove directly against myelin basic protein directed cytotoxic T cells has been developed. Spleen and lymph node cells of myelin basic protein immunized strain 13 guinea pigs were restimulated in vitro with basic protein for 5 days and used as attacking cells. Autologous spleen cells were coated with about 10(7) molecules basic protein by hydrophobic attachment, labelled with 51Cr and used as targets. Concentration dependent lysis (74% corrected lysis) was measured with attackers gained 12 days post immunization. Controls performed using unspecific (PHA) stimulated attackers and non BE coated target showed no lysis. Lysis was shown to be MHC restricted. The test may serve as a tool for the assay of human demyelinating diseases.

The authors report here the case of a female patient who developed symmetrical polyneuropathy of the lower limbs a few months after she was found to have a myeloma with cryoglobulinemia. In musculocutaneous nerve biopsy material from this patient, electron microscopy showed pathological changes in the nervous tissue together with axonal degeneration and demyelination. But the most striking finding was the presence in the endoneurial space, of numerous masses made up of closely packed tubular structures. These masses occurred also in the walls and even in the lumen of all the vasa nervorum. Their morphologic features and dimensions were identical to cryoprecipitate which the authors extracted from the serum and which they examined with electron microscope.

A comprehensive neuropathological study has been undertaken in 53 adult subhuman primates subjected to a single sagittal plane angular acceleration impulse. The types of injury produced correlated well with the input force and were very similar to those seen in fatal nonmissile head injuries in man. There was a fracture of the skull in 14 animals. Subdural haematoma occurred in 33 animals, contusions in the frontal and temporal regions of the brain in 39, and discrete intracerebral haematoma in 4. Brain shift and tentorial herniation were also seen. There was evidence of hypoxic brain damage in 22 animals, and central chromatolysis in Betz cells and in the motor nuclei of the brain stem in 16.