Heredopathia atactica polyneuritiformis (Refsum's disease)

A female patient started to develop deafness and vertigo at the age of 29. In the following years she became atactic and retinitis pigmentosa was discovered. The diagnosis of Refsum's disease was reached on the grounds of the high concentration of phytanic acid in plasma. The patient died 23 years after onset of the first symptons. Liver, spleen and kidney showed lipofuscinosis and pigment-laden macrophages. The retina was atrophic and its pigment discontinuous. The meninges contained lipid-laden macrophages. The nerve cells in brain and spinal cord as well as the astrocytes and perivascular macrophages stored substances weakly PAS-positive and sudanophilic. The nerve cells accumulated lysosomes and residual bodies. In the astrocytes, the residual bodies were extremely polymorphous and contained inclusions with bilamellar ribbon-like structures. In the oligodendroglia the residual bodies displayed high electron density and finger print-like pattern. Peroxisomes were found in glial cells and microperoximes in neurons. The ultrastructural findings in the present case demonstrate that in terminal stages phytanic acid can reach the brain parenchyma passing through the BBB. Further autopsy studies will be necessary to determine whether these changes are consistent findings in Refsum's disease

Lectin histochemistry of mixed gliomas demonstrating an intermediate cell type

18 cases oí' low-graded rnixed gliornas were studied using the two lectins Concanavalin A (Con A) and Peanut lectin (PNA). Con A stained cytoplasm and processes of turnoral astrocytes, whereas PNA stained cell membranes of tumoral oligodendrocytes. Con A and PNA are reliable markers for astrocyte and oligodendrocyte areas of mixed gliornas, respectively. A part of cells were overlappingly positive for both lectins. They expressed an oligosaccharide pattern of both gliorna types and represented a third, interrnediate cell type of rnixed gliomas. The existence of interrnediate cells close to astrocytic and oligodendroglial cell types in mixed gliomas could result frorn transformation processes of neoplastic glial cells or frorn the malignant transformation of a common glial precursor cell

Lectins as differentiation markers of human gliomas

The lectins Concanavalin A (Con A). Ricinus communis agglutinin (RCA-1), Peanut agglutinin (PNA) and Wheat germ agglutinin (WGA) as well as the irnmunomarkers for glial fibrillary acidic protein (GFAP) and myelin basic protein (MBP) were used in a series of 21 glial turnors (4 pylocytic astrocytornas, 5 grade 11 astrocytornas, 3 anaplastic astrocytornas, 4 glioblastornas and 5 oligodendrogliornas). ConA binds to al1 tumoral astrocytes in low grade astrocytomas, as well as to well differentiated tumoral astrocytes in anaplastic astrocytomas and glioblastornas. RCA-1 has a similar behaviour. PNA, and to a lesser degree WGA, binds selectively to the oligodendroglial plasma membrane in well differentiated oligodendrogliomas. The results suggest that these lectins are markers of differentiation in gliomas rather than of malignancy

Encephalopathy with astrocitic residual bodies. Report of a case and rewiev of the literature

Biopsy and autopsy findings in a girl who died at 7% months after having suffered from progressive axial hypotonia, myoclonus, EEG changes and retarded psychomotor development. Inclusions consisting of lamellar profiles, situated in membrane-bound cytosomes were found mainly in astrocytes, but also in neurones and in axons of peripheral nerves. Lipofuscin bodies were also increased in number. The patient belongs in the same category as cases studied by Towfighi et al. (1975) and Martin et al. (1977). Etiology and pathogenesis of this syndrome remain unknown. It is suggested, however, that the pathological changes observed might have been caused by the administration soon after birth of anti-epileptic dmgs (diphenylhydantoin, clonazepam and nitrazepan)

Heart mitochondria in rats submitted to chronic hypoxia

The effect of prolonged exposure to normobaric hypoxia on the mitochondria of myocard of rats exposed for several weeks to 8 and 7% O2 has been morphometrically evaluated. Twelve male Wistar rats housed in Nalgene cages (2 per cage) with a batch of six cages placed in plexiglass chambers were maintained in air/N2 mixtures containing different concentrations of 02. Six animals kept in similar cages under normoxia served as controls. When at day 60 the FIOZ was reduced to 8%, the weight increase stagnated and after the 81st test day, on which the hypoxic animals were subdivided into 8% and 7% groups the weight curve showed a decrease in the mean body weight for both groups. The arrest and the following loss of weight beyond the 85th day may be interpreted as the expression of a limit reached in the compensation capacity. In the 8%-group the shape of the mitochondria varied more markedly often with budding and furrowing of the surface. In the 7%-group bizarre shapes and wide variations in size with a decided shift towards larger mitochondria were noteworthy. While rats kept under 8% oxygen exhibited a numerical increase in myocardial mitochondria compared to controls, the mitochondria of the 7%-group were numerically reduced. The results suggest that hypoxia of 8% oxygen is compensatable, if only to some extent, by an increasing surface of mitochondrial membranes, and that further reduction of oxygen causes compensation mechanisms to fail as seen by the severe alterations of the mitochondrial population of the cardiomyocyte in the 7%-group

La manipulación psicosomática de la personalidad

Medico-biological manipulation is the application of scientific-natural methods and techniques on the part of a human being who is conscious of the fact that he is operating upon another -equally human- being, with the aim of inducing the latter to act in an unconscious manner in any particular direction. Manipulation has to fulfíl, therefore, the following prerequisites: 1) the manipulator has to be conscious of his actuation upon the other human being; 2) the person being manipulated has to be a conscious individual, at least potentially; that is, he must be a human person, but 3) the manipulated subject must not be conscious of the fact that he is being operated upon. Bearing these prerequisites in mind, we can conclude that: 1) genetic manipulation is not possible, nor is cerebral manipulation, the latter form being understood within the framework of direct action on the brain -by means of neuro-surgical or neuro-physiological methods- since apart from being ineffective (at least it is very difficult to achieve concrete planned results). the abolition of conscious knowledg,e is lacking; 2) the utílízation of drugs or the so-called brainwash are much closer to the concept of manipulation, Insofar as their applications and effects are concerned, althoughin these cases also, it is difficult to foresee the final result achieved upon the person being manipulated. In a word, an objective analysis of the biological techniques and of their results demonstrates that any fear in a future -robotization - of personality, founded wholly from a cultural point of view -think, for exampie, of the manipulation carried out by p'ropaganda or through publicity- does not appear to have much chance of being converted into a realíty, at least with reference to medical methods