[Torpid degeneration of the lateral pallidum with Bielschowsky bodies. Light and electronmicroscopic findings (author's transl)]. / Torpide verlaufende Degeneration des äusseren Pallidumgliedes mit Bielschowsky-Körperchen. Licht- und elektronenmikroskopische Befunde

In a 60-year man with a very slowly progressive motor disturbance probably of torsion systonic or choreoathetotic type which started in childhood, we found an isolated degeneration of the exterior pallidum with intraneuronal deposition of Bielschowsky bodies and only slight deficit of neurons. The Bielschowsky bodies show the same structure as corpora amylacea and Lafora bodies. They might also be the result of a cellular disturbance mainly concerning the carbohydrate metabolism. In this case they are localised only in the neurons of the exterior part of the pallidum--in contrast to the organogenitically otherwise deriving (from the nucl. entopeduncularis) intact neurons of the pallidum internum--and they induce, after an excessive accumulation of the metabolites, not till extremely late loss of neurons. This circumstance explains the very torpid progress of this disease.

Dystrophy of Purkinje cell dendrites and vacuolar myelinopathy of the upper brain stem in young Turkish twin girls: secondary encephalopathy or genetic disorder?

Two Turkish twin sisters exhibited alteration of muscle tonus and coordination together with a disturbance of eye motility and a tendency toward central tonoclonic seizures at the age of 2 months. The two children died at the ages of 8 1/2 and 12 months. Microscopic findings in the cerebellum of one of the cases showed cactuslike thickenings of the Purkinje cell dendrites and grumose degeneration in the nucleus dentatus. In addition, vacuolar myelinopathy of the myelinated fiber systems of the upper brain stem was observed. A hereditary disorder of the central nervous system is discussed.

On the clinico-pathological significance of multinucleated giant neurons in the hypothalamic arcuate nucleus.

Several multinucleated giant neurons were demonstrated in the arcuate nucleus of hypothalamus in 5 of 35 autopsied patients. In the 2 male patients, these cells showed intranuclear cytoplasmic inclusions characteristic of a persistent humoro-neural feedback effect in gonadal atrophy. Presumably, they had no pathologic effect on the neuroendocrine function of the arcuate nucleus. In the remaining 3 patients, markedly obese females, multinucleated giant neurons showed dysgenetic, dysplastic features but no evidence of feedback effect. Since severe adiposity associated with giant neurons were present in only these 3 patients, the question of a special form of hypothalamic obesity arises. Likewise, neuronal hypertrophy of the pars subventricularis of the arcuate nucleus, as demonstrated in anorexia nervosa [Hart 1971], suggests a possible participation of this nucleus in the regulation of nutrition and of body weight. Until now, it has been attributed to the ventromedial nucleus and to the lateral portions of the hypothalamus. This appears to support the proposed interpretation in our cases.

On the ultrastructure and immunocytology of arcuate neurons with perpetuated feedback effect.

Electron microscopy reveals rough endoplasmic reticulum and other cytoplasmic organelles in the intranuclear spheroids of human arcuate neurons with feedback phenomenon. After peroxidase-antiperoxidase technique was applied some of these arcuate nerve cells demonstrated luteinizing hormone releasing hormone immunoreactivity.

[Morphologic changes in the myelin-poor hypothalamus in involution and senescence in relation to the involution of the gonads]. / Morphologische Veränderungen im markarmen Hypothalamus während der Involution und im Senium in Abhängigkeit von der Rückbildung der Keimdrüsen.

Caused by diseases or in senescence gonadal atrophy entails morphological alterations in certain hypothalamic neurons particularly in the arcuate and subventricular nucleus. In consequence of an inefficacious and perpetual feedback these cells develop phenomenons of activation on their nucleoli. The continuous nuclear-nucleolar changes are present down to the senium and likely must be seen in relation to the persistently elevated gonadotropin levels in the old age.--Another remarkable finding is the hypertrophy of the subventricular nucleus (Sheehan and Kovacs).

[Melanosis cerebelli. Ultrastructure and infrared absorption of the pigment (author's transl)]. / Melanosis Cerebelli. Elektronenmikroskopische und infrarot-spektrophotometrische Untersuchungen bei der Melanose des Zahnkerns und der cerebellaren Körnerzellschicht.

The pigment present in melanosis cerebelli where it is deposited in the dentate nucleus and within the cerebellar granular layer is similar to that of S. nigra as judged from the typical infrared absorption bands near wave numbers 3.450 cm-1 and 1.650 cm-1, respectively. According to electron microscopic findings, this type of melanin arises within the astrocytes of the dentate nucleus. The astrocytic melanosomes exhibit a relatively monotonous substructure reaching diameters of 30 mu and more, and they differ in this respect from the triadic neuronal melanosomes of S. nigra that are strongly reminiscent of lipofuscin granules. These differences may be due to additional components of melanosomes, apparently specific of a particular cell type, and they are in turn accounted for by the dissimilar cellular metabolic make-up of nigral cells and astrocytes, respectively. This does not dismiss the possibility, however, that the melanin pigments of either type of melanosomes are the same or closely related to each other as far as their basic chemical composition is concerned. The cause of the melanosis of the dentate nucleus is not known. Possibly involutional tissue changes governed by endocrine factors among others may play an important part.

Morphological feedback effect on the nucleoli of the neurons in the nucleus arcuatus (infundibularis) to hypophyseal hypogonadism in juvenile haemochromatosis.

Case report of a man of 22 with juvenile haemochromatosis and hypogonadotropic hypogonadism. There is evidence of siderosis of the basophil cells of the hypophysis, atrophy of the testicles with disappearance of leydig cells and alterations in the nucleoli of the neurones with enlargement, vacuolation and multiplication limited to the nucleus arcuatus tuberis. The nucleolar changes in this hypothalamic centre are interpreted as a feedback effect. They are comparable to matching alterations in the hypertrophy of the nucleus subventricularis (Sheehan and Kovács 1966) in post-partum hypopituitarism.

[Neurosecretoric nerve-cell choristoma in the anterior pituitary (author's transl)]. / Neurosekretorisches Ganglienzell-Choristom in der Adenohypophyse

Report on a blastomatous dysgenesia (choristoma) with dystopic accumulation of neurosecretoric nerve-cells in the anterior lobe of hypophysis of a 65 years old woman without clinical symptoms.

[The so-called filaments in lafora-bodies, corpora amylacea and Bielschowsky-bodies. Fasciolar substructures in accumulated intraplasmatic material of cellular dysmetabolies (author's transl)]. / Die sogenannten Filamente in Lafora-Körperchen, Corpora amylacea und Bielschowsky-Körperchen. Fascioläre Substrukturen in intraplasmatischen Speicherungsprodukten bei cellulären Dysmetabolien

Both the corpora amylacea and Lafora bodies have been described in the literature as filamentous structures. On electron microscopic examination they are often composed of ribbon-like fasciolar units. The ultrastructural appearance of these structures is discussed on the example of an amylopectin model. These fasciolar substructures are also encountered in Bielschowsky bodies and are then considered to be a sign of intracellular accumulation of dysmetabolic products. The histological and electron microscopical similarity of corpora amylacea, Lafora bodies and Bielschowsky bodies suggest that all these structures are an unspecific, nearly identical endproduct of various intracellular metabolic disturbances. The pathognomonic significance of these structures depends on their regional distribution: the common corpora amylacea occur predominantly in the astroglia, the Lafora bodies typical in the neurons and the Bielschowsky bodies are restricted to the neurons of the exterior pallidum.

Morphological feedback effect on neurons of the nucl. arcuatus (sive infundibularis) and nucl. subventricularis hypothalami due to gonadal atrophy.

We have correlated the light-microscopic features in the unmyelinated hypothalamus with gonadal atrophy in 15 women of 30-111 years of age and in 7 men between 29 and 82 years. In the postmenstrual cases there is a distinct concordance of gonadal atrophy and the manifestation of nucleolar changes (augmentation, multiplication and vacuolization) in many nerve cells of the arcuate and subventricular nuclei. In younger, still fertile women this nucleolar finding was seen only rarely and sporadically, was limited to the arcuate nucleus and was absent in the subventricular nucleus. We interpret this nucleolar finding as a feedback effect. In man, too, this coincidence is obvious with age and in gonadal atrophy, with fewer nucleolar changes in old age than are seen in women. This difference is probably caused by a more rapid drop of the estradiol than of the testosterone level. - The hypertrophy of the subventricular nucleus (Sheehan and Kovacs) was also observed in our postmenstrual cases.

Partial transtentorial displacement of the cerebellum and the brain stem in hydrocephalus-a primary condition or a result of treatment?

Two cases of upward displacement of parts of the brain stem and the cerebellum are discussed. In both cases shunts for a hydrocephalus had been operating for a long time (three and a half years in one and seven months in the other). One infant had a meningomyelocele but no complete Arnold-Chiari malformation. The cause of the hydrocephalus in the other case was doubtful, but was thought to be transtentorial displacement of the brain stem and the cerebellum causing traction on the pons and consequent meningeal fibrosis with obstruction of the cisterna pontis. In this case osseous hypoplasia of the posterior fossa was excluded by craniometry, so the upward displacement must have been due to hypoplasia of the tentorium cerebelli and its low attachment to the occiput, bringingg about a narrowing of the infratentorial space. The authors believe the condition to be a primary malformation, and reject the hypothesis of secondary herniation following long standing supratentorial decompression. The concept of a primary malformation is supported by previous descriptions of the condition in cases with spina bifida, Arnold-Chiari malformation, and hydrocephalus without shunting, as well as by other features in the cases which have been recorded.

[Cerebral granular cell tumor in the rat after intraperitoneal application of aflatoxin b1 during pregnancy (author's transl)]. / Granularzelltumor des ZNS bei der Rate nach intraperitonealer Applikation von Aflatoxin B1 während der Tragezeit

On a rat, which has spontaneous died 12 months after 4 times i.p. application of aflatoxin during pregnancy, there has been found a frontal tumor barely of the size of a pepper-corn which came out from the leptomeninx. A comparison to the Abrikossoff tumor concerning the light- and electron-microscope as well as the nucleic-acid concentration is justified. The protein content of the granula of the tumor cells is higher than the values known in the granular-cell myoblastoma of man.

[Malignant granular-cell tumor of the cerebral white matter: morphological, cytophotometrical and neurochemical studies (author's transl)]. / Maligner Granularzelltumor des Grosshirnmarkes: Morphologische, cytophotometrische und neurochemische Befunde

This is a report of the second known case of a primary malignant granular-cell tumor of the cerebral hemispherical white matter. Two cell types may be distinguished apparently representing different developmental stages of otherwise identical tumor cells. Quantitative histochemical and biochemical studies have shown that tumor cells were containing markedly elevated levels of DNA and RNA. Only few ribosomes and polysomes could be detected, however, by electron microscopy. While cytophotometry disclosed only slightly elevated cytoplasmic proteins, there was a distinct increase in total protein concentration of tumor tissue per unit weight, as measured by conventional techniques. It is suggested that this increase is mainly attributable to structural proteins of cellular and subcellular elements of multiplicating malignant cells. A ratio of RNA to DNA in excess of 1 was found for white matter derived from the central tumor, while the ratios of control tissue were lowered to values far below one due to postmortem changes greatly reducing tissue concentrations of RNA. No detailed characterization of RNA was attempted. The nuclear DNA content of tumor cells reached values equivalent to chromosomal hexadekaploidy. This was in sharp contrast to control data from an Abrikossoff tumor of the oral cavity and from a neurohypophyseal tumorette ("choristoma"), respectively, displaying diploidy only associated with a much lesser increase of cytoplasmic RNA and proteins. Qualitative lipid studies were consistent with a marked active demyelination of the tumor centre as indicated by a severe reduction of cerebrosides and sulfatides and the presence of cholesterol esters. In addition there was a striking loss of phosphatidylethanolamine and a lesser one of sphingomyelin of white matter of both the tumor-stricken and the contralateral unaffected hemispherical regions, possibly suggesting a generally disturbed metabolism of myelin. It is not clear whether these general changes were resulting from the presence of the unilateral tumor or from precocious cerebral involution.