Cerebral Whipple's disease with negative jejunal histology.
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Adult↗
Biomedical subjects
B Volk
Publications and source records attributed to B Volk.
[Melanin and melanosomes occurring in the central nervous system in comparison to their extracerebral manifestations and to synthetic melanin derived from dopamine and serotonin (author's transl)].
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Animals↗
Alcohol embryo- and fetopathy. Neuropathology of 3 children and 3 fetuses.
Maternal chronic ethanol abuse during pregnancy causes malformations of the offspring. Three children (aged 6 months, 9 months, 4 1/2 years) and 3 fetuses (17th, 18th, and 20th gestational week) showed a wide spectrum of disorders ranging from severe dysraphic state, arhinencephaly, porencephaly, agenesis of corpus callosum, a range from hydranencephaly to microdysplasias (p.e. reduced gyration of dentate nucleus and inferior olives), and a range from gastrochisis or congenital heart defects to craniofacial dysmorphogenesis and palmar crease anomalies. The patterns of the cerebral malformations were not as uniform as the clinical phenotype of the alcohol embryopathy. The observations did not support the assumption that there exists a specific period for alcohol teratogenicity.
Abnormalities, Drug-Induced↗
Peripheral intraaxonal storage in Tay-Sachs' disease (GM2-gangliosidosis type 1.
Considerable intraaxonal lipid storage was observed in intramuscular nerve fibres mainly of the extraocular muscles and less frequently in limb muscles of a 3-year-old child, which had suffered from Tay-Sachs' disease. Many axons of the small intramuscular nerve twigs and of the terminal and preterminal nerve endings showed spherical and cylindrical enlargements containing granular storage material of the same staining properties as the material in the central nervous system. The identify of the axonal and the neuronal storage material was further confirmed by electron-microscopical demonstration of typical multilamellated cytoplasmic bodies (MCBs) in both of them. In addition, silver impregnation and electron microscopy revealed dystrophic changes with an increase of argyrophilic filamentous material within some of the axonal distensions, indicating that the latter were probably due to both a lipid storage and an unspecific dystrophic process.
Atrophy↗
Amyloid neuropathy due to monoclonal gammopathy. A case report.
A case with peripheral neuropathy due to monoclonal gammopathy is reported. There was striking, dissociated symmetrical sensory loss and paresis, the legs being affected predominantly. The case is compared with the few reported cases of myelomatous neuropathy, and the clinical and histological findings are discussed in view of the pathogenesis.
Amyloidosis↗
[Delayed cerebellar histogenesis in "embryofetal alcohol syndrome". Light microscopic study of the rat cerebellum (author's transl)].
The cerebellar postnatal development of young rats (Wistar) was investigated, whose mothers were given a 12% (W/V) ethyl alcohol solution for 7 days before and during pregnancy. Besides weighing less at birth, the experimental animals had a slower postnatal linear growth and gained weight less rapidly. Histological examination of the cerebellum showed an inpeded histogenesis, which could be observed in the Purkinje cells up to the 10th postnatal day. Moreover, a thick layer of embryonal granular cells with numerous mitoses could still be seen in the experimental animals at 3 weeks of age. After 30 days there was no histological difference between the controls and experimental animals. Because the Purkinje cells in the developing cerebellum represent the units were ribosomes are most abundant, it seems appropriate to ask, whether the observed inhibition of histogenesis could derived from an impaired cerebral protein synthesis. Disturbance of organogenesis was observed in three experimental animals afflicted with an extreme internal hydrocephalus.
Aging↗
[The so-called filaments in lafora-bodies, corpora amylacea and Bielschowsky-bodies. Fasciolar substructures in accumulated intraplasmatic material of cellular dysmetabolies (author's transl)].
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.
Amylopectin↗
[Cerebral granular cell tumor in the rat after intraperitoneal application of aflatoxin b1 during pregnancy (author's transl)].
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.
Aflatoxins↗
The relationship between target, targetoid, and targetoid/core fibers in severe neurogenic muscular atrophy.
In the m. tibialis anterior of a 68-year-old man with rapidly developing denervation atrophy in the legs since 1/2 year prior to death from heart stroke, abundant unifocal concentric fiber changes, such as target, targetoid/core, and targetoid fibers could be observed. Besides, large vacuolized fibers with multiple changes resembling cytoplasmic bodies in the peripheral zone were present as well; they are interpreted as fibers with multicentric target or targetoid formations. The target fibers displayed a broad variation of their outer appearance suggesting a continuous transition to targetoid/core fibers (with a dense center) and targetoid fibers (with a central change to aquous sarcoplasm showing a paucity of fibrillar structures). Very few fibers with a central densification of fibrillar material with or without a thin intermediate zone were fairly akin to core fibers of central core disease; others were more alike the type of targetoid fibers, previously described in the literature, showing a dense target-like center; both were summarized under the term, inaugurated by Engel et al. (1966), "targetoid/core fibers". Simultaneous occurrence of the different kinds of concentric fiber changes suggested a strong relation between all of them in the sense of representing different developmental stages of the same pathogenetic process. Thus, the central core disease, for instance, might be a disorder with a generalization of concentric fiber changes having come to arrest in the earliest stage of development.
Aged↗
[Torpid degeneration of the lateral pallidum with Bielschowsky bodies. Light and electronmicroscopic findings (author's transl)].
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.
Athetosis↗
[Arginase and carbamyl phosphate synthase activities in chronic liver diseases].
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Arginase↗
[Necroses in the granular cell layer of the cerebellum due to methylchloride intoxication in guinea pigs (author's transl)].
QUESTION: Neurotoxic action of methylchloride was reported by various authors. As this solvent is widely used in chemical industry the morphological changes in the CNS of guinea pigs due to methylchloride intoxication were comparatively studied by light- and electron-microscopy. MATERIAL AND METHODS: 19 guinea pigs of both sexes (body weight 300-600 g) were individually exposed to inhalation of methylchloride mixed to normal breathed air in a 2 Vol.-% amount in a pressurised vessel of 20,000 cm3 volume (details on the experimental arrangement see VOLK 1973) for each 10 min 6 times a week. 3 control animals were fed like the experimental animals and exposed to normal breathing air for the same time in the pressurised vessel. The experimental period varied between 7 and 70 days, the number of inhalations was between 6 and 61. At the end of the experiment the animals were sacrificed by perfusion with 3% buffered glutaraldehyde under Thiogenal anesthesia. For light microscopy paraffin sections were prepared from tissue samples of the cerebrum, cerebellum, brain stem, mesencephalon, spinal medulla and spinal ganglia (stainings: HE; Turnbull's blue; Nissl; Klüver-Barrera; Mallory; Bodian and Kossa). For electron microscopy tissue blocks of the cortex, corpus callosum, striatum, mesencephalon and cerebellum as well as of spinal medulla and spinal ganglia were postfixed in 1% OsO4 and embedded in Araldit in the common way. RESULTS: Clinical observations--9 animals showed normal behaviour after 32 inhalations in 37 days.--6 animals displayed staggering, atactic moving of the head, retardation in spontaneous reactions and mobility. Ataxia and paresis of the hind legs occurred earliest after 17 inhalations. 4 animals showed all these symptoms only after 25 days. Morphological findings: Macroscopy: Occurrence of necroses in the cerebellar cortex was mainly observed in the lower part of the vermis; pressings of tissue in the occipital foramen, tissue swellings around the aqueduct and consecutive enlargement of the lateral ventricles occurred. Light microscopy: Changes in the Stratum granulosum in the lower part of the vermis as pyknosis of the cell nuclei and hydropic swelling of the cytoplasm (fig. 1a) were seen after 10 days. Focal necroses of the granular layer as well as "Status spongiosus" and edema occurred after 21 days (fig. 1b). In later phases these alterations were also present in other regions of the vermis and of the cerebellum, too. Furthermore, edema of the Lamina dissecans, hydropic swelling of the Bergmann's glia and marked edematous transformation of the molecular layer were observed. In this phase the hitherto intact Purkinje cells responded to the intoxication by homogenization of the cytoplasm and cell necroses. Electron microscopy: Initially an increase in density of the nuclear chromatin of the granular cells was observed associated with formation of homogeneous electron dense chromatin fragments which were finally released to the perikaryon (figs. 2a, 2b)...
Animals↗