�ber histologische Methoden in der Differentialdiagnose von Leukodystrophien und Lipoidosen

Hirsch, T.; Peiffer, J.

doi:10.1007/bf00392351

Cited by 97 publications

(10 citation statements)

References 25 publications

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“…* To whom all correspondences should be addressed . In the tissues of patients with MLD, sulfatide storage disease due to the defect of sulfatide sulfatase (10) or SAP-i (8), metachromatic deposits stained with acidified cresyl fast violet were observed under light microscopy (17), and specific "heringbone" and "tuffstone" inclusions were observed under electron microscopy (10). Cultured skin fibroblasts from MLD patients, grown under normal conditions, do not show such inclusions.…”

mentioning

confidence: 99%

Correlation of subcellular localization of disease-specific inclusions and sphingolipid activator protein-1(SAP-1) in sulfatide sulfatase-deficient fibroblasts.

Taniike

Inui

Shinoda

et al. 1988

Acta Histochem. Cytochem

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confidence: 99%

Correlation of subcellular localization of disease-specific inclusions and sphingolipid activator protein-1(SAP-1) in sulfatide sulfatase-deficient fibroblasts.

Taniike

Inui

Shinoda

et al. 1988

Acta Histochem. Cytochem

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“…Hinweise auf das Vorliegen von Rosenthal-Fasern in der Eisenhämatoxilinfärbung bzw. von metachromatischem Material mit der Methode nach Hirsch und Peiffer ergaben sich nicht [12]. In der Zusammenschau der Befunde wurde somit eine pigmentierte Form der OLD diagnostiziert.…”

Section: Discussionunclassified

Die pigmentierte Form der orthochromatischen Leukodystrophie

2003

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The pigmentary type of orthochromatic leukodystrophy (van Bogaert-Nyssen disease) is a hardly known neurological disorder usually with late onset that is very difficult to diagnose in vivo. Neuropathologically, the disorder features noninflammatory demyelination and the presence of pigmented macrophages and astrocytes that may contain iron. Clinically, van Bogaert-Nyssen disease can lead to death within a few years and is characterized by dementia, psychiatric abnormalities, epileptic seizures, spastic pareses, and occasionally extrapyramidal motor symptoms. This report presents a typical case and an overview of the literature. Furthermore, galactocerebroside could be documented in remaining macrophages and astrocytes by immunohistochemistry. This possibly indicates a dysfunction in sphingolipid breakdown and could relate the pigmented form of orthochromatic leukodystrophy to the genetically defined globoid cell leukodystrophy (Krabbe's disease). Thus, the rather heterogeneous pool of orthochromatic leukodystrophies could be further narrowed.

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“…Within the limits of sensitivity of the staining method employed (Hirsch and Peiffer, 1955), which are unknown, it implies that the myelin sheath itself does not contain a demonstrable excess of sulphatides, not even in the still intact areas of segments which are already breaking down. The various red droplets and particles observed in sheaths are probably the ',u' granules of Reich (1903), otherwise known as Elzholz granules, a normal finding in myelinated fibres stained with aniline dyes (see illustrations of Doinikow, 1911;Ukai, 1923).…”

Section: Discussionmentioning

confidence: 99%

“…Paraffin sections were stained by conventional methods. Frozen sections, 20 ,z thick, were stained to show metachromasia by Feyrter's enclosure method, and with cresyl violet 1 %, adjusted to pH 3-6 with acetic acid, by the technique of Hirsch and Peiffer (1955).'This paper is a condensation of part of an M.D. thesis submitted to the University of London.…”

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Peripheral neuropathy of metachromatic leucodystrophy: observations on segmental demyelination and remyelination and the intracellular distribution of sulphatide.

Dayan¹

1967

Journal of Neurology, Neurosurgery & Psychiatry

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Peripheral nerve abnormalities in metachromatic leucodystrophy have been noted many times since Jacobi (1947) first reported metachromatic material in the sciatic nerve of her patient. The finding of metachromatic granules in a peripheral nerve biopsy, usually the sural nerve, has been recommended as a method of diagnosing this disease (Thieffry and Lyon, 1959;Hagberg, Sourander, and Thoren, 1962). The occurence of granules, mainly in Schwann cells, and also in macrophages, has been thought to be due to the same metabolic anomaly affecting the cells responsible for myelin formation: oligodendroglia in the central nervous system, and Schwann cells in the peripheral nervous system. Phase-contrast and electron microscope studies (Webster, 1962) and electrophysiological observations (Fullerton, 1964) NERVE TEASING This was done by a method modified from Thomas's (1955) account. Formalin-fixed nerves were washed in water and placed in 1 % aqueous osmic acid for 24 hours. After further washing, they were placed in 60 % glycerol in water until required. Under a dissecting microscope, at a magnification of x 25 or x 50, single nerve fibres were teased out freehand from fascicles, using fine sewing needles for dissection. Individual fibres were placed on clean slides in batches of about 20, straightened, dried in air at 37'C., and mounted in glycerine jelly. Some specimens of nerves after fixation were stained in bulk by the Hirsch-Peiffer method, by immersion in acidified cresyl violet for 24 hours, and then macerated in 60 % glycerine before being teased.

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�ber histologische Methoden in der Differentialdiagnose von Leukodystrophien und Lipoidosen

Cited by 97 publications

References 25 publications

Correlation of subcellular localization of disease-specific inclusions and sphingolipid activator protein-1(SAP-1) in sulfatide sulfatase-deficient fibroblasts.

Correlation of subcellular localization of disease-specific inclusions and sphingolipid activator protein-1(SAP-1) in sulfatide sulfatase-deficient fibroblasts.

Die pigmentierte Form der orthochromatischen Leukodystrophie

Peripheral neuropathy of metachromatic leucodystrophy: observations on segmental demyelination and remyelination and the intracellular distribution of sulphatide.

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