Zentralblatt fur allgemeine Pathologie u. pathologische Anatomie最新文献

Reported is a nasal oncocytoma with locally invasive properties in a 86 year old man. The tumor was histologically found to consist of cells rich in cytoplasm, with striking eosinophilia, granularity, and predominance of glandular architecture. Electron microscopy revealed typical oncocytes with abundant mitochondria in the cytoplasm, with many of them compressing the nuclei of tumor cells. The tumor reported was distinguished from unambiguously benign oncocytomas by its mitotic activity and locally invasive character. These properties should be noted in histologic diagnosis. Locally delimited but radical tumor removal is the adequate therapy.

Peripheral neuropathies are among the most common neurological diseases. Various tissues are available for morphological investigation, depending on the purpose of diagnosis. The sural nerve is most frequently used for nerve biopsy. The nomenclature of neuropathies is described together with prerequisites and techniques for nerve biopsy. Morphologically, a distinction can be made between parenchymatous and interstitial lesions. An account is given of the most important morphological patterns, such as axonal and neuronal degeneration and regeneration, including Waller's degeneration, segmental demyelinisation and remyelinisation as well as hypertrophic alterations. Brief reference is made to conjunctival, dermal, and rectal biopsies.

Hereditary metabolic neuropathies (HMN) are marked by inherited enzyme or other metabolic defects. They comprise lysosomal, mitochondrial, and peroxisomal diseases, i.e. multiorgan, single-organelle system disorders, vitamin E deficiency, porphyrias, and Tangier disease. In addition to non-specific morphological pathology such as demyelinating or axonal lesions certain groups of HMN are marked by disease-specific inclusions only precisely elucidated with the electron microscope, e.g. lysosomal disorders, vitamin E deficiency, and Tangier disease. The lack of clinical and/or electrophysiological abnormalities in some of the HMN, the predominant involvement of the CNS in others and the occurrence of certain HMN in very young children have often delayed systematic investigations of the PNS in HMN and thus also procrastinated knowledge of the morphological and nosological HMN spectrum.

Review is made to an account of contemporary knowledge on cell organelles in an attempt to describe organellopathies known at present together with their relations with diseases and syndromes. Organellopathy is defined as a disease, with its primary effect and/or primary morphological and functional alterations being located in the organelle population of one or several cell types. Mitochondriopathies (mitochondrial disorders), lysosomopathies (lysosomal disease), peroxisomopathies (peroxisomal disorders), ciliopathies (ciliary diseases), and plasma membranopathies (brush border membrane diseases) have so far been most comprehensively characterised and are associated with distinctive clinical pictures. However, unambiguously characterised pathies are almost completely absent with regard to other organelles. With numerous ideas still being of speculative nature, the concept of organellopathies as such may be considered as an element of modern cellular pathology.

Described in this paper are histological, electron-microscopic, and immunocytochemical findings recorded from a duodenal gangliocytic paraganglioma in a 21-year old man. The sessile polypoid tumor consisted of epithelial cells, neuroid spindle cells, and gangliocytic elements. Neurosecretory granules were detected by electron-microscopy in the epithelial cells. The neuroid spindle cells exhibited the ultrastructural feature of Schwann cells. The tumor was immunocytochemically characterized by the S-100-reactivity of the spindle cells and numerous PP-reactive epithelial cells. Gangliocytic paragangliomas of the duodenum are of benign behaviour and should be removed by local excision.

Morin staining is a specific method by which to detect aluminium in the brain. In cases of Alzheimer disease, aluminium was found to occur in neurons and glial cells, dense cores of senile plaques, primitive plaques, and intracortical congophilic vessels. Findings obtained are likely to suggest concomitant presence of aluminium and amyloid. Aluminium is assumed to have high affinity for amyloid. Aluminium is thus capable of overcoming the blood-brain barrier.

Reported in this paper are clinical and morphological findings recorded from two sisters and one brother with familial juvenile nephronophthisis. Coherency in the basic course of the disease was not detectable by histological examinations, in the first place, though infancy developments had been almost identical, and clinical patterns were very similar to each other, with the characteristics including polyuria, polydipsia, hyposthenuria, anaemia, retarded growth, azotaemia, and progressing renal insufficiency. Some of the morphological findings were masked by secondary alterations and organ shrinkage. They were incoherently interpreted following preliminary investigations by different examiners. The pathogenesis of the disease has continued to be obscure. A disorder with tubular basal membranes as primary points of attack is discussed. Autosomal-recessive inheritance seems to be beyond any doubt, following genetic analysis of the family.

Contrasting to the usual measurement of nerve conduction velocity, which only determines the conduction of the fastest fibers, single fiber measurement allows the registration of the conduction velocity of different fiber classes. The present experiment was performed to study whether electroneurophysiological and/or morphometrical parameters of group II fibers have changed after shortterm diabetes mellitus. Diabetes was induced in 28-d-old Lewis 1A-rats by administration of 60 mg/KG b.w. Streptozotocin. 60 d later, with the aid of an oscilloscope VKS 22-16 (VUKO Elektronische Geräte GmbH, Mühlheim) single fiber measurements were performed. Morphometry was carried out on semithin transverse sections of sural nerve with an automatic image analysis system A6471-AMBA/R (Robotron, Dresden). The mean plasma glucose level of diabetic animals was 27.1 +/- 2.7 mmol/l. The mean afferent conduction velocity was significantly reduced in diabetic animals. Furthermore, efferent fibers could be verified in sural nerve which showed also reduced conduction velocity in diabetic rats. Morphometry revealed significant reduction of thickness and area of myelin sheaths, whereas area of axons remained unchanged. Electroneurophysiological and morphometrical changes will be discussed with special emphasis to different fiber groups. It is suggested, that primary Schwann cell lesion is responsible for the observed findings.

A review is given of the skeletal muscle and specific methods required for its examination, before major findings from the neurogenic tissue syndrome are discussed in some detail. These findings, not specific of the diseases involved, may be grouped as follows along three lines: alterations resulting from (repetitive) denervation; re-innervation; secondary myopathic alterations (also called concomitant myopathy). Evaluation and rating of all findings and data relative to percentual incidence are followed by postulation of a guideline for diagnosis.