Hypertrophic perineurial dysplasia in multifocal and generalized peripheral neuropathies

Two cases are described, one with a multifocal cranial and limb neuropathy of adult onset associated with optic neuropathy, and the other with a diffuse demyelinating neuropathy characterized by congenital cataract, mental retardation and progressive lower limb paresis with an onset in childhood. Extensive investigation in both failed to establish the causation. No family history of similar disorder was obtained in either case. Nerve biopsy in both showed similar perineurial abnormalities, the endoneurium being compartmentalized by hypertrophic perineurial cells that exhibited dysplastic features. The appearances resemble those described in a previously reported case of multifocal neuropathy and probably represent an unusual but non-specific response to a peripheral neuropathy.     

Biochemical and therapeutical studies in a case of atrophia gyrata

A thirty-six years old man from an inbred family with the typical clinical picture of Atrophia gyrata chorioideae et retinae was found to have hyperornithinemia and a partial deficiency of ornithin-ketoacid-transaminase activity. The residual activity was stimulated in vitro by high concentrations of pyridoxal phosphate. We have initiated a therapeutic study with vitamine B₆ per os accordingly.Comparitively low dosis may be sufficient for long term treatment. The necessity to start therapy early in life is emphazised. Possible mechanisms of the pathogenesis of Atrophia gyrata are discussed.

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Zusammenfassung Bei einem 36jährigen Patienten aus einer Familie mit mehrfachen Verwandtenehen wurde eine Atrophia gyrata chorioideae et retinae sowie eine Hyperornithinämie und ein Mangel an Ornithinketotransaminase festgestellt. Die Rest-aktivität dieses Enzyms wurde in vitro durch hohe Dosen von Pyridoxalphosphat stimuliert. Eine Therapie mit Vitamin B₆ per os wurde daraufhin eingeleitet.Geringe Dosen scheinen für eine Langzeitbehandlung ausreichend. Auf die Wichtigkeit einer frühzeitigen Therapie wird hingewiesen. Überlegungen zur Pathogenese der Atrophia gyrata werden dargelegt.

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Part of the results was obtained during medical thesis work of U.K.     

Wertigkeit der Feinnadelpunktionszytologie bei Neoplasien der Glandula parotis

BACKGROUND: In many parts of Germany fine-needle aspiration biopsy (FNAB) is still not part of the routine preoperative diagnostic evaluation of salivary gland neoplasms. Most opponents consider the study unnecessary and recommend that all salivary gland neoplasms should be excised. OBJECTIVE: Because of this an evaluated the ability of FNAB to provide an accurate diagnosis of parotid gland neoplasms. PATIENTS AND METHOD: Between January 1992 and October 1995, 336 patients referred for operative therapy of salivary gland neoplasms underwent retrospective analysis of preoperative FNAB compared with the excised tumor histology. RESULTS: Results showed that the FNAB had a sensitivity of 93.1%, a specificity of 99.2%, a positive predictive value of 93.1%, a negative predictive value of 99.2% and an accuracy of 98.6%. Complications were observed in less than 1%. DISCUSSION: Our findings demonstrated that the FNAB is a safe diagnostic tool that has a reliable sensitivity and high specificity for the assessment of salivary gland pathology. Since many malignant salivary gland neoplasms present with a virtual lack of symptoms indicating actual malignancy we believe that there is need for FNAB in routine preoperative diagnostic testing.     

Die Aktivität der UDP-Glucuronsäurepyrophosphatase in der Leber während der Entwicklung

Zusammenfassung Die UDP-Glucuronsäurepyrophosphatase-Aktivität wurde in Lebermikrosomensuspensionen von Meerschweinchen und Ratten bestimmt. In der Leber neugeborener Meerschweinchen beträgt die Aktivität des Enzyms 10%, in der Leber neugeborener Ratten 20% der Aktivität erwachsener Tiere. Bei der Aufbewahrung der Mikrosomen bei -16°C fällt die Enzymaktivität erst vom 7. Aufbewahrungstag an allmählich ab. Die Ergebnisse zeigen, daß die ATP-Aktivierbarkeit der Glucuronyltransferase bei neugeborenen Meerschweinchen nicht auf einer Hemmung der hohen UDP-Glucuronsäure-Hydrolyse in der UDP-Glucuronsäurepyrophosphatase-Reaktion beruhen kann. Auch die Abnahme der ATP-Aktivierbarkeit der Glucuronyltransferase bei der Aufbewahrung hängt vermutlich nicht mit der Höhe der UDP-Glucuronsäurepyrophosphatase-Aktivität zusammen.

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Liver UDP-glucuronic acid pyrophosphatase activity during development

The activity of UDP-glucuronic acid pyrophosphatase has been determined in suspensions of liver microsomes from guinea pigs and rats. In the liver of newborn guinea pigs the enzyme activity exhibits 10 per cent, in the liver of newborn rats 20 per cent of the activity measured in adult animals. During storage of the microsomes at -16°C the enzyme activity remains constant during the first 7 days of storage. The data suggest that in newborn guinea pigs the glucuronyltransferase activation by ATP is not due to the inhibition of a high UDP-glucuronic acid hydrolysis, mediated by the enzyme UDP-glucuronic acid pyrophosphatase. Moreover, there is no correlation between the decline of the glucuronyltransferase activation by ATP during storage and the UDP-glucuronic acid pyrophosphatase activity.

Mit Unterstützung durch die Deutsche Forschungsgemeinschaft, Bad Godesberg.     

CD133 identifies perivascular niches in grade II–IV astrocytomas

The aim of the present study was to investigate the localization and distribution of the putative brain tumour stem cell marker CD133 in formalin fixed paraffin embedded astrocytomas. A retrospective analysis of 114 grade II, III and IV astrocytomas was undertaken. The immunohistochemical expression of CD133 in paraffin sections was analysed using morphometry. In all grades, CD133 was expressed on tumour and endothelial cells. Tumour cells were found in perivascular niches, as dispersed single cells and in pseudopalisade formations around necrosis. There was no correlation between the mean volume fraction of CD133⁺ niches and all CD133⁺ tumour cells and tumour grade. However, the volume fraction of CD133⁺ blood vessels increased significantly from 0.4% in diffuse astrocytomas to 2.2% in glioblastomas. Neither of them was related to patient survival. Double immunofluorescence stainings showed that the CD133⁺ niches both contained CD133⁺ cells with and without co-expression of the intermediate filament protein marker nestin, and only few CD133⁺/MIB-1⁺ proliferating cells were found. In conclusion, a CD133⁺ perivascular stem cell-like entity exists in astrocytomas. CD133⁺ tumour vessels may play an important role in a brain tumour stem cell context, while CD133 alone does not appear to be a specific tumour stem cell marker related to patient survival.     

Furan oligomers and beta-carbolines from terrestrial streptomycetes

2,5-Bis(hydroxymethyl)furan monoacetate (3) and 2,5-bis(hydroxymethyl)furan diacetate (4) were obtained as new natural products from an ethyl acetate extract of the terrestrial Streptomyces sp. isolate GW11/1695. Another Streptomyces isolate, GW21/1313, delivered a dimer (6) and a trimer (7) of (hydroxymethyl)furfural. The latter strain also produced 4-hydroxy-2-(5-(hydroxymethyl)furan-2-ylmethylene)-5-methylfuran-3-one (5), perlolyrin (8), and two new beta-carboline derivatives, 9 and 10. 2,5-Bis(hydroxymethyl)furan diacetate (4) exhibited weak cytotoxic activity against brine shrimp larvae.