Summary
The light and electron microscopical features of two sural nerve biopsies obtained from a nine year old boy with recurrent idiopathic polyneuropathy are described. The biopsies were obtained shortly after an exacerbation of symptoms and six years after the onset of the neuropathy which began acutely following an attack of varicella. Light microscopy revealed active primary demyelination occurring in the presence of an infiltrate of mononuclear cells, lymphocytes and macrophages, with onion-bulb formation discernible in araldite sections. The following fine-structural changes were observed:
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Myelin breakdown appeared to be initiated by macrophages penetrating intact fibres and burrowing into the myelin sheath along minor dense lines. Myelin and Schwann cells not in direct contact with the invading cell appeared normal. Extracellular vesicular dissolution of myelin, as seen in the Landry-Guillain-Barré syndrome, was not observed. A variety of mononuclear cells including cells with the appearance of transformed lymphocytes were present in the endoneurial space and they were frequently observed to contact each other and macrophages.
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The Schwann cells forming onion-bulb lamellae showed little morphological evidence of active protein synthesis, and appeared to be effete cells which had originally subserved the internode and which were cast off during demyelination and the commencement of remyelination.
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The average diameter of axons in nerve fibres showing active or recent demyelination was less than that observed in fibres exhibiting advanced remyelination, suggesting that the diameter of axons increased during remyelination.
The ultrastructural findings are discussed with respect to those observed in other noninfectious inflammatory primary demyelinating diseases of peripheral and central white matter, and it is suggested that recurrent idiopathic polyneuropathy of the type described in the present study and multiple sclerosis may be related disorders.
Zusammenfassung
Licht- und elektronenmikroskopische Befunde von zwei N. suralis-Biopsien eines 9 jährigen Knaben mit rezidivierender idiopathischer Polyneuropathie werden beschrieben. Die Biopsien wurden kurz nach einer klinischen Exazerbation und 6 Jahre nach Beginn der Neuropathie durchgeführt. Die Erkrankung hatte akut im Anschluß an Varizellen begonnen. Lichtoptisch fand sich eine aktive primäre Entmarkung bei Auftreten von Infiltraten aus Mononukleären, Lymphocyten und Makrophagen sowie Zwiebelschalenbildung, die in Araldit-Schnitten erkennbar waren. Folgende ultrastrukturelle Veränderungen wurden beobachtet:
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Der Markzerfall schien durch Makrophagen hervorgerufen, die in intakte Fasern eindringen und in die Markscheiden entlang der minor dense line einbrechen. Myelin und Schwannzellen ohne direkten Kontakt mit den invadierenden Zellen erschienen normal. Extracelluläre bläschenförmige Markauflösung wie beim Landry-Guillain-Barré-Syndrom wurde nicht beobactet. Eine Art von mononukleären Zellen einschließlich solcher nach Art von transformierten Lymphocyten fanden sich im Endoneuralraum und wurden oft in Kontakt untereinander und mit Makrophagen beobachtet.
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Die Schwannzellen, die Zwiebelschalen-Lamellen bildeten, zeigten wenig morphologische Hinweise auf aktive Proteinsynthese. Sie schienen jene Zellen darzustellen, die ursprünglich die Internoden gestützt hatten und während der Entmarkung und am Beginn der Remyelinisation abgerissen waren.
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Der mittlere Durchmesser von Axonen in Nervenfasern mit aktiver oder frischer Demyelinisation war geringer als jener in Fasern mit fortgeschrittener Remyelinisierung. Das weist auf eine Querschnittszunahme der Axone während der Remyelinisation hin.
Die ultrastrukturellen Befunde werden diskutiert und mit jenen bei anderen nicht-infektiösen entzündlichen primären Entmarkungskrankheiten der peripheren und zentralen weißen Substanz verglichen. Es wird vermutet, daß die rezidivierende idiopathische Polyneuropathie des beschriebenen Typs und die multiple Sklerose verwandte Erkrankungen darstellen.
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Read at the meeting of the Australian Association of Neurologists in Canberra, May 19th, 1970.
Wellcome Australian Senior Research Fellow in Clinical Sciences.
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Prineas, J.W. Demyelination and remyelination in recurrent idiopathic polyneuropathy. Acta Neuropathol 18, 34–57 (1971). https://doi.org/10.1007/BF00684474
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DOI: https://doi.org/10.1007/BF00684474