Abstract
A 64 year old woman died at the third attack of MFS. Histological examination demonstrated segmental demyelination and axonal swelling of the peripheral nerves studied, oculomotor included. In the C.N.S. only mild chromatolytic changes and rare pyknosis of the nerve cells in the midbrain were found without signs of primary inflammation. We reviewed the findings in all the 4 anatomoclinical cases of MFS and in 2 cases of GBS with ophthalmoplegia or ataxia. With one exception, they appear to be concordant with those of our case. As the histological examination showed CNS involvement consequent upon peripheral nerve impairment, we are bound to change our opinion on the nosological position of MFS. Any small CT enhancements in the brain in MFS may be due, as in some cases of demyelinating polyneuropathy, to focal rupture of the blood-brain barrier.
Sommario
Una donna di 64 anni è deceduta al terzo attacco di M.F.S. L'esame istologico ha fatto osservare una demielinizzazione segmentale e rigonfiamento assonale dei nervi periferici, inclusi gli oculomotori. Nel S.N.C. si sono osservate solo modeste alterazioni cromatolitiche e rare picnosi dei neuroni dei nuclei mesencefalici, senza segni di flogosi primaria. Abbiamo rivisto i reperti di tutti e 4 i casi anatomo-clinici di M.F.S. e di 2 casi di G.B.S. con oftalmoplegia o atassia. Tali reperti, con una unica eccezione, appaiono concordanti con quelli del nostro caso. Pertanto noi cambiamo l'opinione precedentemente espressa sulla posizione nosologica della M.F.S., dal momento che il controllo istologico ha mostrato che il SNC è coinvolto come conseguenza di una sofferenza dei nervi periferici. Eventuali piccole aree di ipercontrasto alla T.C. sono dovute — come è stato osservato anche in casi di polineuropatia demielinizzante — a rottura focale della BEE.
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Barontini, F., Di Lollo, S., Maurri, S. et al. Localization of the pathological process in Miller Fisher syndrome. Ital J Neuro Sci 13, 221–225 (1992). https://doi.org/10.1007/BF02224393
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DOI: https://doi.org/10.1007/BF02224393