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Porphyric neuropathy: a clinical, neurophysiological and morphological study

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Abstract

A case of neuropathy in the course of an attack of acute intermittent porphyria was studied from the neurophysiological and morphological points of view. The neurophysiological findings (acute neuropathy with almost complete denervation despite normal or slightly reduced conduction velocity) and the morphological findings (no segmental demyelination after teasing, conservation of the linear fiber diameter/internodal distance ratio, mainly axonal damage on ultrastructural study) seem to indicate that the disease process is chiefly an axonal neuropathy.

Somario

Gli autori descrivono un caso di neuropatia nel corso di un attacco di porfiria acuta intermittente. Il caso è stato studiato dal punto di vista neurofisiologico e morfologico.

I dati neurofisiologici (neuropatia acuta con denervazione pressochè completa, malgrado una velocità di condizione normale o poco ridotta) e morfologici (assenza di demielinizzazione segmentale all'esame tramite teasing, conservazione del rapporto lineare fra il diametro delle fibre e la distanza internodale, prevalente sofferenza assonale allo studio ultrastrutturale) sembrano indicare che il processo patologico è soprattutto una neuropatia assonale.

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Authors and Affiliations

  1. Instituto Neurologico “C.Besta”, Milano

    Sghirlanzoni A., Bottacchi E. & Peluchetti D.

  2. Ospedale Bolognini USSL 30, Via C. Battisti, 8, 24068, Seriate (BG)

    Defanti C. A.

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  1. Defanti C. A.
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  2. Sghirlanzoni A.
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  3. Bottacchi E.
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  4. Peluchetti D.
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Defanti, C.A., Sghirlanzoni, A., Bottacchi, E. et al. Porphyric neuropathy: a clinical, neurophysiological and morphological study. Ital J Neuro Sci 6, 521–526 (1985). https://doi.org/10.1007/BF02331049

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