On the nature of the peripheral nerve lesions associated with acute intermittent porphyria

Abstract

A survey of the literature on the anatomical basis for paralysis associated with acute intermittent porphyria yields a confused picture that sheds little light on how this comes about. Erbsloh (1903) originally described changes in the peripheral nerves of a patient paralysed after sulphonal medication which were undoubtedly Wallerian in type. Mason, Courville, and Ziskind (1933), in their compre- hensive review of the subject, stated that axons and myelin sheaths are destroyed in a patchy manner, implying thereby, first that they could see no meaningful pattern in the types of nerve fibres involved, and secondly, that the myelin degeneration was secondary in type. Hierons (1957) echoed this view and in addition stressed that all authors were in agreement that the nerve cells in the spinal cord and root ganglia, although they might be either normal or chromatolytic, were never destroyed.