Abstract

With reference to a patient a description is given of the syndrome of multiple mucosal neuromas — a syndrome which seems to be caused by a disturbance in the development of neural crest derivatives. The syndrome was associated with a number of autonomie dysfunctions; this dysautonomia is correlated with the morphological findings in a sural nerve biopsy. The nerve was hypertrophic and showed few changes in the myelinated fibres; the unmyelinated fibres showed a chronic process of degeneration and regeneration, and small unmyelinated axons were found increased in number. It is suggested that the abnormal reaction to an intradermal histamine injection was due to the dysautonomia shown by this patient.

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