[Adrenomyeloneuropathy in adults. Relationship with adrenoleukodystrophy in children (author's transl)]

The case reported concerns a man in whom appeared gradually, since the age of twenty-seven years, difficulty in walking and sexual impotence. The neurological syndrome consisted of pyramidal signs and objective sensory disorders of the lower limbs, intermittent sphincter disorders and mild mental deterioration, which was progressive. Biopsy of a sensory nerve showed rarefaction of myelinated fibres. The endocrine syndrome consisted of peripheral adrenal insufficiency, which was undoubted but with biologic evidence only. There was no response of plasma cortisol during five stimulation tests with tetracosactide and there was a gradual rise in plasma A.C.T.H. The clinical picture was thus that of an adrenomyeloneuropathy.