Autonomic dysfunction associated with multiple pelvic ganglioneuromas

We describe the case of a 59-year-old male in which several symptoms of autonomic dysfunction resulted associated with the presence of multiple ganglioneuromas in the adrenal glands and in paravertebral spaces. In a 2-year period, the patient developed sexual dysfunctions, micturitional disturbances and severe orthostatic hypotension and was erroneously diagnosed as having primary autonomic failure. However, the examination of all the autonomic functions showed that papillary function and cardiovascular reflexes were normal. CT scan of the abdomen revealed the presence of several small masses in the adrenal glands and along the lumbar sympathetic chain. Cytologic examination of the adrenal glands showed clusters of ganglionlike cells interspersed with Schwann cells, leading to the diagnosis of ganglioneuromas. Patients with signs and symptoms of autonomic dysfunction need an extensive clinical and laboratory examination of all the autonomic functions in order to exclude secondary causes of the symptomatology.