CLINICAL,PHYSIOLOGICAL AND PATHOLOGICAL OBSERVATIONS IN A CASE OF PROGRESSIVE AUTONOMIC NERVOUS SYSTEM DEGENERATION ASSOCIATED WITH HOLMES-ADIE SYNDROME AND PERIPHERAL NEUROPATHY

The case history is reported of a woman aged 40 years who presented with weight loss, postural hypotension and paræsthesia in the hands and feet. The diagnosis of widespread autonomic nervous system degeneration with associated peripheral neuropathy and Holmes-Adie syndrome was made from the results of a series of clinical and physiological tests. Laboratory investigation failed to disclose a cause for the degenerative changes, and symptomatic treatment with 9-alpha fluorohydrocortisone and digitalis was commenced in an effort to combat the patient's postural hypotension. On this régime there was transient improvement in her clinical state, but over a period of six months her general condition gradually deteriorated and she eventually died. At autopsy, widespread amyloid infiltration of the sympathetic ganglia, peripheral nerves, heart and alimentary canal was found, and this appears to have been the pathological basis for the clinical and physiological findings.