pure autonomic failure (PAF)

Introduction

slowly progressive degeneration of the autonomic nervous system.

Epidemiology

sporadic, adult onset

Pathology

• Lewy bodies/inclusions
  • brainstem
  • pre- and post-ganglionic autonomic neurons
  • peripheral sympathetic nerves & parasympathetic nerves

Clinical manifestations

• isolated autonomic impairment
  • orthostatic hypotension

More general terms

• synucleinopathy (includes alpha-synucleinopathy)