Synonyms:
a.k.a. Bradbury-Eggleston syndrome a.k.a. idiopathic orthostatic hypotension
Diagnosis:
A type of alpha-synucleinopathy
Clinical features:
Middle-late life,
Gradual onset and progression of orthostatic hypotension +other features of autonomic failure e.g. Nocturia and erectile dysfunction
Coat hanger pain occurs: shoulder and neck ache on standing
Without other neurologic signs
Findings on investigations:
Noradrenaline:
- After supine for 30 minutes and then after standing for 5 minutes: 50-100% increase is normal. In PAF, levels are low supine and barely increase on standing.
Post ganglionic
Cardiac denervation by:
- Cardiac SPECT 123I –labelled MIBG: impaired uptake
- PET scan
Pathology:
Alpha-synuclein in pre- and post-ganglionic neurons in the sympathetic and parasympathetic nervous system. Lewy bodies and Lewy neurites (nerve fibres with eosinophilic material).
Epicardial space nerve fibres: Tyrosine hydroxylase staining is decreased (cardiac denervation, sympathetic)