Synonyms:
a.k.a. Pandysautonomia a.k.a. idiopathic autonomic neuropathy a.k.a. acute panautonomic neuropathy a.k.a. autoimmune autonomic neuropathy, rare:
Diagnosis:
Clinical features +Anti-ganglionic nAChR antibodies +supported by electrodiagnostic tests
Clinical features:
Failure of sympathetic (orthostatic hypotension, anhidrosis) and parasympathetic (dry eyes, dry mouth, gastroparesis, constipation, urinary retention) nervous system with relative sparing of somatic nerves
Preceded by viral illness
Findings on investigations:
Anti-ganglionic nAChR antibodies
CSF: mildly raised protein sometimes
CT: thymoma, small cell lung cancer
NCS/EMG: normal or near normal somatic nerves
Intact Cardiac innervation by:
- Cardiac SPECT 123I –labelled MIBG, reduced uptake
- PET scan
Pathology:
Epineurium: small mononuclear cell infiltrate and perivascular