Autoimmune Autonomic Ganglionopathy AAG

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

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