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Autoimmune Autonomic Ganglionopathy – Anti-gAChR — SCE Neurology MCQ

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HardPeripheral Neuropathy & NeuromuscularAutoimmune Autonomic Ganglionopathy – Anti-gAChRSCE Neurology

A 60-year-old woman with autoimmune autonomic ganglionopathy presents with severe orthostatic hypotension, anhidrosis, dry eyes and mouth, gastroparesis, urinary retention, and fixed tachycardia. She has anti-ganglionic nicotinic acetylcholine receptor (anti-gAChR) antibodies. What is the pathophysiology?

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Correct answer: D — Autoantibodies against ganglionic nicotinic acetylcholine receptors in autonomic ganglia, impairing synaptic transmission in both sympathetic and parasympathetic ganglia

Autoimmune autonomic ganglionopathy (AAG) is caused by antibodies against the ganglionic (alpha-3-type) nicotinic acetylcholine receptor, which mediates fast synaptic transmission in both sympathetic and parasympathetic autonomic ganglia. This disrupts preganglionic to postganglionic transmission across the entire autonomic nervous system, causing combined sympathetic and parasympathetic failure. Treatment includes plasma exchange, IVIg, or immunosuppression. A: There is autonomic failure, not overactivity. C: The lesion is at the ganglionic synapse, not demyelination. D: This is peripheral, not central. E: The postganglionic fibres are intact — the synapse is blocked.

Reference: EAN Autonomic Disorders Guidelines