Autoimmune Autonomic Ganglionopathy Presenting as Constipation

Area of Science:

• Neurology
• Gastroenterology
• Immunology

Background:

• Autoimmune autonomic ganglionopathy (AAG) is a rare disorder affecting the autonomic nervous system.
• It can manifest with various symptoms, including significant gastrointestinal dysfunction.
• Patients may or may not have antibodies against the nicotinic acetylcholine receptor (nAChR).

Observation:

• A 56-year-old woman with prior sensorimotor neuropathy presented with severe, refractory constipation.
• Diagnostic evaluations revealed diffuse colonic hypomotility, rectal hypersensitivity, and pelvic floor dysfunction.
• The patient was seronegative for nAChR antibodies.

Findings:

• The patient was diagnosed with seronegative autoimmune autonomic ganglionopathy (AAG) 10 months after symptom onset.
• She demonstrated a positive response to treatment with intravenous methylprednisolone and apheresis.

Implications:

• This case underscores the importance of considering seronegative AAG in patients with unexplained gastrointestinal motility disorders.
• Early diagnosis and treatment, including immunotherapy, can lead to significant clinical improvement.
• Further research into seronegative AAG is warranted to understand its pathophysiology and optimize management strategies.