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A 59-year old female was initially diagnosed with hemorrhoids. However, the pathology report from the hemorrhoidectomy revealed an over 20 mm high-grade melanoma with BRAF wild-type mutation and lymphovascular invasion. Staging scans revealed pulmonary nodules. The patient began treatment with dual immune checkpoint inhibitors.

Approximately two months later, the patient presented with a multitude of new symptoms, including constipation, fatigue, hypotension, nausea and weight loss. We admitted the patient for further workup.

Her blood pressure failed to respond to intravenous fluid of 4 liters saline. There were no signs of infection, tachycardia or leukocytosis. The patient was afebrile. A cardiac workup revealed normal ejection fraction and no cardiac abnormalities. The patient has normal TSH, LH, FSH, prolactin, and GH. She was also negative for ANA, Lyme, syphilis, and HIV. Her B12 level was normal. A brain MRI was negative for metastatic lesions.

Formal autonomic testing revealed low supine resting heart rate (52 bpm) and BP (92/52 mmHg). Heart rate ranged from 45-to-53. The patient’s systolic BP dropped from 93 to 61 within 30 seconds upon orthostatic testing.

Are these symptoms suggestive of acute autonomic ganglionopathy?

What additional testing would confirm the diagnosis?

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