Title: Can’t see, can’t pee, can’t stand like me.
Authors: Krokos I, Cohen J, Pierce J, Department of Internal Medicine, University of New Mexico, Albuquerque, NM.
Case Presentation.A 47-year-old Taiwanese male without significant past medical history presented with complaints of dizziness, decreased urination and diarrhea. On exam he was noted to be markedly orthostatic. The abdomen was slightly distended. After voiding, foley catheterization returned approximately one liter of residual urine. Laboratory evaluation was remarkable for hyponatremia. The patient was admitted for intravenous hydration with a presumptive diagnosis of dehydration secondary to gastroenteritis.
Despite aggressive fluid resuscitation his orthostasis did not resolve. He could not tolerate sitting up for more than a few minutes as his systolic blood pressure would acutely drop to 50-60mm Hg. He also exhibited dry skin, dry mouth, dry eyes, blurry vision and poor heart rate variability. An extensive evaluation for causes of autonomic insufficiency did not reveal adrenal insufficiency, paraneoplastic syndrome, myasthenia gravis, Parkinson’s or cancer. A ganglionic acetylcholine receptor antibody test for autoimmune autonomic ganglionopathy was negative. Given the patient’s extensive negative evaluation and his constellation of symptoms consistent with autonomic failure, a diagnosis of autoimmune autonomic ganglionopathy was made. No benefit was noted after treatment with fludrocortisone and midodrine hydrochloride. Plasmapheresis and intravenous immunoglobin (IVIG) was initiated and the patient had significant symptomatic improvement.
Discussion. Autoimmune autonomic ganglionopathy (AAG) is a type of autonomic insufficiency that includes both parasympathetic and sympathetic dysfunction and is thought to have an immunologic basis. It may occur after a viral illness or with cancer, most commonly non-small cell lung cancer. AAG usually develops subacutely over days to weeks. Characteristic clinical features include gastrointestinal dysmotility and an abnormal pupillary response to light and accommodation. Serologic testing may be positive for an autonomic ganglionic acetylcholine receptor antibody, but a negative result does not exclude the diagnosis. Treatment is supportive and fludrocortisone and midodrine hydrochloride help with orthostatic hypotension. There is no definitive course-modifying treatment. Recovery after plasma exchange and IVIG therapy has been reported. Spontaneous recovery may also occur.
Conclusions.Review of this case may help practitioners recognize this rare form of autonomic insufficiency.