We present the case of a 39 year old woman who had been well but presented with rapid onset and progression of nausea, vomiting and postural lightheadedness on a background of poorly controlled type 2 diabetes mellitus diagnosed in 2005. She had hyperglycaemia with ketosis but no acidosis. Antibody testing for type 1 diabetes was negative.
Nausea and vomiting were managed with anti-emetics. Therapy was limited by QT interval prolongation. Gastric emptying study showed severe gastroparesis. A normal duodenal biopsy excluded coeliac disease. Due to 20kg weight loss and poor oral intake, enteral feeding was started through a nasojejunal tube, followed by percutaneous endoscopic gastrostomy tube later converted to a percutaneous endoscopic jejunostomy tube with feeds continuing overnight with the addition of Lantus 80 units.
A persistent drop in systolic blood pressure was recorded up to 70mmHg despite IV fluids, causing impaired mobility and recurrent falls leading to a humerus fracture. Fludrocortisone was commenced and uptitrated. Midodrine (7.5mg/5mg/2.5mg) and pyridostigmine were added with improvement in postural drop (10mmHg) and symptoms. However the patient still requires a walking aid/wheelchair.
Initially gastroparesis and postural hypotension were thought to be due to autonomic neuropathy secondary to poorly controlled diabetes. However the rapid onset and progression in the context of mild stable sensory peripheral neuropathy was not consistent with this. Vasculitic screen, anti-neuronal antibodies, positron emission tomography scan were all negative. However, cerebrospinal fluid showed high protein. A provisional diagnosis of autoimmune autonomic ganglionopathy (AAG) was made in the absence of any evidence of cancer or other causes.
On neurology advice intravenous immunoglobulin was commenced with some improvement. Anti-ganglionic acetylcholine receptor antibodies are pending, but are positive in only 50% of AAG cases.(1) In diabetes with rapidly progressive and severe autonomic neuropathy with minimal peripheral neuropathy causes other than diabetes should be suspected.