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Slowly progressive autonomic neuropathy with antiganglionic acetylcholine receptor antibody
  1. Haruki Koike1,
  2. Shigeru Koyano2,
  3. Saori Morozumi1,
  4. Yuichi Kawagashira1,
  5. Masahiro Iijima1,
  6. Masahisa Katsuno1,
  7. Naoki Hattori1,
  8. Steven Vernino3,
  9. Gen Sobue1
  1. 1Department of Neurology, Nagoya University Graduate School of Medicine, Nagoya, Japan
  2. 2Department of Neurology, Yokohama City University, Yokohama, Japan
  3. 3Department of Neurology, UT Southwestern Medical Center, Dallas, Texas, USA
  1. Correspondence to Dr Haruki Koike and Professor Gen Sobue, Department of Neurology, Nagoya University Graduate School of Medicine, Nagoya 466-8550, Japan; sobueg{at}med.nagoya-u.ac.jp

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The antiganglionic acetylcholine receptor (AChR) antibody is a serological marker for autoimmune autonomic ganglionopathy (AAG).1 2 Typical AAG cases show monophasic progression evolving to peak severity within a few days to weeks.1 3 Before the establishment of AAG as a disease entity, it was called acute pandysautonomia, autoimmune autonomic neuropathy, idiopathic autonomic neuropathy or subacute autonomic neuropathy.1 2 While most cases of AAG show an acute or subacute course to reach its nadir, similar to the somatic counterpart Guillain–Barré syndrome, the nosological position of AAG cases with a chronic course is obscure.

In this report, we describe the clinical features and sural nerve biopsy findings in a case with slowly progressive autonomic neuropathy and positive for antiganglionic AChR antibody followed up for 15 years.

Case report

A male patient noted faintness upon standing at age 61 (in 1994, 7 years before admission to our hospital). Not long afterwards, he felt palpitation, shortness of breath and dizziness during walking. He sometimes lost consciousness while walking after he felt such symptoms. He suffered from diarrhoea 4 years later. Family history was negative. As the loss of consciousness and diarrhoea gradually became frequent, he was admitted to a hospital at age 67, where hypohidrosis and dry …

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