Autoimmune autonomic ganglionopathy in a pediatric patient presenting with acute encephalitis

Published:December 28, 2015DOI:


      Autoimmune autonomic ganglionopathy (AAG) is an acquired immune-mediated disorder that leads to systemic autonomic failure. Autoantibodies to the ganglionic nicotinic acetylcholine receptor (gAChR) are detected in 50% of AAG patients. We report the first pediatric case of AAG presenting with acute encephalitis. The patient was a 13-year-old boy who presented with orthostatic hypotension, followed by rapidly progressing disturbance of consciousness. Cerebrospinal fluid analysis revealed significant pleocytosis and increased neopterin concentration. Head MRI showed hyperintensities in bilateral caudate nuclei, putamen, hippocampus, and insula cortex. Severe autonomic dysfunctions such as severe orthostatic hypotension, bradycardia, dysuria, prolonged constipation and vomiting appeared. These symptoms were successfully controlled by repeated immunomodulating therapy with intravenous methylprednisolone pulse therapy and intravenous immunoglobulin. Autoantibodies to the α3 subunit of gAChR were detected at neurological onset, but were undetectable five months later. This observation indicates that AAG should be suspected in patients manifesting acute encephalitis characterized by preceding and prolonged autonomic symptoms, and immunomodulating therapy from an early stage can be effective.


      AAG (autoimmune autonomic ganglionopathy), gAChR (ganglionic nicotinic acetylcholine receptor), OH (orthostatic hypotension), IVMP (intravenous methylprednisolone pulse therapy), IVIg (intravenous immunoglobulin)


      To read this article in full you will need to make a payment

      Purchase one-time access:

      Academic & Personal: 24 hour online accessCorporate R&D Professionals: 24 hour online access
      One-time access price info
      • For academic or personal research use, select 'Academic and Personal'
      • For corporate R&D use, select 'Corporate R&D Professionals'


      Subscribe to Brain and Development
      Already a print subscriber? Claim online access
      Already an online subscriber? Sign in
      Institutional Access: Sign in to ScienceDirect


        • Vernino S.
        • Low P.A.
        • Fealey R.D.
        • Stewart J.D.
        • Farrugia G.
        • Lennon V.A.
        Autoantibodies to ganglionic acetylcholine receptors in autoimmune autonomic neuropathies.
        N Engl J Med. 2000; 343: 847-855
        • Nakane S.
        • Higuchi O.
        • Koga M.
        • Kanda T.
        • Murata K.
        • Suzuki T.
        • et al.
        Clinical features of autoimmune autonomic ganglionopathy and the detection of subunit-specific autoantibodies to the ganglionic acetylcholine receptor in Japanese patients.
        PLoS One. 2015; 10: e0118312
        • Vernino S.
        • Sandroni P.
        • Singer W.
        • Low P.A.
        Autonomic ganglia: target and novel therapeutic tool.
        Neurology. 2008; 70: 1926-1932
        • Baker S.K.
        • Morillo C.
        • Vernino S.
        Autoimmune autonomic ganglionopathy with late-onset encephalopathy.
        Auton Neurosci. 2009; 146: 29-32
        • McKeon A.
        • Lennon V.A.
        • Lachance D.H.
        • Fealey R.D.
        • Pittock S.J.
        Ganglionic acetylcholine receptor autoantibody: oncological, neurological, and serological accompaniments.
        Arch Neurol. 2009; 66: 735-741
        • Watson R.
        • Jepson J.E.
        • Bermudez I.
        • Alexander S.
        • Hart Y.
        • McKnight K.
        • et al.
        Alpha7-acetylcholine receptor antibodies in two patients with Rasmussen encephalitis.
        Neurology. 2005; 65: 1802-1804
        • Iodice V.
        • Kimpinski K.
        • Vernino S.
        • Sandroni P.
        • Fealey R.D.
        • Low P.A.
        Efficacy of immunotherapy in seropositive and seronegative putative autoimmune autonomic ganglionopathy.
        Neurology. 2009; 72: 2002-2008
        • Gibbons C.H.
        • Vernino S.A.
        • Freeman R.
        Combined immunomodulatory therapy in autoimmune autonomic ganglionopathy.
        Arch Neurol. 2008; 65: 213-217