Abstract
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.
Abbreviations:
AAG (autoimmune autonomic ganglionopathy), gAChR (ganglionic nicotinic acetylcholine receptor), OH (orthostatic hypotension), IVMP (intravenous methylprednisolone pulse therapy), IVIg (intravenous immunoglobulin)Keywords
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Article Info
Publication History
Published online: December 28, 2015
Accepted:
December 18,
2015
Received in revised form:
December 16,
2015
Received:
November 25,
2015
Identification
Copyright
© 2015 The Japanese Society of Child Neurology. Published by Elsevier B.V. All rights reserved.
