A pediatric case of severe pandysautonomia responsive to plasmapheresis.

Abstract:

:We describe a 13-year-old female with abrupt onset urinary retention progressing rapidly to pandysautonomia with symptoms of postural orthostatic tachycardia syndrome, gastroparesis, anhidrosis, pupillary dysfunction, and abdominal pain. Pandysautonomia has been reported frequently in adults, but is less commonly described in children. Autonomic nervous system dysfunction usually has a self-limiting course with gradual near-complete or complete recovery. Most patients with pure pandysautonomia produce an antibody targeted against the ganglionic nicotinic acetylcholine receptor and titers have been shown to correlate with symptom severity. The clinical presentation described in this report is consistent with a progressive form of acute autoimmune autonomic neuropathy, but she was initially seronegative for known autoantibodies. She responded promptly to plasmapheresis. This case report emphasizes the importance of recognizing features of autonomic nervous system dysfunction and discusses the medical evaluation and treatment options for pediatric patients based on symptom severity.

journal_name

J Child Neurol

authors

Clark MB,Davis T

doi

10.1177/0883073812474099

subject

Has Abstract

pub_date

2013-12-01 00:00:00

pages

1716-9

issue

12

eissn

0883-0738

issn

1708-8283

pii

0883073812474099

journal_volume

28

pub_type

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