Open AccessgAChR antibodies in children and adolescents with acquired autoimmune dysautonomia in Japan
Author(s) -
Yamakawa Makoto,
Watari Mari,
Torii KenIchi,
Kuki Ichiro,
Miharu Masashi,
Kawazu Momoko,
Mukaino Akihiro,
Higuchi Osamu,
Maeda Yasuhiro,
Ikeda Tokunori,
Takamatsu Koutaro,
Tawara Nozomu,
Nakahara Keiichi,
Matsuo Hidenori,
Ueda Mitsuharu,
Takahashi Takao,
Nakane Shunya
Publication year - 2021
Publication title -
annals of clinical and translational neurology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.824
H-Index - 42
ISSN - 2328-9503
DOI - 10.1002/acn3.51317
Subject(s) - medicine , dysautonomia , orthostatic vital signs , pediatrics , nausea , vomiting , blood pressure , disease
Objective Patients with acquired autonomic dysfunction may have antibodies specific to the ganglionic nicotinic acetylcholine receptor (gAChR). However, the clinical features of children and adolescents with acquired autonomic dysfunction (AAD) remain unclear. This study aimed to determine the clinical features of pediatric patients with acquired autonomic dysfunction. Methods This study retrospectively examined a series of patients of AAD with serum gAChR antibodies who were referred to our laboratory for antibody testing between January 2012 and April 2019. The study included 200 patients (<20 years, 20 cases; ≥20 years, 175 cases) with clinical features of AAD. Results Upon comparing pediatric and adult patients, we found that antecedent infection and autonomic symptoms at onset with gastrointestinal symptoms occurred more frequently in children with AAD. We confirmed that four children (20.0%) met the diagnostic criteria for postural orthostatic tachycardia syndrome (POTS). A significantly higher number of children than adults had POTS ( P = 0.002). In addition, upper GI dysfunction was more prevalent in children than in adults ( P = 0.042). In particular, nausea and vomiting occurred in 60.0% of children with AAD and in 21.1% of adults ( P < 0.001). The frequency of paralytic ileus was significantly higher in children with AAD (20.0%) relative to adults (6.3%) ( P = 0.030). Regarding extra‐autonomic manifestations, encephalopathy was more frequent in children (15.0%) than in adults (1.1%) ( P < 0.001). Interpretation Pediatric AAD patients have their own clinical characteristics, and these features may be unique to children and adolescents.