[P1-136] Two Pediatric Cases of Autoimmune Autonomic Neuropathy
Autoimmune autonomic neuropathy (AAN) is characterized by acute or subacute onset immune-mediated dysautonomia, which is regarded as a subgroup of Guillain-Barre syndrome. Anti-ganglionic acetylcholine receptor (AChR) antibody in serum is positive in about half of these cases. The diagnostic criteria of AAN has not been established yet, and reported pediatric cases are rare. We report two children with AAN successfully treated by intravenous Immunoglobulin (IVIG). The first patient was a 9-year-old boy who had headache and hands and feet pain after mumps infection. He gradually had general fatigue and became bed ridden several days later. Although no abnormalities were seen in neurological findings including nerve conduction study, coefficient of variation of R-R intervals (CVRR) in ECG were decreased. Standing load test showed postural orthostatic tachycardia syndrome. Cerebrospinal fluid examination showed albuminocytologic dissociation. His symptoms were markedly improved by IVIG administration. The second patient was a 13-year-old boy who had agitation, generalized convulsion after sinusitis. Paroxysmal supraventricular tachycardia was noted at emergency arrival, then he had headache, hypertension, tachycardia, general fatigue persisting for one week. He had neck stiffness, and high voltage slow waves were noted in frontal region on EEG. Motor nerve conduction study showed increased latency of F waves. CVRR were decreased. Cerebrospinal fluid showed albuminocytologic dissociation. His symptoms had been improved following IVIG administration. Serum anti-ganglionic AChR antibody was negative in both patients. AAN should be considered when acute or subacute onset autonomic symptoms are observed.