[A case of acute idiopathic pandysautonomia with SIADH]

We reported a 6-year-old girl with acute idiopathic pandysautonomia (AIPD) associated with syndrome of inappropriate secretion of anti-diuretic hormone (SIADH). She showed various symptoms indicating the impairment of widespread sympathetic and parasympathetic nervous functions and SIADH, which followed common cold. Sural nerve biopsy showed severe reduction of both myelinated and unmyelinated fibers. The main site of the lesion of AIPD has been assumed to be the peripheral autonomic nervous system. However, there are a few reports indicating the involvement of the central nervous system in AIPD. The present case also indicates the involvement of hypothalamus in addition to the peripheral autonomic nervous system.