Acute idiopathic autonomic neuropathy with local autonomic failure in a child

Abstract A 5-year-old girl presented with flushing and sweating on the left arm with coldness on the left palm that had persisted for approximately 24 hours. She had a fever and chicken pox-like exanthemas on her skin. She had no weakness, sensory disturbance or other autonomic dysfunction, such as orthostatic hypotension. Physical, neurological, blood and cerebrospinal fluid findings, including those of a viral study, were normal. A spinal MRI revealed no abnormal signals. Motor nerve conduction velocity, compound muscle action potential and sensory nerve conduction velocity in both medial nerves were normal, although compound sensory nerve action potential was low in the left medial nerve. F waves were absent in both medial nerves. The amplitude of the sympathetic skin response was low in the left palm. The cold-induced vasodilatation test showed bilateral sympathetic nerve dysfunction, especially on the left side. The coefficient of variation of RR intervals was low. Aciclovir was administered until chicken pox was ruled out. Subsequently, her symptoms improved. However, a sympathetic skin response and cold-induced vasodilatation findings 9 months later revealed sympathetic nerve dysfunction. These findings suggested autonomic neuropathy with local sympathetic dysfunction and a mild sensory nerve disturbance.