Acute Pandysautonomic Neuropathy 2 Cases

급성 범자율신경장해성신경병증 (Acute Pandysautonomic Neuropathy) 2개증례

Acute pandysautonomic neuropathy(APN) is an uncommon clinical entitiy involving vasomotor, sudomotor, pupilomotor, secretomotor and other autonomic systems. Both sympathetic or parasympathetic fibers are involved with relative preservation of somatic sensory and motor function. Although APN shares several clinical features with GBS, it is not clear whether APN is a subvariety of GBS. We report two young patients with APN. Patient 1 was a 18-year-old girl with recurrent fainting spells. Patient 2 was a 23-year-old man sufferring from unexplained nausea and vomiting. Both had a history of previous upper respiratory infection. They presented with gastroparesis, anhydrosis and orthostatic hypotension. Mild numbness and tingling sense was present, but motor power was intact. Neurologic examination showed bilateral tonic pupil, decreased pain and vibration sense, and absent tendon reflexes. Nerve conduction study indicated diffuse sensorimotor polyneuropathy. Nerve biopsy in patient 2 revealed axonal degeneration. After conservative management, gastrointestinal symptoms were improved in patient 2, however, patient 1 suffered from the symptoms lasting more than several months. These cases suggest that post-infectious dysautonomic symptoms in young patient may indicate the diagnosis of APN. Although the natural course is generally benign, accurate diagnosis and proper management may be mandatory for the better clinical outcome.