G.P.300: Pandysautonomia: A rare variant of Guillain–Barre syndrome

• Published in: Neuromuscular disorders : NMD Vol. 24; no. 9-10; pp. 911 - 912
• Main Authors: Güngör, M., Esmeray, P., Öncel, İ., Topaloglu, H.
• Format: Journal Article
• Language: English
• Published: Elsevier B.V 01.10.2014
• ISSN: 0960-8966; 1873-2364
• DOI: 10.1016/j.nmd.2014.06.390

Gullain-Barre sydrome (GBS) is the most common cause of acute childhood paralysis worldwide, affecting approximately 0.3–2 per 100,000 children per year. This illness is clinically characterized by acute onset of generalized, symmetrical and ascending muscle weakness and areflexia. Beyond motor weakness, autonomic nervous system dysfunction may be prominent during the course of GBS. Pandysautonomia is a very rare form of GBS and charactirezed by pure autonomic involvement without motor weakness. We report the case of a 7year-old boy who developed this form of GBS, the pandysautonomia variant. He was admitted to our hospital with vomitting, swallowing difficulty, colic and midriasis with a gradual onset of two months. He was operated twice, first for a possible appendicitis and then days later for ileus. His examination showed a cachectic appearance, fixed dilated pupils, bilaterally negative pupilllary light reflexes, and abdominal distention. There was urinary retention, too. Motor power was preserved. Deep tendon reflexes were normoactive. Blood biochemistry, cerebrospinal fluid, NCV studies and cranial MRI were normal. Acetylcholine receptor antibody was negative. He was diagnosed with pandysautonomia, a variant of GBS. Initially, after a treatment lag of 4months he was treated with IVIg twice. There was no improvement gained. At the 5th month, oral prednisolone and azathiopurine treatment was launched. Starting from the 15th day, this boy started to show a beneficial response. Swallowing difficulty disappeared, normal feeding resumed, nasogastric catheter was removed, light reflexes of the pupils came back. He was discharged thereafter.