A Case of Melkersson-Rosenthal syndrome

J Korean Neurol Assoc > Volume 17(4); 1999 > Article

Journal of the Korean Neurological Association 1999;17(4): 602-604.

Melkersson-Rosenthal syndrome 1예

임준성, 정상준 박운규 이영주

한양대학교 의과대학 신경과학교실

A Case of Melkersson-Rosenthal syndrome

Joon-Sung Yim, M.D., Sang-Joon Jung, M.D., Woon-Gyu Park, M.D., Young-Joo Lee, M.D.,

Department of Neurology, Hanyang University Hospital

Abstract

Melkersson-Rosenthal syndrome (MRS) is a rare form of hereditary angioedema characterized by a triad of orofacial swelling, relapsing facial paralysis, and a fissured tongue. However, the classic triad is not frequently seen in its complete form, and monosymptomatic or oligosymptomatic forms are more common. Case: A 20-year-old man presented with recurrent labial swelling 9 months ago and recently with a fissured tongue. The surface of the tongue showed deep furrows characteristic of lingua plicata, and an edematous enlargement was observed on the lower lips. Upon neurologic examination, a left sided facial palsy of a peripheral type was noted without abnormalities in the taste sense or lacrimation. An electromyography of the left frontal muscle showed positive sharp waves and fibrillation potentials. Biopsies performed on the lower lips revealed the congested vessels and perivascular inflammatory cells. We report a 20-year-old man with a classical triad of symptoms of Melkersson-Rosenthal syndrome. J Kor Neurol Ass 17(4):602~604, 1999 Key Words : Orofacial Swelling, Facial Paralysis, Fissured Tongue