Double Seronegative Myasthenia Gravis with Anti-LRP4 Antibodies Presenting with Dropped Head and Acute Respiratory Insufficiency

作者：Beck Goichi^*; Yabumoto Taiki; Baba Kousuke; Sasaki Tsutomu; Higuchi Osamu; Matsuo Hidenori; Mochizuki Hideki

来源：Internal Medicine, 2016, 55(22): 3361-3363.

DOI：10.2169/internalmedicine.55.7030

• myasthenia gravis
• anti-LRP4 antibodies
• acute respiratory insufficiency
• dropped head
• steroid pulse therapy

We herein report the case of a 72-year-old man demonstrating myasthenia gravis (MG) with a dropped head and acute respiratory insufficiency. There was no ocular, bulbar, or limb involvement. The patient was seronegative for anti-acetylcholine receptor (AChR) antibodies and anti-muscle-specific tyrosine kinase (MuSK) antibodies. Subsequent tests showed seropositivity for anti-low-density lipoprotein receptor-related protein 4 (LRP4) antibodies. The addition of steroid pulse therapy resulted in a full remission of his respiratory symptoms. This presentation suggests that LRP4-positive MG should be considered in the differential diagnosis of patients presenting with acute respiratory insufficiency without either cranial or limb involvement.

• 出版日期2016