A Patient with Fulminant Myasthenia Gravis is Seropositive for Both AChR and LRP4 Antibodies, Complicated by Autoimmune Polyglandular Syndrome Type 3

Hiroyasu Inoue; Kentaro Yamada; Asami Fujii; Tatsuya Tomonari; Kotaro Mizuno; Keiko Mita; Osamu Higuchi; Masaya Akao; Noriyuki Matsukawa

doi:10.2169/internalmedicine.4708-20

This article has now been updated. Please use the final version.

A Patient with Fulminant Myasthenia Gravis is Seropositive for Both AChR and LRP4 Antibodies, Complicated by Autoimmune Polyglandular Syndrome Type 3

Hiroyasu Inoue, Kentaro Yamada, Asami Fujii, Tatsuya Tomonari, Kotaro Mizuno, Keiko Mita, Osamu Higuchi, Masaya Akao, Noriyuki Matsukawa

Author information

Hiroyasu Inoue
Department of Neurology, Nagoya City East Medical Center, Japan
Department of Neurology, Nagoya City University Graduate School of Medical Sciences, Japan
Kentaro Yamada
Department of Neurology, Nagoya City East Medical Center, Japan
Asami Fujii
Department of diabetes & Endocrinology, Nagoya City East Medical Center, Japan
Tatsuya Tomonari
Department of Nephrology, Nagoya City East Medical Center, Japan
Kotaro Mizuno
Department of Surgery, Nagoya City East Medical Center, Japan
Keiko Mita
Department of Surgery, Nagoya City East Medical Center, Japan
Osamu Higuchi
Department of Clinical Research, Nagasaki Kawatana Medical Center, Japan
Masaya Akao
Department of diabetes & Endocrinology, Nagoya City East Medical Center, Japan
Noriyuki Matsukawa
Department of Neurology, Nagoya City University Graduate School of Medical Sciences, Japan

Keywords: acetylcholine receptor, autoimmune polyglandular syndrome, low-density lipoprotein receptor, myasthenia gravis

JOURNAL OPEN ACCESS Advance online publication

Article ID: 4708-20

DOI https://doi.org/10.2169/internalmedicine.4708-20

The final version of this article is now available: Vol. 59 (2020), No. 17 pp. 2177-2181

Details

Abstract

This article describes the first reported case of myasthenia gravis (MG) seropositive for both acetylcholine receptor antibody and low-density lipoprotein receptor-related protein 4 antibody, complicated by autoimmune polyglandular syndrome (APS) type 3. The patient exhibited myasthenic weakness restricted to the ocular muscles and ptosis. Severe clinical deterioration ensued with predominant bulbar symptoms. MG rapidly worsened, the patient was intubated, and agranulocytosis due to thiamazole was also present, so it was necessary to perform thyroidectomy with tracheostomy and thymectomy in two phases. Both the double-seropositive MG and the APS were involved in the patient's rapid deterioration.

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