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Case Reports
. 2018;57(20):3021-3024.
doi: 10.2169/internalmedicine.0966-18. Epub 2018 Oct 15.

Late-onset Myasthenia Gravis Accompanied by Amyotrophic Lateral Sclerosis with Antibodies against the Acetylcholine Receptor and Low-density Lipoprotein Receptor-related Protein 4

Keiko Ohnari  1 , Kazumasa Okada  1 , Osamu Higuchi  2 , Hidenori Matsuo  3 , Hiroaki Adachi  1
Affiliations
Case Reports

Late-onset Myasthenia Gravis Accompanied by Amyotrophic Lateral Sclerosis with Antibodies against the Acetylcholine Receptor and Low-density Lipoprotein Receptor-related Protein 4

Keiko Ohnari et al. Intern Med. 2018.

Abstract

An 82-year-old woman developed neck weakness and dysarthria with antibodies against acetylcholine receptor (AChR) and low-density lipoprotein receptor-related protein 4 (LRP4). Myasthenia gravis (MG) was diagnosed by edrophonium and repetitive nerve stimulation tests. Her symptoms resolved completely by immunotherapy. One year later, she presented with muscle weakness and bulbar palsy accompanied by atrophy and fasciculation. Her tendon reflexes were brisk, and Babinski's sign was positive. She was diagnosed with probable amyotrophic lateral sclerosis (ALS). Immunotherapy did not improve her symptoms, and she ultimately died of respiratory failure. MG and ALS may share a pathophysiology, including anti-LRP4 antibodies at the neuromuscular junction.

Keywords: amyotrophic lateral sclerosis; anti-acetylcholine receptor antibody; anti-low-density lipoprotein receptor-related protein 4 antibody; myasthenia gravis; neuromuscular junction.

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Figures

Figure 1.
Figure 1.
Repetitive nerve stimulation at 3 Hz showed a 26.3% decrease in the nasal muscle amplitude. The decrement response was calculated using the following formula: decrement (%)=(amplitude of 1st response-amplitude of 4th response) ×100/amplitude of 1st response.
Figure 2.
Figure 2.
The clinical course and medications. Note that the muscle weakness trace shows weakness in the extremities (upper and lower).
See this image and copyright information in PMC

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