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CC BY-NC-ND 4.0 · Arq Neuropsiquiatr 2017; 75(03): 197-198
DOI: 10.1590/0004-282X20160192
IMAGES IN NEUROLOGY

Facial and bulbar muscle atrophy in acetylcholine receptor antibody-positive myasthenia gravis

Atrofia de musculatura facial e bulbar em miastenia gravis com presença do anticorpo Anti-AChR
Ronnyson Susano Grativvol
1   Universidade de São Paulo, Faculdade de Medicina, Departamento de Neurologia, São Paulo, Brasil.
,
André Macedo Serafim da Silva
1   Universidade de São Paulo, Faculdade de Medicina, Departamento de Neurologia, São Paulo, Brasil.
,
Bruno Fukelmann Guedes
1   Universidade de São Paulo, Faculdade de Medicina, Departamento de Neurologia, São Paulo, Brasil.
,
Eduardo de Paula Estephan
1   Universidade de São Paulo, Faculdade de Medicina, Departamento de Neurologia, São Paulo, Brasil.
,
Rodrigo de Holanda Mendonça
1   Universidade de São Paulo, Faculdade de Medicina, Departamento de Neurologia, São Paulo, Brasil.
,
Antçnio Alberto Zambon
1   Universidade de São Paulo, Faculdade de Medicina, Departamento de Neurologia, São Paulo, Brasil.
,
Carlos Otto Heise
1   Universidade de São Paulo, Faculdade de Medicina, Departamento de Neurologia, São Paulo, Brasil.
,
Edmar Zanoteli
1   Universidade de São Paulo, Faculdade de Medicina, Departamento de Neurologia, São Paulo, Brasil.
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A 62-year-old man presented with seven years of progressive dysphagia, dysphonia and difficulty in closing both eyes. His examination showed weakness and atrophy of facial and bulbar muscles without ocular involvement ([Figure A, B and C]). Single-fiber electromyography revealed increased jitter ([Figure D]). To evaluate for concurrent myopathy, a muscle biopsy was performed and showed angulated atrophic type II fibers, a particular finding described in patients with myasthenia gravis[1],[2]([Figure E]). Acetylcholine receptor antibody was positive (2.2 nmol/L). Pronounced facial and tongue atrophy is uncommon in myasthenia gravis and usually associated with the muscle-specific receptor tyrosine kinase antibody, which was negative in this patient[3],[4].

Zoom Image
Figure Clinical, electrophysiological and left biceps brachii muscle biopsy findings. Bilateral facial weakness, Bell’s sign (A) and atrophy of the tongue (B), temporal and masseter muscles (arrows) (C). Repetitive stimulation was unremarkable (upper image) and the single-fiber electromyography demonstrated increased jitter (bottom image) (D). Muscle ATPase (pH 9.4) stain revealed angulated fiber II atrophy (arrowhead) (E). Bar = 100μm.


Publication History

Received: 26 July 2016

Accepted: 31 October 2016

Article published online:
05 September 2023

© 2023. Academia Brasileira de Neurologia. This is an open access article published by Thieme under the terms of the Creative Commons Attribution-NonDerivative-NonCommercial License, permitting copying and reproduction so long as the original work is given appropriate credit. Contents may not be used for commecial purposes, or adapted, remixed, transformed or built upon. (https://creativecommons.org/licenses/by-nc-nd/4.0/)

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