Mitochondrial disorder should be considered as a differential of late-onset myasthenia gravis
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COMMENTARY AND EDITORIAL
Mitochondrial disorder should be considered as a differential of late‑onset myasthenia gravis J. Finsterer1 · F. A. Scorza2 · C. A. Scorza2 · A. C. Fiorini3,4 Received: 16 February 2020 / Accepted: 20 March 2020 © Belgian Neurological Society 2020
Keywords Myasthenia · Nonagenarian · Mitochondrial · Repetitive nerve stimulation · Ptosis · Double vision
Commentary With interest, we read the article by Oliveria et al. about a 93-year-old female with a 2 months history of generalised weakness, ptosis, double vision, dysarthria, dysphagia, neck pain, and hypophonia, being diagnosed as myasthenia gravis [1]. The authors concluded that the outcome would be more favourable if the diagnosis would have been established earlier. The study has a number of shortcomings. We do not agree with the conclusions. The outcome was excellent. Missing myasthenia is a frequent event. If the diagnosis would have been established, for instance 4 weeks earlier, the outcome, most likely, would have been the same. Myasthenia is often overlooked for weeks or months before being correctly diagnosed. Nevertheless, it is surprising that myasthenia was not considered earlier in thus 93-year-old * J. Finsterer [email protected] F. A. Scorza [email protected] C. A. Scorza [email protected] A. C. Fiorini [email protected] 1
Krankenanstalt Rudolfstiftung, Messerli Institute, Postfach 20 1180 Vienna, Austria
2
Disciplina de Neurociência, Universidade Federal de São Paulo/Escola Paulista de Medicina (UNIFESP/EPM), São Paulo, Brazil
3
Programa de Estudos Pós‑Graduado Em Fonoaudiologia, Pontifícia Universidade Católica de São Paulo (PUC-SP), São Paulo, Brazil
4
Departamento de Fonoaudiologia, Escola Paulista de Medicina/Universidade Federal de São Paulo (EPM/UNIFESP), São Paulo, Brazil
patient with ptosis, double, vision, dysarthria, dysphagia, and quadruparesis. Missing are the exact titers of the acetyl-choline receptor antibodies and the appropriate reference limits. Considering that acetyl-choline receptor antibodies were only slightly elevated, differential diagnoses should be discussed, as a decremental response on repetitive nerve stimulation may not only be found in myasthenia gravis and as pyridostigmine may not only be beneficial in myasthenia gravis. Particularly excluded should be a mitochondrial disorder (MID), as the latter may be easily misdiagnosed as myasthenia [2]. Needle electromyography (EMG) in myasthenia gravis patients may not only show a myogenic pattern [1]. Needle EMG using concentric needles may be also normal, non-specifically abnormal, or neurogenic in myasthenia [3, 4]. Since the EMG was myogenic, myopathy should be appropriately excluded. Was the family history positive for neuromuscular disorders? Missing is an extensive individual history and the current medication the patent was taking prior to the development of myasthenic symptoms. Knowing the current medication is crucial as drugs can strongly influence the clinical presentation and may even trigger the de
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