Unusual occurrence of amyotrophic lateral sclerosis in myasthenia gravis

Naik, Karkal Ravishankar; Saroja, Aralikatte Onkarappa; Mahajan, Manik

doi:10.3109/17482968.2012.711836

Cited by 9 publications

(11 citation statements)

References 8 publications

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“…This suggests a more general defect in MG patient's immune or motor system influencing ALS pathogenesis. Co-occurrence of ALS and MG has previously been reported [19][20][21][22]33], with heterogeneity in MG subtypes and clinical condition.…”

Section: Discussionmentioning

confidence: 93%

“…Such molecular mechanisms may be parallel alterations that account for selective vulnerability of motor neurons rather than causative events. MG and ALS have been reported to co-exist in a few patients [19][20][21][22]. False positive AChR antibodies are very uncommon, and a positive titer together with muscle weakness is specific for MG [1,2,23].…”

Section: Introductionmentioning

confidence: 95%

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Myasthenia gravis and amyotrophic lateral sclerosis: A pathogenic overlap

Gotaas

Skeie

Gilhus

2016

Neuromuscular Disorders

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Section: Discussionmentioning

confidence: 93%

Section: Introductionmentioning

confidence: 95%

Myasthenia gravis and amyotrophic lateral sclerosis: A pathogenic overlap

Gotaas

Skeie

Gilhus

2016

Neuromuscular Disorders

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“…Retrospectively, we only identified six cases with co-occurrence of ALS and MG during a twelve years period. Even if we cannot exclude that some cases may not have been referred to one of the French reference centers for ALS, this association seems rare, as supported by the small number of similar cases reported in the literature [10][11][12]. The combination of these two rare diseases may be a by chance association.…”

Section: Discussionmentioning

confidence: 94%

Unusual association of amyotrophic lateral sclerosis and myasthenia gravis: A dysregulation of the adaptive immune system?

Amador

Vandenberghe

Berhoune

et al. 2016

Neuromuscular Disorders

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Myasthenia gravis is an autoimmune disorder affecting neuromuscular junctions that has been associated with a small increased risk of amyotrophic lateral sclerosis (ALS). Here, we describe a retrospective series of seven cases with a concomitant diagnosis of ALS and myasthenia gravis, collected among the 18 French reference centers for ALS in a twelve year period. After careful review, only six patients strictly met the diagnostic criteria for both ALS and myasthenia gravis. In these patients, limb onset of ALS was reported in five (83%) cases. Localization of myasthenia gravis initial symptoms was ocular in three (50%) cases, generalized in two (33%) and bulbar in one (17%). Median delay between onset of the two conditions was 19 months (6-319 months). Anti-acetylcholine receptor antibodies testing was positive in all cases. All patients were treated with riluzole and one had an associated immune-mediated disease. In the one last ALS case, the final diagnosis was false-positivity for anti-acetylcholine receptor antibodies. The co-occurrence of ALS and myasthenia gravis is rare and requires strict diagnostic criteria. Its demonstration needs thoughtful interpretation of electrophysiological results and exclusion of false positivity for myasthenia gravis antibody testing in some ALS cases. This association may be triggered by a dysfunction of adaptive immunity.

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“…In the present study, we show that the distribution of decrements is very similar between ALS and MG. Clinically, proximal muscles are more frequently engaged in both ALS and MG, which may suggest a lower safety factor for neuromuscular transmission in proximal muscles25 ) . Hence, ALS patients with positive decrements should distinguished from those with MG.…”

Section: Discussionmentioning

confidence: 99%

Correlations between slow-rate repetitive nerve stimulation and characteristics associated with amyotrophic lateral sclerosis in Chinese patients

et al. 2017

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[Purpose] To clarify the features associated with decrements in compound muscle action potentials (CMAP) during slow-rate repetitive nerve stimulation (RNS) of muscles involved in amyotrophic lateral sclerosis (ALS) in mainland China. [Subjects and Methods] A retrospective study of decremental responses to slow-rate RNS was performed to compare patients with ALS to those with myasthenia gravis (MG). [Results] A significant decrement (>5%) was observed in at least one muscle in 54% of ALS patients. The trapezius muscle was the most commonly affected (67%). In the ALS group, the CMAP amplitude evoked by the first stimulus was negatively correlated with the CMAP decrement in ulnar but not accessory nerves. Additionally, a positive decrement was associated with disease progression but not gender, age at onset, disease duration, region of onset, ALSFRS-R scores, or ALS diagnostic subgroup in ALS. Furthermore, the incidence of positive decrements and the decremental percentages were significantly higher in myasthenia gravis (MG) than in ALS. [Conclusions] The lower CMAP amplitude by the first RNS stimulus was more likely to induce a positive decrement in the ulnar nerve in ALS patients. The positive decremental responses to RNS observed in ALS indicate the faster progress of the disease, which is helpful for evaluating prognoses.

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Unusual occurrence of amyotrophic lateral sclerosis in myasthenia gravis

Cited by 9 publications

References 8 publications

Myasthenia gravis and amyotrophic lateral sclerosis: A pathogenic overlap

Myasthenia gravis and amyotrophic lateral sclerosis: A pathogenic overlap

Unusual association of amyotrophic lateral sclerosis and myasthenia gravis: A dysregulation of the adaptive immune system?

Correlations between slow-rate repetitive nerve stimulation and characteristics associated with amyotrophic lateral sclerosis in Chinese patients

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