ALS and myasthenia: An unusual association in a patient treated with riluzole

Restivo, Domenico A.; Bianconi, C.; Ravenni, Roberta; Grandis, D. De

doi:10.1002/(sici)1097-4598(200002)23:2<294::aid-mus25>3.0.co;2-g

Cited by 20 publications

(11 citation statements)

References 7 publications

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“…Although profuse fibrillations and PSWs can be seen in some myopathies, such as polymyositis, our patient presented with distal‐dominant limb weakness and normal serum CK; furthermore, he lacked other features of myopathy in electrophysiological and histological studies. MG and muscle atrophy have been associated with polyneuropathy or amyotrophic lateral sclerosis in some patients 8, 10, 17, 21. However, the normal nerve conduction and improvement of muscle atrophy in our patient makes an association with such disorders unlikely.…”

Section: Discussionmentioning

confidence: 57%

Myasthenia gravis presenting with unusual neurogenic muscle atrophy

et al. 2007

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We report a patient with myasthenia gravis who had neurogenic muscle atrophy in association with external ophthalmoplegia and weakness of the upper limbs. Neurogenic changes in the limb muscles were found on needle electromyography and histological studies. Symptoms improved and atrophy of the limbs diminished after intravenous immunoglobulin and oral corticosteroid therapy. We concluded that functional interruption of the neuromuscular junction caused the neurogenic muscle atrophy and that this was relieved by appropriate therapy.

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Section: Discussionmentioning

confidence: 57%

Myasthenia gravis presenting with unusual neurogenic muscle atrophy

et al. 2007

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“…However, Bernstein et al3 reported that disease activity and speed of progression might be more important factors for these findings. Restivo et al7 reported significant decrements in facial muscles in one ALS patient, but this case seem-ed to be a true MG patient with ALS, as evidenced by seropositivity and abnormal decrements in the deltoid muscle.…”

Section: Discussionmentioning

confidence: 74%

Repetitive Nerve Stimulation Test in Amyotrophic Lateral Sclerosis with Predominant Oropharyngeal Manifestations

et al. 2011

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Background and PurposeAmyotrophic lateral sclerosis (ALS) patients display easy fatigability and abnormal decrements on repetitive nerve stimulation (RNS) test of clinically involved limb muscles, which can result in ALS being misdiagnosed as myasthenia gravis. We retrospectively analyzed the RNS tests of ten ALS patients with only or predominant oropharyngeal symptoms without ocular or facial weakness.MethodsRNS tests were performed on the abductor digiti quinti, flexor carpi ulnaris, orbicularis oculi (OO), nasalis and trapezius muscles at low-rate stimulation frequencies of 3 and 5-Hz. Decrements greater than 10% of the compound muscle action potential amplitude on the fifth stimulation compared to the first was regarded as abnormal.ResultsSix patients complained of muscular fatigue or diurnal fluctuation. Among the ten patients, three exhibited abnormal decrements during low-rate stimulation in the facial muscles but not in the limb muscles, two exhibited abnormal decrements in the OO and nasalis muscles, and one exhibited abnormal decrements in the OO muscle.ConclusionsThese findings show that the facial muscles may be involved in some early oropharyngeal forms of ALS, although facial weakness may not be clinically evident. We confirm herein that abnormal decrement of facial muscles to RNS test cannot make a definite diagnose for myasthenia gravis.

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“…A total of 23 full-text articles were assessed for eligibility. Four studies had an obscure diagnosis of either ALS or MG or lack of important information (5–8), five differentiated the two diseases but not their coexistence (9–13), two reported coexistence of MG and lower motor neuron syndrome (14, 15), and one described an ALS patient who developed myasthenia possibly due to the use of riluzole (16). Finally, 11 articles that reported cases diagnosed as coexistence of ALS and MG were identified (4, 17–26).…”

Section: Resultsmentioning

confidence: 99%

Amyotrophic Lateral Sclerosis and Myasthenia Gravis Overlap Syndrome: A Review of Two Cases and the Associated Literature

et al. 2017

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ObjectiveTo describe the characteristics of patients with amyotrophic lateral sclerosis (ALS) and myasthenia gravis (MG) overlap syndrome and explore the relationship between the two diseases.MethodsWe conducted a search of medical records at Peking Union Medical University Hospital from 1983 to 2015 for coexistence of ALS and MG and searched the PubMed database for all literature describing ALS and MG overlap syndrome published through December 2016. We analyzed the clinical and neurophysiological characteristics of patients by groups according to strict diagnostic criteria.ResultsWe presented 2 patients in our database with combined ALS and MG, and together with 25 cases reported in the literature, the patients were divided into 4 groups: 12 patients with MG followed by ALS, 8 patients with ALS followed by MG, 5 ALS patients with false-positive anti-acetylcholine receptor, and the other 2 ALS patients with only myasthenia symptoms. Most patients had limb onset ALS, and myasthenia symptoms mainly affected ocular and bulbar muscles. Clinical and neurophysiological characteristics were summarized.ConclusionThese findings support the conclusion that immunological mechanisms and alterations in the neuromuscular junction are related to ALS pathogenesis.

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ALS and myasthenia: An unusual association in a patient treated with riluzole

Cited by 20 publications

References 7 publications

Myasthenia gravis presenting with unusual neurogenic muscle atrophy

Myasthenia gravis presenting with unusual neurogenic muscle atrophy

Repetitive Nerve Stimulation Test in Amyotrophic Lateral Sclerosis with Predominant Oropharyngeal Manifestations

Amyotrophic Lateral Sclerosis and Myasthenia Gravis Overlap Syndrome: A Review of Two Cases and the Associated Literature

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