The clinical limits of myasthenia gravis and differential diagnosis

Having droopy eyelids is a common problem. Causes may range from disorders that are simple and not progressive, such as congenital ptosis, to progressive, multisystemic disorders, such as mitochondrial disease. Myasthenia gravis (MG) is a very significant and treatable cause of ptosis that should always be part of the differential diagnosis. Apraxia of the eyelid opening can be confused with ptosis or blepharospasm, leading to a delay in reaching the correct diagnosis. Horner’s syndrome has different causes and management approaches. Knowledge of the anatomy and physiology of the eyelids is crucial for understanding the pathological processes that affect them.

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Myasthenia gravis: pathophysiology, diagnosis, differential diagnosis and management

Clinical Eye and Vision Care ◽

10.1016/0953-4431(95)00101-8 ◽

1995 ◽

Vol 7 (1) ◽

pp. 3-13 ◽

Cited By ~ 6

Author(s):

Kelly H. Thomann ◽

Shruti Pandya

Keyword(s):

Differential Diagnosis ◽

Myasthenia Gravis ◽

Diagnosis And Management

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P038 Is it all in the eyes?

Rheumatology ◽

10.1093/rheumatology/keab247.035 ◽

2021 ◽

Vol 60 (Supplement_1) ◽

Author(s):

Anil Gharatya ◽

Julekha Wajed ◽

Natalia Cernovschi ◽

Dimitrios Christidis

Keyword(s):

Differential Diagnosis ◽

Myasthenia Gravis ◽

Giant Cell Arteritis ◽

Giant Cell ◽

Inflammatory Markers ◽

British Society ◽

Ischemic Optic Neuropathy ◽

Temporal Artery Biopsy ◽

Blurred Vision ◽

Ocular Symptoms

Abstract Background/Aims The ocular manifestations of giant cell arteritis (GCA) and ocular myasthenia gravis (OMG) have significant overlap, which can lead to clinical uncertainty in the absence of awareness and routine diagnostic testing. Methods We present the case of a 63-year-old male patient with a strong family history of autoimmune disease. He presented to the Rheumatology department in 2009 with symptoms of polymyalgia rheumatica (PMR) and was treated successfully with a reducing regime of Prednisolone. In 2013, he presented to the Ophthalmology department with blurred vision and diplopia. Giant cell arteritis was suspected and a high dose of Prednisolone was started. His temporal artery biopsy was normal and inflammatory markers were not raised, but there was a complete resolution of his symptoms since starting steroids. Under the care of the Rheumatology department, reducing regimes of Prednisolone were attempted and a steroid sparing agent, Methotrexate was introduced. Unfortunately, he continued to have recurring symptoms of blurred vision and diplopia, particularly at Prednisolone doses less than 20mg. Over the course of a few years, there was a progression of his ocular symptoms. He developed a horizontal diplopia, monocular ptosis and a 4th nerve palsy. He was referred back to the Ophthalmology department, where alternate diagnoses were investigated. Anti-acetylcholine receptor antibodies (AChR) were strongly positive at 35 mU/L (normal <0.44 mU/L). MRI head was normal, and CT chest showed no thymoma. Single fibre EMG confirmed severe OMG. Our patient was subsequently commenced on Pyridostigmine, with which symptoms improved. Results The literature on OMG mimicking GCA is sparse. The prevalence of an exclusively ophthalmic presentation in patients with GCA is 1 in 5. Ocular signs and symptoms in GCA can vary drastically due to the propensity of the disease to affect different structures within the eye. Transient and permanent visual loss, affects 10-30% of GCA patients due to anterior ischemic optic neuropathy. Diplopia affects 5% of GCA patients. In comparison, OMG can be the only presenting feature in 15% of patients with Myasthenia Gravis, and is characterised by unilateral ptosis, oculomotor paresis and binocular diplopia. Conclusion This case highlights the clinical similarities of OMG and GCA, and that OMG should be considered as a differential diagnosis when the clinical picture of GCA does not truly fit. In patients presenting with GCA with ocular symptoms, normal inflammatory markers and no systemic features, OMG should be considered and AChR antibody testing be performed. More awareness of OMG is needed. We also propose that OMG should be included as a differential diagnosis, in the British Society of Rheumatology guidelines for GCA. Disclosure A. Gharatya: None. J. Wajed: None. N. Cernovschi: None. D. Christidis: None.

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The Myotonic Plot Thickens: Electrical Myotonia in Antimuscle-Specific Kinase Myasthenia Gravis

Case Reports in Neurological Medicine ◽

10.1155/2015/242691 ◽

2015 ◽

Vol 2015 ◽

pp. 1-4

Author(s):

Marcus Magnussen ◽

Ioannis Karakis ◽

Taylor B. Harrison

Keyword(s):

Differential Diagnosis ◽

Respiratory Failure ◽

Tyrosine Kinase ◽

Myasthenia Gravis ◽

Nerve Stimulation ◽

Inflammatory Myopathies ◽

Proximal Muscle Weakness ◽

Proximal Muscle ◽

Repetitive Nerve Stimulation ◽

Early Recruitment

Electrical myotonia is known to occur in a number of inherited and acquired disorders including myotonic dystrophies, channelopathies, and metabolic, toxic, and inflammatory myopathies. Yet, electrical myotonia in myasthenia gravis associated with antibodies against muscle-specific tyrosine kinase (MuSK) has not been previously reported. We describe two such patients, both of whom had a typical presentation of proximal muscle weakness with respiratory failure in the context of a significant electrodecrement in repetitive nerve stimulation. In both cases, concentric needle examination revealed electrical myotonia combined with myopathic motor unit morphology and early recruitment. These findings suggest that MuSK myasthenia should be included within the differential diagnosis of disorders with electrical myotonia.

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An undiagnosed myasthenia gravis presenting as isolated recurrent acute respiratory failure

Journal of Neurosciences in Rural Practice ◽

10.4103/0976-3147.91961 ◽

2012 ◽

Vol 03 (01) ◽

pp. 80-82

Author(s):

Ram Shri Sharma ◽

Nalini Sharma ◽

ME Yeolekar

Keyword(s):

Emergency Department ◽

Differential Diagnosis ◽

Critical Care ◽

Respiratory Failure ◽

Acute Respiratory Failure ◽

Myasthenia Gravis ◽

Care Setting ◽

Signs And Symptoms ◽

Initial Presentation ◽

Speech Therapist

ABSTRACTAcute respiratory failure is an uncommon initial presentation of myasthenia gravis (MG). In our case a 22-year-old woman of unrecognized MG presented to the emergency department with isolated respiratory failure as the first presenting symptom. Initially she presented with dysphonia and was managed by speech therapist and ENT surgeons for 3 months. Subsequently, she presented with signs and symptoms of sepsis and went into acute respiratory failure. This case highlights the need to consider MG in the differential diagnosis of an otherwise unexplained respiratory failure in the critical care setting.

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Early Motor Unit Disease Masquerading as Psychogenic Breathy Dysphonia: A Clinical Case Presentation

Journal of Speech and Hearing Disorders ◽

10.1044/jshd.3601.115 ◽

1971 ◽

Vol 36 (1) ◽

pp. 115-124 ◽

Cited By ~ 9

Author(s):

Arnold E. Aronson

Keyword(s):

Differential Diagnosis ◽

Myasthenia Gravis ◽

Motor Unit ◽

Clinical Features ◽

Muscular Strength ◽

Clinical Case ◽

Neurologic Disease ◽

Voice Change ◽

Case Presentation ◽

Voice Changes

This is a study of a 20-year-old girl who developed mild, breathy dysphonia which, because of its nonspecificity, had been previously diagnosed as psychogenic; in actuality her voice change was a sign of early myasthenia gravis. The case is presented to alert the clinician to the fact that voice changes can be one of the first and only signs of early neurologic disease. Differential diagnosis requires careful laryngologic, psychiatric, speech, and neurologic examinations. In this instance, the laryngologic and psychiatric examinations were nonproductive, but the speech examination elicited a marked increase in breathiness, hypernasality, and articulatory imprecision as the consequence of prolonged, effortful speaking. This finding, along with the neurologist’s demonstration of an increase in muscular strength by means of injecting edrophonium chloride (Tensilon) led to the diagnosis of myasthenia gravis. A summary of the incidence, clinical features, methods of examination, and treatment of this disease follow the case presentation.

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Application of the Technique of Extracorporeal Membrane Oxygenation in a Patient With Respiratory Distress Syndrome Associated With Myasthenia Gravis

Russian Sklifosovsky Journal Emergency Medical Care ◽

10.23934/2223-9022-2021-10-2-393-400 ◽

2021 ◽

Vol 10 (2) ◽

pp. 393-400

Author(s):

N. M. Kruglyakov ◽

D. G. Levitova ◽

G. I. Bagzhanov ◽

K. K. Gubarev ◽

S. S. Ochkin ◽

...

Keyword(s):

Differential Diagnosis ◽

Respiratory Failure ◽

Extracorporeal Membrane Oxygenation ◽

Myasthenia Gravis ◽

Muscle Weakness ◽

High Mortality ◽

Respiratory Support ◽

Myasthenic Crisis ◽

Social Significance ◽

Membrane Oxygenation

Myasthenia gravis is an autoimmune neuromuscular disease characterized by pathologically rapid fatigue of striated muscles [1]. The main symptom of myasthenia gravis is the presence of pathological muscle weakness with involvement of the ocular, bulbar and skeletal muscles in the pathological process. The provoking factors for the development of myasthenia gravis can be infectious diseases, surgery, drugs [2, 3]. The main danger is represented by myasthenic and cholinergic crises, which are characterized by a severe course and high mortality; therefore, the problems of treating myasthenia gravis are still of high medical and social significance. The prevalence of myasthenia gravis is 17.5–20.3 per 100 thousand population, and the number of patients is increasing by 5–10% annually [4, 5]. In recent years, there has been a steady increase in morbidity with an increase in age over 50 years [6, 7]. Myasthenia gravis is a serious disease with a high mortality rate of up to 30–40% [3]. There are difficulties in the early differential diagnosis of muscle weakness in patients with respiratory failure between myasthenia gravis, myasthenic syndrome and critical illness polyneuropathy. These difficulties and insufficient awareness of patients and doctors of various specialties about myasthenia gravis can lead to the choice of the wrong treatment tactics and the development of myasthenic crisis, which is manifested by respiratory failure, requiring respiratory support. The progression of respiratory failure against the background of myasthenic crisis may require the use of extracorporeal membrane oxygenation (ECMO).It is necessary to expand the differential diagnosis of muscle weakness in a patient during the period of resolution of respiratory failure, allowing to move away from compulsory respiratory support, termination of ECMO.

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