Ocular myasthenia: diagnostic and therapeutic problems

Evoli, Amelia; Tonali, P.; Bartoccioni, E.; Monaco, Mauro Lo

doi:10.1111/j.1600-0404.1988.tb06970.x

Cited by 119 publications

(61 citation statements)

References 15 publications

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“…Another explanation may be related to the rather long initial disease duration in our patients. The risk of secondary generalization is well known to have a strong inverse correlation with the duration of ocular disease [3,4,[16][17][18]. Therefore, it is likely that the long initial disease duration in our patients could affect the subsequent generalization rate, i.e, a longer delay before presentation would imply a better prognosis.…”

Section: Discussionmentioning

confidence: 99%

“…This issue needs to be addressed with respect to certain limitations probably inherent in a retrospective study like the current one. First, the diagnosis of OMG can be difficult in patients with atypical or subtle signs, particularly when diagnostic tests, such as AChR antibody assay and Tensilon testing, yield negative or equivocal results [17,20]. Therefore, we suspect that OMG may be underdiagnosed, particularly in seronegative and mild cases.…”

Section: Discussionmentioning

confidence: 99%

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Prognosis of ocular myasthenia in Korea: A retrospective multicenter analysis of 202 patients

Hong

Kwon

Kim

et al. 2008

Journal of the Neurological Sciences

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Objectives: The aims of this study were to obtain data on the frequency with which Korean patients with autoimmune myasthenia gravis (MG) present solely with ocular disturbances and progress to develop generalized disease and to identify the prognostic factors associated with secondary generalization. Methods: We conducted a retrospective multicenter survey in which a total of 376 adult patients who were newly diagnosed with MG from 2000 through 2005 were reviewed for analysis. Patients with ocular MG at the time of symptom presentation (n = 202, 53.7%) were divided into two subgroups according to their prognosis: the patients whose disease remained ocular throughout the follow-ups were placed in the OMG-R group, and the patients who progressed to develop generalized disease were placed in the OMG-G group. Clinical characteristics and laboratory findings were compared between the two subgroups. Results: Secondary generalization developed in 47 (23.3%) of the 202 study subjects, mostly within the first 6 months after symptom presentation, while the disease remained ocular throughout the follow-up duration (median 11.8 months) in the remaining 155 patients (76.7%). AChR antibody, abnormal repetitive nerve stimulation tests (RNST) and thymoma were more frequently observed in the patients in the OMG-G group than in those in the OMG-R group (p b 0.01 in all). In seropositive cases, the titers of AChR antibody were also significantly higher in the OMG-G group than in the OMG-R group (median, 3.8 nM vs. 6.4 nM; p b 0.05). Cox proportional hazards regression analyses showed that early oral prednisolone treatment significantly reduced the risk of secondary generalization (HR, 0.24; 95% CI, 0.11-0.56), whereas abnormal AChR antibody (HR, 5.34; 95% CI,) and thymoma (HR, 2.32; 95% CI, 1.21-4.45) were predictive of the development of secondary generalization. Conclusions: Our findings suggest that several factors, including the AChR antibody, thymoma, early corticosteroid treatment, and possibly latent neuromuscular abnormality revealed by RNST, may have an impact on the risk of developing generalized disease in Korean patients presenting with ocular myasthenia.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Prognosis of ocular myasthenia in Korea: A retrospective multicenter analysis of 202 patients

Hong

Kwon

Kim

et al. 2008

Journal of the Neurological Sciences

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“…6 Like other reports, LPS was the most commonly affected muscle followed by orbicularis oculi. 5 The use of local cooling in a patient with ptosis who is suspected of having myasthenia is a rapid, simple, and inexpensive test with a high degree of specificity and sensitivity. 7,8 A transient improvement of symptoms after injection of edrophonium (Tensilon test) is generally considered fundamental in the diagnosis of MG. Because of the transient nature of the ocular (and systemic) changes in muscle strength that occur following the administration of edrophonium, the neostigmine bromide test remains an exceptionally valuable method of diagnosing MG, particularly in patients with diplopia but without ptosis.…”

Section: Discussionmentioning

confidence: 99%

“…The diagnosis of ocular myasthenia was based on the association of typical history and signs together with at least one of these features: unequivocal improvement of ocular signs after injection of Tensilon test, positive response to Electromyography tests, detection of serum antibodies to AChR 5 . We excluded all patients who had symptoms of difficulty in swallowing or chewing, fatigue, limb weakness or respiratory muscle weakness which suggested features of generalized myasthenia gravis (GMG) at the onset of disease.…”

Section: Methodsmentioning

confidence: 99%

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A clinical approach to ocular myasthenia gravis

Sitaula¹,

Sharma²,

Thapa³

et al. 2015

J Chitwan Med Coll

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Myasthenia gravis is a common disorder of the neuromuscular junction characterized clinically by muscle weakness and fatigability. Ocular myasthenia is often misdiagnosed in the initial stages due to the fluctuating nature of ptosis and diplopia. Here, we describe the clinical characteristics of ocular myasthenia gravis along with the diagnostic difficulties in a tertiary eye care center in Nepal. We retrospectively reviewed the charts of the patients diagnosed as ocular myasthenia gravis in Neuro-ophthalmology clinic, BPKLCOS, from 2010 to 2013. The diagnosis of ocular myasthenia was based on the association of typical history and signs together with at least one of these features: unequivocal improvement of ocular signs after injection of neostigmine, positive response to electrophysiological tests and detection of serum antibodies to acetylcholine receptors. There were 24 cases of diagnosed ocular myasthenia gravis during the 3 years. Among them 11 cases (45.8%) were males and 13 cases (54.1%) were females. The mean age was 32.04±19.56 years with the age range from 9 years to68 years. The most common symptoms among patients were drooping of the lids (45.8%, n=11), followed by both double vision and drooping of the lids (37.5%, n=9) and only intermittent double vision in16.6% (n=4). In two patients (9.09%) CT-scan findings were suggestive of thymoma. The symptoms were resolved with anticholinesterases alone in 66.6% (n=16) and anticholinesterases were supplemented with immunosuppressive drugs in 33.3% (n=8) patients. Ocular myasthenia is often misdiagnosed in the initial stages due to its fluctuating nature. With a high index of clinical suspicion simple clinical tests accompanied by pharmacological tests can be useful in diagnosing the condition.DOI: http://dx.doi.org/10.3126/jcmc.v4i3.11931Journal of Chitwan Medical College 2014; 4(3): 5-8

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Beneficial Effects of Corticosteroids on Ocular Myasthenia Gravis

Kupersmith

Moster²,

Bhuiyan³

et al. 1996

Archives of Neurology

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Moderate-dose daily prednisone for 4 to 6 weeks, followed by low-dose alternate-day therapy as needed, can control the diplopia in patients with ocular myasthenia gravis. The frequency of deterioration to generalized myasthenia gravis at 2 years may be reduced; 9.4% in this study compared with more than 40% previously reported frequency. Corticosteroids may be useful even when ocular motor dysfunction is not normalized.

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Ocular myasthenia: diagnostic and therapeutic problems

Cited by 119 publications

References 15 publications

Prognosis of ocular myasthenia in Korea: A retrospective multicenter analysis of 202 patients

Prognosis of ocular myasthenia in Korea: A retrospective multicenter analysis of 202 patients

A clinical approach to ocular myasthenia gravis

Beneficial Effects of Corticosteroids on Ocular Myasthenia Gravis

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