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1.
Journal of the Korean Ophthalmological Society ; : 1284-1289, 2019.
Artigo em Coreano | WPRIM | ID: wpr-916355

RESUMO

PURPOSE@#To investigate the clinical features of acute ophthalmoplegia associated with anti-GQ1b antibody in the Republic of Korea.@*METHODS@#From January 2011 to July 2018, we retrospectively reviewed the medical records of all patients who tested positive for anti-GQ1b antibody, and who had been concurrently diagnosed with acute ophthalmoplegia at a tertiary hospital in the Republic of Korea. Ophthalmic and neurological features were carefully reviewed. Laboratory results including ganglioside antibody panels and treatment outcomes were analyzed.@*RESULTS@#Thirty-three patients were enrolled in the study and 8 (24%) of them showed pure Miller-Fisher syndrome with all three cardinal symptoms of ophthalmoplegia, ataxia, and areflexia. One patient (3%) showed ophthalmoplegia, areflexia, and pharyngeal- cervical-brachial weakness without ataxia and 2 patients (6%) showed only ophthalmoplegia and pharyngeal-cervical- brachial weakness without ataxia and areflexia. Twenty-two patients (67%) showed only ophthalmoplegia without any other neurological symptom. Early immunosuppressant treatment was used to treat 17 patients, and observation only was conducted as the initial treatment for 16 patients (48.5%). Twenty-four patients (72.7%) showed improvement and 9 patients showed persistent strabismus and diplopia. The average duration until recovery was 2.52 ± 2.11 months.@*CONCLUSIONS@#The symptoms of acute ophthalmoplegia associated with anti-GQ1b antibody can manifest in various forms that overlap with clinical features of demyelinating neuropathy, so it is important to recognize such characteristics when treating acute ophthamoplegia.

2.
Journal of the Korean Ophthalmological Society ; : 1301-1306, 2019.
Artigo em Coreano | WPRIM | ID: wpr-916352

RESUMO

PURPOSE@#Primary localized amyloidosis is a disease characterized by the deposition of abnormal protein fibrils in a specific tissue without systemic involvement. We report a rare case of primary amyloidosis involving the extraocular muscle.CASE SUMMARY: A 51-year-old female visited our clinic due to diplopia. There was a 3 mm exophthalmos and restricted ocular motility in infraduction. Right hypertropia with 25 prism diopters was observed in the primary position. Orbital imaging scans showed fusiform enlargement of the inferior and medial rectus muscles with significant contrast enhancement in the right eye. A calcified nodule embedded in the medial rectus muscle was observed. Empirical steroid pulse therapy was conducted, but the patient showed little response. Subsequently, an incisional biopsy of inferior rectus muscle was performed. A histopathological examination showed amyloid deposits in hematoxylin and eosin staining, and a positive birefringence in Congo-red staining under polarized light, confirming amyloidosis. A systemic work up showed no evidence of systemic amyloidosis besides increased levels of rheumatoid factor.@*CONCLUSIONS@#When infiltrative myopathy of the extraocular muscle is suspected in a patient with exophthalmos and enlargement of extraocular muscles, primary amyloidosis should be considered in the differential diagnosis.

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