Visual Recovery of Dysthyroid Optic Neuropathy With Teprotumumab

2021 ◽  
Vol Publish Ahead of Print ◽  
Author(s):  
Michael J. Lopez ◽  
Jordan L. Herring ◽  
Ciera Thomas ◽  
Bradley A. Bertram ◽  
Dilip A. Thomas
Medicina ◽  
2021 ◽  
Vol 57 (3) ◽  
pp. 202
Author(s):  
Rasa Liutkeviciene ◽  
Agne Sidaraite ◽  
Lina Kuliaviene ◽  
Brigita Glebauskiene ◽  
Neringa Jurkute ◽  
...  

Leber hereditary optic neuropathy (LHON) is one of the most common inherited mitochondrial optic neuropathies, caused by mitochondrial DNA (mtDNA) mutations. Three most common mutations, namely m.11778G>A, m.14484T>G and m.3460G>A, account for the majority of LHON cases. These mutations lead to mitochondrial respiratory chain complex I damage. Typically, LHON presents at the 15–35 years of age with male predominance. LHON is associated with severe, subacute, painless bilateral vision loss and account for one of the most common causes of legal blindness in young individuals. Spontaneous visual acuity recovery is rare and has been reported in patients harbouring m.14484T>C mutation. Up to date LHON treatment is limited. Idebenone has been approved by European Medicines Agency (EMA) to treat LHON. However better understanding of disease mechanisms and ongoing treatment trials are promising and brings hope for patients. In this article we report on a patient diagnosed with LHON harbouring rare m.11253T>C mutation in MT-ND4 gene, who experienced spontaneous visual recovery. In addition, we summarise clinical presentation, diagnostic features, and treatment.


2021 ◽  
pp. 112067212110620
Author(s):  
Lihua Luo ◽  
Dongmei Li ◽  
Lixin Gao ◽  
Wei Wang

Purpose To compare the diagnostic accuracy of peripapillary retinal nerve fiber layer with macular ganglion cell complex thickness as an auxiliary tool for the early diagnosis of dysthyroid optic neuropathy and help assess the effectiveness of the treatment. Methods In this retrospective case–control study, a total of 58 thyroid-associated opthalmopathy patients and 58 healthy participants were enrolled in the study. Thyroid-associated opthalmopathy patients were divided according to the European Group Graves’ Orbitopathy severity classification. The thicknesses of peripapillary nerve fiber layer and macular ganglion cell complex were measured using optical coherence tomography and their correlation with the severity of the disease as well as the effect of the treatment was investigated. Results No statistically significant differences were found between the mild thyroid-associated opthalmopathy group and the control group in both peripapillary nerve fiber layer and macular ganglion cell complex thickness. In the moderate-to-severe thyroid-associated opthalmopathy group, however, Temporal and Nasal peripapillary nerve fiber layer thicknesses were lower compared to the control group ( p = 0.041, p = 0.012), whereas in the sight-threatening thyroid-associated opthalmopathy group Temporal Inferior, Nasal Superior, and mean (G) peripapillary nerve fiber layer thicknesses were larger ( p = 0.000, p = 0.004, p = 0.000). No significant differences were observed in the macular ganglion cell complex thickness among the different severity groups and the control groups ( p > 0.05). After treatment, the mean peripapillary nerve fiber layer thickness decreased whereas mean macular ganglion cell complex thickness showed no significant change in the sight-threatening group. A correlation was established between exophthalmos, best corrected visual acuity, clinical activity score, disease course, and the mean peripapillary nerve fiber layer thickness. The area under curve analysis indicated that mean peripapillary nerve fiber layer thickness can be used as a powerful diagnostic tool in early stage dysthyroid optic neuropathy in thyroid-associated opthalmopathy patients. Conclusion Our study indicates that peripapillary nerve fiber layer act as an auxiliary tool for the early diagnosis of dysthyroid optic neuropathy and helps assess the effectiveness of the treatment.


2019 ◽  
pp. 3-8
Author(s):  
Matthew J. Thurtell ◽  
Robert L. Tomsak

Optic neuritis is the most frequent cause of acute-onset optic neuropathy in young adults and is often encountered in clinical practice. In this chapter, we begin by reviewing the cardinal signs of optic neuropathy. We review the clinical characteristics and workup of optic neuritis. We review factors that increase the risk for developing multiple sclerosis. We discuss atypical clinical and imaging findings that should prompt further evaluation for other causes of optic neuritis, such as neuromyelitis optica. Lastly, we discuss the management options for optic neuritis, with reference to the findings from the Optic Neuritis Treatment Trial, and the prognosis for visual recovery.


Eye ◽  
2020 ◽  
Vol 34 (9) ◽  
pp. 1702-1709 ◽  
Author(s):  
Aylin Garip Kuebler ◽  
Caroline Wiecha ◽  
Lukas Reznicek ◽  
Annemarie Klingenstein ◽  
Kathrin Halfter ◽  
...  

Clinics ◽  
2008 ◽  
Vol 63 (3) ◽  
pp. 301-306 ◽  
Author(s):  
Mário L. R. Monteiro ◽  
Allan C. P. Gonçalves ◽  
Carla T. M. Silva ◽  
Janete P. Moura ◽  
Carolina S. Ribeiro ◽  
...  

Author(s):  
Peerooz Saeed ◽  
Shahzad Tavakoli Rad ◽  
Peter H. L. T. Bisschop

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