Pure Eye Muscle Involvement in Endocrine Orbitopathy

2008 ◽  
Vol 60 (2) ◽  
pp. 67-72 ◽  
Author(s):  
Marion Gerlach ◽  
Andreas Ferbert
Keyword(s):  
Muscle Involvement ◽  
Eye Muscle ◽  
Endocrine Orbitopathy

scholarly journals AB0854 A CASE REPORT OF BROWN SYNDROME COMPLICATING THE MANAGEMENT OF SCLEROMYOSITIS

2021 ◽  
Vol 80 (Suppl 1) ◽  
pp. 1451.1-1451
Author(s):  
P. Arora ◽  
L. Croot
Keyword(s):  
Case Report ◽  
Myasthenia Gravis ◽  
Muscle Weakness ◽  
Tendon Sheath ◽  
Motility Disorder ◽  
Ocular Motility ◽  
Muscle Involvement ◽  
Eye Muscle ◽  
Brown Syndrome ◽  
Superior Oblique

Background:Brown syndrome is a rare ocular motility disorder which has been reported in JRA, RA and SLE but never in a patient with scleromyositis.Objectives:To report the first case of Brown syndrome in a patient with scleromyositis and increase awareness of this condition.Methods:A case report and discussion.Results:The patient was diagnosed with scleromyositis, at the age of 34, after presenting with arthralgia, sclerodactyly, skin pigmentation, Raynaud’s phenomenon, mild muscle weakness and dyspnoea. His labs were CRP 47 mg/L, CK 868 IU/L, ANA strongly positive; anticentromere Ab negative and Anti-PM/Scl-75 and Anti- PM/Scl-100 Ab positive. HRCT chest showed extensive pulmonary fibrosis with lower lobe honeycombing. TLCO was 3.98 (33% of predicted).He was initially managed with high dose steroids and pulsed IV cyclophosphamide with azathioprine for maintenance therapy. His lung disease stabilised and myositis resolved but he continued to develop calcinosis cutis so was switched to 6 monthly IV rituximab.6 years later, he developed morning headaches with intermittent diplopia, described as double vision in vertical gaze with one image being above the other. Episodes lasting 10 minutes to 2 hours. Examination showed normal visual acuity and fundoscopy, no peripheral or eye muscle weakness.Investigations to exclude myasthenia gravis, cerebral vasculitis and atypical infection were organised (MRI, AChR antibody, lumbar puncture, MRA) and were normal.Because of intermittent nature of his episodes, his eye examination was always normal but he captured images in disconjugate gaze with right eye looking upwards and outwards when trying to look straight (Figure 1). Occasionally this was associated with orbital pain and an audible click. These features are suggestive of Brown syndrome.He continues to have recurrent episodes despite immunosuppression but prednisolone 20mg daily for 1-2 days at onset of each attack causes rapid resolution of symptoms.Figure 1.Right eye looking upwards and outwards when trying to look straightConclusion:Scleromyositis is an overlap syndrome of scleroderma and dermatomyositis. Muscle involvement is mild and clinical presentation can be variable. The PM/Scl antibodies are highly characteristic of the syndrome. (1)Brown syndrome is an ocular motility disorder, first described in 1950, characterized by the inability to fully elevate the affected eye in adduction due to pathology of the superior oblique tendon sheath. (2)It can be congenital or acquired, viz, trauma, surgery or sinusitis and also been described in RA, JIA and SLE. (3)If superior oblique tendon cannot relax or slide freely through the trochlea then the affected eye cannot depress completely, leading to diplopia on upward gaze. (4) In inflammatory disease it is thought that swelling of the posterior part of the superior oblique tendon or tenosynovitis are likely causes of the tendon sheath abnormality. (4) This is likely to be the case in this patient because his symptoms are recurrent, respond to steroids and tend to occur more towards the end of rituximab cycles.Recognition of this syndrome is important because invasive investigations can be avoided. Also, intermittent diplopia in a patient with autoimmune disease is suggestive of myasthenia gravis which maybe incorrectly diagnosed.Finally, this case demonstrates the syndrome can be easily managed with short courses of oral steroids, although patients who are already on immunosuppressant treatment may need this in addition.References:[1]Török L, Dankó K, Cserni G, Szûcs G. PM-SCL autoantibody positive scleroderma with polymyositis (mechanic’s hand: clinical aid in the diagnosis). JEADV 2004; 18: 356–359[2]Brown H W. Congenital structural muscle anomalies. In:Alien J H, ed. Strabismus ophthalmic symposium I. St Louis:CV Mosby, 1950: 205-6.[3]Cooper C, Kirwan JR, McGill NW, Dieppe PA. Brown’s syndrome: an unusual ocular complication of rheumatoid arthritis. Ann Rheum Dis 1990; 49:188-9.[4]Sandford-Smith JH. Superior oblique tendon syndrome and its relationship to stenosing tenosynovitis. Br JOphthalmol 1973; 57:859-65.Disclosure of Interests:None declared

scholarly journals Beneficial Effect of Pentoxifylline on Thyroid Associated Ophthalmopathy (TAO)*: a pilot study

1997 ◽  
Vol 82 (6) ◽  
pp. 1999-2002 ◽  
Author(s):  
Cs. Balazs ◽  
E. Kiss ◽  
A. Vamos ◽  
I. Molnar ◽  
N.R. Farid
Keyword(s):  
Pilot Study ◽  
Soft Tissue ◽  
Beneficial Effect ◽  
Tnf Alpha ◽  
Thyroid Peroxidase ◽  
Thyroid Antibodies ◽  
Muscle Involvement ◽  
Eye Muscle ◽  
Hla Dr ◽  
Thyroid Associated Ophthalmopathy

Abstract We have previously found that pentoxifylline (Ptx) inhibited cytokine induced HLA-DR expression and glycosaminoglycan (GAG) synthesis by retroorbital fibroblasts. We have now tested the clinical efficacy of Ptx in treating TAO. Ten patients with moderately severe ophthalmopathy were selected for study. All patients were euthyroid before and during the 12 weeks of the Ptx therapy. Serum GAG, TNF-alpha, anti-TSH-receptor, anti-eye muscle, anti-thyroglobulin and anti-thyroid peroxidase antibodies were determined sequentially. At the end of 12 weeks eight of the ten patients showed improvement in soft tissue but not in proptosis or extraocular muscle involvement. At baseline the levels of GAG (5.2±0.92 mg/dl v.s. 0.7±0.14 mg/dl, p<0.001) and TNF-alpha (33.6±6.6 pg/ml v.s. 5.4±1.3 pg/ml, p<0.001) were increased in patients compared to controls. They gradually decreased in the eight patients who responded to Ptx: after 4, 8 and 12 weeks of therapy serum GAG was 3.4±0.42 mg/dl, 2.5±0.77 mg/dl (p<0.01) and 1.1±0.2 mg/dl (p<0.001), respectively and serum TNF-alpha was 20.9±4.8 pg/ml, 14.9±2.2 pg/ml (p<0.05) and9.7±1.8 pg/ml (p<0.01), respectively. Serum GAG and TNF alpha did not fall in the two patients who did not respond. The titre of anti-eye muscle antibodies but not anti-thyroid antibodies were lower at 12 weeks. Ptx has a beneficial effect on inflammatory symptoms of TAO and associated laboratory parameters in the majority of patients.

Dosage of Eye Muscle Surgery in Endocrine Orbitopathy

2020 ◽  
pp. 265-269
Author(s):  
K.-P. Boergen ◽  
G. Rudolph ◽  
O. Ehrt ◽  
P. Kalpadakis
Keyword(s):  
Eye Muscle ◽  
Endocrine Orbitopathy ◽  
Eye Muscle Surgery

scholarly journals Graves' ophthalmopathy: eye muscle involvement in patients with diplopia

2000 ◽  
Vol 142 (6) ◽  
pp. 591-597 ◽  
Author(s):  
EV Nagy ◽  
J Toth ◽  
I Kaldi ◽  
J Damjanovich ◽  
E Mezosi ◽  
...  
Keyword(s):  
Relaxation Time ◽  
Connective Tissue ◽  
Relaxation Times ◽  
Tissue Expansion ◽  
Muscle Thickness ◽  
T2 Relaxation Time ◽  
T2 Relaxation ◽  
Muscle Involvement ◽  
Eye Muscle ◽  
Orbital Connective Tissue

BACKGROUND: Diplopia identifies patients with eye muscle involvement in Graves' ophthalmopathy (GO). OBJECTIVE: To identify clinical parameters that could eliminate the need for magnetic resonance imaging (MRI) to assess the activity of inflammation in the eye muscles of GO patients with diplopia. METHODS: In 43 patients with GO with recently developed diplopia, orbital ultrasound and MRI were performed. Muscle diameters and MRI T2 relaxation times were measured, and the amount of orbital connective tissue was calculated from MRI scans and compared with ultrasound readings, diplopia grades, degree of protrusion, ocular pressure, tear production, antibody levels and hormonal parameters of thyroid function. RESULTS: No correlation was found between diameters of 233 extraocular muscles measured by MRI and by ultrasound. For each of the four muscles, there was a diameter above which ultrasound was always unreliable. MRI data were used in further analysis. Of the muscles examined, the inferior rectuses were the most frequently enlarged - at least one, in 93% of cases. Medial, lateral and superior rectuses were enlarged in 59%, 37% and 34% of the orbits respectively. The pattern of muscle involvement of the two orbits tended to be symmetric (r=0.49, P=0.003), particularly for the medial rectuses (r=0.90, P=0.000). Proptosis correlated with the sum of the muscle diameters for a given eye (right eye: r=0.54, P=0.003; left eye: r=0.57, P=0.001), but it failed to correlate with the amount of orbital connective tissue. In 53% of the patients, normal T2 relaxation times were found in all eight muscles. There was only a weak correlation between muscle thickness and T2 relaxation time (r=0.49, P=0.003), indicating that muscle enlargement alone is not a sign of disease activity. The severity of diplopia was independent of T2 relaxation time. The amount of orbital connective tissue showed a negative correlation with the greatest T2 relaxation time for a given eye (r= -0.52, P=0.004); this suggests that disease types exist that have predominant muscle involvement and predominant connective tissue expansion. No correlation between connective tissue expansion and proptosis, diplopia grade, muscle thickness or disease duration was found - that is, connective tissue expansion is not a major factor in diplopia. Both muscle and connective tissue findings were independent of thyroid function. CONCLUSION: Ultrasound and MRI eye muscle diameter readings do not correlate, because of the inherent inaccuracy of orbital ultrasound. Muscle enlargement alone does not mean oedematous swelling and active disease. Neither ultrasound, nor any combination of 11 clinical and laboratory parameters provided the degree of information on muscles and connective tissue that was obtainable by MRI. In unclear cases of recently developed diplopia, before orbital decompression surgery, in the case of treatment failure or if, for any other reason, imaging is needed in GO, MRI is the method of choice.

Detection and immunomodulation of eye muscle antibodies in Graves' ophthalmopathy

1987 ◽  
Vol 116 (3_Suppl) ◽  
pp. S12
Author(s):  
G. KAHALY ◽  
R. MONCAYO ◽  
U. BEMETZ ◽  
U. KRAUSE ◽  
J. BEYER
Keyword(s):  
Eye Muscle ◽  
Graves Ophthalmopathy
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