Inferior oblique myositis presenting as superior oblique muscle palsy

A 14-year-old girl presented with progressive vertical strabismus simulating superior oblique muscle palsy. However, because of the presence of limitation of infraduction, computed tomography was done; it showed a large mass in the region of the right inferior oblique muscle. A biopsy specimen of the enlarged muscle showed mainly degenerative and some regenerative changes. The diagnosis was orbital myositis, which should be considered in the differential diagnosis of strabismus, especially if the clinical findings are atypical of strabismus.     

Thyroid ophthalmopathy presenting as superior oblique paresis.

Six patients with thyroid ophthalmopathy presented with what appeared to be a unilateral superior oblique paresis by the three-step test, which was eventually followed by more typical findings of thyroid disease. This early motility defect in thyroid ophthalmopathy may be caused by a restrictive process due to involvement of the inferior rectus muscle. Clues to the proper diagnosis included an increase in vertical deviation in upgaze, elevation of intraocular tension in upgaze, and the lack of excyclodeviation. These features should be assessed in patients with isolated superior oblique paresis.     

Ocular rotation as a tool for superior oblique tendon exposure

In several clinical studies involving superior oblique surgery it was noticed that the posterior insertion of the superior oblique tendon can be moved outside from the undersurface of the superior rectus muscle. This was achieved by rotating the eye in a maximum nasal and downwards direction while avoiding intorsion or retraction of the globe. Traction was applied on the superior temporal quadrant of the globe.     

Ocular myasthenia

Ocular myasthenia is a special form of general myasthenia gravis characterized by unilateral or bilateral ptosis and eye muscle pareses of distinct variability, depending on the time of day and the state of fatigue of the patient. Most important for diagnosis is the Tensilon test, which can, however, produce negative results. In such cases a combination of the Tensilon test with electromyography is indispensable. In ocular myasthenia there is not always an increase in the antibody titer against acetylcholine receptors in the blood. The treatment of ocular myasthenia is based on the application of cholinesterase inhibitors. The drug of choice is Mestinon; however, the reaction of the eye muscles to this drug is often unsatisfactory. Local application of cholinesterase inhibitors in the form of Eserine, Prostigmin etc. is an additional important therapy. Also in ocular myasthenia the modern treatment with Cortisone (alternate-day therapy with 100 mg Prednisone every second day) has proved very useful. Another possible method of interfering with the immunological systems of myasthenia is immunosuppression with Azathioprin or Cyclophosphamide. The pathognomonic significance of the thymus in the autoimmune process of myasthenia gravis is demonstrated by the good results obtained by thymectomy, which can also be performed successfully in ocular myasthenia, not only in young patients in whom the condition is severe, but also in older patients in whom it is chronic. Often, the therapeutic measures mentioned have to be tested one after another or in combination in order to achieve an optimal therapeutic effect.     

Abduction paralysis with hypotropia: sign of weakness of a contralateral superior oblique

background The clinical diagnosis of extraocular motor paralysis that is caused by severe cranial trauma can often be complicated. The resulting clinical picture can make the identification of all the components of potentially treatable oculomotor problems difficult. methods We examined five cases of complete abducens nerve paralysis with marked downshoot in attempted abduction seen after severe cranial trauma. results With the patients looking in the field of gaze of the paralysis, a marked infraductive movement of the paralytic eye occurred while the other eye maintained fixation. Other clinical findings confirmed this to be a secondary deviation due to a paresis of the contralateral superior oblique. conclusion Patients with a paralysis of the lateral rectus following a severe cranial trauma who demonstrate a marked downshoot of the involved eye should be suspected of having a paresis of the contralateral superior oblique. This diagnosis has helped us effectively to treat this vertical incomitance by a simple weakening procedure of the contralateral inferior oblique.     

Abduction paralysis with hypotropia: Sign of weakness of a contralateral superior oblique

background The clinical diagnosis of extraocular motor paralysis that is caused by severe cranial trauma can often be complicated. The resulting clinical picture can make the identification of all the components of potentially treatable oculomotor problems difficult. methods We examined five cases of complete abducens nerve paralysis with marked downshoot in attempted abduction seen after severe cranial trauma. results With the patients looking in the field of gaze of the paralysis, a marked infraductive movement of the paralytic eye occurred while the other eye maintained fixation. Other clinical findings confirmed this to be a secondary deviation due to a paresis of the contralateral superior oblique. conclusion Patients with a paralysis of the lateral rectus following a severe cranial trauma who demonstrate a marked downshoot of the involved eye should be suspected of having a paresis of the contralateral superior oblique. This diagnosis has helped us effectively to treat this vertical incomitance by a simple weakening procedure of the contralateral inferior oblique.     

Ocular myasthenia gravis

Ocular myasthenia gravis may mimic any pupil-spared, painless, nonproptotic ophthalmoplegia with or without ptosis. This review references recent papers regarding clinical features, diagnostic techniques, and treatment for ocular myasthenia gravis.     

Ocular myasthenia gravis

Myasthenia gravis (MG) is probably the best studied autoimmune disease caused by autoantibodies against the acetylcholine receptor (AChR) at the neuromuscular junction, subsequently leading to abnormal fatigability and weakness of skeletal muscle. Extraocular muscle weakness with droopy eyelids and double vision is present in about 90% of MG patients, being the initial complaint in about 50%. In approximately 20% of the patients the disease will always be confined to the extraocular muscles. The single most important diagnostic test is the detection of serum antibodies against AChR which is positive in 90% of patients with generalized MG, but only in 65% with purely ocular MG. Electromyographic studies and the Tensilon test are of diagnostic value in clear-cut cases, but may be equivocal in purely ocular myasthenia, especially the latter not rarely producing false-positive results. Treatment response to corticosteroids and anti-cholinesterase agents is satisfactory in many patients with ocular MG, however other immunosuppressive drugs may also be needed. Pathogenetically relevant steps of the underlying autoimmune process have been elucidated during the last few years; nevertheless a number of questions remain open, especially what starts off the autoimmune process, and why are eye muscles so frequently involved in MG?     

Ocular myasthenia gravis

Myasthenia gravis may be limited solely to the eyes (ocular myasthenia) or may have systemic manifestations (generalized myasthenia). Most patients display blepharoptosis or ophthalmoparesis, resulting in diplopia, but the ocular motility disorder may take many forms. The diagnosis is often elucidated by variable muscle weakness with fatigability noted in the history and on examination. The ice test has supplanted the need for the edrophonium (Tensilon) test in many cases of ocular myasthenia. Therapy may be challenging, because many ocular myasthenics do not achieve significant clinical improvement with acetylcholinesterase inhibitors alone. Immunomodulatory treatment,such as systemic corticosteroids, is frequently effective, and emerging data suggest that this type of therapy may reduce the likelihood of ocular myasthenia becoming generalized.     

Isolated superior oblique tucking: an effective procedure for superior oblique palsy with profound superior oblique underaction.

PURPOSE: To compare efficacy and complications of isolated unilateral superior oblique tucking in patients with unilateral superior oblique palsy (SOP). METHOD: A retrospective analysis of 24 cases of unilateral SOP, 13 Acquired (group 1), and 11 Congenital (group 2), who underwent isolated unilateral superior oblique tuck over a 13-year period was performed. RESULTS: The mean preoperative vertical deviation in primary gaze was 10 +/- 3 PD for group 1 and 12 +/- 5 PD for group 2 and mean vertical deviation in lateral gaze of affected superior oblique was 19 +/- 5 PD for group 1 and 21 +/- 9 PD for group 2. The mean postoperative vertical deviation in primary gaze for group 1 after a mean follow-up period of 15 +/- 21 months was 1 +/- 3 PD; for group 2 after a mean follow-up period of 17 +/- 13 months was 2 +/- 3 PD, and in lateral gaze of affected superior oblique was 3 +/- 5 PD for group 1 and 5 +/- 6 PD for group 2. The mean correction of vertical deviation in primary gaze at last follow-up was 8 +/- 2 PD for group 1 and 9 +/- 5PD for group 2 ( P > 0.05) and in the lateral gaze field of affected superior oblique muscle was 16 +/- 4 PD for group 1 and 15 +/- 5 PD for group 2 ( P > 0.05). The mean preoperative torsion was 9 +/- 4 degrees for group 1 and 9 +/- 2 degrees for group 2; mean postoperative torsion was 1.2 +/- 2.2 degrees for group 1 and 1 +/- 1 degrees for group 2. The mean torsion corrected for group 1 was 8 +/- 3 degrees and for group 2 was 8 +/- 2 degrees ( P > 0.05). Only one patient in group 1 and three patients in group 2 required reoperation to correct residual deviation. A mild postoperative limitation to elevation in adduction was seen in all cases but was asymptomatic and lessened over time. CONCLUSION: Isolated unilateral superior oblique tucking corrected a large amount of the vertical deviation and torsion with minimal complications in selective patients of both congenital and acquired superior oblique palsy. Superior oblique tucking is a safe and effective procedure and can be considered in patients with SOP meeting selective criteria.     

Treatment of superior oblique palsy with superior oblique tendon tuck and inferior oblique muscle myectomy

Twelve patients underwent quantitated superior oblique tendon tuck and ipsilateral inferior oblique muscle myectomy in the primary treatment of unilateral superior oblique muscle palsy. Preoperatively, each patient exhibited a vertical deviation of 30-55 prism diopters in at least one position of gaze. Following a single operation, satisfactory ocular alignment was obtained in all but one case, with fusion demonstrable in clinically significant gaze positions. Eight of 12 patients had no measurable vertical deviation in primary position, and two others had residual hyperphorias of only two prism diopters. No tucks were removed for postoperative Brown's syndrome. Superior oblique tendon tuck combined with ipsilateral inferior oblique muscle myectomy appears to be a safe and effective treatment for large angle superior oblique muscle palsy.     

Orbital mantle cell lymphoma presenting as myasthenia gravis

A 69-year-old man, previously treated with pyridostigmine for myasthenia gravis (manifesting as ptosis and diplopia) was evaluated for several concomitant bilateral anterior orbital masses. Imaging revealed 3 discrete, solid masses within and around the orbits. An incisional biopsy demonstrated atypical lymphocytes positive for CD20 and Cyclin-D1, consistent with mantle cell lymphoma. The patient received induction chemotherapy with a rituximab-based regimen. He experienced resolution of his diplopia and ptosis after one cycle of chemotherapy and achieved complete remission of the orbital masses and myasthenia symptoms after 6 cycles. Myasthenia gravis is most commonly associated with thymoma, but may also be observed with other malignancies. Recognition that orbital lymphoma may coexist with myasthenia gravis will help in expediting the diagnosis of future cases and in guiding treatment decisions.