Journal of Neuro-Ophthalmology, March 2012, Volume 32, Issue 1

Letters to the Editor

Monocular Elevation Deficiency ("Double Elevator" Palsy): A Cautionary Note (Reprinted from J Neuro-Opthalmol. 2011:31;291-292) I read with interest the article by Brodsky and Karlsson (1) and the authors' caution that tethering and buckling of the central lower eyelid in down gaze of patients with mon-ocular elevation deficiency can simulate impaired infraduc-tion in the involved eye. However, I wish to express a different opinion on the description of the figure shown by the authors. Indeed, contrary to the description submitted, figure 1C does in fact demonstrate a deficit of infraduction of the abnormal right eye. Two clear landmarks can be used to come to that conclusion: first, the relative alignment of the upper limbus of each eye that clearly shows a lack of adequate depression movement of the affected eye. Second, the rounded pupil image of the right eye, as opposed to its oval-shaped counterpart (0.5 mm difference in vertical diameter), is in keeping with the difference in downward gaze position of the 2 eyes. Unfortunately, in this case, the pictures do not show which eye is fixating in down gaze due to the lack of corneal light reflection. Furthermore, there is no photographic documentation of ductions. A review of some of the key publications on double elevator palsy shows a dramatic similarity with the documen-tation by Brodsky and Karlsson. For example, 4 of 5 cases with adequate pictorial documentation show a hyperdeviation in down gaze of the abnormal eye in textbooks by both Rosenbaum and Santiago (2) and von Noorden and Campos (3). Surprisingly, this deviation in down gaze is poorly dis-cussed throughout the literature, most of the attention being directed at the classical findings of good alignment in primary position, a deficit of elevation in adduction and abduction, and the presence in some patients of a Bell phenomenon. Finally, this deficit in depression of the abnormal eye referred to here and illustrated by the authors could be an indicator of a miswiring of either the superior rectus or the inferior rectus, another example of the ever-growing spectrum of congenital primary extraocular cranial neuropathies. G. Robert La Roche, MD, FRCSC Division of Pediatric Ophthalmology and Ocular Motility Department of Ophthalmology and Visual Sciences Dalhousie University, Halifax, Nova Scotia, Canada The author reports no conflicts of interest. REFERENCES 1. Brodsky MC, Karlsson V. Monocular elevation deficiency ("double elevator" palsy): a cautionary note. J Neuroophthalmol. 2011;31:56-57. 2. Rosenbaum A, Santiago A. Clinical strabismus management. Philadelphia, PA: Saunders, 1999:273. 3. von Noorden G, Campos E. Binocular vision and ocular motility. 2002442-443. Reply-Monocular Elevation Deficiency: A Cautionary Note I thank Dr LaRoche for his correspondence (1) about our article (2). His cited pictures of patients with monocular elevation deficiency do indeed show diminished excursion of the involved eye in downgaze. This finding can be confirmed by extending a clear transparent ruler from one lateral can-thus to the other to compare the amount of visible sclera superior to each upper limbus. These photographs depict versions, so it cannot be assumed that any of these patients have the "deficit of infraduction" that he posits. Nevertheless, Dr LaRoche has made an astute observation that has man-aged to elude the strabismus community. Our patient differed from these cases, in that most of his apparent vertical misalignment in downgaze was due to an exaggerated lower lid retraction in downgaze, which also produced an apparent infraduction deficit. His true hyperde-viation in extreme downgaze was clinically insignificant, as demonstrated by his normal vertical alignment in physiologic downgaze. As congenital deficiency of motor innervation leads to the paradoxical combination of extraocular muscle hypoplasia and fibrosis, I agree with Dr LaRoche that con-genital cranial dysinnervation provides the most plausible explanation for this finding (3). Michael C. Brodsky, MD Departments of Ophthalmology and Neurology, Mayo Clinic, Rochester, Minnesota [e-mail] The author reports no conflicts of interest. REFERENCES 1. LaRoche GR. Monocular elevation deficiency ("double elevator" palsy): a cautionary note (letter to the editor). J Neuro- Ophthalmol. 2011;31:291-292. 2. Brodsky MC, Karlsson V. Monocular elevation deficiency ("double elevator" palsy): a cautionary note. J Neuro- Ophthalmol. 2011;31:56-57. 3. Brodsky MC. Hereditary external ophthalmoplegia, synergistic divergence, jaw winking, and oculocutaneous hypopigmentation: a congenital fibrosis syndrome caused by deficient innervation to extraocular muscles. Ophthalmology. 1998;105:717-725. 92 Letter to the Editor: J Neuro-Ophthalmol 2012; 32: 92-96 Letters to the Editor Copyright © North American Neuro-Ophthalmology Society. Unauthorized reproduction of this article is prohibited. Central Retinal Artery Occlusion Secondary to Orbital Inflammation in Lupus Erythematosus Profundus In a recent issue of this journal, Kao et al (1) reported a patient with lupus erythematosus profundus (LEP) who presented with a violaceous periocular discoloration of the right upper and lower eyelids. Precontrast orbital T1 MRI showed diffuse loss of fat signal, and postcontrast fat-suppressed T1 MRI showed enhancement of the orbital fat and extraocular muscles. After treatment with oral pred-nisone, the patient developed dramatic orbital soft tissue lipoatrophy and enophthalmos. We recently examined a patient who displayed another neuro-ophthalmic manifestation of LEP: central retinal artery occlusion (CRAO). MRI showed intraconal fat and intraorbital optic nerve sheath inflammation on the affected side, which may have been contributory. A 51-year-old African American woman with history of biopsy-proven LEP for the past 20 years presented with acute loss of vision in the left eye. A punch biopsy of a left arm lesion 2 years earlier had shown hyperkeratosis, basement membrane thickening with basilar vasculopathy, perivascular inflammation in the dermis, sclerosing septal and hyalinizing lobular panniculitis with deep nodular lymphoid aggregates, and rare lymphoid follicles consistent with a diagnosis of LEP (Fig. 1). There was disfiguring hyperpigmentation and surface irregularity of her nose and fingertips with depressed lipoatrophic areas in the left midface and left arm. Visual acuity was 20/20 in the right eye and counting fingers in the left eye, with a left relative afferent pupillary defect. Extraocular motility was full in both eyes, and the eyes appeared to be aligned. There was no proptosis or resistance to globe retropulsion. Anterior segment examination and intraocular pressures were normal bilaterally. Fundus exami-nation was normal in the right eye but showed cloudy retinal swelling and a cherry red spot in the left macula consistent with CRAO. Neurological examination was otherwise normal. FIG. 1. Skin biopsy demonstrates hyperkeratosis, dermal perivascular inflammation, septal panniculitis (white arrows) and lobular panniculitis with lobular hyaline fat necrosis (black arrow), and lymphoid nodules in the subcutis. These abnormalities are consistent with LEP (hematoxylin & eosin, ·20). FIG. 2. Postcontrast T1 axial (A) and coronal (B) MRI show nodular enhancement of left orbital fat, perioptic dura, and lacrimal gland. The extraocular muscles also are enlarged in the left orbit. Letter to the Editor: J Neuro-Ophthalmol 2012; 32: 92-96 93 Letters to the Editor Copyright © North American Neuro-Ophthalmology Society. Unauthorized reproduction of this article is prohibited. Postcontrast fat-suppressed axial and coronal MRI showed abnormal enhancement of the preseptal and intraconal soft tissue and intraorbital optic nerve sheath and enlargement of all extraocular muscles in the left orbit (Fig. 2). Despite a course of methylprednisone, ophthalmologic examination and MRI findings remained unchanged 2 months later. She was slowly weaned off the corticosteroid. We presume that the orbital inflammation in our patient led to the CRAO. Such a phenomenon has often been reported in other orbital inflammatory conditions, such as optic perineuritis (2) and idiopathic orbital inflammation (3). It has been reported only once in LEP in a patient who developed ischemic optic neuropathy and CRAO which led, months later, to melting of the entire orbital contents, including the eye (4)! A unique aspect of our case is the inclusion of orbital inflammatory findings on MRI. This inflammatory response presumably led to CRAO and underscores the vision-threatening nature of LEP. Padmaja Sudhakar, MD Department of Ophthalmology and Visual Sciences Gaurang V. Shah, MD Department of Radiology (Neuroradiology) Fiorella Saponara, MD Department of Ophthalmology and Visual Sciences Douglas R. Fullen, MD Departments of Pathology and Dermatology Jonathan D. Trobe, MD Department of Ophthalmology and Visual Sciences W. K. Kellogg Eye Center University of Michigan, Ann Arbor [e-mail] The authors report no conflicts of interest. REFERENCES 1. Kao TY, Yoon MK, McCulley TJ, Ruben BS, Hwang TN. Acquired enophthalmos in lupus erythematosus profundus. J Neuroophthalmol. 2010;30:64-66. 2. Spierer O, Ben Sira L, Leibovitch I, Kesler A. MRI demonstrates restricted diffusion in distal optic nerve in atypical optic neuritis. J Neuroophthalmol. 2010;30:31-33. 3. Foroozan R. Combined central retinal artery and vein occlusion from orbital inflammatory pseudotumour. Clin Experiment Ophthalmol. 2004;32:435-437. 4. Arthurs BP, Khalil MK, Chagnon F, Lindley SK, Anderson DP, Burnier M Jr. Orbital infarction and melting in a patient with systemic lupus erythematosus. Ophthalmology. 1999;106:2387-2390. Central Retinal Artery Occlusion and Recurrent Papillitis in a Patient With Incomplete Behçet Disease: A Comment I read with great interest the article by Tian et al (1): "Central retinal artery occlusion and recurrent papillitis in a patient with incomplete Behçet disease." However, I wish to express a different opinion regarding the diagnosis suggested by the authors. In Figure 2A, there is minimal filling of the choroid and no filling of the optic disc vessels supplied by the choroid. A diagnosis of central retinal artery occlusion (CRAO) cannot be well established because CRAO should not affect the choroidal circulation. Radial vascular filling on the surface of the optic disc is seen, which represents some blood supply from the central retinal artery. A single photograph of the fluorescein angiogram does not offer enough evidence to support the diagnosis of CRAO. Using published diagnostic criteria (2), the authors diagno-sed their patient with incomplete Behçet disease (BD) based on the finding of CRAO, recurrent papillitis, and recurrent oral ulcers. But one has to have at least 3 attacks or oral ulcers in 1 year in order for the recurrent oral ulcers to be a major criterion. In addition, CRAO and recurrent papillitis are not actually minor features. I suggest that a diagnosis of suspected BD may be more appropriate for this case. Ying Liu, MD, PhD Aier Eye Hospital of Changsha Changsha, China [e-mail] The author reports no conflicts of interest. REFERENCES 1. Tian G, Lu N, Yan R, Zhang X. Central retinal artery occlusion and recurrent papillitis in a patient with incomplete Behcet disease. J Neuroophthalmol. 2011;31:244-247. 2. Suzuki KM, Suzuki N. Behcet's disease. Clin Exp Med. 2004;4:10-20. Central Retinal Artery Occlusion and Recurrent Papillitis in a Patient With Incomplete Behçet Disease: A Response We thank Dr Liu for the interest in our article and will respond to the questions raised (1). First, Dr Liu pointed out that a single photograph of the fluorescein angiogram does not offer enough evidence for a diagnosis of central retinal artery occlusion (CRAO). We have already extensively discussed this with the reviewers and editor of 94 Letter to the Editor: J Neuro-Ophthalmol 2012; 32: 92-96 Letters to the Editor Copyright © North American Neuro-Ophthalmology Society. Unauthorized reproduction of this article is prohibited. the journal before our article was accepted. When we did this, we carefully reviewed the fluorescein angiogram and found in the early phase of the study that there was good choroidal filling (Fig. 1). In the Photo Essay, Figure 2A shows a later stage of the angiogram when filling of the choroid was complete, yet there was no perfusion of the retinal vessels. Furthermore, the appearance of the vascular occlusion in Figure 2A is actually more suggestive of an occlusive vasculitis than a CRAO caused by emboli, adding further support for the diagnosis of Behçet disease (BD). Second, regarding the issue of diagnostic criteria for BD, our patient admitted to recurrent oral ulcers more than 5 times per year. Finally, we agree that CRAO and recurrent papillitis are not listed as minor features in the diagnostic criteria of BD (2), but "vasculitis" and "central nervous system symptoms" are included. If we accept that the vas-cular occlusion observed in each eye of our patient was caused by vasculitis and that the optic nerve is part of the central nervous system, then we have 2 minor features of BD. While recurrent oral ulcers are a major feature, it is noteworthy that diagnostic criteria for BD vary and have been revised many times. The International Study Group for BD proposed that the diagnosis be considered when recurrent oral ulcers plus 2 other features are present, in the absence of other clinical explanations (3). In our patient, we performed a wide range of ancillary tests to exclude other disorders, which can cause an obliterative vasculitis. Most importantly, we wish to remind clinicians that CRAO and recurrent papillitis can be caused by a vasculitis, such as BD. Prompt treatment can be helpful to prevent further loss of visual function. Xiaojun Zhang, MD, PhD Guohong Tian, MD, PhD Department of Neurology Beijing Tongren Hospital Capital Medical University Beijing, China [e-mail] The authors report no conflicts of interest. REFERENCES 1. Liu Y. Central retinal artery occlusion and recurrent papillitis in a patient with incomplete Behçet disease: a comment. J Neuroophthalmol. Epub 2012. 2. Suzuki KM, Suzuki N. Behçet's disease. Clin Exp Med. 2004;4:10-20. 3. International Study Group for Behcet's Disease. Criteria for diagnosis of Behcet's disease. Lancet. 1990;335:1078-1080. Leber Hereditary Optic Neuropathy Mimicking Thyroid-Related Optic Neuropathy We read with great interest the article "Leber Hered-itary Optic Neuropathy Mimicking Neuromyelitis Optica" by McClellend et al (1). We recently evaluated a similar patient with Leber hereditary optic neuropathy whose symptoms were initially suggestive of dysthyroid optic neuropathy and whose older age and optic nerve enhancement on MRI resulted in extensive testing for neu-romyelitis optica (NMO) and other etiologies that delayed diagnosis. A 67-year-old Caucasian man presented in with a 3- month history of painless progressive vision loss. His medical history was significant for hypertension, diabetes mellitus, kidney failure, and Graves disease. The patient had undergone radioactive iodine thyroid ablation 15 years previously. He had bilateral hand numbness. MRI of the cervical spine in October 2010 showed severe dege-nerative disease at the levels of C5-C7 with neuroforaminal stenosis, central canal stenosis, and compressive myelopathy. MRI of the brain revealed few nonspecific periventricular white matter changes consistent with small vessel ischemic disease. When the patient was referred for neuro-ophthalmic examination, visual acuity was 20/400 bilaterally, without a relative afferent pupillary defect. Ocular motility was normal. External examination showed superior and inferior scleral show and bilateral upper lid retraction consistent with thyroid eye disease; on slit-lamp biomicroscopy, there were mild, bilateral nuclear sclerotic cataracts with normal FIG. 1. Early phase of fluorescein angiogram demonstrates choroidal filling. Letter to the Editor: J Neuro-Ophthalmol 2012; 32: 92-96 95 Letters to the Editor Copyright © North American Neuro-Ophthalmology Society. Unauthorized reproduction of this article is prohibited. intraocular pressure in each eye. Funduscopic examination showed normal optic nerves and mild nonproliferative diabetic retinopathy with scattered microaneurysms bilater-ally. Automated (24-2) visual fields showed central defects in each eye. Erythrocyte sedimentation rate and C-reactive protein levels were normal. Orbital computed tomography showed prominent intraconal and extraconal fat with proptosis and optic nerve straightening bilaterally (Fig. 1). A diagnosis of bilateral optic neuropathy secondary to thyroid eye disease was made, and the patient elected to undergo bilateral orbital decompression surgery. Over the ensuing 6 months, the patient continued to experience vision loss to no light perception, right eye, and bare light perception, left eye. The pupils were nonreactive to light, and both optic discs became pale. The patient was admitted to hospital and started on empiric steroid therapy. Repeat MRI of the brain and orbits was unremark-able, except for minimal right optic nerve enhancement. Cerebrospinal fluid analysis was normal as were paraneo-plastic and NMO serologies. Testing for LHON showed a 14484 mtDNA mutation. McClellen et al (1) presented a case of LHON with multiple sclerosis-like symptoms masquerading as NMO. The patient presented with vision loss, contiguous myelop-athy in more than 3 spinal cord segments, and was NMO-IgG seronegative. Interestingly, both the cases presented by McClellen et al and another published report (2) of LHON with an NMO-like presentation bear many similarities to our patient. All 3 cases harbored the 14484 LHON muta-tion, had evidence of diffuse cervical spine myelopathy, presented at an age later than that typically associated with LHON (range, 39-65 years), and had subacute, slowly pro-gressive bilateral visual loss, which is atypical for LHON. In both our patient and the one reported by McClellen et al, there was a notable absence of circumpapillary telangiectatic microangiopathy classically associated with LHON. Our case was additionally complicated by the presence of thyroid eye disease that led to the mistaken diagnosis of dysthyroid optic neuropathy due to stretching of the nerves. Kobayashi et al (3) documented a case of LHON in a woman with hyperthyroidism and speculated that thyroid disease may act as a potential trigger for LHON given the role of thyroid hormone in mitochondrial biogenesis. The spectrum of LHON is variable, and the caveat remains that clinicians must consider this diagnosis regardless of older age, female gender, slowly progressive vision loss, and ini-tially normal-appearing optic nerves with minimal enhance-ment on MRI. Nafiseh Hashemi, MD Sushma S. Yalamanchili, MD Department of Ophthalmology The Methodist Hospital, Houston, Texas Jason Zhang, BA Department of Ophthalmology The Methodist Hospital, Houston, Texas Baylor College of Medicine, Houston, Texas Andrew G. Lee, MD Department of Ophthalmology The Methodist Hospital, Houston, Texas Baylor College of Medicine, Houston, Texas Departments of Ophthalmology, Neurology, and Neurosurgery Weill Cornell Medical College, New York, New York Department of Ophthalmology The University of Texas Medical Branch, Galveston, Texas Department of Ophthalmology The University of Iowa Hospitals and Clinics, Iowa City, Iowa The authors report no conflicts of interest. REFERENCES 1. McClelland CM, Van Stavern GP, Tselis AC Leber hereditary optic neuropathy mimicking neuromyelitis optica. J Neuroophthalmol. 2011;21:265-268. 2. Jaros E, Mahad DJ, Hudson G, Birchall D, Sawcer SJ, Griffiths PG, Sunter J, Perry RH, Chinnery PF Primary spinal cord neurodegeneration in Leber hereditary optic neuropathy. Neurology. 2007;69:214-216. 3. Kobayashi Y, Endo Y, Ito N, Iijima Y, Mizuki N A case of Leber's hereditary optic neuropathy in a female patient with the recrudescence of hyperthyroidism [in Japanese]. Nihon Ganka Gakkai Zasshi. 2007;111:905-910. FIG. 1. Noncontrast CT of the orbits showing prominent intraconal and extraconal fat with proptosis and optic nerve straightening. 96 Letter to the Editor: J Neuro-Ophthalmol 2012; 32: 92-96 Letters to the Editor Copyright © North American Neuro-Ophthalmology Society. Unauthorized reproduction of this article is prohibited.