|Year : 2021 | Volume
| Issue : 3 | Page : 596-598
Bilateral external ophthalmoplegia due to uremia in Goodpasture syndrome: A case report
Niranjan K Pehere1, Aulakh Simranjeet2, Megha S Uppin3
1 Liberia Eye Center (L V Prasad Eye Institute Liberia Inc) John F Kennedy Memorial Medical Center, Monrovia, Liberia
2 Aulakh Simranjeet (MS), Fellow in Pediatric Ophthalmology and Strabismus, Jasti V Ramanamma Children's Eye Care Centre, Kallam Anji Reddy Campus, Hyderabad, India
3 Megha S. Uppin, MD Associate Professor, Department of Pathology, Nizam's Institute of Medical Sciences, Punjagutta, Hyderabad, India
|Date of Submission||22-Aug-2020|
|Date of Acceptance||04-Feb-2021|
|Date of Web Publication||02-Jul-2021|
Dr. Niranjan K Pehere
Head, Liberia Eye Center (L V Prasad Eye Institute Liberia Inc), John F Kennedy Memorial Medical Center, Monrovia - - 1000
Source of Support: None, Conflict of Interest: None
Several ophthalmic manifestations have been reported in patients with uremia due to chronic kidney disease (CKD). These include chronic sore eyes, ischemic optic neuropathy, and retinal detachment. In this case report, we discuss a patient of Goodpasture syndrome undergoing hemodialysis for CKD who presented with a complaint of diplopia. The patient had bilateral near-total external ophthalmoplegia, which initially progressed and remained constant at the last follow-up. The patient was neurologically stable with normal neuroimaging; however, his serum creatinine and blood urea levels remained persistently elevated despite regular hemodialysis. After considering several differential diagnoses, we finally arrived at a diagnosis of bilateral external ophthalmoplegia secondary to uremia and propose potential mechanisms for its pathogenesis.
Keywords: Chronic kidney disease, external ophthalmoplegia, Goodpasture syndrome, ophthalmic manifestations, uremia
|How to cite this article:|
Pehere NK, Simranjeet A, Uppin MS. Bilateral external ophthalmoplegia due to uremia in Goodpasture syndrome: A case report. Indian J Ophthalmol Case Rep 2021;1:596-8
|How to cite this URL:|
Pehere NK, Simranjeet A, Uppin MS. Bilateral external ophthalmoplegia due to uremia in Goodpasture syndrome: A case report. Indian J Ophthalmol Case Rep [serial online] 2021 [cited 2021 Jul 26];1:596-8. Available from: https://www.ijoreports.in/text.asp?2021/1/3/596/320008
The ocular manifestations of chronic kidney disease (CKD) and hemodialysis (HD) include dry eye, band-shaped keratopathy, cataract, raised intraocular pressure (IOP), macular edema, retinal detachment, ischemic optic neuropathy, and uremic optic neuropathy., Locke et al. reviewed neurological complications of uremia in 13 patients and reported nystagmus (in 8 patients), anisocoria (2), ptosis (2), visual field loss (1), and optic disc edema (1). We found only one report by Can et al. that describes ophthalmoplegia as a sequel to uremia. In the present report, we describe a case of acute onset bilateral near-total external ophthalmoplegia in a patient with CKD secondary to Goodpasture syndrome.
| Case Report|| |
A 56-year-old man presented with a one-week history of binocular diplopia. There was no history of diurnal variation. He also described gradual onset impaired vision in both eyes for 3 months. He had previously developed angle-closure glaucoma in his right eye, necessitating laser peripheral iridotomy in both eyes, and was using a combination of timolol 0.5% and brimonidine 0.1% in the right eye. He had a 3-year history of Goodpasture syndrome, proven by high serum levels of anti-glomerular basement membrane IgG antibody and a positive renal biopsy [Figure 1]. He had earlier received immunosuppression therapy. At the time of consultation, he was receiving anti-hypertensive medication and undergoing hemodialysis. His serum creatinine level was 14 mg/dL and blood urea level was 140 mg/dl.
|Figure 1: The renal biopsy showed cellular and fibrous circumferential crescents highlighted by (a) PAS and (b) SM.PAS stains. (c) Direct Immunofluorescence showing linear staining for IgG along the capillary basement membrane|
Click here to view
His best-corrected visual acuities (BCVA) were 20/80 in the right eye (with -1.50 diopter cylinder at 90 degrees) and 20/125 in the left, not improving with any refractive correction. He manifested a 30-prism diopter (PD) of esotropia on Krimsky's test, limited abduction and elevation in both eyes which did not improve with doll's eye movements and Bell's phenomenon was absent. Forced duction test (FDT) and force generation test (FGT) were normal in both eyes. There was posterior subcapsular cataract in both eyes with normal pupillary reactions. Dilated fundus examination revealed a vertical cup: disc ratio of 0.6 in the right eye and 0.3 in the left, and intraocular pressures were normal in both eyes. On examination, there was no variability in the amount of strabismus. He did not have any history of dysthyroidism and there were no signs of thyroid ophthalmopathy. An initial diagnosis of bilateral abducens nerve palsy with upgaze palsy was made. An MRI brain and orbit scan was advised which was normal. One week later the ophthalmoplegia had progressed to affect all positions of gaze [Figure 2]. A provisional diagnosis of bilateral rapid onset progressive external ophthalmoplegia of undetermined etiology was made. To establish a definitive etiological diagnosis, a muscle biopsy was planned, but was deferred owing to the end-stage renal failure and uncontrolled systemic hypertension. Monocular occlusion was advised to avoid diplopia. At six-month follow-up, his ocular alignment and motility remained unchanged, serum creatinine level was 14.05 mg/dL and blood urea level was 105 mg/dL. Later the patient was lost to follow-up due to systemic issues and finally passed away.
|Figure 2: Nine gaze photograph showing esotropia in primary gaze and limitation of ocular movements in all directions of gaze|
Click here to view
| Discussion|| |
Before ascribing ophthalmoplegia to uremia, we considered other differential diagnoses. There was no history of variability in diplopia, no ptosis, no variability of deviation and motility, decreasing the likelihood of myasthenia gravis. Tensilon test was deferred in view of patient's unstable systemic condition. Though clinically myasthenia gravis was less likely, single fiber electromyography (EMG) and testing for anti-acetylcholine receptor antibodies would have further helped to rule it out more firmly. A normal MRI excluded the possibility of brainstem stroke. Adult-onset chronic progressive external ophthalmoplegia (CPEO) was also considered as a differential; however, in view of unstable systemic condition, the muscle biopsy was deferred. There were no signs of any inflammatory pathology affecting the extraocular muscles, either clinically or on MRI. Hence it was deemed that the ophthalmoplegia was likely related to uremia.
Only one similar case of external ophthalmoplegia, with additional ptosis, has been described by Can U et al. in a patient with CKD due to chronic pyelonephritis. Her blood urea nitrogen (BUN) level was 166 mg/dL and serum creatinine level was 19.3 mg/dL. By the third day of peritoneal dialysis, the ophthalmoplegia and ptosis resolved completely, and BUN level diminished to 69 mg/dL. Thus, her ophthalmoplegia was ascribed to uremia and it resolved dramatically with peritoneal dialysis. Our patient did not show complete recovery of his extraocular movements. The patient reported by Can U et al. had an acute presentation of uremia, while our patient was known to have had CKD for 3 years and had been on hemodialysis throughout this time. His levels of serum creatinine and blood urea were almost unaltered from the time of onset of ophthalmoplegia to his last follow-up at 6 months. The effect of persistent uremic toxicity may thus have contributed to the persistent deficits in ocular motility.
There are two reports in literature concerning ophthalmoplegia in uremia in association with Wernicke's encephalopathy (WE). These patients manifested the clinical triad of ophthalmoplegia, ataxia, and confusion., Our patient had only ophthalmoplegia but the classical triad of WE is seen only in 16% of patients and WE should be thought of in any patient on peritoneal or hemodialysis presenting with encephalopathy or unexplained neurological symptoms. Hence this possibility was ruled out in our patient consultation with a Neurologist.
Miller Fisher's syndrome (MFS) characterized by the triad of ataxia, areflexia, and ophthalmoplegia, has also been reported in a case of CKD. Ophthalmoparesis can be an early finding in MFS, as in our patient, and ataxia or areflexia may develop later. But over a follow-up period of 6 months, he did not develop these features. Testing for antibodies to GQ1b gangliosides would have helped to rule out this possibility more definitively, but it was done since the testing facility was not available.
It is known that uremia may cause myopathy as a result of several mechanisms including oxidative stress, vitamin D deficiency, impaired potassium metabolism, and the accumulation of uremic toxins. It is plausible that the extra-ocular muscles can also be involved in this uremic myopathy. High metabolic demand of extraocular muscles may predispose them to the oxidative stress induced by uremic metabolic status to a greater degree than other skeletal muscles. In addition, diplopia giving more bothersome symptoms tends to be noticed more easily. Thus, uremic myopathy may be considered as a contributory cause for the ophthalmoplegia in our case.
| Conclusion|| |
In summary, an uncontrolled uremic status can potentially lead to acute onset bilateral near-complete external ophthalmoplegia. So, one should keep this differential diagnosis in mind while dealing with a patient with CKD who develops ophthalmoplegia.
Ethics committee and Institutional Review Board clearance was obtained for this case report.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published, and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
| References|| |
Evans RD, Rosner M. Ocular abnormalities associated with advanced kidney disease and hemodialysis. Semin Dial 2005;18:252-7.
Mullaem G, Rosner MH. Ocular problems in the patient with end-stage renal disease. Semin Dial 2012;25:403-7.
Locke S, Merrill JP, Tyler HR. Neurologic complications of acute uremia. Arch Intern Med 1961;108:519-30.
Can U, Aydin P, Kansu T. Bilateral external ophthalmoplegia in uremia. Nephron 1994;68:391.
Ihara M, Ito T, Yanagihara C, Nishimura Y. Wernicke's encephalopathy associated with hemodialysis: Report of two cases and review of literature. Clin Neurol Neurosurg 1999;101:118-21.
Arnold R, Issar T, Krishnan AV, Pussell BA. Neurological complications in chronic kidney disease. JRSM Cardiovasc Dis 2016;5:1-13. doi: 10.1177/2048004016677687.
Sechi G, Serra A. Wernicke's encephalopathy: New clinical settings and recent advances in diagnosis and management. Lancet Neurol 2007;6:442-55.
Teener JW. Miller Fisher's syndrome. Semin Neurol 2012;32:512-6.
[Figure 1], [Figure 2]