Journal of Neuro-Ophthalmology, June 1989, Volume 9, Issue 2

Uremic optic neuropathy.

A rare case of documented reversible bilateral optic neuropathy as the presenting manifestation of chronic renal failure is discussed. Repeated hemodialysis in the early stages of the disorder was useful for recovery of vision.

Journal of Clinical Neuro- ophthalmology 9( 2): 131- 133, 1989 Uremic Optic Neuropathy J. S. Saini, M. D., I. S. Jain, F. R. C. S., S. Dhar, M. S., and K. Mohan, M. S. © 1989 Raven Press, Ltd" New York Arare case of documented reversible bilateral optic neu­ropathy as the presenting manifestation of chronic renal failure is discussed. Repeated hemodialysis in the early stages of the disorder was useful for recovery of vision. Key Words: Optic neuropathy- Uremia- Bilateral chronic renal failure. From the Postgraduate Institute of Medical Education and Research, Chandigarh, India. Address correspondence and reprint requests to Dr. ]. S. Saini at Department of Ophthalmology, Postgraduate Institute of Medical Education, Chandigarh- 160012, IndIa. 131 Metabolic disorders known to manifest as optic neuropathy include diabetes mellitus ( 1- 5) and dysthyroidism ( 6,7), Uremia- related optic neurop­athy is a rare entity, and only a few cases have been documented in the literature ( 8,9), although auditory and other cranial nerve involvement is known ( 10). We present a case of documented bi­lateral simultaneously occurring optic neuropathy due to uremia. CASE REPORT KM., an apparently healthy man of 24 years of age presented with sudden diminution of vision in both eyes of 3 days duration. This fall of vision had been painless, rapid, and progressive. He was not aware of any neurological deficit, and he had no past history of tuberculosis or diabetes mellitus, nor had he received any medication known to cause toxic effects on the optic nerves. He was a total abstainer with no addictions. One month prior to his illness, he had pain and swelling over his left foot and received nonsteroidal antiinflam­matory analgesics, multivitamins, and local hepa­rin ointment. He was never diagnosed as having renal disease before the present illness. On clinical examination, the patient was of av­erage build, pale, but otherwise an apparently healthy adult man. He was afebrile, had a blood pressure of 140/ 80 mm Hg, and his pulse was 96 beats/ min and regular. There was no neurological deficit either motor or sensory. Ocular examination revealed a visual acuity of counting fingers at a half meter in the right eye and counting fingers close to the face in the left eye. Intraocular pressure was normal in both eyes, and ocular movements were full. Pupillary light re­flexes were ill sustained in both eyes. Ophthalmos­copy of both eyes revealed only mild disc hypere­mia. He was investigated for etiology, and high doses of systemic steroids were administered. 132 J. S. SAINI ET AL. INVESTIGATIONS Hemoglobin was 8.2 g%; Westergren sedimen­tation rate was 38 mm/ h. The peripheral blood film revealed a normocytic, normochromic anemia with mild anisocytosis and adequate platelets. The total leukocyte count was 2,200/ mm3 ; the differential leukocytic count was as follows: polymorphs 75%, lymphocytes 20%, and monocytes 5%. Micro­scopic examination of urine revealed 1- 2 leuko­cytes/ high power field. Urine culture was sterile. The 24- h urinary protein was 120 mg% and 24- h urinary creatinine was 62 mg%. Blood urea nitro­gen was 140 mg%, serum creatinine 19 mg%, fast­ing blood sugar 142 mg%, serum uric acid 10 mg%, serum sodium 135 mg%, potassium 3.9 mg%, chloride 10.5 mg%, bicarbonate 22 mg%, inorganic phosphate 11.4 mg%, alkaline phosphate 13 King­Armstrong U, and serum calcium 7.7 mg%. Anti­streptolysin titer was < 100 IU. VORL was nonre­active. Serum protein was 7.2 g%, serum albumin 3.2 g%, and albumin- to- globulin ratio ( A/ G) 1: 3. Serum cryoglobulins were negative; and anti­nuclear factor was negative. Complement C 3 levels were 55 mg%. Mantoux test was negative. Coag­ulogram was normal. Liver function tests were normal. Roentgenograms of chest and head were normal, and computed tomography of head re­vealed no abnormality. Visual evoked responses revealed initially no wave pattern formation. After 3 weeks when vi­sual acuity had improved, visual fields were done that showed bilateral central scotomas in both eyes and a parecentral scotoma on left nasal field with bilateral constriction of the peripheral fields. Color vision tested on Ishihara charts demon­strated color deficiency. Fluorescein angiography did not reveal any leak in the early stages. CLINICAL COURSE Contrast studies detected a nonfunctional left kidney, and a hydronephrosis with hydroureter and a positive vesicourinary reflux on the right side. The patient received prednisolone 80 mg/ day for 3 days, but showed no improvement. The dose of steroids was then enhanced to 100 mg/ day for the next 5 days but without any improvement. Ad­renocorticotropic hormone ( ACTH) gel 20 U i. m. every 6 h was administered for the next 10 days. There was no improvement in vision, and the ACTH was discontinued. Six hours after receiving his first hemodialysis fnr chronic renal failure on the 19th day of illness, [- J", hI, ,,, I ;-,-':) n.~ '~"' · .-, h'linc were lowered from / elill Neuro- ophthalmol, Vol. 9, No. 2, 1989 128 and 12 to 80 and 5 mg%, respectively. The visual acuity recorded at the same time improved to 20/ 120 in the right eye and counting fingers at 1 m in the left eye. Visual acuity further improved to 20/ 40 in the right eye and 20/ 200 in the left eye over the next 10 days. After the second hemodialysis on the 29th day of illness, visual acuity improved to 20/ 40 in the right eye and 20/ 200 in the left eye. Following the third hemodialysis, the patient's vi­sion improved to 20/ 40 in both eyes. A repeat vi­sual evoked response revealed in the right eye a latency of 202 ms and amplitude of 4- 5 j. LV, and in the left eye a latency of 146 ms and amplitude of 3- 4 j. LV. Visual fields were repeated and showed marked improvement in both eyes. A renal trans­plant surgery was then performed on the patient. Following the transplant, the patient has done well. DISCUSSION Among the causes of metabolic optic neuropa­thy, renal diseases are rare. The only case report in literature known to us was reported by Nikoskel­ainen ( 8) in 1975. In her large series of 105 cases of optic neuritis, she described nephritis with ure­mia- related polyneuropathy in only one case. Op­tic neuritis due to glomerulonephritis with or with­out hypertension, and chronic pyelonephritis have also been documented ( 10,11). Neurological in­volvement due to uremia is known. Cranial nerves other than optic nerve are more frequently affected in uremic patients ( 12). Transient nystagmus, mi­osis, and heterophoria are occasionally observed ( 12). Bilateral optic neuritis is more frequently known to be a manifestation of demyelinating disorders and metabolic neuropathies ( 13- 19). All investiga­tions on our patient except renal function tests were within normal range. In spite of high doses of systemic steroids and later ACTH, patient showed no improvement, which is most unusual for optic neuritis due to demyelinating disorders. The only remarkable improvement in vision was seen after the first hemodialysis. Following this, the vision improved steadily and showed rapid im­provement after every hemodialysis. Other visual parameters like color vision, visual fields, and vi­sual evoked response also showed improvement. In this patient, optic neuropathy was evidently re­lated to some metabolic end product of uremia. Elimination of the metabolic toxic product from the body by hemodialysis resulted in improvement in optic nerve conductivity. To the best of our knowledge, this is the first documented case where optic neuropathy caused UREMIC OPTIC NEUROPATHY 133 by uremia has improved with dialysis. [ The report of Knox et aI. ( 9) was added to this article after submission- Editor. See Editorial in this issue.] Nerve conduction defects are demonstrable in up to 60-- 65% of patients with uremia on nerve conduction studies. The uremic neuropathy is be­lieved to be due to dialysable toxic metabolites ( 20­23). Many of the uremia- related toxins are incrim­inated and include guanidino compounds, raised parathyroid hormones, myo- inositol and middle molecules of 300- 2, OOO- dalton range. These com­pounds inhibit activity of a vitamin- B- dependent enzyme, transketolase, formed in the nerves, con­sequently leading to manifest neuropathy ( 24). Cranial nerves have been known to be involved in uremic polyneuropathy, although this is rare. Among them, auditory nerve involvement is well documented ( 18,24). In our patient, the nonresponsiveness to sys­temic steroids and the quick improvement in vi­sion following hemodialysis indicates the uremic origin of the optic neuropathy. We suggest that all patients with manifest bilateral optic neuropathy not responding to steroids should be investigated for renal disorders because this can be the first presentation of renal dysfunction, as happened in our patient. REFERENCES 1. Appen RE, et al. Diabetic papillopathy. Am J Ophtha/ mo/ 1980; 90: 203- 9. 2. Lubow M, Makely TA. Pseudopapilledema of juvenile di­abetes mellitus. Arch Ophthalmol 1971; 85: 417- 22. 3. Sykowski P. Diabetic retrobulbar neuritis. Am J Ophthalmol 1949; 32: 1589- 90. 4. Topilow A, Bisland T. Diabetes mellitus as a cause of papil­litis. Am J Ophthalmol 1952; 85: 855-- 8. 5. Foulds WS. Visual disturbances in systemic disorders. Op-tic neuropathy and systemic disease. Trans Ophthalmol Soc UK 1970; 89: 125- 46. 6. Freud M, Carmon A, Cohen AM. Papilledema and papilli­tis in diabetics-- a report of two cases. Am J Ophthalmol 1965; 60: 18-- 22. 7. Day RM, Carroll FD. Optic nerve involvement associated with thyroid dysfunction. Arch OphthalmoI1962; 67: 289- 97. 8. Nikoskelainen E. Symptoms, signs and early course of op­tic neuritis. Acta Ophthalmol 1975; 53: 254- 71. 9. Knox, DL et al. Uremic optic neuropathy. Arch Ophthalmol 1988; 106: 50- 4. 10. Roy FH. Ocular differential diagnosis in optic nerve. 3rd ed. Philadelphia: K. M. Varghese Company. 1984: 391. 11. Goldzieher MA, McGarack TH, Peterson CA, Goldzieher JW, Miller HR. Retrobulbar neuritis associated with hyper­thyroidism. AMA Arch Neural Psychiat 1951; 65: 189- 96. 12. Arieff AI. Neurological manifestations of uremia. In: Bren­ner BM, Rector Jr FC eds. The kidney. 3rd ed. Philadelphia: WB Saunders Co., 1986: 1731- 56. 13. Carroll FD. Optic neuritis-- a 15 year study. Am J Ophthal­mol 1952; 35: 75-- 82. 14. Jain IS, Dhirs P, Munjal VP. A profile of optic neuritis in Chandigarh and surrounding areas. Indian J Ophthalmol 1981; 28: 18-- 22. 15. Behrman S. Optic neuritis, papillitis and neuronal retinop­athy. Br J OphthalmoI1964; 48: 209- 17. 16. Lynn BH. Retrobulbar neuritis: a survey of the present con­dition of cases occurring over the last fifty- six years. Trans Ophthalmol Soc UK 1959; 79: 701- 16. 17. Nikoskelainen E. Late course and prognosis of optic neu­ritis. Acta OphthalmoI1975; 53: 273- 9I. 18. Miller NR. Walsh and Hoyt's clinical neuro- ophthalmology, vol. 1. 4th ed. Baltimore: Williams and Wilkins, 1983: 227- 48. 19. Henson RA, Urich H. Metabolic neuropathies. In: Vinken PJ, Bruyn GW, eds. Handbook of clinical neurology, vol. 8, part II. Amsterdam: North Holland Publishing Company, 1970. 20. Asbury AK. Uremic neuropathy. In Dyck PJ, Thomas PK, Lambert EH, Bunge, R, eds. Peripheral neuropathy, vol. II. 2nd ed. Philadelphia: WB Saunders Co., 1984: 1811. 21. Forno L, Alston W. Uremic polyneuropathy. Acta Neural Scand 1967; 43: 640- 54. 22. Asbury AK, Victor M, Adams RD. Uremic polyneuropathy. Arch Neural 1963; 8: 413- 28. 23. Tenckhoff HA, Boen FST, Jebsen RH, Spiegler JH. Polyneu­ropathy in chronic renal insufficiency. JAMA 1965; 192: 1121- 4. 24. Parson V. Renal and eye disease. Trans Ophtha/ mol Soc UK 1970; 89: 156-- 66. r Clin Neuro- ophthalmol, Vol. 9, No. 2, 1989