| OCR Text |
Show J. Clin. Neuro-ophthalmol. 3: 109-110, 1983. Isolated Sixth Nerve Palsy as Initial Manifestation of Systemic Lupus Erythematosus A Case Report LYN A. SEDWICK, M.D. RONALD M. BURDE, M.D. Abstract A 35-year-old woman with an isolated, transient sixth cranial nerve palsy was found to have a positive fluorescent antinuclear antibody test with a pattern and titer most consistent with systemic lupus erythematosus. Mild anemia and leukopenia were also present. Over 2 months she improved without treatment. Nervous system disease in systemic lupus erythematosus and the unusual nature of presentation in this case are discussed. Systemic lupus erythematosus frequently affects the nervous system at some time during the illness. Involvement of the nervous system may occur before or shortly after the diagnosis is made, but such findings rarely occur in isolation. Usually they are associated with other more diagnostic signs such as skin rash or arthritis. We report a case of a transient sixth nerve paresis in an otherwise healthy woman in whom the ultimate diagnosis was systemic lupus erythematosus. Case Reports A 35-year-old, white woman was first seen in the Neuro-ophthalmology Consult Office at Washington University Medical Center in June 1982, with a 4-day history of horizontal diplopia. The patient reported that for 1 year she had experienced rare episodes of diplopia of a similar nature, lasting approximately 2 hours before resolving completely. During her pregnancy, she had a similar episode which was treated with prisms in her glasses until complete resolution of her symptoms after the delivery of her baby. At the time of this examination the patient stated that she was in excellent health and was taking no medications. From the Departments of Ophthalmology (LAS, RMB). Neurology, and Neurological Surgery (RMB). Washington UnIversity School of Medicine, 5t. Louis. MiSSOUri. June 1983 Neuro-ophthalmologic examination revealed a best-corrected visual acuity of 20/20 in each eye. She had normal kinetic visual fields (Goldmann perimeter). All of her abnormalities were limited to the extraocular motility examination. The patient had a right head turn and single binocular vision only in left lateral gaze. There was a 6-prism diopter right esotropia in primary position, increasing to 10-prism diopters in right lateral gaze. A small right hypertropia was present intermittently as well as horizontal nystagmus with a fast phase component in the direction of gaze and occasional upbeating nystagmus. Abduction of the right eye was mildly deficient, but her neurologic examination was otherwise normal. A tensilon test and forced duction testing were normal. The family history revealed that both a maternal uncle and a paternal aunt of this patient had known adult onset diabetes mellitus. In addition, the son of her father's sister developed diabetes in his 20s. The patient was, therefore, carefully studied to exclude diabetes mellitus as having been a factor in the pathogenesis of her sixth nerve palsy. A fasting blood sugar was 92, a random blood sugar on an other occasion was 85, and a 2-hour postprandial blood sugar was 91. A formal oral glucose tolerance test revealed a fasting blood sugar of 85, a 30-minute value of 140: I hour = 130; 2 hours = 98; 3 hours = 84; and 4 hours = 78 mg%. All urine tests done on the half hour .lOd last 3 hours were negative for glucose. Cranial high-resolution computerized tomogrilphy with and without contrast was obtained with special attention being directed to the middle and posterior fossae. This study was entirely normal. Serum tests included hematocrit of 35.8%, white blood cell count of 3,600 with a normal differential. erythrocyte sedimentation rate of 20 mmlhour, VORL and FTA-ABS nonreactive, and a 3+ positive fluorescent antinuclear antibody (ANA) with a homogeneous, rimmed pattern and subsequent titering to 1:640. On further questioning the patient denied a history of skin rash, alopecia, Raynaud's phenom- 109 Isolated VI Nerve Palsy and SLE enon, pericarditis, pleuritis, seizures, or other neurologic or psychiatric problems. Rheumatologic consultation was obtained to evaluate a history of periodic shoulder discomfort and the recent onset of "tennis elbow." Further laboratory studies were negative for rheumatoid factor, extractable nuclear antigen, and DNA antibodies; however, ANA on repeat testing was positive to 1:1280 with homogeneous, rimmed pattern. Liver function studies showed mild elevation of serum aspartate aminotransferase (SCOT) to 56 I.U./liter (normal, 5-35 I.U./liter) but were otherwise normal. Her serum creatinine was 0.8 mg/dl. Over the ensuing 2 months the patient had a spontaneous resolution of her nystagmus. At the time of her last examination in September 1982 she had only right esotropia of several diopters in extreme right gaze. Her epicondylitis had improved on Clinoril®, and no further symptoms were reported. Comment Since Kaposi's original description of systemic lupus erythematosus in the early 1870s, which included two patients with recurrent delirium, I neurologic and psychiatric complications in systemic lupus erythematosus are reported in about 40-50% of cases. 2 - 5 Psychoses and other psychiatric disease account for approximately 25% of these cases, while seizures occur in another 15%.2 Longtract syndromes, cranial nerve palsies, and peripheral neuropathies each occur in abouth 5-15% of patients with systemic lupus erythematosus2 . " Frequently cranial and peripheral neuropathies are concurrent events.2 A myasthenia-like syndrome is also described in association with systemic lupus erythematosus.4 Though both central and peripheral nervous system involvement is common in systemic lupus erythematosus, it is decidedly uncommon to make a diagnosis of systemic lupus erythematosus in the milie~ of an isolated cranial nerve palsy. Feinglass et al.- reviewed 140 cases of systemic lupus erythematosus, of which 52 had systemic lupus erythematosus- related neurologic or psychiatric symptoms. They found that in 33 patients, nervous system disease either preceded the diagnosis of systemic lupus erythematosus or occurred in the first year after the diagnosis was made. However, in those with neuropsychiatric symptoms preceding the diagnosis (two cases of seizures; one each of hemiparesis, chorea, and papilledema) all but one occurred simultaneously with clinical symptoms more diagnostic of systemic lupus erythematosus. We are unaware of any previously reported case of systemic lupus erythematosus presenting as an isolated sixth cranial nerve palsy. 110 In the case presented here, the lesion responsible for the transient paresis is probably a microinfarction in the brain stem, which explains the finding of nystagmus and a possible intermittent skew deviation. However, the possibility of a microinfarction of the peripheral nerve can also be postulated, although not as convincingly. Johnson and Richardson:J report a transient, pupil-sparing, third nerve palsy in a patient with systemic lupus erythematosus who, at autopsy 7 years later, had a totally normal brain stem. Unfortunately the peripheral nerve was not sectioned. Neuropathologic examinations in the other 23 cases of systemic lupus erythematosus patients with neurologic deficits in the report of Johnson and Richardson uniformly demonstrate microinfarcts without vasculitis in the cerebral cortex and brain stem. Systemic lupus erythematosus is a disease that may eventually affect many organ systems. Although most patients demonstrate the classic diagnostic signs of skin rash and arthritis, the case reported here of an isolated sixth cranial nerve palsy suggests that such uncharacteristic presentation of systemic lupus erythematosus is possible. The implication of this finding in terms of the ultimate prognosis of this patient or similar patients remains unknown. References 1. Kaposi, M. (as quoted by R.T. Johnson and E.P. Richardson): The neurological manifestations of systemic lupus erythematosus: A clinical-pathological study of 24 cases and review of the literature. Medicine -17: 337-3b9, 19b8. 2. Feinglass, E.J., Arnett, F.C, Dorsch, CA., Zizic, T.M., and Stevens, M.B.: Neuropsychiatric manifestations of systemic lupus erythematosus: Diagnosis, clinical spectrum, and relationship to other features of the disease. Medicine 55: 323-339, 1976. 3. Johnson, R.T., and Richardson, E.P.: The neurological manifestations of systemic lupus erythematosus: A clinical-pathological study of 24 cases and review of the literature. Medicine 47: 337-369 1968 4. Lessell, S.: Some ophthalmological and 'neuro·logical aspects of systemic lupus erythematosus. ]. Rheumatol. 7: 398-404, 1980. 5. Yancey, CL., Doughty, R.5., and Athreya, B.H.: Central nervous system involvement in childhood systemic lupus erythematosus. Arthritis Rheum. 24: 1389-1395, 1981. Acknowledgment This work was supported in part by a grant from Research to Prevent Blindness, Inc., New York, New York (Department of Ophthalmology). Write for reprints to: Ronald M. Burde, M.D., Department of Ophthalmology, Box 8096, 660 South Euclid Avenue, St. Louis, Missouri 63110. Journal of Clinical Neuro-ophthalmology |
