| OCR Text |
Show Journal of Neuro- Ophthalmology 16( 4): 274- 276, 1996. 1996 Lippincott- Raven Publishers, Philadelphia The One- and- a- Half Syndrome in Systemic Lupus Erythematosus Ay tag Yigit, M. D., Ay § e Bingol, M. D., Nermin Mutluer, M. D., and Nida Ta § gilar, M. D. We report a case of one- and- a- half syndrome occurring as the first manifestation of central nervous system ( CNS) involvement in systemic lupus erythematosus ( SLE). The lesion in the pons was documented with magnetic resonance imaging ( MRI). The patient responded quite satisfactorily to high- dose i. v. methyl-prednisolone therapy. Key Words: Systemic lupus erythematosus- One- and-a- half syndrome- Central nervous system involvement- Magnetic resonance imaging. The " one- and- a- half" syndrome is characterized by the combination of a lateral gaze palsy in one direction with an internuclear ophthalmoplegia ( INO) in the other direction. It is usually due to a unilateral lesion in the dorsal pontine tegmentum, involving the ipsilateral paramedian pontine reticular formation, the abducens nucleus, and the internuclear fibers of the medial longitudinal fasciculus. Brainstem infarcts and multiple sclerosis are the most common causes ( 1). We report a patient with systemic lupus erythematosus ( SLE) who developed a one- and- a- half syndrome as the first manifestation of the central nervous system ( CNS) involvement. Manuscript received November 13, 1995; accepted November 30, 1995. From the Department of Neurology, Faculty of Medicine, University of Ankara, Ankara, Turkey Address correspondence and reprint requests to Dr. Aytac Yigit, Akdeniz caddesi 11/ 4, 06580 Yiicetepe/ Ankara, Turkey. CASE REPORT A 68- year- old woman with a long- standing history of mild SLE presented with acute- onset diplopia and dysarthria, accompanying the activation of discoid rash and photosensitivity. Neurologic examination revealed a slight right hemiparesis and abnormalities of extraocular movements. In forward gaze, both eyes were in the midline, with no exotropia. On attempted leftward gaze, neither eye moved laterally to pass the midline. On rightward gaze, the left eye did not adduct, but the right eye abducted fully with a lateral nystagmus. Convergence and downward gaze were intact. An upbeat nystagmus occurred on upward gaze. The right hemiparesis resolved spontaneously in 1 day. Laboratory investigations showed normal values for complete blood counts, erythrocyte sedimentation rate, blood glucose, urea and creatinine, and urinalysis. Serum complement C3c and C4 levels and anticardiolipin antibodies were also within normal limits. Antinuclear antibodies ( ANA) were positive (+ + + +), and anti- double stranded 274 ONE- AND- A- HALF SYNDROME IN SLE 275 FIG. 1. Transverse T2- weighted MRIs showing hyperintense lesion in the pons, extending from the midportion to the left and lower parts. Left: pretreatment, with dimensions of 2 x 1.5 x 0.5 mm. Right: following treatment with high- dose i. v. methylprednisolone, with dimensions of 1.3 x 0.75 x 0.4 mm. DNA ( anti- dsDNA) was 58.0 IU/ ml ( normal, < 7.0 dysfunction ( Fig. 2 top). Consequently, we admin- IU/ ml). Cerebrospinal fluid analysis was normal. istered intravenous methylprednisolone 1 g/ day Cranial T2- weighted magnetic resonance imag- for the following 3 days. The one- and- a- half syn-ing ( MRI) revealed a hyperintense lesion in the drome resolved into an incomplete left INO ( Fig. 2 pons ( Fig. 1 left). We treated the patient with bottom). A repeat cranial MRI showed that the methylprednisolone 1 mg/ kg/ day for 10 days and pontine lesion was reduced ( Fig. 1 right). The oral observed only a slight improvement of extraocular methylprednisolone at 1 mg/ kg/ day was continued FIG. 2. Extraocular movements. Top: one- and- a- half syndrome on day 10 of oral prednisolone. Left, middle, and right photographs are rightward, forward, and leftward gazes, respectively. Note that both eyes pass the midline to the leftonly slightly on leftward gaze. Bottom: incomplete left internuclear ophthalmoplegia following treatment with high- dose i. v. methylprednisolone. Left, middle, and right photographs are rightward, forward, and leftward gazes, respectively. / Neuro- Ophthalmol, Vol. 16, No. 4, 1996 276 A. YIGIT ET AL. for 1 week, without recurrence of the neuro-ophthalmological signs. The medication was then changed to an alternate- day regimen, dosage tapering in 10 mg decrements every 3 days, and discontinued at the end of 1 month without reactivation of the neuro- ophthalmological signs. DISCUSSION Our patient presented with a one- and- a- half syndrome characterized by lateral gaze palsy on leftward gaze and INO on rightward gaze. She also fulfilled the American Rheumatism Association criteria for classifying SLE patients ( 2): positive ANA, anti- dsDNA, discoid rash, and photosensitivity. Neuro- ophthalmological manifestations as INO are rarely associated with SLE. Cogen et al. ( 3) reviewed six patients with documented unilateral INO in the literature, and described a patient with bilateral INO ( 3). Another patient with bilateral INO was reported by Jackson et al. ( 4). To our knowledge, our patient is the first reported case of one- and- a- half syndrome in association with SLE. Brainstem lesions are infrequently documented in SLE patients ( 5). In our patient, the T2- weighted MRI showed a hyperintense lesion in the pons that partially resolved following high- dose i. v. steroid therapy. The reduction of one- and- a- half syndrome into incomplete left INO may reflect the resolution of edema surrounding focal ischemia or inflammation ( 5,6). In our patient, we obtained the most beneficial effect with high- dose i. v., rather than oral, meth-ylprednisolone administration. This result disagrees with that of Bell et al. ( 6), who reported that focal CNS manifestations of SLE do not respond to steroid therapy. Although, in some series, brainstem infarcts are associated with high mortality rate ( 7), patients with only extraocular dysfunction occurring late during mild SLE, may have a more favorable outcome ( 3,4). REFERENCES 1. Wall M, Wray SH. The one- and- a- half syndrome. A unilateral disorder of the pontine tegmentum: A study of 20 cases and review of the literature. Neurology 1983; 33: 971- 80. 2. Tan EM, Cohen AS, Fries JF. The 1982 revised criteria for the classification of systemic lupus erythematosus. Arthritis Rheum 1982; 25: 1271- 7. 3. Cogen MS, Kline LB, Duvall ER. Bilateral internuclear ophthalmoplegia in systemic lupus erythematosus. / Clin Neuro- Ophthalmol 1987; 7: 69- 73. 4. Jackson G, Miller M, Littlejohn G, Helme R, King R. Bilateral internuclear ophthalmoplegia in systemic lupus erythematosus. / Rheumatol 1986; 13: 1161- 2. 5. Stimmler MM, Coletti PM, Quismorio FP. Magnetic resonance imaging of the brain in neuropsychiatric lupus erythematosus. Sem Arthritis Rheum 1993; 22: 335- 49. 6. Bell CL, Partington C, Robbins M, Graziano F, Turshi P, Kornguth S. Magnetic resonance imaging of central nervous system lesions in patients with lupus erythematosus: correlation with clinical remission and antineurofilament and anticardiolipin antibody titers. Arthritis Rheum 1991; 34: 432- 11. 7. Wong KL, Woo EKW, Yu YL, Wong RWS. Neurological manifestations of systemic lupus erythematosus: a prospective study. Quart J Med 1991; 81: 857- 70. / Neuro- Ophthalmol, Vol. 16, No. 4, 1996 |
