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Table of Contents    
LETTER TO EDITOR
Year : 2014  |  Volume : 62  |  Issue : 3  |  Page : 307-308

The one-and-a-half syndrome as the presenting sign of systemic lupus erythematosus


Department of Neurology, Mashhad University of Medical Sciences, Mashhad, Iran

Date of Submission08-Apr-2014
Date of Decision12-Apr-2014
Date of Acceptance29-May-2014
Date of Web Publication18-Jul-2014

Correspondence Address:
Nahid Olfati
Department of Neurology, Mashhad University of Medical Sciences, Mashhad
Iran
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0028-3886.136983

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How to cite this article:
Sasannejad P, Olfati N, Sabi MS, Juibary AG. The one-and-a-half syndrome as the presenting sign of systemic lupus erythematosus. Neurol India 2014;62:307-8

How to cite this URL:
Sasannejad P, Olfati N, Sabi MS, Juibary AG. The one-and-a-half syndrome as the presenting sign of systemic lupus erythematosus. Neurol India [serial online] 2014 [cited 2021 Jan 16];62:307-8. Available from: https://www.neurologyindia.com/text.asp?2014/62/3/307/136983


Sir,

One-and-a-half syndrome (OHS) is characterized by an internuclear ophthalmoplegia (INO) and lateral gaze palsy due to the involvement of the pontine gaze center and ipsilateral medial longitudinal fasciculus (MLF). Most frequent causes include multiple sclerosis and brainstem ischemic stroke. We report a patient of systemic lupus erythematosus (SLE) presenting with one-and-a-half syndrome.

In September 2013, a 34-year-old man with a history of bicytopenia, seizures, and periodic delusions was brought with complaints of diplopia and dizziness. Previous testing for anti nuclear antibody (ANA) and other SLE serum markers was negative. The patient also had a history of tingling sensation, pain, and weakness in his legs, non-erosive arthritis of hands and feet, skin plaques on extensor sides of both elbows, and a history of bloating that responded to gluten-free regimen despite a negative duodenal biopsy for celiac disease. Neurologic examination revealed a one-and-a-half syndrome with freezing of the left eye and restricted adduction of the right eye in conjugate horizontal gaze [Figure 1]. Incomplete ptosis of the left eyelid was also evident. Diplopia was reported by patient in right horizontal gaze. No other cranial nerve palsies was found. Bilateral foot drop was also noted. Laboratory evaluation at this admission revealed a positive ANA (+), borderline Anti-ds-DNA (value: 96 IU/ml; normal: <100), positive nRNP/Sm (+), positive SS-A (++), positive Rib.P-protein (+), positive AMA-M2 (+), negative P- and C-ANCA, low C3 (value 67; normal: 89-187) and C4 (value: 13; normal: 16-38) levels, negative Lupus Anti-coagulant and Beta-II GPI, and positive Anti-Cardiolipin antibodies. Examination of the cerebrospinal fluid was normal. Complete blood picture revealed mild anemia (hemoglobin: 11.9), leukopenia (WBC: 2000 per ml; 55% lymphocytes, 35% neutrophils), and mild thrombocytopenia (100,000 per ml).
Figure 1: Ocular movements after second methylprednisolone pulse therapy. On rightward gaze (left) both eyes only pass slightly to the right from midline position and on leftward gaze (right) restricted adduction of the right eye is evident

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Electrodiagnostic studies revealed axonal sensorimotor polyneuropathy. Cranial magnetic resonance imaging (MRI) showed no evidence of diffusion restriction in brainstem or in brain hemispheres [Figure 2]. A follow-up brain MRI done at two weeks was essentially normal. One-gram intravenous infusion of methylprednisolone was given for three days. However, considering the patient's symptoms (cytopenia, relapse of delusions, and cerebral involvement), we administered an additional one-gram dose of cyclophosphamide and continued the treatment with oral prednisolone. The patient responded to the treatment: Remission of diplopia, improvement in hemoglobin and white blood cell counts, and improved eye movements.
Figure 2: Axial brain MRI at the time of presentation. No pathologic signal changes are evident

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Our patient fulfilled the criteria for SLE stipulated by American College of Rheumatology and the presenting feature was OHS, which is a rare presentation of SLE. Literature review revealed two cases of OHS in known patients of SLE. [1],[2] Yigit et al., [2] reported a case of SLE presenting with diplopia and dysarthria with pontine infarction on MR imaging. Kunavarapu et al., [1] reported OHS in a patient with SLE with infarction zone in lower midbrain on MRI. In contrast to these patients, no pathologic signal changes in related regions of gaze control were seen on MRI in the present patients.

The OHA is a subtype of INO, an uncommon condition in patients with SLE as well. [3],[4],[5],[6] In the two patients of SLE with OHA, the cause was ischemic infarct. [7] Other possible mechanisms have also been proposed for central nervous system involvement in lupus and they include vasculopathy, autoantibodies, and cytokines. [8] In their review, Cogen et al., argue that unilateral INO is more frequent than the bilateral form in SLE and the probable cause could be selective localization of the blood supply to the medial longitudinal fasciculi in contrast to plaques of multiple sclerosis which are nonselective and bilateral INO is more frequent in multiple sclerosis. Immune complex deposition and microvascular injury might probably be the basis of the clinical presentation in this patient. This possible mechanism was further supported by low complement levels and multiple positive autoantibodies observed in this patient.

 
  References Top

1.Kunavarapu C, Kesavan RB, Pevil-Ulysee M, Mohan SS. Systemic lupus erythematosus presenting as "one-and-a-half syndrome". J Rheumatol 2001;28:874-5.  Back to cited text no. 1
    
2.Yiðit A, Bingöl A, Mutluer N, Taþçilar N. The one-and-a-half syndrome in systemic lupus erythematosus. J Neuroophthalmol 1996;16:274-6.  Back to cited text no. 2
    
3.Cogen MS, Kline LB, Duvall ER. Bilateral internuclear ophthalmoplegia in systemic lupus erythematosus. J Clin Neuroophthalmol 1987;7:69-73.  Back to cited text no. 3
    
4.Dutta I, Gohar S, Balakrishnan C, Mangat G, Mittal G, Bhaba SK, et al. Unilateral inter-nuclear ophthalmoplegia in systemic lupus erythematosus. J Assoc Physicians India 2000;48:1210-1.  Back to cited text no. 4
    
5.Galindo M, Pablos JL, Gomez-Reino JJ. Internuclear ophthalmoplegia in systemic lupus erythematosus. Semin Arthritis Rheum 1998;28:179-86.  Back to cited text no. 5
    
6.Jackson G, Miller M, Littlejohn G, Helme R, King R. Bilateral internuclear ophthalmoplegia in systemic lupus erythematosus. J Rheumatol 1986;13:1161-2.  Back to cited text no. 6
    
7.Keane JR. Eye movement abnormalities in systemic lupus erythematosus. Arch Neurol 1995;52:1145-9.  Back to cited text no. 7
    
8.Efthimiou P, Blanco M. Pathogenesis of neuropsychiatric systemic lupus erythematosus and potential biomarkers. Mod Rheumatol 2009;19:457-68.  Back to cited text no. 8
    


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