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  • Neuro-Ophthalmology/Orbit

    The authors report the case of a 37-year-old woman with Balint syndrome as the initial presentation of posterior reversible encephalopathy syndrome (PRES). They believe this to be the first time that Balint syndrome has been described in association with PRES. The patient also had acute hypertension and systemic lupus erythematosus (SLE), and reports of PRES associated with SLE are rare.

    She presented with complaints of abdominal pain, nausea, vomiting, alopecia, malar rash and arthritis. Her serum was positive for anti-double-stranded DNA and anticardiolipin antibodies. SLE was diagnosed and treated with intravenous methylprednisolone. The patient's blood pressure was elevated, and lupus nephritis was presumed responsible. However, serum creatinine was normal (64 µmol/L; normal range, 50-90 µmol/L), and renal biopsy was not performed.

    Two weeks after initial presentation, the patient rapidly developed bilateral impairment of vision. Blood pressure was 190/150 mmHg. Visual acuity was difficult to assess because she made erroneous saccades toward optotypes but was at least 20/400 in each eye. She was able to count fingers in the left hemifield of each eye but perceived only hand movement in the right hemifields. Although she had full range of volitional saccades to directional commands, she could not make visually-guided saccades to small or large objects in any direction. Saccades were generated in directions that grossly missed fixation of visual objects presented in portions of the visual fields where they could be seen. Hand movements were dysmetric when she attempted to grasp a viewed object but accurate toward her own body parts. She was unable to recognize more than one item at a time when presented with the cookie theft picture. Three days later, automated visual field testing showed a right inferior homonymous quadrantanopia. MRI showed extensive FLAIR hyperintensity within the parietal, occipital and frontal lobes. Diffusion-weighted imaging and apparent diffusion coefficient demonstrated restricted diffusion in some of the involved areas.

    Hypertension was treated with angiotensin-converting enzyme inhibitors. SLE was treated with prednisone, mycophenolic acid and hydroxychloroquine. Optic ataxia resolved six days after the initiation of treatment. Simultanagnosia and impaired visually-guided saccades resolved after three months. Six months following initial presentation, the patient's vision improved to 20/80 in each eye, and the right inferior homonymous quadrantanopia resolved.

    The outcome of Balint syndrome is typically favorable, as illustrated by the rapid resolution of Balint syndrome in this patient. However, the authors conclude that the relative roles of acute hypertension and cerebral SLE in the pathogenesis of PRES in this patient are unknown and either or both could have been responsible. The role of SLE in the pathogenesis of PRES is often unclear. Often multiple factors associated with PRES are found among SLE patients, including hypertension, nephritis and immunosuppressive drugs.