SUMMARY:Brain MR imaging abnormalities in primary Sjö gren syndrome (pSS) are generally discrete white matter lesions. We describe a 50-year-old woman with recurrent neurologic deficits. MR imaging revealed a large brain lesion. A diagnosis of pSS was made on the basis of clinical features, positive anti-Ro and anti-La antibodies, abnormal Schirmer test findings, and salivary gland scintigraphy. The patient was treated with oral prednisone with good response. Large tumefactive brain lesions are a complication of pSS. Sjögren syndrome is an autoimmune disorder that may be primary or secondary to other connective tissue diseases. It is characterized by lymphocytic infiltration and destruction of salivary and lachrymal glands leading to xerostomia and xerophthalmia. Similar mononuclear infiltrates or vasculitic lesions can have extraglandular manifestations.1 The prevalence of central nervous system (CNS) disease and MR imaging abnormalities in primary Sjögren syndrome (pSS) is controversial.2-4 We present a patient with pSS and a pseudotumoral lesion in the brain. Case ReportA 50-year-old woman was referred to our unit with a 6-month history of headache associated with vomiting, impaired visual perception in left visual fields, and difficulty retrieving some words. The patient had a 1-year history of arthralgias, dry mouth, and a 3-kg weight loss. Findings of a neurologic examination were normal. Blood analysis showed antinuclear antibody titers of 1/160 and positive anti-Ro and anti-La antibodies. Findings of serologic tests were negative. MR imaging revealed a demyelinating-inflammatory lesion in the right temporo-occipital white matter. Cerebral angiography did not demonstrate vasculitis. CSF did not show oligoclonal bands. Visual evoked potentials were normal. Symptoms and the radiologic lesion improved spontaneously within 1 month.Three months later, the patient was admitted because of left facial and brachial paresis and left hemibody hypoesthesia. A new MR imaging showed a large right frontoparietal lesion with ring contrast enhancement surrounding edema and notable mass effect, without restricted diffusion (Fig 1A, -B). Findings of thoracoabdominal CT, mammography, and tumor markers were negative. Schirmer test results were Ͻ2 mm. Salivary gland scintigraphy was compatible with Sjögren syndrome grade 3. A diagnosis of pSS was made according to accepted criteria. 5 Oral prednisone 1 mg/kg daily was started with dramatic clinical and radiologic improvement (Fig 1C, -D). In the subsequent months, the oral prednisone dosage was tapered to 5 mg daily. The patient remained stable until 2 years later, when she experienced right hemifacial paresthesias. MR imaging revealed 2 small hyperintense lesions in the bulb and in the cervical spinal cord on T2-weighted images. Her oral prednisone dosage was increased to 1 mg/kg daily again, and hydroxychloroquine was added with good response. DiscussionThe frequency and type of CNS involvement in pSS is a matter of discussion. The reported prevalence of CNS manifestations r...
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