Primary Sjögren Syndrome with Tumefactive Central Nervous System Involvement: Fig 1.

Abstract

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...

A New Kind of and Reversible Brainstem Involvement in Primary Sjögren's Syndrome as an Initial Manifestation

“…Hence, intensive and early treatment is needed in cases of CNS involvement. In some case reports of pSS with CNS involvement, clinical and radiological improvement have been suggested (Hirohata, Yasukawa, Ishida, Komai, & Yamada, 2005;Kadota et al, 2002;Sanahuja et al, 2008). However, severe clinical outcomes have been also reported (Hayashi et al, 2008;Ichikawa et al, 2004;Owada et al, 2002;Yaguchi et al, 2008;Yoon et al, 2000).…”

“…Brain MRI abnormalities in pSS are predominantly in the periventricular and subcortical white matter (Morgen et al, 2004). Tumeactive brain lesion, central pontine myelinolysis, dentate nucleus of the cerebellum bilaterally, medulla oblangata and cervical spinal cord involvement, severe cerebellar and cerebral atrophy, and optic nerve involvement have been also reported in unique cases of pSS (Kadota et al, 2002;Owada et al, 2002;Sanahuja, Ordoñez-Palau, Begué, Brieva, & Boquet, 2008;Yaguchi et al, 2008;Yoon, Fong, Koh, & Suri, 2000). The underlying pathogenesis of CNS complications in pSS remains unknown.…”

Resonancia magnética en hipoacusia y vértigo

“…Se caracteriza por la infiltración linfocítica y la destrucción de las glándulas salivales y lacrimales que conducen a la xerostomía y xeroftalmía, puede tener manifestaciones extraglandulares [52]. La prevalencia de la afectación del sistema nervioso central y las alteraciones en RM en el síndrome de Sjögren primario son controvertidas [53]. Las manifestaciones más graves recuerdan a la esclerosis múltiple (EM), aunque normalmente los hallazgos de la RM sugieren discretos daños del tejido cerebral, apreciando pequeñas tumoraciones en la sustancia blanca, que son inespecíficas pues pueden confundirse con tumoraciones asociadas a la edad o factores de riesgo cerebrovascular, como la hipertensión.…”

Immune Mediated Diseases and Immune Modulation in the Neurocritical Care Unit

“…Although most of the neurological manifestations of Sjögren's syndrome are not severe enough to warrant ICU admission, acute myelopathy can occur and potentially necessitate ventilatory support [203,204]. Tumefactive brain lesions are also reported in Sjögren's syndrome [205,206].…”