Inflammatory Pseudotumor of the Choroid Plexus in Sjögren's Disease

Chen, Yuan; Horoupian, Dikran S.; Lane, Barton; Fross, Robin D.; Smyth, Lawrence T.; Seiling, Robert J.

doi:10.1227/00006123-199108000-00023

Cited by 40 publications

(23 citation statements)

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“…Some authors propose that the immunological response could be triggered by a viral infection, since the EpsteinBarr virus has been associated with up to 40% of plasma cell granuloma cases 1,16) . Some articles, in discussing associations with Sjögren syndrome, suggests an auto-immune pathogenesis 5) . In our case, there was neither any evidence of autoimmune or immunodeficiency disorder nor any medical history of lung disease.…”

Section: Discussionmentioning

confidence: 99%

Intracranial Plasma Cell Granuloma

Kim

Choi

Song

et al. 2009

J Korean Neurosurg Soc

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J Korean Neurosurg Soc 46 : [161][162][163][164] 2009 Plasma cell granuloma is a tumor-like disease characterized by non-neoplastic polyclonal proliferation of plasma cells and other mononuclear cells. This disease occurs most frequently in the lung and upper respiratory tract, while the involvement of the central nervous system is very rare. A 44-year-old female patient presented with nausea and progressive visual disturbance. Brain magnetic resonance imaging (MRI) revealed the mass along the right tentorium with low signal intensity in the T2 weighted image (T2WI) and fluid-attenuated inversion recovery (FLAIR) sequence, and an isosignal intensity in T1 weighted image (T1WI), the latter of which was enhanced after administration of gadoliniumdiethylenetriamine penta-acetic acid (Gd-DTPA). The thickest portion of the tentorium was partially excised via the combined suboccipital and infratentorial approach. The histopathological examination indicated a diagnosis of plasma cell granuloma. Postoperative steroid therapy was administered for remnant tumor control. Although a follow up MRI scan taken 20 months after the operation showed a slight decrease in tumor size, the lesion had extended to the falx and left frontal convexity along with parenchymal edema at 32 months after the operation and the clinical status was aggravated. The mass was removed from the left frontal convexity. Radiation therapy was given, together with steroid administration.

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Section: Discussionmentioning

confidence: 99%

Intracranial Plasma Cell Granuloma

Kim

Choi

Song

et al. 2009

J Korean Neurosurg Soc

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“…Pseudolymphoma or pseudotumoral lesions affecting several organs like the liver, kidney, lung, pancreas, and choroid plexus have been reported in patients with pSS. [12][13][14][15] In our patient, improvement with corticosteroids, the pattern of contrast enhancement thicker medially, and a relapsing-remitting course of the lesions suggest an inflammatory-demyelinating mechanism as the underlying pathogenic process. …”

Section: Discussionmentioning

confidence: 83%

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

Sanahuja¹,

Ordoñez-Palau²,

Begué³

et al. 2008

AJNR Am J Neuroradiol

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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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“…The acute form consists of a polymorphnuclear leucocyte infiltration that progressively leads to lymphocytes and fibrotic cell absence of systemic or specific symptoms, especially for sarcoidosis and Sjö gren's syndrome, and of autoantibodies or respiratory and renal tract lesions typical of Wegener granulomatosis, may help physicians to avoid a wrong diagnosis. [8][9][10][11] Recently, several studies have reported a relationship between IPTs and the IgG4-related sclerosing disease (IgG4-RSD), a systemic condition characterized by IgG4-positive plasma cells and T lymphocyte infiltration. 12,13 Katsura et al 14 reported a case of an IgG4-related trigeminal IPT without any other localizations, confirming the association of the two pathological conditions.…”

Section: Discussionmentioning

confidence: 99%

Bilateral inflammatory pseudotumour of the trigeminal nerve: a diagnostic challenge

Romano

Espagnet²,

Galassi³

et al. 2012

Dentomaxillofacial Radiology

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Inflammatory pseudotumour (IPT) is an uncommon disease with undefined pathogenesis. It is often characterized by local aggressiveness with compressive and displacing effects on surrounding structures. It may appear in different regions of the body, rarely involving perineural structures unilaterally. We present a case of a bilateral IPT around trigeminal branches in a patient with a long-term history of periorbital swelling and proptosis. Dentomaxillofacial Radiology (2012) 41, 432-435. doi: 10.1259/dmfr/43068020Keywords: X-ray tomography, computed; magnetic resonance imaging; pseudotumour; inflammatory; trigeminal nerve Case reportA 42-year-old man presented with a long-term history of bilateral painless periorbital swelling and proptosis, mainly in the left side. The values of thyroid hormones were in the normal range and thyroid antibodies were negative.CT and MRI revealed bilateral, space-occupying masses in both orbits encasing optic nerves (Figure 1), especially on the left side. There was extension along the infraorbital nerves to the pterygopalatine fossa bilaterally (Figure 1d,e) and to the right cavernous sinus and Meckel's cave. Evidence of bilateral bone remodelling was present in the pterygopalatine fossa and infraorbital canal, mainly on the left side (Figures 1f, 2a-d).The mass was hypointense on T 2 weighted images (Figure 1a,b) and showed a mild contrast enhancement (Figure 1c,d).The patient underwent left Caldwell-Luc intervention with removal of part of the pathological tissue in the left supraorbital region. Histopathological evaluation showed inflammation sustained by polyclonal B and T (75% cluster of differentiation 4) lymphocytes and eosinophils with signs of bilateral perineuritis and chronic sinusitis (Figure 1e,f).The patient refused surgery or radiotherapy. DiscussionInflammatory pseudotumour (IPT) is a rare idiopathic condition which can occur in nearly every site of the body, although it most commonly involves the lung, the liver and the orbit and is the third most common primary orbital tumour. [1][2][3] IPTs may represent a diagnostic challenge because of their tumour-emulating radiological and clinical behaviour. To our knowledge, only a few cases of IPT originating from cranial nerves have been reported, but none with bilateral involvement have been described until now. 4,5 Even if the aetiology remains obscure, IPT may arise after trigger events such as traumas, infections or drug abuse that lead to a common response pathologically dominated by inflammatory cells (like lymphocytes and plasma cells), histiocytes and spindle cells, and a mixture of fibroblasts and myofibroblasts. 6 The detection of Epstein-Barr virus (EBV) antigens on immunohistochemical staining validates the hypothesis of an autoimmune post-infective reaction also sustained by corticosteroids responsiveness, even if laboratory test results for autoimmune disorders appear normal. 5 IPT is not a static condition but dynamically changes over time, assuming two main aspects that are associated with different progno...

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Inflammatory Pseudotumor of the Choroid Plexus in Sjögren's Disease

Cited by 40 publications

References 0 publications

Intracranial Plasma Cell Granuloma

Intracranial Plasma Cell Granuloma

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

Bilateral inflammatory pseudotumour of the trigeminal nerve: a diagnostic challenge

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