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Background: Intracranial inflammatory pseudotumours (IPT) are rare entities that frequently lead to misdiagnosis with malignant lesions. The identification of these lesions is difficult, but important to avoid inadvertent iatrogenicity and to adjust therapeutic protocols. Case Presentation: We report the case of a 30-year-old man who presented a single tonic-clonic seizure. Brain imaging showed a right frontal lesion with intra and extra axial components. Facing the radiologic presentation, a brain tumor was suspected, thus the patient underwent surgery. Pathological exam concluded to a plasma cell granuloma. A whole-body CT-scan showed only a thoracic aortitis. Complete blood work studies came back negative. The patient was also tested for an array of antibodies among which antinuclear antibodies were positive (blood level superior to 1/100). CSF evaluation revealed clear fluid with normal glucose concentration, normal protein levels and lymphocytic pleocytosis. Finally, IgG-4 plasma levels were elevated which led to the diagnosis of an IgG4-RD. The patient was put under prednisolone with a favorable outcome. Conclusion: IPT have several etiologies, among which IgG4 related disease may be one of the less known as only 2 cases have previously been reported. Herein, we report a new case of a young man who presented for seizures related to an intracranial lesion of an IgG4 related disease. The challenge is to suspect such conditions to avoid unnecessary surgeries.

Background: Intracranial inflammatory pseudotumors (IIPT) are one of the differential diagnosis for the central nervous system (CNS) tumors. They represent a rare condition that may mimic clinically and radiologically intracranial tumors and induce their complications. Among their etiologies, neurosarcoidosis is one of the less known and less frequent. To the best of our knowledge, only two cases of posterior fossa IIPT have been reported in the literature. We present here the 3rd case related to a neurosarcoidosis. Case presentation: We report the case of a 55-year-old female patient who presented with an altered state of consciousness associated to severe intracranial hypertension syndrome for four months. Glasgow coma scale on admission was 14/15. Brain imaging revealed bilateral cerebellar micronodular meningeal enhancement regarding the mesencephalon and the pons, as well as a nodular lesion of the 4th ventricle causing a triventricular acute hydrocephalus. The patient had a ventriculo-peritoneal shunt with a favorable outcome. Afterwards, she underwent a salivary gland biopsy which confirmed the diagnosis of neurosarcoidosis. Conclusion: Posterior fossa IIPT are very rare, mainly when located in the posterior fossa, leading to confusion with other pathologies. MRI has an important role in the diagnosis of these lesions, and the determination of their etiology. It shows other than the IIPT itself, many other signs such as leptomeningeal enhancement, nodular lesions or pituitary stalk thickening. These signs can orientate towards the diagnosis. Treatment may associate to symptomatic approach, corticosteroids. Surgical resection may be proposed when the diagnosis remains doubtful.