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Neurosarcoidosis and multiple intracerebral hematomas: An unusual clinical presentation

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Neurosarcoidosis (NS) represents a heterogeneous clinical entity accountable for the variety of neurological and other symptoms and signs. Pathological changes (non-caseating granulomas, vasculitic and ischaemic changes) can affect meningeas, brainstem,… Click to show full abstract

Neurosarcoidosis (NS) represents a heterogeneous clinical entity accountable for the variety of neurological and other symptoms and signs. Pathological changes (non-caseating granulomas, vasculitic and ischaemic changes) can affect meningeas, brainstem, spinal cord and peripheral nerves [1]. Based on the Zajicek criteria diagnosis can be classified as definitive, probable andpossible. In addition to a clinicalfindings suggestive of NS, definitive diagnosis requires a pathohistological confirmation of CNS granuloma and exclusion of other causes of neurological symptoms. Probable NS lacks a pathohistological confirmation, but in addition to typical presentation, can be supported by MRI findings, elevated CSF protein levels and/or pleocytosis, oligoclonal bands, confirmed systemic sarcoidosis and elevated serum angiotensin converting enzyme level (ACE). Possible NS requires a clinical findings suggestive of NS and exclusion of other causes of neurological symptoms [2]. Intracerebral hemorrhage in neurosarcoidosis is rare. To our knowledge, only twenty cases have been recorded so far [3]. Our patient had a single granulomatous change in the pons which showed significant radiological regression after the introduction of steroids, followed by a clinical improvement, too. Upon gradual discontinuation of steroids multiple cerebral and cerebellar hematomas were observed. Neurosarcoidosis treatment is less effective in comparison to sarcoidosis without CNS involvement and it's not supported by evidence-based guidelines or controlled randomised trials. Algorithm created by Nozaki and Judson, can be helpful especially in treatment of severe cases, comprises of methylprednisolone (500–1000 mg/day 3–5 days), followed by prednisone (40–100 mg/day) and another immunomodulatory drug, with methotrexate as a first choice [4].

Keywords: neurological symptoms; presentation; hematomas; multiple intracerebral; neurosarcoidosis multiple; neurosarcoidosis

Journal Title: Journal of the Neurological Sciences
Year Published: 2017

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