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      A case of neurosarcoidosis presenting with multiple cranial neuropathies

      case-report

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          Abstract

          Purpose

          We report a case of neurosarcoidosis that presented simultaneously with oculomotor nerve palsy, contralateral abducens nerve palsy, and paresthesia of both lower limbs.

          Observations

          A 69-year-old Japanese woman who suffered from repeated diplopia and lower-limb paresthesia was referred to our hospital. Ophthalmic findings included oculomotor nerve and contralateral abducens nerve palsies. No remarkable abnormalities were detected via enhanced brain magnetic resonance imaging (MRI), chest X-ray, and cerebrospinal fluid analysis. Chest computed tomography (CT) was performed to exclude neoplastic lesions; this revealed right hilar lymphadenopathy, and positron emission tomography MRI showed strong 18-F fluorodeoxyglucose uptake in the hilar lymph node. Biopsy of the lymph node showed non-caseating epithelioid granulomatous tissue, leading to a diagnosis of probable neurosarcoidosis. After the initiation of oral prednisolone treatment, the patient experienced complete remission without any recurrence.

          Conclusions and importance

          When examining a patient presenting with multiple cranial neuropathies of unknown cause, neurosarcoidosis should be considered as a differential diagnosis and chest CT should be performed even when the chest X-ray and angiotensin-converting enzyme appears normal.

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          Most cited references18

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          Definition and Consensus Diagnostic Criteria for Neurosarcoidosis

          The Neurosarcoidosis Consortium Consensus Group, an expert panel of physicians experienced in the management of patients with sarcoidosis and neurosarcoidosis, engaged in an iterative process to define neurosarcoidosis and develop a practical diagnostic approach to patients with suspected neurosarcoidosis. This panel aimed to develop a consensus clinical definition of neurosarcoidosis to enhance the clinical care of patients with suspected neurosarcoidosis and to encourage standardization of research initiatives that address this disease.
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            Clinical features, treatment and outcome in neurosarcoidosis: systematic review and meta-analysis

            Background Neurosarcoidosis is a rare variant of sarcoidosis and is only described in small cohort studies. We define clinical features, treatment and outcome of patients with neurosarcoidosis over the last 35 years. Methods We performed a systematic review and meta-analysis of studies on neurosarcoidosis published between 1980 and 2016. Studies were included if they reported at least 5 cases. Studies describing one specific neurological presentation were excluded. Results We identified 29 articles describing 1088 patients diagnosed between 1965 and 2015. Neurosarcoidosis occurred in 5% of patients with systemic sarcoidosis. Mean age at presentation was 43 years and neurological symptoms were the first clinical manifestation of sarcoidosis in 52%. The most commonly reported feature of neurosarcoidosis was cranial neuropathy in 55%, with the facial and optic nerve most commonly affected, followed by headache in 32%. Pleiocytosis and elevated CSF protein were found in 58 and 63%. MRI of the brain showed abnormalities in 70%. Chest X-ray, chest CT, or gallium-67-scintigraphy showed findings consistent with sarcoidosis in 60%, 70% and 69%, respectively. First line therapy with corticosteroids was initiated in 434 of 539 patients (81%). Second and third line therapy was started in 27 and 9%. Outcome consisted of complete remission in 27%, incomplete remission in 32%, stable disease in 24%, deterioration in 6% and death in 5%. Conclusion Neurosarcoidosis has a heterogeneous clinical presentation and the diagnosis can be difficult because of low sensitivity of ancillary investigations. New treatments have emerged, but nevertheless one third of patients do not respond to treatment. Prospective cohort studies and RCTs on treatment are urgently needed. Electronic supplementary material The online version of this article (doi:10.1186/s12883-016-0741-x) contains supplementary material, which is available to authorized users.
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              Neurosarcoidosis: a review of its intracranial manifestation.

              Sarcoidosis is a multisystem disease of unknown cause and with a worldwide distribution. Involvement of the central nervous system occurs in a relatively small number of patients with sarcoidosis. Isolated neurosarcoidosis without signs of systemic disease is a rarity. Because of its non-specific clinical presentation and neuroradiological imaging characteristics, intracranial neurosarcoidosis remains a very difficult diagnosis, particularly in the absence of systemic signs of the disease. Intracranial neurosarcoidosis has a predilection for the basal leptomeninges commonly affecting the cranial nerves, but any part of the brain may be involved, resulting in a wide spectrum of clinical syndromes. Cranial nerve involvement is the most common single symptom. Intracranial sarcoid manifests as nodular or diffuse leptomeningeal thickening and extraor intra-axial parenchymatous lesions. Intracranial sarcoid may mimic various forms of meningitis, including carcinomatous and intracranial mass lesions such as meningioma, lymphoma and glioma, based on neuroradiological imaging. Magnetic resonance imaging is a very sensitive diagnostic tool for detecting intracranial abnormalities due to neurosarcoidosis. Lumbar puncture is useful in ruling out other neurological disorders, in particular infectious, but cerebrospinal fluid findings are not specific. Angiotensin-converting enzyme in serum and cerebrospinal fluid may be increased, decreased or normal. Therapy consists of immunosuppressive agents and should be initiated with corticosteroids. Other immunosuppressive drugs should be added in severe cases or after frequent recurrences. We review here all aspects of intracranial neurosarcoidosis from the clinical point of view, with special emphasis on presentation, diagnostic procedures, differential diagnostic considerations and treatment.
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                Author and article information

                Contributors
                Journal
                Am J Ophthalmol Case Rep
                Am J Ophthalmol Case Rep
                American Journal of Ophthalmology Case Reports
                Elsevier
                2451-9936
                27 June 2020
                September 2020
                27 June 2020
                : 19
                : 100796
                Affiliations
                [a ]Division of Ophthalmology, Department of Surgery, Kobe University Graduate School of Medicine, Kobe, Japan
                [b ]Division of Neurology, Kobe University Graduate School of Medicine, Kobe, Japan
                [c ]Department of Neurology, Graduate School of Medicine, The University of Tokyo, Tokyo, Japan
                Author notes
                []Corresponding author. Division of Ophthalmology, Department of Surgery Kobe University Graduate School of Medicine 7-5-2 Kusunoki-cho, Chuo-ku, Kobe, 650-0017, Japan. kuri1201@ 123456med.kobe-u.ac.jp
                Article
                S2451-9936(20)30132-8 100796
                10.1016/j.ajoc.2020.100796
                7330492
                32637734
                a777b40e-c1e1-46a9-9c76-ce7422a9656b
                © 2020 Published by Elsevier Inc.

                This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/).

                History
                : 26 February 2020
                : 17 June 2020
                : 17 June 2020
                Categories
                Case Report

                neurosarcoidosis,oculomotor nerve palsy,abducens nerve palsy,computerized tomography,positron emission tomography,multiple cranial nerve palsies

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