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      Multiple Cranial Nerve Palsies as the First Presentation of Sarcoidosis

      case-report

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          Abstract

          Sarcoidosis is a disease process which predominantly affects the lungs but can involve virtually any organ in the human body. Neurosarcoidosis is a rare manifestation which can present in a variety of ways. There is no single diagnostic test for sarcoidosis; hence, the diagnosis is based on combined clinical, laboratorial, and radiological grounds. We describe a rare case where a patient presented with dysphagia, hoarseness, hearing loss, and unsteadiness.

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          Presentations and outcomes of neurosarcoidosis: a study of 54 cases.

          Sharon H Moses, Peruvemba Sriram, S Pawate (2009)
          To report on the clinical presentations, laboratory abnormalities, treatment and outcomes in 54 patients with neurosarcoidosis (NS). Sarcoidosis is an inflammatory granulomatous disease affecting multiple organ systems. Neurosarcoidosis (CNS involvement) is seen in approximately 25% of patients with systemic sarcoidosis, although it is subclinical in most of these cases. Because of its rarity, exposure of neurologists to the clinical spectrum of NS is limited to case reports or short case series. A database of 3900 patients treated at the Vanderbilt Multiple Sclerosis Clinic between 1995 and 2008 was searched for 'neurosarcoidosis', 'neurosarcoid', 'sarcoidosis' and 'sarcoid'. Of the 162 patient records that were retrieved, 54 patients were found to meet the criteria for definite, probable or possible neurosarcoidosis and were reviewed, including their clinical presentation, Cerebrospinal fluid (CSF) findings, Magnetic resonance imaging (MRIs), biopsy results, treatment, and where available, outcomes 4 months to 20 years after onset of the presenting illness. Clinical presentations and imaging findings in NS were varied. Cranial nerve abnormalities were the most common clinical presentation and involvement of the optic nerve in particular was associated with a poor prognosis for visual recovery. Isolated involvement of lower cranial nerves had a more favorable outcome. T(2) hyperintense parenchymal lesions were the most common imaging finding followed by meningeal enhancement. Long-term treatment consisted of prednisone and/or other immunomodulators (azathioprine, methotrexate or mycophenolate mofetil). Unlike systemic sarcoidosis, there is difficulty in making tissue diagnosis when involvement of CNS is suspected. MRI and CSF studies are sensitive in the detection of CNS inflammation but lack specificity, making the ascertainment of neurosarcoidosis a clinical challenge. In addition the low prevalence of the disease makes clinical trials difficult and therapeutic decisions are likely to be made from careful reporting from case studies.
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            Neurosarcoidosis: a clinical dilemma.

            Birgit C G Faber, Marjolein Drent, Elske Hoitsma … (2004)
            Sarcoidosis is an inflammatory multisystem disorder of unknown cause. Practically no organ is immune to sarcoidosis; most commonly, in up to 90% of patients, it affects the lungs. The nervous system is involved in 5-15% of patients. Neurosarcoidosis is a serious and commonly devastating complication of sarcoidosis. Clinical diagnosis of neurosarcoidosis depends on the finding of neurological disease in multisystem sarcoidosis. As the disease can present in many different ways without biopsy evidence, solitary nervous-system sarcoidosis is difficult to diagnose. Corticosteroids are the drug of first choice. In addition, several cytotoxic drugs, including methotrexate, have been used to treat sarcoidosis. The value of new drugs such as anti-tumour necrosis factor alpha will be assessed. In this review we describe the clinical manifestations of neurosarcoidosis, diagnostic dilemmas and considerations, and therapy.
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              Neurosarcoidosis: Clinical manifestations, diagnosis and treatment.

              Kenkichi Nozaki, Marc Judson (2012)
              Sarcoidosis is an idiopathic granulomatous disease affecting multiple organs. Neurosarcoidosis, involving the central and/or peripheral nervous systems, is a relatively rare form of sarcoidosis. Its clinical manifestations include cranial neuropathies, meningitis, neuroendocrinological dysfunction, hydrocephalus, seizures, neuropsychiatric symptoms, myelopathy and neuropathies. The diagnosis is problematic, especially when occurring as an isolated form without other organ involvement. Distinguishing neurosarcoidosis from other granulomatous diseases and multiple sclerosis is especially important. Although biopsy of neural tissue is the gold standard for the diagnosis of neurosarcoidosis, this is often not practical and the diagnosis must be inferred though other tests, often coupled with biopsy of extraneural organs. Corticosteroids and other immuno-suppressants are frequently used for the treatment of neurosarcoidosis. This article reviews the epidemiology, pathogenesis, pathology, clinical features, diagnosis, diagnostic tests, diagnostic criteria, and therapy of neurosarcoidosis.
              Article 0 comments Cited 35 times – based on 0 reviews     Review now
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                Author and article information

                Journal
                Journal ID (nlm-ta): Case Rep Otolaryngol
                Journal ID (iso-abbrev): Case Rep Otolaryngol
                Journal ID (publisher-id): CRIOT
                Title: Case Reports in Otolaryngology
                Publisher: Hindawi Publishing Corporation
                ISSN (Print): 2090-6765
                ISSN (Electronic): 2090-6773
                Publication date (Print): 2014
                Publication date (Electronic): 26 March 2014
                Volume: 2014
                Electronic Location Identifier: 592510
                Affiliations
                1Auckland City Hospital, Auckland, New Zealand
                2Manukau Superclinic, Auckland, New Zealand
                Author notes

                Academic Editors: N. Perez-Fernandez and A. Rapoport

                Author information
                http://orcid.org/0000-0003-4754-9706
                Article
                DOI: 10.1155/2014/592510
                PMC ID: 3984804
                PubMed ID: 24790763
                SO-VID: 45660f9a-cd2e-4477-a534-7235064e5a46
                Copyright © Copyright © 2014 Oliver Rose et al.
                License:

                This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

                History
                Date received : 7 January 2014
                Date accepted : 27 February 2014
                Categories
                Subject: Case Report

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