Neurosarcoidosis: guidance for the general neurologist

Abstract

Sarcoidosis is an inflammatory multisystem disorder of unknown etiology with a worldwide distribution that can affect lungs, lymphatic system, skin, liver, eyes, and nervous system 1 . We owe the first recorded and illustrated description of sarcoidosis to Jonathan Hutchinson in 1878, when he reported a patient with a dermatologic disease, which comprised purplish, symmetric, and non-tender skin plaques, initially considered a manifestation of gout 2 . The term sarcoidosis comes from histological studies performed by Cesar Boeck, in 1899, who described pathological findings of skin lesions that resembled those of sarcoma, which he called 'sarkoid ' or 'sarcoma-like' 3 . Involvement of the nervous system was first recognized, in 1905, by Winkler, and, in 1909, Heerfordt described three patients in the context of facial nerve palsies, uveitis, parotid enlargement, and fever 1,3 . Neurosarcoidosis (NS) may involve any part of the nervous system with acute and chronic courses 1,4 . Cranial nerves, hypothalamus, and pituitary gland are the most commonly regions involved, but meninges, parenchyma, brainstem, and spinal cord may also be affected 4 . Clinical differentiation from other neurological diseases may be difficult when an isolated involvement of the nervous system is present 3,4 . In this review, we highlighted its clinical forms, pathogenesis, and treatment guidelines for NS, as guidance for general neurologists. neUROLOGiCaL ManiFesTaTiOns OF saRCOidOsisNeurologic manifestations are found in 5 to 20% of cases and symptoms may be mild or even severe, requiring a more aggressive intervention. About one-half of patients with NS can develop neurologic manifestations before systemic sarcoidosis is evident absTRaCT Neurosarcoidosis (NS) more commonly occurs in the setting of systemic disease. The diagnosis is based on a clinical history suggestive of NS, presence of noncaseating granulomas, and supportive evidence of sarcoid pathology, laboratory, and imaging studies. NS could involve any part of the nervous system and often demands high doses of steroids for symptom control. It presents low response to isolated steroids administration and frequently requires immunosuppressive agents. In NS, lymphocytes are polarized toward an excessive Th1 response, leading to overproduction of TNF-alpha and INF-gama, as well as lL-2 and IL-15. Infliximab, a chimeric monoclonal antibody that neutralizes the biological activity of TNF-alpha, is a new option in the NS treatment. We revised pathophysiology, clinical manifestations, diagnostic work up, and treatment of NS as guidance for the general neurologist.Key words: sarcoidosis, neurosarcoidosis, methotrexate, azathioprine, cyclophosphamide, infliximab. ResUMOA neurosarcoidose (NS) ocorre frequentemente no contexto de doença sistêmica. O diagnóstico é baseado na história clínica sugestiva de NS, presença de granulomas não-caseosos e achados anatomopatológicos, laboratoriais e radiológicos de sarcoidose. A NS causa manifestações neurológicas variadas, que apresentam, em ge...

Neurosarcoidosis in a Tertiary Referral Center

“…5,9,10 The efficacy of these treatments, however, has not been studied systematically. In our hospital, a national neurosarcoidosis referral center has been established in 2014.…”

Multiple cerebral infarcts: a rare complication of neurosarcoidosis

“…Combination therapy with corticosteroid-sparing agents is therefore recommended early in patients with significant CNS involvement to reduce the risk of relapse on steroid weaning. Dutra et al 9 highlighted three studies showing that fewer than 40% of patients with neurosarcoidosis treated with corticosteroids alone either stabilised or improved, further supporting the need for additional immunosuppressive treatment.…”

Not everything is as it seems: neurosarcoidosis presenting as leptomeningitis

“…Analysis of the CSF can be helpful, although it is not specific . Those patients with meningeal disease may have hypoglycorrhachia as low as 30 mg/dL, high opening pressure, and mononuclear pleocytosis ranging from 10 to 100 cells/mm 3 .…”

“…It is crucial to rule out infectious diseases, malignancy, and other autoimmune inflammatory diseases. The most common differential diagnoses are tumors such as lymphoma, meningioma carcinomatous meningitis, and gliomas; infections including neurosyphilis, HIV, tuberculosis, herpes family viruses, toxoplasmosis, and others such as multiple sclerosis, Guillain–Barré syndrome, systemic lupus erythematosus, amyloidosis, and porphyria .…”