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Neurosarcoidosis occurs in 3% to 10% of patients with sarcoidosis.
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Any part of the nervous system can be affected with cranial neuropathy and meningeal involvement being the most common manifestations.
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Glucocorticoids are the main therapy, although immunosuppressive agents are also often required because of the high rate of relapse.
Neurosarcoidosis
Section snippets
Key points
Clinical features
Any part of the nervous system can be affected by sarcoidosis, and multiple lesions are often noted.11, 12, 13 The frequency of each neurologic manifestation is summarized in Table 1. Neurologic abnormalities are one of the first clinical manifestations that lead to the diagnosis of sarcoidosis in 70% to 80% of patients with neurosarcoidosis.13, 14, 15 Isolated neurosarcoidosis is uncommon, because more than 90% of patients also have sarcoidosis in other organs, especially the lungs and
Diagnostic tests
Similar to systemic sarcoidosis, diagnosis of neurosarcoidosis requires the presence of noncaseating granuloma and compatible clinical presentations after exclusion of alternative diagnoses.50 The presence of noncaseating granuloma itself is not sufficient to make a definite diagnosis because it could be seen in other conditions, such as foreign body reaction, tuberculosis, and fungal infection.11, 25
Given the invasive nature of biopsy of the nervous system, histopathologic confirmation is
Treatments
Unlike pulmonary sarcoidosis, spontaneous resolution of neurosarcoidosis is uncommon (except for facial nerve palsy), and treatment is generally indicated to minimize morbidity and mortality.6 Unfortunately, there have been no randomized, placebo-controlled trials in neurosarcoidosis to guide treatment. Therefore, all recommendations are based on clinical experience and limited data from observation studies. Doses, adverse reactions, and monitoring of the commonly used medications in
Summary
Neurosarcoidosis is an uncommon manifestation of sarcoidosis that is associated with significant morbidity and mortality. Diagnosis of neurosarcoidosis is often challenging, because clinical manifestations and findings on imaging studies can be mimicked by several other diseases. Glucocorticoids are the cornerstone for the treatment of neurosarcoidosis; immunosuppressive agents are also often required. Clinical trials are needed to establish the efficacy of these treatments.
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Disclosure Statement: The authors have no financial or nonfinancial potential conflicts of interest to declare.