Abstract
Sarcoidosis is a rare but important cause of neurological morbidity, and neurological symptoms often herald the diagnosis. Our understanding of neurosarcoidosis has evolved from early descriptions of a uveoparotid fever to include presentations involving every part of the neural axis. The diagnosis should be suspected in patients with sarcoidosis who develop new neurological symptoms, those presenting with syndromes highly suggestive of neurosarcoidosis, or neuro-inflammatory disease where more common causes have been excluded. Investigation should look for evidence of neuro-inflammation, best achieved by contrast-enhanced brain magnetic resonance imaging and cerebrospinal fluid analysis. Evidence of sarcoidosis outside the nervous system should be sought in search of tissue for biopsy. Skin lesions should be identified and biopsies taken. Chest radiography including high-resolution computed tomography is often informative. In difficult cases, fluorodeoxyglucose positron emission tomography and gallium-67 imaging may identify subclinical disease and a target for biopsy. Symptomatic patients should be treated with corticosteroids, and if clinically indicated other immunosuppressants such as hydroxychloroquine, azathioprine, cyclophosphamide or methotrexate should be added. Anti-tumour necrosis factor alpha therapies may be considered in refractory disease but caution should be exercised as there is evidence to suggest they may unmask disease.
Highlights
Purpose of reviewNeurosarcoidosis (NS) is a rare neuro-inflammatory disorder with protean manifestations which presents a diagnostic challenge to general physicians and neurologists alike
The diagnosis should be suspected in patients with sarcoidosis who develop new neurological symptoms, those presenting with syndromes highly suggestive of neurosarcoidosis, or neuro-inflammatory disease where more common causes have been excluded
Anti-tumour necrosis factor alpha therapies may be considered in refractory disease but caution should be exercised as there is evidence to suggest they may unmask disease
Summary
Neurosarcoidosis (NS) is a rare neuro-inflammatory disorder with protean manifestations which presents a diagnostic challenge to general physicians and neurologists alike. In 2007, we highlighted how case series continued to refine our understanding, and that anti-tumour necrosis factor alpha (TNF-a) therapies may hold promise as steroid-sparing agents in difficult disease [1, 2]. We provide a clinically focused update on the management of NS, guided by knowledge from new case series, refinements in diagnostic criteria and further reports on anti-TNF-a and other immunosuppressive therapies. Neurosarcoidosis, the involvement of the nervous system by sarcoid granulomata, is uncommon, occurring symptomatically in 5–16% of patients with sarcoidosis [5,6,7,8]. Our regional hospital-based study of patients in South West England and South Wales estimated a prevalence of 1/100,000 [9]. A significant proportion of NS is subclinical as confirmed by autopsy studies; one showed nervous system involvement in 14% of those with sarcoidosis [10]; another found only in 50% was NS diagnosed in life [11]
Sign-in/Register to view highlights & summary Talk to us
Join us for a 30 min session where you can share your feedback and ask us any queries you have
Schedule a callDisclaimer: All third-party content on this website/platform is and will remain the property of their respective owners and is provided on "as is" basis without any warranties, express or implied. Use of third-party content does not indicate any affiliation, sponsorship with or endorsement by them. Any references to third-party content is to identify the corresponding services and shall be considered fair use under The CopyrightLaw.
