Abstract
Sarcoidosis is a systemic immune-mediated inflammatory disease which significantly affects three major organs: the skin, pulmonary tract, and lymph nodes. Up to 10 % of patients with sarcoidosis develop neurosarcoidosis, and in a minority of patients, neurologic manifestations constitute the presenting clinical picture. The inflammatory non-caseating granulomas of sarcoidosis are the salient pathologic features and can affect the nervous system at several locations. Neurosarcoidosis generates a wide spectrum of clinical manifestations; therefore, its diagnosis requires a systemic approach. The conclusive diagnosis of sarcoidosis rests on pathologic examination of the biopsied tissue; however, imaging techniques, including MRI of central nervous system with contrast, play a significant role in the diagnosis of neurosarcoidosis. There is no cure for sarcoidosis or neurosarcoidosis. Presently, the treatment is based on chronic immunosuppression utilizing TNF-α antagonists, corticosteroids, and a number of cytotoxic medications. Symptomatic treatments of the neurologic complications of neurosarcoidosis involve multiple treatment teams and use of various medications.
This is a preview of subscription content, log in via an institution to check access.
Access this chapter
Tax calculation will be finalised at checkout
Purchases are for personal use only
References
Spencer TS, Campellone JV, Maldonado I, Huang N, Usmani Q, Reginato AJ. Clinical and magnetic resonance imaging manifestations of neurosarcoidosis. Semin Arthritis Rheum. 2005;34(4):649–61.
Ricker W, Clark M. Sarcoidosis: a clinicopathologic review of 300 cases, including 22 autopsies. Am J Clin Pathol. 1949;19(8):725–49.
Iannuzzi MC, Rybicki BA, Teirstein AS. Sarcoidosis. N Engl J Med. 2007;357(21):2153–65.
Nozaki K, Scott TF, Sohn M, Judson MA. Isolated neurosarcoidosis: case series in 2 sarcoidosis centers. Neurologist. 2012;18:373–7.
Chapelon C, Ziza JM, Piette JC, Levy Y, Raguin G, Wechsler B, Bitker MO, Bletry O, Laplane D, Bousser MG. Neurosarcoidosis: signs, course and treatment in 35 confirmed cases. Medicine (Baltimore). 1990;69:261–76.
La Mantia L, Erbetta A. Headache and inflammatory disorders of the central nervous system. Neurol Sci. 2004;25(Suppl 3):S148–53.
Krumholz A, Stern BJ, Stern EG. Clinical implications of seizures in neurosarcoidosis. Arch Neurol. 1991;48:842–4.
Degardin A, Devos P, Vermersch P, de Seze J. Cerebrovascular symptomatic involvement in sarcoidosis. Acta Neurol Belg. 2010;110(4):349–52.
Caplan L, Corbett J, Goodwin J, Thomas C, Shenker D. Schatz n. neuro-ophthalmologic signs in the angiitic form of neurosarcoidosis. Neurology. 1983;33:1130–5.
Byrne JV, Lawton CA. Meningeal sarcoidosis causing intracranial hypertension secondary to dural sinus thrombosis. Br J Radiol. 1983;56:755–7.
Bihan H, Christozova V, Dumas JL, Jomaa R, Valeyre D, Tazi A, Reach G, Krivitzky A, Cohen R. Sarcoidosis: clinical, hormonal, and magnetic resonance imaging (MRI) manifestations of hypothalamic-pituitary disease in 9 patients and review of the literature. Medicine (Baltimore). 2007;86(5):259–68.
Polydefkis M, Allen RP, Hauer P, Earley CJ, Griffin JW, McArthur JC. Subclinical sensory neuropathy in late-onset restless legs syndrome. Neurology. 2000;55:1115–21.
May MC, Deng JC, Albores J, Zeidler M, Harper RM, Avidan AY. Hypocretin deficiency associated with narcolepsy type 1 and central hypoventilation syndrome in neurosarcoidosis of the hypothalamus. J Clin Sleep Med. 2015;11(9):1063–5.
Servan J, Marchand F, Garma L, Seilhean D, Hauw JJ, Delattre JY. Narcolepsy disclosing neurosarcoidosis. Rev Neurol (Paris). 1995;151:281–3.
Rubinstein I, Gray TA, Moldofsky H, Hoffstein V. Neurosarcoidosis associated with hypersomnolence treated with corticosteroids and brain irradiation. Chest. 1988;94:205–6.
Verbraecken J, Hoitsma E, van der Grinten CP, Cobben NA, Wouters EF, Drent M. Sleep disturbances associated with periodic leg movements in chronic sarcoidosis. Sarcoidosis Vasc Diff Lung Dis. 2004;21:137–46.
Turner GA, Lower EE, Corser BC, Gunther KL, Baughman RP. Sleep apnea in sarcoidosis. Sarcoidosis Vasc Diff Lung Dis. 1997;14:61–4.
Pihtili A, Bingol Z, Kiyan E, Cuhadaroglu C, Issever H, Gulbaran Z. Obstructive sleep apnea is common in patients with interstitial lung disease. Sleep Breath. 2013;17:1281–8.
Shah R, Roberson GH, Curé JK. Correlation of MR imaging findings and clinical manifestations in neurosarcoidosis. AJNR Am J Neuroradiol. 2009;30(5):953–61.
Zajicek JP, Scolding NJ, Foster O, et al. Central nervous system sarcoidosis – diagnosis and management. QJM. 1999;92(2):103–17.
Burke WM, Keogh A, Maloney PJ, Delprado W, Bryant DH, Spratt P. Transmission of sarcoidosis via cardiac transplantation. Lancet. 1990;336(8730):1579.
Bakkers M, Merkies IS, Lauria G, Devigili G, Penza P, Lombardi R, Hermans MC, van Nes SI, De Baets M, Faberm CG. Intraepidermal nerve fiber density and its application in sarcoidosis. Neurology. 2009;73:1142–8.
Prabhakar HB, Rabinowitz CB, Gibbons FK, O’Donnell WJ, Shepard JA, Aquino SL. Imaging features of sarcoidosis on MDCT, FDG PET, and PET/CT. AJR Am J Roentgenol. 2008 Mar;190(3 Suppl):S1–6.
Author information
Authors and Affiliations
Corresponding author
Editor information
Editors and Affiliations
Rights and permissions
Copyright information
© 2017 Springer International Publishing AG
About this chapter
Cite this chapter
Aachi, R., Fowler, M., Gonzalez-Toledo, E., McGee, J., Minagar, A. (2017). Neurosarcoidosis: Clinical Features, Pathogenesis, and Management. In: Minagar, A., Alexander, J. (eds) Inflammatory Disorders of the Nervous System. Current Clinical Neurology. Humana Press, Cham. https://doi.org/10.1007/978-3-319-51220-4_5
Download citation
DOI: https://doi.org/10.1007/978-3-319-51220-4_5
Published:
Publisher Name: Humana Press, Cham
Print ISBN: 978-3-319-51218-1
Online ISBN: 978-3-319-51220-4
eBook Packages: MedicineMedicine (R0)