Abstract
Background
The clinical manifestations of neurosarcoidosis are highly variable and it should be considered as a potential differential diagnosis in any neurological presentation.
Aim
This study was designed to describe the clinical, diagnostic, and treatment patterns and functional outcome in a Caucasian neurosarcoidosis population.
Design
A retrospective analysis was performed on prospectively recorded data in patients attending our neurology clinic between 2008 and 2014 with a diagnosis of definite or probable neurosarcoidosis according to Zajiek criteria.
Methods
Detailed clinical features, baseline demographic data, results of investigations, treatment type and duration, and clinical outcomes were collated.
Results
Eleven patients were identified (55% men) with mean age 39 years (range 21–63). Four had a prior history of systemic sarcoidosis leading to earlier diagnosis (6.7 vs 13.1 months). Six were found to have evidence of systemic sarcoidosis on further investigation and one was biopsy proven isolated neurosarcoidosis. The commonest site of CNS involvement was the cranial nerves (64%), and headache (45%) was the most frequent presenting symptom. MRI abnormalities included leptomeningeal enhancement, white matter lesions, acute arteritis, spinal cord lesion, and cauda equina enhancement. The commonest CSF finding was raised protein (n = 6) and a lymphocytic pleocytosis (n = 7). Serum ACE was only elevated in three cases. Ten patients were treated with both corticosteroids and steroid-sparing agents 8 of whom went into remission.
Conclusions
This series highlights the diverse nature of neurosarcoidosis. Early introduction of aggressive therapy with corticosteroids and steroid-sparing agents appears to improve clinical outcome.
Similar content being viewed by others
References
Baughman RP, Culver DA, Judson MA (2011) A concise review of pulmonary sarcoidosis. Am J Respir Crit Care Med 183(5):573–581. doi:10.1164/rccm.201006-0865CI
Valeyre D, Prasse A, Nunes H, Uzunhan Y, Brillet PY, Muller-Quernheim J (2014) Sarcoidosis. Lancet 383(9923):1155–1167. doi:10.1016/S0140-6736(13)60680-7
Mirfakhraee M, Crofford MJ, Guinto Jr FC, Nauta HJ, Weedn VW (1986) Virchow-Robin space: a path of spread in neurosarcoidosis. Radiology 158(3):715–720. doi:10.1148/radiology.158.3.3945745
Schley D, Carare-Nnadi R, Please CP, Perry VH, Weller RO (2006) Mechanisms to explain the reverse perivascular transport of solutes out of the brain. J Theor Biol 238(4):962–974. doi:10.1016/j.jtbi.2005.07.005
Morimoto T, Azuma A, Abe S, Usuki J, Kudoh S, Sugisaki K, Oritsu M, Nukiwa T (2008) Epidemiology of sarcoidosis in Japan. Eur Respir J 31(2):372–379. doi:10.1183/09031936.00075307
Iannuzzi MC, Rybicki BA, Teirstein AS (2007) Sarcoidosis. N Engl J Med 357(21):2153–2165. doi:10.1056/NEJMra071714
Zajicek JP, Scolding NJ, Foster O, Rovaris M, Evanson J, Moseley IF, Scadding JW, Thompson EJ, Chamoun V, Miller DH, McDonald WI, Mitchell D (1999) Central nervous system sarcoidosis–diagnosis and management. QJM Mon J Assoc Phys 92(2):103–117
Joseph FG, Scolding NJ (2009) Neurosarcoidosis: a study of 30 new cases. J Neurol Neurosurg Psychiatry 80(3):297–304. doi:10.1136/jnnp.2008.151977
Stern BJ, Krumholz A, Johns C, Scott P, Nissim J (1985) Sarcoidosis and its neurological manifestations. Arch Neurol 42(9):909–917
Gascon-Bayarri J, Mana J, Martinez-Yelamos S, Murillo O, Rene R, Rubio F (2011) Neurosarcoidosis: report of 30 cases and a literature survey. Eur J Intern Med 22(6):e125–132. doi:10.1016/j.ejim.2011.08.019
Pawate S, Moses H, Sriram S (2009) Presentations and outcomes of neurosarcoidosis: a study of 54 cases. QJM Mon J Assoc Phys 102(7):449–460. doi:10.1093/qjmed/hcp042
Chapelon C, Ziza JM, Piette JC, Levy Y, Raguin G, Wechsler B, Bitker MO, Bletry O, Laplane D, Bousser MG et al (1990) Neurosarcoidosis: signs, course and treatment in 35 confirmed cases. Medicine 69(5):261–276
Ricker W, Clark M (1949) Sarcoidosis; a clinicopathologic review of 300 cases, including 22 autopsies. Am J Clin Pathol 19(8):725–749
Sohn M, Culver DA, Judson MA, Scott TF, Tavee J, Nozaki K (2014) Spinal cord neurosarcoidosis. Am J Med Sci 347(3):195–198. doi:10.1097/MAJ.0b013e3182808781
Nozaki K, Scott TF, Sohn M, Judson MA (2012) Isolated neurosarcoidosis: case series in 2 sarcoidosis centers. Neurologist 18(6):373–377. doi:10.1097/NRL.0b013e3182704d04
Segal BM (2013) Neurosarcoidosis: diagnostic approaches and therapeutic strategies. Curr Opin Neurol 26(3):307–313. doi:10.1097/WCO.0b013e3283608459
Nozaki K, Judson MA (2013) Neurosarcoidosis. Curr Treat Options Neurol 15(4):492–504. doi:10.1007/s11940-013-0242-9
Santos E, Shaunak S, Renowden S, Scolding NJ (2010) Treatment of refractory neurosarcoidosis with Infliximab. J Neurol Neurosurg Psychiatry 81(3):241–246. doi:10.1136/jnnp.2008.149989
Riancho-Zarrabeitia L, Delgado-Alvarado M, Riancho J, Oterino A, Sedano MJ, Rueda-Gotor J, Perez-Martin I, Gonzalez-Vela MC, Berciano J, Gonzalez-Gay MA, Blanco R (2014) Anti-TNF-alpha therapy in the management of severe neurosarcoidosis: a report of five cases from a single centre and literature review. Clin Exp Rheumatol 32(2):275–284
Bonita R, Beaglehole R (1988) Recovery of motor function after stroke. Stroke J Cereb Circ 19(12):1497–1500
O’Dwyer JP, Al-Moyeed BA, Farrell MA, Pidgeon CN, Collins DR, Fahy A, Gibney J, Swan N, Dempsey OJ, Kidd DP, Reid JM, Smyth S, McCabe DJ (2013) Neurosarcoidosis-related intracranial haemorrhage: three new cases and a systematic review of the literature. Eur J Neurol Off J Eur Fed Neurol Soc 20(1):71–78. doi:10.1111/j.1468-1331.2012.03783.x
Baughman RP, Teirstein AS, Judson MA, Rossman MD, Yeager H, Bresnitz EA, DePalo L, Hunninghake G, Iannuzzi MC, Johns CJ, McLennan G, Moller DR, Newman LS, Rabin DL, Rose C, Rybicki B, Weinberger SE, Terrin ML, Knatterud GL, Cherniak R, Case Control Etiologic Study of Sarcoidosis research g (2001) Clinical characteristics of patients in a case control study of sarcoidosis. Am J Respir Critic Care Med 164(10 Pt 1):1885–1889
Tavee JO, Stern BJ (2014) Neurosarcoidosis. Continuum 20 (3 Neurology of Systemic Disease). p 545–559. doi:10.1212/01.CON.0000450965.30710.e9
Nozaki K, Judson MA (2012) Neurosarcoidosis: clinical manifestations, diagnosis and treatment. Presse Medicale 41(6 Pt 2):e331–348. doi:10.1016/j.lpm.2011.12.017
Dale JC, O’Brien JF (1999) Determination of angiotensin-converting enzyme levels in cerebrospinal fluid is not a useful test for the diagnosis of neurosarcoidosis. Mayo Clin Proc 74(5):535. doi:10.4065/74.5.535
Scott TF, Yandora K, Valeri A, Chieffe C, Schramke C (2007) Aggressive therapy for neurosarcoidosis: long-term follow-up of 48 treated patients. Arch Neurol 64(5):691–696. doi:10.1001/archneur.64.5.691
Keijsers RG, Grutters JC, Thomeer M, Du Bois RM, Van Buul MM, Lavalaye J, Van Den Bosch JM, Verzijlbergen FJ (2011) Imaging the inflammatory activity of sarcoidosis: sensitivity and inter observer agreement of (67)Ga imaging and (18)F-FDG PET. Q J Nucl Med Mol Imaging Off Publ Italian Assoc Nucl Med 55(1):66–71
Hoyle JC, Jablonski C, Newton HB (2014) Neurosarcoidosis: clinical review of a disorder with challenging inpatient presentations and diagnostic considerations. Neurohospitalist 4(2):94–101. doi:10.1177/1941874413519447
Stern BJ, Aksamit A, Clifford D, Scott TF (2010) Neurologic presentations of sarcoidosis. Neurol Clin 28(1):185–198. doi:10.1016/j.ncl.2009.09.012
Riancho-Zarrabeitia L, Delgado-Alvarado M, Riancho J, Oterino A, Sedano MJ, Rueda-Gotor J, Perez-Martin I, Gonzalez-Vela MC, Berciano J, Gonzalez-Gay MA, Blanco R (2013) Anti-TNF-alpha therapy in the management of severe neurosarcoidosis: a report of five cases from a single centre and literature review. Clin Exp Rheumatol 32(2):275–284
Author information
Authors and Affiliations
Corresponding author
Ethics declarations
Funding
There was no funding for this study.
Conflict of interest
KOC has no conflict of interest to declare. LW has no conflict of interest to declare. JJ has no conflict of interest to declare. DMC has no conflict of interest to declare. DM has no conflict of interest to declare. RK has no conflict of interest to declare. SOR has no conflict of interest to declare. NT has received travel bursaries and consultancy fees for Novartis. CMG has received research grants from Biogen, Novartis, Teva and Genzyme and has also received honoria from Biogen, Novartis, Roche and Genzyme.
Ethical approval
This article is an observational study and does not contain any studies with human participants or animals performed by any of the authors.
Rights and permissions
About this article
Cite this article
O’Connell, K., Williams, L., Jones, J. et al. Neurosarcoidosis: clinical presentations and changing treatment patterns in an Irish Caucasian population. Ir J Med Sci 186, 759–766 (2017). https://doi.org/10.1007/s11845-016-1539-y
Received:
Accepted:
Published:
Issue Date:
DOI: https://doi.org/10.1007/s11845-016-1539-y