Q J Med 1999; 92: 103-117
© 1999 Association of Physicians
Central nervous system sarcoidosis—diagnosis and management
1 Department of Neurology, Derriford Hospital, Plymouth, 2 University of Cambridge Neurology Unit, Addenbrooke's Hospital, Cambridge, 3 Department of Neurology, St George's Hospital Medical School, London, 4 MRI Research Unit, National Hospital for Neurology and Neurosurgery, London, 5 National Hospital for Neurology and Neurosurgery, London, and 6 Royal Brompton Hospital, London, UK
Received 16 March 1998 and in revised form 3 December 1998
Dr J.P. Zajicek, Department of Neurology, Derriford Hospital, Plymouth, Devon PL6 8DH
A series of 68 patients with neurosarcoidosis is reported, with particular emphasis on clinical aspects, diagnosis and treatment. A classification system based on clinical diagnostic probability is proposed, consisting of probable and definite disease, the latter being dependent on finding sarcoid granulomas on nervous system histology, which was obtained in 12 patients (18%). The role of investigations, including magnetic resonance imaging (MRI), chest radiography, Kveim skin test, Gallium 67 isotope scanning and cerebrospinal fluid (CSF) studies, is considered. Sixty-two percent of patients presented with nervous system disease, most commonly affecting the optic nerve and chiasm. Other common presentations included cranial nerve palsies, spinal cord and brainstem manifestations. Investigations yielding most diagnostic information included the Kveim test (41/48, 85% positive), raised CSF protein and/or cells (50/62, 81%) and gallium 67 scan (14/31, 45%). Eleven out of 29 patients (38%) patients showed meningeal enhancement on MRI scanning and 43% of scans demonstrated multiple white-matter lesions. Mean follow-up for the group was 4.6 years. Forty-seven patients were seen for >18 months, and over half of these patients progressed despite corticosteroid and other immunosuppressive therapies. The benefit of a large patient database prospectively studied, with extended follow-up is discussed in order to learn more about prognosis and advance therapy in neurosarcoidosis.
CiteULike 
Connotea 
Del.icio.us What's this?
This article has been cited by other articles:
|
|
 |
|
  S. Pawate, H. Moses, and S. Sriram Presentations and outcomes of neurosarcoidosis: a study of 54 cases QJM, July 1, 2009; 102(7): 449 - 460. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 N. M Savage, H. Shah, C. H Alleyne, J. A Switzer, J. R Lee, J. Steele, and S. Sharma Neurosarcoidosis with necrotising sarcoid granulomatosis mimicking meningiomatosis cerebri: case report and literature search BMJ Case Reports, May 25, 2009; 2009(may25_1): bcr1120081187 - bcr1120081187. [Abstract] [Full Text]
|
|
|
|
 |
|
 M C Brouwer, J de Gans, R B Willemse, and D van de Beek Sarcoidosis presenting with hydrocephalus J. Neurol. Neurosurg. Psychiatry, May 1, 2009; 80(5): 550 - 551. [Full Text] [PDF]
|
|
|
|
 |
|
 R. Shah, G.H. Roberson, and J.K. Cure Correlation of MR Imaging Findings and Clinical Manifestations in Neurosarcoidosis AJNR Am. J. Neuroradiol., May 1, 2009; 30(5): 953 - 961. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
F G Joseph and N J Scolding Neurosarcoidosis: a study of 30 new cases J. Neurol. Neurosurg. Psychiatry, March 1, 2009; 80(3): 297 - 304. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 D. Cros, R. G. Gonzalez, and E. J. Mark Case 6-2009 -- A 37-Year-Old Woman with Vertigo, Facial Weakness, and a Generalized Seizure N. Engl. J. Med., February 19, 2009; 360(8): 802 - 809. [Full Text] [PDF]
|
|
|
|
 |
|
 M. Moravan and B. M. Segal Treatment of CNS sarcoidosis with infliximab and mycophenolate mofetil Neurology, January 27, 2009; 72(4): 337 - 340. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
S. D. Brass, M. L. Durand, J. H. Stone, J. W. Chen, and J. R. Stone Case 36-2008 -- A 59-Year-Old Man with Chronic Daily Headache N. Engl. J. Med., November 20, 2008; 359(21): 2267 - 2278. [Full Text] [PDF]
|
|
|
|
 |
|
 F G Joseph and N J Scolding Sarcoidosis of the nervous system Practical Neurology, August 1, 2007; 7(4): 234 - 244. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 T. F. Scott, K. Yandora, A. Valeri, C. Chieffe, and C. Schramke Aggressive Therapy for Neurosarcoidosis: Long-term Follow-up of 48 Treated Patients Arch Neurol, May 1, 2007; 64(5): 691 - 696. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
J. M. Noble, C. T. Anderson, M. Etienne, O. Williams, and D. J. Adams Sarcoid Meningitis With Fulminant Delirium and Markedly Abnormal Cerebrospinal Fluid Arch Neurol, January 1, 2007; 64(1): 129 - 131. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 J. C. Grutters and J. M. M. van den Bosch Corticosteroid treatment in sarcoidosis. Eur. Respir. J., September 1, 2006; 28(3): 627 - 636. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
T Fukushima, M Shirota, M Waragai, T Yonemitsu, T Yamaguchi, K Yamada, and M Tannno Spinal endoscopic biopsy in the diagnosis of central nervous system neurosarcoidosis J. Neurol. Neurosurg. Psychiatry, May 1, 2006; 77(5): 702 - 702. [Full Text] [PDF]
|
|
|
|
 |
|
 S. E. Campbell, C. M. Reed, L. T. Bui-Mansfield, and E. Fillman Vertebral and Spinal Cord Sarcoidosis Am. J. Roentgenol., May 1, 2005; 184(5): 1686 - 1687. [Full Text] [PDF]
|
|
|
|
 |
|
 J. A. Bourgeois, R. J. Maddock, L. Rogers, C. M. Greco, R. S. Mangrulkar, and S. Saint Neurosarcoidosis and Delirium Psychosomatics, April 1, 2005; 46(2): 148 - 150. [Full Text] [PDF]
|
|
|
|
 |
|
 S. Sollima, F. Croce, C. Scalamogna, I. Caramma, and S. Antinori Sarcoidosis Presenting with Massive Involvement of the Nervous System Ann Intern Med, March 15, 2005; 142(6): 475 - 476. [Full Text] [PDF]
|
|
|
|
|
|
J Overell and A Lindahl Neuro-otological syndromes for the neurologist J. Neurol. Neurosurg. Psychiatry, December 1, 2004; 75(suppl_4): iv53 - iv59. [Full Text] [PDF]
|
|
|
|
|
|
R. K. Tikoo, M. J. Kupersmith, and J. L. Finlay Treatment of Refractory Neurosarcoidosis with Cladribine N. Engl. J. Med., April 22, 2004; 350(17): 1798 - 1799. [Full Text] [PDF]
|
|
|
|
 |
|
 T. Koyama, H. Ueda, K. Togashi, S. Umeoka, M. Kataoka, and S. Nagai Radiologic Manifestations of Sarcoidosis in Various Organs RadioGraphics, January 1, 2004; 24(1): 87 - 104. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 R. J. Baumann and W. C. Robertson Jr Neurosarcoid Presents Differently in Children Than in Adults Pediatrics, December 1, 2003; 112(6): e480 - 486. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
A. Minagar, M. Hardjasudarma, and R. E. Kelley Neurosarcoidosis Neurology, August 13, 2002; 59(3): 477 - 477. [Full Text] [PDF]
|
|
|
|
|
|
J. W Jennings, A. M Rojiani, S. S Brem, and F. R. Murtagh Necrotizing Neurosarcoidosis Masquerading as a Left Optic Nerve Meningioma: Case Report AJNR Am. J. Neuroradiol., April 1, 2002; 23(4): 660 - 662. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
R Al-Shahi, C P Warlow, G H Jansen, C. Frijns, and J van Gijn A 59 year old man with progressive spinal cord and peripheral nerve dysfunction culminating in encephalopathy: Edinburgh advanced clinical neurology course, 1999 J. Neurol. Neurosurg. Psychiatry, November 1, 2001; 71(5): 696 - 703. [Full Text] [PDF]
|
|
|
|
 |
|
 E. S. White and J. P. Lynch III Sarcoidosis Involving Multiple Systems : Diagnostic and Therapeutic Challenges Chest, May 1, 2001; 119(5): 1593 - 1597. [Full Text] [PDF]
|
|