Objective: To describe a group of patients with neurosarcoidosis and to highlight diagnostic difficulties based on current diagnostic criteria. Methods: The patient database of a general neurological department was searched for patients with established or suspected diagnosis of neurosarcoidosis. Twenty-four patients were identified with definite (n = 3), probable (n = 10) and possible neurosarcoidosis (n = 10). History and clinical, laboratory and imaging data of patients with definite and probable neurosarcoidosis were analyzed. Results: Cranial nerve symptoms were a dominant clinical feature, with the optic nerve being affected most frequently. Cerebrospinal fluid pleocytosis was found in more than half of the patients. Intrathecal IgG synthesis and oligoclonal bands were less frequent. There was a wide array of MRI lesions in both groups. Chest X-ray was false negative in 2 of 5 patients who also underwent a thoracic CT. Therapy with prednisolone was initiated in all patients. After a median of 36 months, 6 of 8 patients with follow-up data of >24 months were still in remission. Aggravation of symptoms required therapy escalation in 2 patients. Conclusion: There is a wide range of clinical symptoms and test results in patients with ‘definite’ or ‘probable’ neurosarcoidosis. Because systemic involvement is a crucial diagnostic criterion, extensive medical work-up may be necessary. Prognosis under corticosteroid treatment may be better than previously thought.

1.
American Thoracic Society (ATS): Statement on sarcoidosis, adopted by the ATS Board of Directors and by the ERS Executive Committee, February 1999. Am J Respir Crit Care Med 1999;160:736–755.
2.
Stern BJ, Krumholz A, Johns C, Scott P, Nissim J: Sarcoidosis and its neurological manifestations. Arch Neurol 1985;42:909–917.
3.
Delaney P: Neurologic manifestations in sarcoidosis: Review of the literature, with a report of 23 cases. Ann Intern Med 1977;87:336–345.
4.
Scott TF: Neurosarcoidosis: Progress and clinical aspects. Neurology 1993;43:8–12.
5.
Cipri S, Gambardella G, Campolo C, Mannino R, Consoli D: Unusual clinical presentation of cerebral-isolated sarcoidosis. Case report and review of the literature. J Neurosurg Sci 2000;44:140–144.
6.
Uchino M, Nagao T, Harada N, Shibata I, Hamatani S, Mutou H: Neurosarcoidosis without systemic sarcoidosis – case report. Neurol Med Chir (Tokyo) 2001;41:48–51.
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;69:261–276.
8.
Oksanen V: Neurosarcoidosis: Clinical presentations and course in 50 patients. Acta Neurol Scand 1986;73:283–290.
9.
Salazar A, Mana J, Albareda JM, Badrinas F, Fernandez-Nogues F: Neurosarcoidosis: presentation of 14 cases. Med Clin (Barc) 1993;100:292–295.
10.
Sharma OP: Neurosarcoidosis: A personal perspective based on the study of 37 patients. Chest 1997;112:220–228.
11.
Nowak DA, Widenka DC: Neurosarcoidosis: A review of its intracranial manifestation. J Neurol 2001;248:363–372.
12.
Zajicek JP, Scolding NJ, Foster O, Rovaris M, Evanson J, Moseley IF, Scadding JW, Thompson EJ, Chamoun V, Miller DH, McDonald WI, Mitchell D: Central nervous system sarcoidosis – diagnosis and management. QJM 1999;92:103–117.
13.
Galassi G, Gibertoni M, Mancini A, Nemni R, Volpi G, Merelli E, Vacca G: Sarcoidosis of the peripheral nerve: Clinical, electrophysiological and histological study of two cases. Eur Neurol 1984;23:459–465.
14.
Nemni R, Galassi G, Cohen M, Hays AP, Gould R, Singh N, Bressman S, Gamboa ET: Symmetric sarcoid polyneuropathy: Analysis of a sural nerve biopsy. Neurology 1981;31:1217–1223.
15.
Borucki SJ, Nguyen BV, Ladoulis CT, McKendall RR: Cerebrospinal fluid immunoglobulin abnormalities in neurosarcoidosis. Arch Neurol 1989;46:270–273.
16.
Dale JC, O’Brien JF: Determination of angiotensin-converting enzyme levels in cerebrospinal fluid is not a useful test for the diagnosis of neurosarcoidosis. Mayo Clin Proc 1999;74:535.
17.
Tahmoush AJ, Amir MS, Connor WW, Farry JK, Didato S, Ulhoa-Cintra A, Vasas JM, Schwartzman RJ, Israel HL, Patrick H: CSF-ACE activity in probable CNS neurosarcoidosis. Sarcoidosis Vasc Diffuse Lung Dis 2002;19:191–197.
18.
Schweisfurth H, Schioberg-Schiegnitz S, Kuhn W, Parusel B: Angiotensin I converting enzyme in cerebrospinal fluid of patients with neurological diseases. Klin Wochenschr 1987;65:955–958.
19.
Oksanen V, Fyhrquist F, Somer H, Gronhagen-Riska C: Angiotensin converting enzyme in cerebrospinal fluid: A new assay. Neurology 1985;35:1220–1223.
20.
Jones DB, Mitchell D, Horn DB, Edwards CR: Cerebrospinal fluid angiotensin converting enzyme levels in the diagnosis of neurosarcoidosis. Scott Med J 1991;36:144–145.
21.
Zajicek JP: Neurosarcoidosis. Curr Opin Neurol 2000;13:323–325.
22.
Mana J: Magnetic resonance imaging and nuclear imaging in sarcoidosis. Curr Opin Pulm Med 2002;8:457–463.
23.
Miller DH, Kendall BE, Barter S, Johnson G, MacManus DG, Logsdail SJ, Ormerod IE, McDonald WI: Magnetic resonance imaging in central nervous system sarcoidosis. Neurology 1988;38:378–383.
24.
Pickuth D, Spielmann RP, Heywang-Kobrunner SH: Role of radiology in the diagnosis of neurosarcoidosis. Eur Radiol 2000;10:941–944.
25.
Allen RK, Sellars RE, Sandstrom PA: A prospective study of 32 patients with neurosarcoidosis. Sarcoidosis Vasc Diffuse Lung Dis 2003;20:118–125.
26.
Pavese P, Brion JP, Chabre O, Fauconnier J, Pasquier B: Neurologic involvement in sarcoidosis. Federation of Systemic Diseases of Grenoble. Presse Méd 1999;28:168–172.
Copyright / Drug Dosage / Disclaimer
Copyright: All rights reserved. No part of this publication may be translated into other languages, reproduced or utilized in any form or by any means, electronic or mechanical, including photocopying, recording, microcopying, or by any information storage and retrieval system, without permission in writing from the publisher.
Drug Dosage: The authors and the publisher have exerted every effort to ensure that drug selection and dosage set forth in this text are in accord with current recommendations and practice at the time of publication. However, in view of ongoing research, changes in government regulations, and the constant flow of information relating to drug therapy and drug reactions, the reader is urged to check the package insert for each drug for any changes in indications and dosage and for added warnings and precautions. This is particularly important when the recommended agent is a new and/or infrequently employed drug.
Disclaimer: The statements, opinions and data contained in this publication are solely those of the individual authors and contributors and not of the publishers and the editor(s). The appearance of advertisements or/and product references in the publication is not a warranty, endorsement, or approval of the products or services advertised or of their effectiveness, quality or safety. The publisher and the editor(s) disclaim responsibility for any injury to persons or property resulting from any ideas, methods, instructions or products referred to in the content or advertisements.
You do not currently have access to this content.