Charles Tyshkov1, Siddharama Pawate1, Michael J Bradshaw1, Dorlan J Kimbrough1, Tanuja Chitnis1, Jeffrey M Gelfand1, Lana Zhovtis Ryerson1, Ilya Kister1. 1. New York University Langone Medical Center (CT, LZR, IK), Multiple Sclerosis Comprehensive Care Center, New York, NY; the Vanderbilt University Medical Center (SP), Neuroimmunology Division, Nashville, TN; the Brigham and Women's Hospital (MJB, DJK, TC), Massachusetts General Hospital, Harvard Medical School, Boston, MA; and the Division of Neuroinflammation and Glial Biology (JMG), UCSF Department of Neurology, Weill Institute for Neurosciences, University of California, San Francisco, CA.
Abstract
BACKGROUND: Patients with biopsy-proven systemic sarcoidosis who develop a chronic CNS disorder are often presumed to have neurosarcoidosis (NS), however, the possibility of comorbid neurologic disease, such as MS, must be considered if presentation and course are not typical for NS. METHODS: Retrospective chart review across 4 academic MS centers was undertaken to identify patients with diagnosis of MS (2017 McDonald criteria) and biopsy-confirmed extraneural sarcoidosis. Data were abstracted from each chart using a case report form that systematically queried for demographic, clinical, and paraclinical characteristics relevant to NS and MS. RESULTS: Ten patients met our inclusion criteria (mean age 47.7 [±5.9] years; 80% female). Noncaseating granulomas consistent with sarcoidosis were found on biopsy in all cases (lung 7/10, mediastinum 2/10, liver 1/10, spleen 1/10, and skin 1/10). Diagnosis of MS was based on clinical history of MS-like relapses and MRI findings characteristic of demyelination and typical disease evolution during follow-up (average of 7 years). No patient developed features of NS that could be considered a "red flag" against the diagnosis of MS (such as meningeal enhancement, hydrocephalus, and pituitary involvement). All patients were treated with disease-modifying therapy for MS. CONCLUSIONS: We propose a rational diagnostic approach to patients with sarcoidosis who may have comorbid MS. When the clinical picture is equivocal, the presence of multiple "MS-typical lesions" and the absence of any "NS-typical lesions" on MRI favor diagnosis of MS. Close follow-up is required to ascertain whether clinical and radiologic disease evolution and response to MS therapies conform to the proposed diagnosis of MS.
BACKGROUND: Patients with biopsy-proven systemic sarcoidosis who develop a chronic CNS disorder are often presumed to have neurosarcoidosis (NS), however, the possibility of comorbid neurologic disease, such as MS, must be considered if presentation and course are not typical for NS. METHODS: Retrospective chart review across 4 academic MS centers was undertaken to identify patients with diagnosis of MS (2017 McDonald criteria) and biopsy-confirmed extraneural sarcoidosis. Data were abstracted from each chart using a case report form that systematically queried for demographic, clinical, and paraclinical characteristics relevant to NS and MS. RESULTS: Ten patients met our inclusion criteria (mean age 47.7 [±5.9] years; 80% female). Noncaseating granulomas consistent with sarcoidosis were found on biopsy in all cases (lung 7/10, mediastinum 2/10, liver 1/10, spleen 1/10, and skin 1/10). Diagnosis of MS was based on clinical history of MS-like relapses and MRI findings characteristic of demyelination and typical disease evolution during follow-up (average of 7 years). No patient developed features of NS that could be considered a "red flag" against the diagnosis of MS (such as meningeal enhancement, hydrocephalus, and pituitary involvement). All patients were treated with disease-modifying therapy for MS. CONCLUSIONS: We propose a rational diagnostic approach to patients with sarcoidosis who may have comorbid MS. When the clinical picture is equivocal, the presence of multiple "MS-typical lesions" and the absence of any "NS-typical lesions" on MRI favor diagnosis of MS. Close follow-up is required to ascertain whether clinical and radiologic disease evolution and response to MS therapies conform to the proposed diagnosis of MS.
Authors: J P Zajicek; N J Scolding; O Foster; M Rovaris; J Evanson; I F Moseley; J W Scadding; E J Thompson; V Chamoun; D H Miller; W I McDonald; D Mitchell Journal: QJM Date: 1999-02
Authors: Pietro Maggi; Martina Absinta; Pascal Sati; Gaetano Perrotta; Luca Massacesi; Bernard Dachy; Caroline Pot; Reto Meuli; Daniel S Reich; Massimo Filippi; Renaud Du Pasquier; Marie Théaudin Journal: Mult Scler Date: 2019-09-19 Impact factor: 6.312