Defining the course of neurosarcoidosis according to presentation at onset and disease modifying treatment: a cohort study of 84 patients

Inès Bekkour; Edouard Courtin; Cécile Dulau-Metras; Pierre Duffau; Laurent Kremer; Guillaume Mathey

doi:10.1177/17562864231205954

Defining the course of neurosarcoidosis according to presentation at onset and disease modifying treatment: a cohort study of 84 patients

Ther Adv Neurol Disord. 2023 Dec 23:16:17562864231205954. doi: 10.1177/17562864231205954. eCollection 2023.

Authors

Inès Bekkour¹, Edouard Courtin², Cécile Dulau-Metras², Pierre Duffau^{3

4}, Laurent Kremer^{5

6}, Guillaume Mathey^{7

1

8}

Affiliations

¹ Department of Neurology, Nancy University Hospital, Nancy, France.
² Department of Neurology, Bordeaux University Hospital, Bordeaux, France.
³ Department of Internal Medicine and Clinical Immunology, Bordeaux University Hospital, Bordeaux, France.
⁴ CNRS, ImmunoConcEpT, UMR 5164, University of Bordeaux, Bordeaux, France.
⁵ Department of Neurology, Strasbourg University Hospital, Strasbourg, France.
⁶ INSERM U1119, Strasbourg, France.
⁷ Service de Neurologie, Hopital Central, CHRU de Nancy, 1 Avenue du Maréchal de Lattre de Tassigny, Nancy 54000, France.
⁸ CIC-Epidémiologie Clinique, Nancy University Hospital, INSERM, Université de Lorraine, Nancy, France.

Abstract

Background: Neurosarcoidosis is a rare manifestation of sarcoidosis with heterogeneous presentations. Patient management is challenging due to the current lack of knowledge about the long-term disease course.

Objective: To identify specific disease courses of neurosarcoidosis according to the clinical and paraclinical presentations at onset.

Methods: We conducted an observational multicenter cohort study by retrospectively collecting data from the medical records of 84 patients diagnosed with definite, probable, or possible neurosarcoidosis in three tertiary referral centers in France (Nancy, Strasbourg, and Bordeaux). We collected demographic characteristics, clinical and paraclinical data at the beginning of patient management, and during follow-up under the different treatment lines. Two expert neurologists determined disease course profiles.

Results: The mean follow-up was 6.6 years. Almost every patient (96.4%) received steroids at some point of their follow-up. Tumor Necrosis Factor-alpha blockers were given in 10.7% as first-line treatment and in 33.3% during follow-up. Every patient presented with a relapsing disease, often monophasic (75%) and sometimes polyphasic with the recurrence of identical manifestations (11.9%). Patients developing new neurological symptoms during follow-up were a minority (13.1%). No patients exhibited a progressive course. Patients with isolated cranial nerves injury or aseptic meningitis always exhibited a monophasic course, and 62.5-75% of them had a full recovery after first-line treatments. This proportion was 15.6% in other forms of the disease. Those with peripheral presentations were more likely to present a polyphasic course than patients with other forms of neurosarcoidosis. Spinal cord presentations were monophasic, but resulted in sequelae and exhibited poor response to first-line treatments despite frequent use of TNF-alpha blockers.

Conclusion: Identification of these disease course profiles, based on the initial clinical and paraclinical presentation, could guide the clinician to select the optimal therapeutic approach and follow-up modalities for their patients with neurosarcoidosis.

Keywords: cohort; disease course; disease modifying treatments; long term; neurosarcoidosis.