Neurosarcoidosis : Initial Presentation and Disease Course (NEUROSARC)

Neurosarcoidosis : Initial Presentation and Disease Course, a Multicenter Retrospective Study

Neurosarcoidosis represents up to 10% of sarcoidosis cases. Little is known about its long-term course, even if the disease remains mainly monophasic with/w.o. sequelae, or if bouts of new symptoms may arise over years (polyphasic).

Using retrospective data from patients diagnosed with neurosarcoidosis in three French referral centers for neuro-inflammation, the investigators aim to determine patterns of disease course, according to the initial presentation and the treatments used.

Study Overview

• Status: Unknown
• Conditions: Neurosarcoidosis
• Intervention / Treatment: Other: none (observational)

Detailed Description

Sarcoidosis is a systemic inflammatory disease which a 10-20/100.000/year incidence rate. The nervous system is found to be affected in 5-10% in clinical series, rising to 14-27% in post-mortem studies. Mortality is as high as 5% mainly due to cardiac, pulmonary or neurological injury. The treatment of the disease uses cortico-steroids, and often steroid-sparing immunosuppressive agents such as methotrexate or cyclophosphamide. More recently, TNFalpha-blockers have been found to be highly effective.

Long-term data about the clinical and paraclinical course of the disease are lacking. "Bouts" or "relapse" of the disease are barely described, with no substantial information about their frequency, their sequelae, or their response to the treatments when initiated. Less is known about the risk of recurrence in each presentation of neurosarcoidosis (peripheral, spinal, brain or meningeal presentations). In the end, if a truly relapsing-remitting form of the disease exist is not sure, many patients presenting with a lond-standing pattern of invariant symptoms.

Due to this lack of knowledge about the natural history of neurosarcoidosis, even a "good treatment response" cannot be defined, and in many cases, it is difficult to attribute to treatments a seemingly favourable course af the disease.

In the current study, the investigators aim to build a retrospective cohort of patients diagnosed with neurosarcoidosis and seen at least once in three French referral centers for neuro--inflammation from Jan-1st-2000 to date : Nancy (Lorraine, North-Eastern), Bordeaux (Aquitaine, South-Western) and Strasbourg (Alsace, North-Eastern).

Demographics, clinical and paraclinical data at presentation will be collected(magnetic resonance imaging of the spine and brain, electroneuromyography if available, data of blood and cerebro-spinal fluid). After having clustered patients by presentation pattern, data about lines and types of treatments, change in clinical and paraclinical data will be collected. From this perspective, patterns of disease course on the mid/long-term will be established.

Estimated number of patients : 60-100. Estimated duration of follow-up : 5-10 years. Intervention : none (existing data).

• Study Type: Observational
• Enrollment (Anticipated): 80

Contacts and Locations

• Study Contact: Name: Guillaume Mathey, MD; Phone Number: +33383851688; Email: g.mathey@chru-nancy.fr
• Study Contact Backup: Name: Ines Bekkour, Student; Phone Number: +33383851688; Email: i.bekkour@chru-nancy.fr

Study Locations

• France
  • Lorraine
    • Nancy, Lorraine, France, 54000
      • Recruiting
      • Guillaume Mathey
      • Contact: Phone Number: 0634397921; Email: guillaumemathey@yahoo.fr
      • Principal Investigator: Cecile Dulau, MD
      • Principal Investigator: Laurent Kremer, MD

Participation Criteria

Eligibility Criteria

• Ages Eligible for Study: ADULT; OLDER_ADULT; CHILD
• Accepts Healthy Volunteers: No
• Genders Eligible for Study: All

• Sampling Method: Non-Probability Sample

Study Population

Every patient of the three centers meeting the inclusion criteria

Description

Inclusion Criteria:

• To have at least one documented visit in one of the three referral neurological centers (Nancy, Bordeaux, Strasbourg) between Jan-1st-2000 and Jan-1st-2021
• To be diagnosed with possible/probable/definite neurosarcoidosis

Exclusion Criteria:

- Denial to participate to the study

Study Plan

How is the study designed?

What is the study measuring?

Primary Outcome Measures

Outcome Measure	Measure Description	Time Frame
change in disease characteristics from Baseline to end of study	composite variable with three categories : monophasic course / polyphasic course with the same symptoms / polyphasic course with different symptoms This variable is composite and its evaluated by the investigators by reviewing the medical file with every available document : MRI results, clinical evaluation	from baseline to end of study (an average of 6 years)

Collaborators and Investigators

• Sponsor: Central Hospital, Nancy, France

Study record dates

Study Major Dates

• Study Start (ANTICIPATED): June 1, 2021
• Primary Completion (ANTICIPATED): August 1, 2021
• Study Completion (ANTICIPATED): August 1, 2021

Study Registration Dates

• First Submitted: February 18, 2021
• First Submitted That Met QC Criteria: May 27, 2021
• First Posted (ACTUAL): June 3, 2021

Study Record Updates

• Last Update Posted (ACTUAL): June 3, 2021
• Last Update Submitted That Met QC Criteria: May 27, 2021
• Last Verified: February 1, 2021

More Information

Terms related to this study

• Additional Relevant MeSH Terms: Pathologic Processes; Nervous System Diseases; Lymphoproliferative Disorders; Lymphatic Diseases; Disease Attributes; Disease Progression; Sarcoidosis; Central Nervous System Diseases
• Other Study ID Numbers: 2020PI207

Plan for Individual participant data (IPD)

• Plan to Share Individual Participant Data (IPD)?: NO

Drug and device information, study documents

• Studies a U.S. FDA-regulated drug product: No
• Studies a U.S. FDA-regulated device product: No