Abstract Details

Title New-onset Headache in Neurosarcoidosis: An Early Sign of Leptomeningeal Involvement.

Topic Autoimmune Neurology

Presentation(s) P11 - Poster Session 11 (11:45 AM-12:45 PM)

Poster/Presentation
Number 9-004

Background Sarcoidosis is a multisystem inflammatory granulomatous disease. Neurologic involvement occurs in approximately 5-10% of patients with sarcoidosis. The diagnosis of neurosarcoidosis is challenging due to lack of sensitive and specific biomarkers. Headaches are reported as a symptom, but the association between headache with disease activity in neurosarcoidosis has not been investigated.

Objective To investigate the association between headache symptoms and disease activity in neurosarcoidosis.

Design/Methods A retrospective review was performed by searching the patient database for ICD-10 diagnosis of neurosarcoidosis (n=20). Patients with probable (n=11) or definite (n=4) neurosarcoidosis based on the modified Zajicek criteria were included in the analysis. The information on presenting symptoms, prior migraine history, imaging findings, and treatment response was extracted from the medical records.

Results New-onset daily headaches were present in 67% (10/15) of patients and occurred before the onset of other neurologic symptoms. Headache features include unilateral/bilateral tension-like pain (70%; 7/10), retroorbital pain (30%; 3/10), and neck pain (30%; 3/10). The most common radiographic feature associated with headache was the presence of leptomeningeal enhancement and evidence of radiographic progression correlated with worsening headache (60%; 6/10). Traditional analgesic therapies for headaches provided only partial symptom relief in 40% (4/10) and no relief in the remaining patients. Treatment with corticosteroids led to completed resolution of symptoms in 30% (3/10) of patients and partial resolution in 70% (7/10) patients. Treatment with infliximab was associated with complete resolution of headache symptoms in all patients (5/5). Relapse of headache occurred in all patients (3/3) who stopped infliximab therapy and was associated with radiographic progression of leptomeningeal disease.

Conclusions New-onset daily headache is an early clinical sign of disease activity in neurosarcoidosis as evidenced by its association with leptomeningeal enhancement, improvement with immunosuppressive therapy, and relapse after treatment discontinuation.

Authors/Disclosures
Danwei Wu, MD (Stanford University) PRESENTER	Dr. Wu has nothing to disclose.
Anna J. Tomczak, MSc (Stanford)	Miss Tomczak has nothing to disclose.
Neda Sattarnezhad Oskouei, MD (Stanford Univesrity)	Dr. Sattarnezhad has received personal compensation in the range of $500-$4,999 for serving as a Consultant for EMD-Serono. Dr. Sattarnezhad has received research support from National MS Society.
Jamie C. McDonald, MD (Stanford University)	Dr. McDonald has nothing to disclose.
Julia Sumera (Stanford Neurology)	Miss Sumera has nothing to disclose.
Lucas Kipp, MD	The institution of Dr. Kipp has received research support from Biogen. The institution of Dr. Kipp has received research support from Genentech.
Christopher Lock, MD, MBBS, PhD (Stanford University)	Dr. Lock has received personal compensation for serving as an employee of InterX Inc. Dr. Lock has received personal compensation for serving as an employee of Diagnose Early . Dr. Lock has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Biogen. Dr. Lock has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Sanofi . Dr. Lock has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for EMD Serono . Dr. Lock has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Alexion Pharmaceuticals. Dr. Lock has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Bristol Myers Squib. Dr. Lock has received personal compensation in the range of $5,000-$9,999 for serving on a Speakers Bureau for Biogen. Dr. Lock has received personal compensation in the range of $10,000-$49,999 for serving as an Expert Witness for OGLE, WORM & TRAVIS, PLLP.
Jeffrey E. Dunn, MD, FAAN (Stanford University Medical Center)	Dr. Dunn has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Alexion. Dr. Dunn has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Janssen. Dr. Dunn has received personal compensation in the range of $500-$4,999 for serving as a Consultant for BMS. Dr. Dunn has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Genzyme. The institution of Dr. Dunn has received personal compensation in the range of $0-$499 for serving on a Scientific Advisory or Data Safety Monitoring board for Progentec Diagnostics.
May Han, MD (Stanford University)	Dr. Han has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Roche. Dr. Han has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Arena Pharmaceuticals.