Pachymeningitis in Biopsy-Proven Sarcoidosis: Clinical Course, Radiographic Findings, Response to Treatment, and Long-Term Outcomes

Objective To study the clinicoradiographic features of pachymeningeal involvement in neurosarcoidosis and its evolution over time response to treatment. Background Meningeal inflammation is one most common forms neurosarcoidosis, occurring 16-69% affected patients. While clinical radiographic leptomeningitis are well known, those pachymeningitis far less clear. Design/Methods Patients with a diagnosis seen at Emory University [01/2011-8/2021] were included if was evident by MRI patient's sarcoidosis pathologically confirmed (from neural or extraneural site). Results 26/215 (12.1%) patients qualified for inclusion. Pathological confirmation came from tissue 50%. Median age onset 43.5 years; male (16/26, 61.5%). Symptoms primarily related 20/26 (76.9%). Headache (19/26, 73.1%), visual dysfunction (12/26, 46.2%), seizures (7/26, 26.9%) symptoms. All had cranial pachymeningitis; only single patient undergoing spinal imaging (1/11, 9.1%) pachymeningitis. The falx cerebri 61.5%) commonly dural structure, but anterior middle fossae tentorium frequently involved (12/26 each, 46.2%). lesions unifocal (11/26, 42.3%) multifocal (15/26, 57.7%) distribution, nodular morphologically (23/25, 92.0%), homogeneously enhancing (24/25, 96.0%). Symptomatic improvement occurred steroids initially 22/25 (88.0%). Ultimately, 23/26 (88.5%) required initiation steroid-sparing immunosuppressants, including 8/26 (30.8%) eventually TNF inhibition. Pachymeningeal relapses 7/26 (26.9%). follow-up 48 months. mRS last improved 1.0 2.0 presentation. Conclusions Sarcoid often presents headaches dysfunction, usually affects fossae, tends require immunosuppressants. It has potential relapse, prospect recovery excellent.