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

Abstract

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