The Spectrum of Intracranial Hypertrophic Pachymeningitis at an Eastern Indian Tertiary Care Center.

Abstract

Hypertrophic pachymeningitis (HP) is a treatable, rare inflammatory disease, either primary or secondary to systemic causes. To characterize the etiology, clinical manifestations, and treatment outcomes of HP patients and determine the factors influencing the radiological resolution of the pachymeningeal enhancement and recurrence of symptoms within the follow-up period. We collected data for this prospective observational study between March 1, 2021 and May 31, 2022, at the Bangur Institute of Neurosciences, Kolkata, and the patients were followed for a 6-month period. Demographic, clinical, laboratory, and treatment-related data were collected. A univariate logistic regression model was used for comparison between patients with and without radiological resolution of pachymeningitis and between patients with and without symptom recurrence. Among 44 patients, the male: female ratio was 1.2:1. The median age at disease onset was 35.5 (28.5-49.5) years. The etiologies were idiopathic (56.8%), tuberculosis (22.8%), immunoglobulin G subtype 4 (IgG4) disease (9.2%), other infections (6.8%), and neoplastic (4.4%). Headache was the most common presentation (95.4%), followed by cranial neuropathies (68.2%). Optic and oculomotor neuropathies were the most common. In terms of radiological features, 27.27, 29.54, and 43.18% of patients had diffuse, focal regular, and focal irregular enhancement, respectively. Temporal (50%), followed by cavernous sinus (38.63%) enhancement, was the most common. Recurrence occurred in 36 and 50% of idiopathic and IgG4-related HP cases, respectively. Mycophenolate mofetil was added to their steroid regimen with no further recurrences. The cohort had a marked absence of (antineutrophil cytoplasmic antibodies) ANCA-associated HP. The severity of clinical manifestations or distribution of pachymeningitis did not differ significantly among the etiological groups. The presence of idiopathic etiology and focal regular enhancement had a significantly higher chance of radiological resolution. The response to therapy was satisfactory. Recurrence was significantly related to shorter steroid courses (<median duration of 5.2 months).