Hypertrophic pachymeningitis

Hypertrophic Pachymeningitis (HP) denotes inflammation and thickening of the dura mater that can be idiopathic or secondary to a wide variety of conditions. Clinically, HP can present as debilitating headaches and cranial nerve defects but in other cases may be completely asymptomatic. We aimed to determine the relative incidence of different etiologies of HP and compare their associated imaging findings. Additionally, we sought to compare the clinical features of the underlying syndromes. We retrospectively examined twenty-two consecutive cases of HP seen in a single practitioner neurology practice over a ten-year time period. The most common etiologies were idiopathic HP and neurosarcoidosis. No imaging features were completely specific to any etiology. Nonetheless, idiopathic HP typically demonstrated diffuse regular enhancement whereas neurosarcoidosis was more likely to display a nodular enhancement pattern. Headache and cranial neuropathies were the most common clinical presentation. HP symptoms were often responsive to steroids but complete responses were rare. HP is a diagnostic challenge without specific findings on minimally or non-invasive diagnostic studies. Biopsy is often required and serves as the basis for effective therapy.