Highlights
- •Most cases of HP were idiopathic or related to neurosarcoidosis.
- •No imaging features were unique to each etiology of HP.
- •Regular, diffuse enhancement was most common for idiopathic HP.
- •Symptoms of HP were often responsive to treatment with steroids.
Abstract
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.
Abbreviations:
HP (hypertrophic pachymeningitis)Keywords
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Article info
Publication history
Published online: June 10, 2016
Accepted:
June 9,
2016
Received in revised form:
May 19,
2016
Received:
December 10,
2015
Identification
Copyright
© 2016 Published by Elsevier B.V.
