Hypertrophic pachymeningitis is a condition where there is localized or diffuse inflammatory thickening of the dura. On imaging, it presents as a localized, multiple, or diffuse enhancing dural thickening commonly forming mass-like lesions.
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Clinical presentation
The clinical presentation may be varied 7. Common clinical features include headache and cranial nerve palsies 7.
Pathology
Etiology
It can result from a number of causes which include 5-7:
- idiopathic
- infective
- inflammatory
- other
- Rosai-Dorfman disease
- haemodialysis
- mucopolysaccharidoses
Radiographic features
MRI
- localized, or less often, diffuse dural thickening
- may uncommonly depict mass-like thickening, termed tumefactive hypertrophic pachymeningitis 1
Signal characteristics:
- T1: thickened areas are hypointense to brain parenchyma 6
- T1 C+ (Gd): dural enhancement
- T2: thickened areas are hypointense to brain parenchyma 6
Treatment and prognosis
Management depends on the underlying cause, and includes immunosuppression in idiopathic cases 7.
Differential diagnosis
General imaging differential considerations include:
- meningeal metastases
- meningeal involvement with CNS lymphoma
- multiple meningiomas including lymphoplasmacyte-rich meningioma
- intracranial involvement with Erdheim Chester disease