- •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.
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)
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- Clinical significance of diffuse dural enhancement detected by magnetic resonance imaging.J. Neurosurg. 1996; 85: 777-783
- Idiopathic hypertrophic pachymeningitis.Neurology. 2004; 62: 686-694
- IgG4-related disease and hypertrophic pachymeningitis.Medicine (Baltimore). 2013; 92: 206-216
- A nationwide survey of hypertrophic pachymeningitis in Japan.J. Neurol. Neurosurg. Psychiatry. 2013;
- Tuberculous cranial pachymeningitis.Neurology. 2007; 68: 298-300
- Uncommon findings in idiopathic hypertrophic cranial pachymeningitis.J. Neurol. 2004; 251: 548-555
- Intracranial hypotension: improved MRI detection with diagnostic intracranial angles.AJR Am. J. Roentgenol. 2013; 200: 400-407
- P-ANCA cranial pachymeningitis: a case report.Clin. Rheumatol. 2005; 24: 174-177
- Hypertrophic pachymeningitis with MPO-ANCA-positive vasculitis.Clin. Rheumatol. 2010; 29: 111-113
- IgG4-related disease.Lancet. 2015; 385: 1460-1471
- Hypertrophic spinal pachymeningitis: idiopathic vs. IgG4-related.J. Neurol. Sci. 2014; 347: 398-400
- Diagnostic value of IgG4 indices in IgG4-related hypertrophic pachymeningitis.J. Neuroimmunol. 2014; 266: 82-86
- Neurosarcoidosis.Clin. Chest Med. 2008; 29 (ix): 475-492
- Clinical characteristics of patients in a case control study of sarcoidosis.Am. J. Respir. Crit. Care Med. 2001; 164: 1885-1889
- Neurosarcoidosis: presentations and management.Neurologist. 2010; 16: 2-15
- Imaging manifestations of neurosarcoidosis.AJR Am. J. Roentgenol. 2004; 182: 289-295
- Epidemiology of sarcoidosis in Japan.Eur. Respir. J. 2008; 31: 372-379
- Magnetic resonance imaging of pachymeningeal enhancement in Vogt-Koyanagi-Harada disease.Neurol. Sci. 2010; 31: 785-788
- Hypertrophic pachymeningitis as a result of a retropharyngeal inflammatory pseudotumor: case report.Neurosurgery. 2002; 51 (discussion 1064-1065): 1061-1064
- Inflammatory pseudotumors of the central nervous system.Hum. Pathol. 2009; 40: 1611-1617
- Inflammatory pseudotumor of temporal bone with pachymeningitis, cranial neuropathies and uveitis.Eur. Neurol. 2004; 51: 238-240
- Intracranial aneurysms in three patients with disseminated Lyme borreliosis: cause or chance association?.J. Neurol. Neurosurg. Psychiatry. 1998; 64: 636-642
- Intracranial dural metastases.Cancer. 2009; 115: 1947-1953
- Dural metastases.J. Neuro-Oncol. 2005; 75: 57-61
- Surgical implications of magnetic resonance-enhanced dura.Neurosurgery. 1994; 35 (discussion 377): 370-377
- Idiopathic hypertrophic cranial pachymeningitis: case report with 7 years of imaging follow-up.AJNR Am. J. Neuroradiol. 2003; 24: 119-123
- Idiopathic hypertrophic pachymeningeal lesions: correlation between clinical patterns and neuroimaging characteristics.Eur. Neurol. 1998; 39: 49-56
- Neurosarcoidosis: a clinical dilemma.Lancet Neurol. 2004; 3: 397-407
- Neurosarcoidosis: a study of 30 new cases.J. Neurol. Neurosurg. Psychiatry. 2009; 80: 297-304
- Idiopathic hypertrophic cranial pachymeningitis. Report of three cases.J. Neurosurg. 1993; 79: 270-276
- Idiopathic hypertrophic cranial pachymeningitis.J. Clin. Neurosci. 2008; 15: 465-469
- Neurosarcoidosis: clinical presentations and course in 50 patients.Acta Neurol. Scand. 1986; 73: 283-290
- MR of CNS sarcoidosis: correlation of imaging features to clinical symptoms and response to treatment.AJNR Am. J. Neuroradiol. 1999; 20: 655-669
- Idiopathic hypertrophic pachymeningitis - case report and literature review.Folia Neuropathol. 2007; 45: 36-42
Published online: June 10, 2016
Accepted: June 9, 2016
Received in revised form: May 19, 2016
Received: December 10, 2015
© 2016 Published by Elsevier B.V.