Idiopathic hypertrophic pachymeningitis

Kupersmith, Mark J.; Martín, Vanina; Heller, Gordon; Shah, Ashish H.; Mitnick, Hal J.

doi:10.1212/01.wnl.0000113748.53023.b7

Cited by 356 publications

(384 citation statements)

References 37 publications

Supporting

Mentioning

308

Contrasting

Unclassified

Order By: Relevance

“…It is realized that intracranial infections, neoplastic diseases and some autoimmune disorders such as sarcoidosis and immunoglobulin G4 (IgG4)-related disease contribute to the development of HP, although cases of 'idiopathic HP' where etiology is uncertain still exist. 1,2 Considering the etiology of HP, which is associated with autoimmune disorders, anti-neutrophil cytoplasmic antibody (ANCA)-related HP is the most frequently recognized. 2,3 ANCA is implicated in small vessel vasculitis, which is designated as ANCA-associated vasculitis (AAV), and is distinct from immune complexmediated vasculitis.…”

Section: Introductionmentioning

confidence: 99%

Hypertrophic pachymeningitis is a characteristic manifestation of granulomatosis with polyangiitis: A retrospective study of anti‐neutrophil cytoplasmic antibody‐associated vasculitis

et al. 2017

View full text Add to dashboard Cite

Aim: To elucidate the characteristics of patients with hypertrophic pachymeningitis (HP) in a population with anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV). Methods:We retrospectively investigated the clinical records of 39 patients who were diagnosed with AAV. To determine the characteristics of HP in AAV, the epidemiological and clinical data from patients with HP were statistically compared with those from patients without HP.Results: Of 39 patients with AAV, seven (17.9%) had associated HP. All patients with HP were classified as having granulomatosis with polyangiitis (GPA), whereas only five of 32 patients without HP were diagnosed as having GPA (P < 0.0001). The frequencies of myeloperoxidase (MPO)-ANCA and proteinase 3-ANCA positivity in patients with HP were equivalent, while MPO-ANCA positivity was obviously dominant in patients without HP. HP occurred as the initial clinical episode of AAV in three patients (7.7% of all AAV). Frequent significant characteristics of patients with HP were headache, cranial neuropathy and paranasal involvement (P < 0.05), and histopathological findings from paranasal involvement were useful for the diagnosis of GPA in some patients with HP. Combination therapy of corticosteroid and an immunosuppressant, such as methotrexate, cyclophosphamide or rituximab, was effective for achieving remission and improving radiographic findings of HP.Conclusion: AAV is a common cause of HP; epidemiological features of AAV patients with HP are different from those of patients without HP. Additionally, HP impacts diagnosis because it may be an initial clinical sign of AAV.

show abstract

Section: Introductionmentioning

confidence: 99%

Hypertrophic pachymeningitis is a characteristic manifestation of granulomatosis with polyangiitis: A retrospective study of anti‐neutrophil cytoplasmic antibody‐associated vasculitis

et al. 2017

View full text Add to dashboard Cite

show abstract

“…Demographically, the median age of patients with idiopathic hypertrophic pachymeningitis is 58.3 years (standard deviation, 15.8; range, 37-88 years). [4][5][6] Only a few paediatric patients have been reported, with the youngest age being 2 years and 11 months in India. 7 Our patient had early-onset disease.…”

Section: Discussionmentioning

confidence: 99%

“…Multiple cranial nerve involvement, ataxia, cortical blindness, psychosis, motor function disturbance, fever, convulsion, and/or loss of consciousness have all been reported. 5,6 Yamada et al 2 thought that the inflammatory thickening of the dura may cause damage to the superior hypophyseal artery resulting in subarachnoid haemorrhage and apoplexy in the anterior lobe of the pituitary gland.…”

Section: Discussionmentioning

confidence: 99%

Idiopathic hypertrophic pachymeningitis mimicking prolactinoma with recurrent vision loss

Lok¹,

Yip²,

Chong³

et al. 2015

Hong Kong Med J

View full text Add to dashboard Cite

A B S T R A C TIdiopathic hypertrophic pachymeningitis is a rare inflammatory condition with diffuse thickening of the dura mater, which may cause a compressive effect or vascular compromise. We report on a 28-year-old Chinese woman with a history of granulomatous mastitis 7 years previously and oligomenorrhoea, headache, blurred vision, and raised prolactin level 2 years previously, that was diagnosed as prolactinoma and treated conservatively with bromocriptine. However, she had recurrent bilateral vision loss when the bromocriptine was stopped. Her symptoms were resolved by high-dose steroid injection but remained steroid-dependent. Serial magnetic resonance imaging scan showed progressive diffuse thickening of the pachymeningitis with disappearance of pituitary apoplexy. Lumbar puncture showed lymphocytosis with no organisms. Open biopsy of the meninges was performed and histology showed Idiopathic hypertrophic pachymeningitis mimicking prolactinoma with recurrent vision loss

show abstract

“…Idiopathic hypertrophic pachymeningitis is a chronic, progressive, diffuse, inflammatory fibrosis of the dura mater [13][14][15]22]. Idiopathic hypertrophic spinal pachymeningitis (IHSP) is a rare inflammatory disorder that leads to spinal cord compression.…”

Section: Introductionmentioning

confidence: 99%

Long-term outcome of idiopathic hypertrophic thoracic pachymeningitis

et al. 2011

View full text Add to dashboard Cite

Idiopathic hypertrophic spinal pachymeningitis (IHSP) is a comparatively rare disease characterized by hypertrophic inflammation of the dura mater and clinical symptoms that progress from local pain to myelopathy. We report a case of IHSP followed up for 20 years in a 46-year-old man. Expansive laminoplasty was performed in 1991, and this case has been previously reported by a co-author. After 17 years, the patient's gait disturbance returned. Physical examination and imaging confirmed IHSP that had developed into syringomyelia at the T2-L1 conus level. This case was diagnosed as adhesive spinal arachnoiditis due to pachymeningitis caused by syringomyelia. T1-T4 laminectomy, a syringo-subarachnoid shunt (S-S shunt), and L2-L3 laminectomy were performed. The patient again developed dysesthesia and gait disturbance 3 years after the second operation. Most reports of IHSP have limited their focus to short-term follow-up after initial treatment with no long-term results. At present, there are only five reports referring to long-term results of greater than 5 years. All but one case needed additional surgery. To the best of our knowledge, this is the first case in which syringomyelia occurred in a patient with IHSP. It is important to note that syringomyelia may be a cause of symptom recrudescence during long-term follow-up in IHSP patients.

show abstract

Idiopathic hypertrophic pachymeningitis

Cited by 356 publications

References 37 publications

Hypertrophic pachymeningitis is a characteristic manifestation of granulomatosis with polyangiitis: A retrospective study of anti‐neutrophil cytoplasmic antibody‐associated vasculitis

Hypertrophic pachymeningitis is a characteristic manifestation of granulomatosis with polyangiitis: A retrospective study of anti‐neutrophil cytoplasmic antibody‐associated vasculitis

Idiopathic hypertrophic pachymeningitis mimicking prolactinoma with recurrent vision loss

Long-term outcome of idiopathic hypertrophic thoracic pachymeningitis

Contact Info

Product

Resources

About