+ Site Statistics
References:
54,258,434
Abstracts:
29,560,870
PMIDs:
28,072,757
+ Search Articles
+ Subscribe to Site Feeds
Most Shared
PDF Full Text
+ PDF Full Text
Request PDF Full Text
+ Follow Us
Follow on Facebook
Follow on Twitter
Follow on LinkedIn
+ Translate
+ Recently Requested

A case of myeloperoxidase anti-neutrophil cytoplasmic antibody (MPO-ANCA)-associated hypertrophic pachymeningitis presenting with multiple cranial nerve palsies and diabetes insipidus



A case of myeloperoxidase anti-neutrophil cytoplasmic antibody (MPO-ANCA)-associated hypertrophic pachymeningitis presenting with multiple cranial nerve palsies and diabetes insipidus



Rinsho Shinkeigaku 56(5): 334-337



A 61-year-old woman developed hearing difficulties and became thirsty after experiencing cold symptoms. A neurological examination revealed a loss of odor sensation, facial palsy, dysphasia, and dysarthria. Vocal cord palsy was observed during pharyngoscopy. Brain magnetic resonance imaging (MRI) showed a thickened pituitary stalk and swelling of the pituitary gland, but no high signal intensity regions were seen in the posterior portion of the pituitary gland. Gadolinium-enhanced MRI demonstrated a thickened dura mater over the anterior cranial fossa. A biopsy specimen of the thickened dura mater showed fibrosis, granulomatous inflammation, and necrotic foci. Blood tests detected myeloperoxidase anti-neutrophil cytoplasmic antibody (MPO-ANCA). The patient's urine osmolarity was low even though she exhibited hypernatremia. We diagnosed her with hypertrophic pachymeningitis associated with MPO-ANCA and diabetes insipidus. The patient received two courses of 5-day high-dose intravenous methylprednisolone (1.0 g/day), and was subsequently administered oral prednisolone, which gradually relieved her symptoms. However, the patient's symptoms recurred despite the high-dose prednisolone treatment. It was difficult to control the patient's symptoms in this case with oral prednisolone monotherapy, but combined treatment with cyclosporine resulted in sustained remission. It is considered that patients with MPO-ANCA-positive hypertrophic pachymeningitis require combination therapy with prednisolone and immunosuppressive agents at an early stage.

(PDF emailed within 0-6 h: $19.90)

Accession: 057052042

Download citation: RISBibTeXText

PMID: 27098904

DOI: 10.5692/clinicalneurol.cn-000859


Related references

A case of slowly progressive type 1 diabetes mellitus developing myeloperoxidase-specific anti-neutrophil cytoplasmic antibody-associated vasculitis with hypertrophic pachymeningitis manifesting as multiple cranial nerve palsy. Nihon Rinsho Men'eki Gakkai Kaishi 34(6): 510-515, 2012

Hypertrophic pachymeningitis with anti-neutrophil cytoplasmic antibody (p-ANCA), and diabetes insipidus. Acta Neurologica Scandinavica 104(6): 397-401, 2001

Multiple cranial nerve palsy revealing hypertrophic pachymeningitis with positive myeloperoxidase-antineutrophil cytoplasmic antibody. Reumatismo 65(5): 248-252, 2015

A case of idiopathic hypertrophic pachymeningitis presenting with chronic headache and multiple cranial nerve palsies: A case report. Medicine 96(29): E7549, 2017

Hypertrophic pachymeningitis: significance of myeloperoxidase anti-neutrophil cytoplasmic antibody. Brain 137(Pt 2): 520-536, 2014

Anti-neutrophil Cytoplasmic Antibody-associated Vasculitis Complicated by Periaortitis and Cranial Hypertrophic Pachymeningitis: A Report of an Autopsy Case. Internal Medicine 57(1): 107-113, 2017

Teaching Neuro Images : Myeloperoxidase-anti-neutrophil cytoplasmic antibody-positive hypertrophic pachymeningitis. Neurology 89(21): E253, 2018

Intracranial hypertrophic pachymeningitis with high perinuclear anti-neutrophil cytoplasmic antibody (p-ANCA) occurred in a patient on hemodialysis. Rinsho Shinkeigaku 42(3): 243-246, 2002

Case of systemic sclerosis presenting with alveolar hemorrhage and positive anti-neutrophil cytoplasmic myeloperoxidase antibody(MPO-ANCA) without pathological renal involvement. Ryumachi 43(4): 690-695, 2003

Anti-neutrophil cytoplasmic antibody-associated hypertrophic cranial pachymeningitis and otitis media: a review of literature. European Archives of Oto-Rhino-Laryngology 275(12): 2915-2923, 2018

A case of optic neuropathy, recurrent transverse myelopathy and hypertrophic pachymeningitis associated with perinuclear anti-neutrophil cytoplasmic antibodies (p-ANCA). Rinsho Shinkeigaku 35(5): 513-515, 1995

P-ANCA-positive Wegener's granulomatosis presenting with hypertrophic pachymeningitis and multiple cranial neuropathies: case report and review of literature. Neuropathology 20(1): 23-30, 2000

Case report of Wegener's granulomatosis presenting with multiple cranial nerve palsy and hypertrophic cranial pachymeningitis. Rinsho Shinkeigaku 47(2-3): 85-89, 2007

Rheumatoid factor positive hypertrophic cranial pachymeningitis in association with hypopituitarism and multiple cranial nerve palsies. Internal Medicine 40(9): 964-967, 2001

Neuro-Ophthalmic Manifestations of Hypertrophic Cranial Pachymeningitis Associated With Antineutrophil Cytoplasmic Antibody for Myeloperoxidase. Neuro-Ophthalmology 34(4): 308-310, 2010