Multiple cranial nerve palsy revealing hypertrophic pachymeningitis with positive myeloperoxidase-antineutrophil cytoplasmic antibody

Aoud, S. El; Frikha, F.; Salah, Raida Ben; Snoussi, M.; Loukil, H.; Bahloul, Z.

doi:10.4081/reumatismo.2013.248

Cited by 3 publications

(2 citation statements)

References 18 publications

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“…The etiology of HP is multifaceted and varied, encompassing factors such as autoimmune diseases (e.g., rheumatoid arthritis), infections (e.g., tuberculosis, syphilis), systemic conditions (e.g., IgG4-related diseases), neoplasms (e.g., lymphoma), and other confounding elements. Instances of HP without a clear etiology are termed idiopathic HP (IHP) [9][10][11]. Predominantly, headache constitutes the principal clinical manifestation [12,13], and the second most frequent presentation involves cranial nerve deficits, with the optic nerve being the most commonly affected, followed by cranial nerves III, IV, V, VI, and VIII [14].…”

Section: Discussionmentioning

confidence: 99%

Hypertrophic Pachymeningitis: An Unusual Cause of Headache

Enabi,

Sharif,

Venkatesan

et al. 2024

Cureus

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Hypertrophic pachymeningitis (HP) is a rare condition characterized by inflammation and thickening of the dura mater. It can be idiopathic or secondary to various causes, including infections, tumors, or systemic inflammatory diseases. Diagnosis is challenging due to its rarity and the overlap of symptoms with other conditions. We present the case of a 42-year-old Hispanic woman with diabetes mellitus type 2 and end-stage kidney disease who presented with chest pain, dry cough, mild dyspnea, and chronic occipital headaches. Physical examination revealed cranial VI nerve palsy. Imaging showed pulmonary cavitary lesions and mediastinal lymphadenopathy. Elevated inflammatory markers and positive autoimmune tests, including rheumatoid factor and antineutrophil cytoplasmic antibody (ANCA), led to further investigation. Brain imaging revealed dural thickening, confirming HP. The patient's medical history revealed double ANCA positivity and a lung biopsy confirmed granulomatous pneumonitis. A diagnosis of ANCA-associated vasculitis (granulomatosis with polyangiitis (GPA)) was established, and treatment with rituximab and high-dose corticosteroids led to symptom improvement. GPA rarely involves meningeal inflammation, but severe and persistent headaches are common early symptoms. Inflammatory markers are often elevated, and around two-thirds of HP cases related to GPA have positive serum ANCA. MRI is the primary diagnostic tool, with characteristic findings of dural thickening and contrast enhancement. This case highlights HP as a rare cause of chronic headaches and the importance of a comprehensive medical history in diagnosis. Early recognition and treatment are crucial for improving outcomes in GPA-related HP.

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Section: Discussionmentioning

confidence: 99%

Hypertrophic Pachymeningitis: An Unusual Cause of Headache

Enabi,

Sharif,

Venkatesan

et al. 2024

Cureus

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“…1 The inflammation may be idiopathic or secondary to other pathological processes including infective processes (commonly tuberculosis, neurosyphylis, fungal infections), underlying neoplasms (commonly lymphomas), autoimmune conditions (rheumatoid arthritis, polyarteritis nodosa, antineutrophil cytoplasmic autoantibody (ANCA)-related vasculitides) or systemic illness (sarcoid, tuberous sclerosis, immunoglobulin G (IgG)4-related systemic disease). 2,3 The combination of systemic lupus erythematosus (SLE) and HP is extremely rare, with only six other cases reported in the literature according to our knowledge. 4–9 Herein, we describe the first case of multiple cranial palsies as a complication of underlying SLE pachymeningitis.…”

Section: Introductionmentioning

confidence: 99%

SLE pachymeningitis and multiple cranial nerve palsies: a case report and review of the literature

John

Plessis

et al. 2019

Lupus

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Hypertrophic pachymeningitis (HP) is a relatively uncommon disease associated with focal or diffuse thickening of the dura mater secondary to underlying chronic inflammation. The link between systemic lupus erythematosus (SLE) and hypertrophic pachymeningitis (HP) is extremely rare, with only six other cases reported in the literature. We, however, report the first case of SLE pachymeningitis presenting with multiple cranial nerve palsies. The patient showed good response to steroids and cyclophosphamide therapy. One should maintain a high index of suspicion to make the diagnosis in patients with SLE presenting with neurological dysfunction. Prompt therapy prevents long-term neurological sequelae.

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A case report: Diagnosis and treatment of idiopathic hypertrophic pachymeningitis

Luo

Cao

et al. 2023

Ibrain

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A 57‐year‐old man who suffered from a headache for 1 year, accompanied by blurred vision for 7 months and numbness in his left face for 1 week was admitted to the Affiliated Hospital of Zunyi Medical University on May 7, 2022. One year ago, the patient had no obvious precipitating factor of paroxysmal stabbing pain in the whole skull with dizziness, which could be relieved by oneself after lasting for 1–2 min each time, with about 20 episodes per day. The cranial magnetic resonance imaging revealed changes in bilateral frontal lobe ischemic foci, bilateral frontal, ethmoid, sphenoid and maxillary sinusitis, and retinal macular degeneration. After hormone shock treatment, the condition improved. He suffered from headaches again with blurred vision in the right eye 7 months ago and was initially diagnosed with multiple sclerosis. He then was discharged after improvement due to hormone shock therapy. Oral hormone therapy was continued outside the hospital, but he stopped it due to drug side effects (details remained unclear). After cutting off, he developed a headache and visited our hospital once more, the relevant tests were performed and the patient was diagnosed with idiopathic hypertrophic pachymeningitis (IHP). The symptoms were slightly abated after hormone therapy. We hope that through this case report, we can deepen the clinicians' understanding of IHP, and improve the diagnosis rate of the disease through relevant examinations in future clinical work, so that patients can receive timely treatment and the mental pressure and economic burden caused by the disease on patients are reduced.

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Multiple cranial nerve palsy revealing hypertrophic pachymeningitis with positive myeloperoxidase-antineutrophil cytoplasmic antibody

Cited by 3 publications

References 18 publications

Hypertrophic Pachymeningitis: An Unusual Cause of Headache

Hypertrophic Pachymeningitis: An Unusual Cause of Headache

SLE pachymeningitis and multiple cranial nerve palsies: a case report and review of the literature

A case report: Diagnosis and treatment of idiopathic hypertrophic pachymeningitis

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