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Related Concept Videos

Cranial and Spinal Meninges01:19

Cranial and Spinal Meninges

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The cranial and spinal meninges are complex protective structures surrounding the central nervous system (CNS), consisting of the brain and spinal cord. These meninges consist of the dura mater, the arachnoid mater, and the pia mater. They protect the CNS, provide structural support, and aid in circulating cerebrospinal fluid (CSF).
Cranial Meninges
These meningeal layers cover the cranium. The dura mater is the outermost layer of cranial meninges. It is a thick and durable membrane of dense...
1.7K

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Related Experiment Video

Updated: Sep 12, 2025

Inducing Meningococcal Meningitis Serogroup C in Mice via Intracisternal Delivery
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Seronegative pachymeningitis.

Bette K Kleinschmidt-DeMasters

    Clinical Neuropathology
    |August 8, 2025
    PubMed
    Summary

    Diagnosing hypertrophic pachymeningitis is complex. This study highlights two seronegative cases with features of granulomatosis with polyangiitis (GPA), emphasizing the need for clinical-serological correlation in autoimmune conditions.

    Area of Science:

    • Neuropathology
    • Immunohistochemistry
    • Autoimmune Diseases

    Background:

    • Hypertrophic pachymeningitis (HP) diagnosis is challenging, often requiring exclusion of infectious and neoplastic etiologies.
    • Autoimmune conditions like granulomatosis with polyangiitis (GPA) and IgG4-related disease (IgG4-RD) are key differentials for HP.
    • Potential overlap between GPA and IgG4-RD complicates diagnosis.

    Purpose of the Study:

    • To present two seronegative cases of pachymeningitis with histological features mimicking GPA.
    • To compare these cases with a seropositive GPA-associated pachymeningitis case.
    • To discuss the diagnostic challenges and implications for autoimmune conditions.

    Main Methods:

    • Histopathological analysis of surgical pachymeningitis specimens.

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  • Clinical and serological data review.
  • Immunohistochemical assessment for IgG4-expressing plasma cells.

    • Main Results:

    • Two male patients (73 and 75 years) presented with neurological symptoms and dural thickening.
    • Histology revealed features identical to GPA, including fibrosis, lymphoplasmacytic infiltrates, giant cells, and necrosis.
    • Vessel vasculitis was noted in one case; elevated IgG4+ cells were observed in both, with varying IgG4:IgG ratios.

    Conclusions:

    • Histological findings in HP require careful interpretation and clinical-serological correlation for definitive diagnosis.
    • Seronegative HP cases with GPA-like features raise questions about disease variants or overlap syndromes.
    • Distinguishing between GPA, IgG4-RD, or co-existing disorders is crucial for patient management.