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Updated: Sep 13, 2025
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Published on: October 14, 2022
Reversible dementia associated with communicating hydrocephalus secondary to spinal hypertrophic pachymeningitis in
Yuki Terashima1, Yoshitaka Ueda1, Naoto Yokogawa1
1Department of Rheumatic Diseases, Tokyo Metropolitan Tama Medical Center, Fuchu, Tokyo, Japan.
Abstract:
Microscopic polyangiitis (MPA) is a subtype of antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV), a small-vessel vasculitis that can cause organ-threatening complications. Hypertrophic pachymeningitis is a rare, central nervous system manifestation of AAV rarely involving the spine. We herein report a patient with myeloperoxidase-ANCA-positive MPA presenting with progressive cognitive decline and gait disturbance associated with communicating hydrocephalus secondary to spinal hypertrophic pachymeningitis. The patient responded well to high-dose prednisolone and rituximab and showed significant clinical and radiological improvement without surgery. The present case not only demonstrated that spinal hypertrophic pachymeningitis in AAV can cause reversible dementia associated with communicating hydrocephalus but also highlighted the potential of timely immunosuppressive therapy to induce remission.
Insights
Microscopic polyangiitis (MPA), a form of ANCA-associated vasculitis, can cause spinal hypertrophic pachymeningitis. This rare condition led to reversible dementia and hydrocephalus in a patient, successfully treated with immunosuppressants.
Area of Science:
- Neurology
- Rheumatology
- Immunology
Background:
- Microscopic polyangiitis (MPA) is an ANCA-associated vasculitis (AAV) affecting small vessels.
- Spinal hypertrophic pachymeningitis is a rare central nervous system manifestation of AAV.
Purpose of the Study:
- To report a case of MPA presenting with spinal hypertrophic pachymeningitis causing cognitive decline and hydrocephalus.
- To highlight the efficacy of immunosuppressive therapy in managing this rare AAV complication.
Main Methods:
- Case report of a myeloperoxidase-ANCA-positive MPA patient.
- Clinical presentation, diagnostic findings, and treatment response were documented.
Main Results:
- The patient exhibited progressive cognitive decline and gait disturbance due to spinal hypertrophic pachymeningitis and communicating hydrocephalus.
- High-dose prednisolone and rituximab led to significant clinical and radiological improvement without surgical intervention.
Conclusions:
- Spinal hypertrophic pachymeningitis in AAV can cause reversible dementia and hydrocephalus.
- Prompt immunosuppressive therapy is effective in achieving remission for this rare manifestation of AAV.
