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Hypertrophic Pachymeningitis (HP). Overview of the Underlying etiology, presentation & management
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| Author:
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DR. AHMED, DR. MUHAMMED HAMEED FAEADH. AL JUMAILY, DR. RANA HANI MOHAMMED ALI AL-SHAIKH HAMED
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| Abstract:
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Hypertrophic pachymeningitis (HP) is a rare chronic inflammatory dural pathology. It is classified into idiopathic by
exclusion, or associated with underlying pathology like tuberculous meningitis, rheumatoid arthritis, syphilis,
Wegener’s granulomatosis, IgG4 related disease and neoplasm. Objective: we try to review the etiology, clinical
presentation & management & to review the literature on HP. this was retrospective study of 12 patients (7 men, 5
women), with age range between 20 to 75 years), they were diagnosed as HP (8 cranial & 4 spinal) by imaging studies
& histopathology. The clinical features, serological, biochemical & radiological studies, treatment & outcome were
followed over one year. The underlying etiology were so that 5 cases were idiopathic (IHP), 3 cases were associated
with tuberculous meningitis, 2 cases were diagnosed in patients with rheumatoid arthritis & 2 cases were confirmed
to be IgG4 related disease. The main clinical features at presentation were headache, cranial nerves neuropathy in
cranial HP & limb sensory/motor dysfunction in spinal HP. On MRI, the enhancement & thickening of the dura mater
correlated with the clinical presentation. Histopathological study from 8 patients demonstrated infiltrates of small
mature lymphocytes, plasma cells, macrophages and epithelioid histiocytes. Corticosteroid therapy improved or
stabilized the condition in 9 of 12 cases. Four cases need surgical intervention & have improvement. Three cases had
progressive worsening & deterioration despite steroid therapy, two of them refused surgery & referred for
chemotherapy but we missed them from our study without a feedback. Follow-up MRI performed in all patients. the
diagnosis of HP is based on serological, MRI & histopathological studies. Idiopathic cases were diagnosed by exclusion.
Steroid therapy control the disease progression. In symptomatic cases with mass effect & neurodeficit, surgery is
indicated.
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| Keyword:
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Pachymeningits, IgG4 Related Disease.
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| EOI:
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| DOI:
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https://doi.org/10.31838/ijpr/2019.11.01.082
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| Download:
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