Is Pachymeningitis fatal?
The lesion responded poorly to pulsed steroids or any immunosuppressants. The brainstem lesion grew rapidly and formed various nodules that ultimately resulted in brain death. This is the first fatal case of HP in a child.
What is idiopathic hypertrophic Pachymeningitis?
Idiopathic hypertrophic pachymeningitis (IHP) is a rare disorder causing inflammation and thickening of the outer layer (dura) of the brain and/or spinal cord. [13281][13282] IHP can be widespread or cause tumor-like lesions.
What are the symptoms of Pachymeningitis?
Typical symptoms are chronic headaches, facial pain, cerebella ataxia, cranial nerve failure, neuro-ophthalmic complications, including papilloedema.
What causes hypertrophic Pachymeningitis?
HP is a rare chronic fibrosing inflammatory process that involves the cerebral and/or spinal dura mater. Causes include vascular abnormality, trauma, infection, neoplasm, and neuro-inflammatory conditions. IHP is diagnosed if no cause can be identified.
Is Pachymeningitis curable?
Previous reports found a favorable response of IgG4-RP to steroids, suggesting that this disease is benign and treatable. However, the long-term treatment response and clinical course remain unknown.
What causes Pachymeningitis?
As indicated by the presence of abundant pus, pachymeningitis most often results from a bacterial infection (usually staphylococcal or streptococcal) that is localized to the dura.
What causes thickening of the dura?
Localized or diffuse thickening of the dura mater may be seen in hypertrophic pachymeningitis that may be associated with rheumatoid arthritis, syphilis, Wegener’s granulomatosis, tuberculosis, and cancer [22–26].
What does Pachymeningitis mean?
Medical Definition of pachymeningitis : inflammation of the dura mater.
What are the treatment options for idiopathic hypertrophic pachymeningitis?
Idiopathic hypertrophic pachymeningitis: a report of two patients and review of the literature Although prompt and extensive surgery has been recommended for this condition, the results from our second patient indicate that complete remission can be achieved in some patients with biopsy and steroid therapy.
What is hypertrophic pachymeningitis?
Access to this article can also be purchased. Background: Hypertrophic pachymeningitis is an uncommon disorder that causes a localized or diffuse thickening of the dura mater and has been associated with rheumatoid arthritis, syphilis, Wegener’s granulomatosis, tuberculosis, and cancer.
Do MRI findings correlate with the clinical course of idiopathic hypertrophic pachymeningitis?
Objective: To investigate the clinical and laboratory evaluation, course, and treatment of patients with idiopathic hypertrophic pachymeningitis (IHP), to correlate the MRI findings with the clinical course, and to review the literature on IHP.
Is there a cure for hypertrophic cardiorespiratory disease?
Although prompt and extensive surgery has been recommended for this condition, the results from our second patient indicate that complete remission can be achieved in some patients with biopsy and steroid therapy. This also supports the view that autoimmune mechanisms underlie idiopathic hypertrophi …