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"Idiopathic" cranial hypertrophic pachymeningitis responsive to antituberculous therapy: case report
I F Parney1, E S Johnson, P B Allen
1Department of Surgery, University of Alberta, Edmonton, Canada.
Objective And Importance:
We present a case similar to previously described cases of idiopathic hypertrophic cranial pachymeningitis. However, our patient responded to antituberculous therapy. This raises the possibility that some cases of "idiopathic" hypertrophic cranial pachymeningitis may represent occult tuberculous disease.
Clinical Presentation:
A 55-year-old woman presented with a right fourth nerve palsy and a 5-month history of headaches. Magnetic resonance imaging with gadolinium revealed thick enhancing dura on the right half of the tentorium cerebelli, with edema of the adjacent midbrain, pons, and cerebral peduncle.
Intervention:
Open biopsy of the tentorial lesion revealed only dense fibrosis with histiocytic infiltration. An exhaustive search failed to demonstrate an underlying cause. In particular, mycobacterial stains/cultures were negative, there was no granuloma formation, and the chest x-ray was unremarkable. However, because of a strongly positive purified protein-derivative skin test and residence in an area endemic for tuberculosis, the patient was placed on antituberculous medications.
Conclusion:
The patient's symptoms and signs resolved with antituberculous therapy. Resolution of the tentorial lesion was confirmed by gadolinium-enhanced magnetic resonance imaging. We conclude that this case represented occult tuberculous disease. An empiric trial of antituberculous therapy may be warranted in other cases of apparently idiopathic hypertrophic cranial pachymeningitis.
Insights
A case of hypertrophic cranial pachymeningitis initially deemed idiopathic responded to tuberculosis treatment. This suggests occult tuberculous disease may underlie some idiopathic cases, warranting empiric antitubercular therapy.
Area of Science:
- Neurology
- Infectious Diseases
- Radiology
Background:
- Idiopathic hypertrophic cranial pachymeningitis (IHCP) is a rare condition characterized by thickening of the dura mater.
- Diagnosis of IHCP typically involves excluding other causes of pachymeningitis.
- The etiology of IHCP remains largely unknown, leading to varied treatment approaches.
Observation:
- A 55-year-old woman presented with a fourth nerve palsy and chronic headaches.
- MRI revealed thickened, enhancing dura mater over the tentorium cerebelli with adjacent brainstem edema.
- Biopsy showed fibrosis and histiocytic infiltration, with negative stains/cultures for infection and no granuloma formation.
Findings:
- Despite negative initial investigations for tuberculosis, a positive purified protein-derivative (PPD) skin test and endemic residence prompted antituberculous therapy.
- The patient experienced complete resolution of symptoms and radiographic evidence of dural thickening after treatment.
- This outcome strongly suggests the underlying cause was an occult tuberculous infection.
Implications:
- This case challenges the traditional definition of IHCP by demonstrating a treatable infectious etiology.
- Occult tuberculous pachymeningitis should be considered in the differential diagnosis of IHCP, especially in endemic areas.
- Empiric antituberculous therapy may be beneficial for patients with suspected idiopathic hypertrophic cranial pachymeningitis.