Secondary trigeminal autonomic cephalgia with cavernous sinus involvement: a diagnostic challenge

Area of Science:

• Neurology
• Immunology
• Rheumatology

Background:

• Chronic headaches refractory to treatment require investigating underlying causes.
• IgG4-related disease (IgG4-RD) can present with hypertrophic pachymeningitis (HPM), affecting cranial nerves and mimicking other headache disorders.
• Extrapulmonary tuberculosis is a common mimic in endemic areas, complicating diagnosis.

Purpose of the Study:

• To highlight a rare case of chronic hemicranial headache caused by IgG4-related hypertrophic pachymeningitis.
• To emphasize the diagnostic challenges posed by co-existing conditions like tuberculosis.
• To underscore the importance of advanced diagnostic tools and personalized treatment for IgG4-related HPM.

Main Methods:

• Detailed clinical case presentation.
• Review of advanced neuroimaging findings.
• Serological marker analysis for IgG4-related disease.
• Consideration of differential diagnoses including tuberculosis.

Main Results:

• A patient with chronic, refractory hemicranial headache was diagnosed with IgG4-related hypertrophic pachymeningitis.
• The diagnosis was complicated by concurrent extrapulmonary tuberculosis.
• Advanced neuroimaging and serological markers were key to accurate diagnosis.

Conclusions:

• IgG4-related hypertrophic pachymeningitis is a rare but important cause of refractory headaches.
• Diagnostic challenges necessitate a high index of suspicion and comprehensive workup.
• Personalized therapeutic strategies and long-term follow-up are essential due to relapse potential.