See no evil, hear no evil…
Sarah Driscoll1, Ann P Murchison2, Jurij R Bilyk2
1Wills Eye Institute, Philadelphia, Pennsylvania, USA.
Abstract:
A 32-year-old woman presented with right-sided visual loss, hearing loss and diffuse headache. Examination confirmed CN-II, V1/2, and VIII neuropathies. MRI showed an infiltrative skull-base process involving the pachymeninges. Serology and histopathology confirmed idiopathic hypertrophic pachymeningitis.
Insights
Idiopathic hypertrophic pachymeningitis caused cranial nerve palsies, including visual and hearing loss, in a 32-year-old woman. This rare condition presented as an infiltrative skull-base mass on MRI.
Area of Science:
- Neurology
- Neuro-oncology
- Inflammatory diseases
Background:
- Idiopathic hypertrophic pachymeningitis (IHP) is a rare, chronic inflammatory condition characterized by thickening of the dura mater.
- It can mimic neoplastic or infectious processes, leading to diagnostic challenges.
- Cranial nerve involvement is a significant manifestation of IHP.
Observation:
- A 32-year-old woman presented with progressive right-sided visual loss, hearing loss, and diffuse headache.
- Clinical examination revealed deficits in cranial nerves II (optic), V1/2 (trigeminal), and VIII (vestibulocochlear).
- Magnetic resonance imaging (MRI) demonstrated an infiltrative process at the skull base, involving the pachymeninges.
Findings:
- Serological tests and histopathological examination of the affected meninges confirmed the diagnosis of idiopathic hypertrophic pachymeningitis.
- The findings indicated a non-neoplastic, inflammatory etiology for the dural thickening and associated neuropathies.
- The case highlights the diverse neurological presentations of IHP.
Implications:
- This case underscores the importance of considering IHP in the differential diagnosis of skull-base masses and cranial neuropathies.
- Accurate diagnosis of IHP is crucial for appropriate management and to avoid unnecessary aggressive treatments.
- Further research into the pathogenesis and treatment of IHP is warranted.
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