Idiopathic hypertrophic craniocervical pachymeningitis.

PURPOSE: Hypertrophic craniocervical pachymeningitis (HCP) is a rare disease causing chronic inflammatory hypertrophy of the cranial and spinal dura mater. To increase awareness of this condition, we report the details of a case here.

METHODS

We reviewed the case of a 78-year-old man presenting with a rare case of HCP and summarized the clinical features, laboratory evaluations and treatment of the case.

RESULTS

In this case, the HCP involved the intracranial dura and high cervical regions, manifesting as lower cranial nerve palsies, headache, and neck pain, developing over 7 months. Magnetic resonance imaging revealed thickening of the dura in the craniocervical region with peripheral enhancement. Steroid therapy was commenced and the symptoms improved rapidly.

CONCLUSIONS

HCP can be diagnosed by MRI and laboratory investigations. In this case corticosteroid treatment was effective, although care must be taken when slowly reducing the dose. This case highlights HCP as a cause of progressive cerebellomedullar and cervical spinal cord symptoms.