Headache to a Patient with the Coexistence of Chiari I Malformations and Primary Empty Sella Syndrome: A Case Report

Abstract

Background: Chiari I malformations (CM-I) and Primary Empty Sella syndrome (PESS) are rare disorders of structural defects within a skull. Headache is the most common presenting symptom in both conditions. However, CM-I and ESS comorbidity in patients with severe headaches is yet to be reported.

Case description: The patient was a 38-year-old woman with a one-year history of headaches at various localisations (occipital and bilateral frontal) preceded by mood agitation. Physical examination was significant for obesity. Magnetic resonance imaging (MRI) revealed a 6.4 mm cerebellar tonsil descent through the foramen magnum and hypointense (CSF-filled) flattened sella. The lipid panel showed elevated cholesterols (total) and low-density lipoprotein (LDL) levels.  She was medically managed, with no report of symptoms relapses at one-month and two-month follow-ups.

Conclusion: This is the first report on comorbid CM-I and PESS in adults presenting with cephalalgia. PESS and CM-I co-occurrence may present with mixed headache localisation; careful history-taking and imaging are mandatory for diagnosis confirmation. Future extensive studies are warranted to analyse the pathophysiological interplay between these two rare disorders.