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Idiopathic hypertrophic pachymeningitis mimicking hemicrania continua: An unusual clinical case
Antonio Russo1,2, Marcello Silvestro1,2, Mario Cirillo2,3
11 Headache Center, Department of Medical, Surgical, Neurological, Metabolic and Aging Sciences, University of Campania 'Luigi Vanvitelli', Naples, Italy.
Insights
Hemicrania continua (HC) can mimic other headache disorders. This case report details a patient whose HC symptoms were caused by idiopathic hypertrophic pachymeningitis (IHP), a rare inflammatory condition.
Area of Science:
- Neurology
- Neuroscience
- Inflammatory Disorders
Background:
- Hemicrania continua (HC) is a primary headache disorder with continuous unilateral pain and exacerbations.
- Similar clinical presentations can arise from secondary causes, necessitating differential diagnosis.
- Indomethacin responsiveness is a hallmark of HC, but not exclusive to it.
Observation:
- A 62-year-old man presented with a year-long headache consistent with HC, fully responsive to indomethacin.
- The patient subsequently developed diplopia due to sixth cranial nerve palsy ipsilateral to the headache.
- Clinical, laboratory, and neuroimaging findings indicated idiopathic hypertrophic pachymeningitis (IHP).
Findings:
- This report describes the first documented instance of a hemicrania continua phenotype caused by idiopathic hypertrophic pachymeningitis (IHP).
- Idiopathic hypertrophic pachymeningitis is a rare inflammatory condition causing dura mater thickening.
- IHP can manifest as progressive headache and cranial nerve involvement.
Implications:
- This case highlights the importance of considering secondary causes, such as IHP, in patients presenting with hemicrania continua.
- Accurate diagnosis of IHP is crucial for appropriate management and to prevent potential complications.
- Further research may elucidate the mechanisms linking IHP to headache syndromes.
Abstract:
Background Hemicrania continua (HC) is a primary headache syndrome characterized by a unilateral, moderate, continuous headache with exacerbations marked by migrainous and cranial autonomic symptoms. However, clinical phenotypes similar to primary HC may be subtended by several disorders. Case report We report the case of a 62-year-old man experiencing, over the previous year, a headache completely consistent with HC and its absolute responsiveness to indomethacin therapy. Later, the patient developed diplopia caused by sixth cranial nerve palsy ipsilateral to headache. In this frame, clinical, laboratory and neuroimaging characteristics supported the diagnosis of idiopathic hypertrophic pachymeningitis (IHP). Conclusions IHP is a rare fibrosing inflammatory disorder leading to a localized or diffuse dura mater thickening. IHP clinical manifestations are a progressively worsening headache and signs related to cranial nerves involvement and venous sinus thrombosis. Here, we report, for the first time, a HC phenotype subtended by IHP.
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