- •Idiopathic hypertrophic cranial pachymeningitis (IHCP) is a rare, chronic inflammation of the dura.
- •Seizures and status epilepticus are an uncommon presentation of this entity.
- •Two cases are presented of patients manifesting focal status epilepticus secondary to IHCP.
- •Anti-inflammatory treatment is an effective addition to antiepileptic drugs in these patients.
Idiopathic hypertrophic cranial pachymeningitis (IHCP) is an uncommon disease of unknown etiology characterized by thickening of the cerebral dura mater with possible associated inflammation. The most frequently described clinical symptoms include headache, cranial nerve palsy, and cerebellar dysfunction. Epilepsy and/or status epilepticus as main presentation is very uncommon.
Two consecutive cases are presented of patients manifesting focal status epilepticus secondary to IHCP, with clinical, laboratory [blood test and cerebrospinal fluid (CSF) analysis], neuroradiologic [magnetic resonance imaging (MRI) at 3 Tesla and digital subtraction angiography (DSA)], and therapeutic data. One patient underwent meningeal biopsy; pathology findings are also included. Corticosteroid therapy resulted in clinical improvement in both cases, and neuroimaging showed decreased abnormal morphology, compared to initial findings.
In the diagnostic approach to focal status epilepticus or epilepsy, IHCP must be considered a potential, although extremely infrequent, cause. Anti-inflammatory treatment is an effective addition to antiepileptic drug therapy in patients with IHCP.
Abbreviations:IHCP (Idiopathic hypertrophic cranial pachymeningitis), CNS (central nervous system), MRI (magnetic resonance imaging), DSA (digital subtraction angiography), CSF (cerebrospinal fluid), ELISA (enzyme-linked immunosorbent assay)
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Published online: June 10, 2016
Accepted: June 9, 2016
Received in revised form: May 27, 2016
Received: December 24, 2015
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