We highlight an under-recognized epileptic pathology in a 56-year-old left-handed female with progressive right facial numbness and weekly focal seizures characterized by episodic aphasia. Additionally, we provide a thorough literature review of reported IDME patients along with their clinical presentation and outcomes.

56-year-old left-handed female presented with progressive right facial numbness and focal seizures characterized by episodes of aphasia for a year. Episodes are characterized by worsening right facial numbness followed by garbled speech, word-finding difficulty, and grammatical errors when she attempts to talk or text over the phone, while she remains conscious and is aware of her surroundings. Her only epilepsy risk factor was minor head trauma ten years prior to presentation. Episodes were evaluated in the Epilepsy Monitoring Unit at Cleveland Clinic Foundation. An ictal event characterized by rhythmic sharply contoured delta and theta activity in the left hemisphere, most prominently in the left frontocentral region, This was clinically associated with numbness in the right side of the face progressing into an aphasic seizure.  She was found to have left frontoparietal intradiploic meningoencephalocele (IDME) on imaging studies.  Positron emission tomography (PET) showing mild metabolism corresponding to Intradiploic meningoencephalocele in left parietal bone, and subtle hypometabolism in the adjacent posterior left frontal lobe operculum.  She had a craniotomy for encephalocele resection and mesh cranioplasty. At 3-year follow-up visit, patient was seizure free without residual neurological deficits. She remains on lacosamide 100 mg BID as seizures recurred after attempting to discontinue it.