Episodic lingual movements are subtle and difficult to detect.

Cerebral cavernous malformations (CCMs) are irregular clusters of enlarged blood vessels in the brain, with 20% being familial. Autosomal dominant KRIT1, CCM2, and PDCD10 account for 80% of familial cases. Focal seizures are the presenting symptom in 36% of cases. CCMs are best seen on magnetic resonance imaging (MRI) brain T2 gradient echo sequence.

A 4-year-old right-handed boy with long-standing expressive speech delay presented with new-onset, subacute, episodic, and involuntary tongue movements. The tongue movements involved the right lip and lasted 2-3 minutes, occurring every 30-45 minutes. Consciousness was preserved. He reported an alteration in orobuccal sensation. Neurological exam was normal except expressive language delay. Importantly on exam, his tongue movements were normal as was his palate. Family history was positive for paternal congenital hearing loss.

MRI brain showed multiple CCMs with the largest in the right posterior frontal lobe with subacute hemorrhage and mild edema. Video EEG showed right central epileptiform discharges and interictal slowing. Seizures were well controlled with levetiracetam 30mg/kg/day. Further investigation of the family revealed CCMs in four paternal relatives. CCM gene panel determined KRIT1 mutation. Genetics provided counseling of immediate family members. Surgery is pending language functional MRI mapping due to CCM proximity to speech and language areas.

Lingual seizures with normal clinical examination may mimic orobuccal seizures attributed to Rolandic epilepsy. However, epilepsia partialis continua should raise concern for lesional etiologies. As in this case, primary lingual seizures localize to the posterior frontal lobe. Autosomal dominant CCMs and other focal structural lesions should not be overlooked as a cause for focal seizures and should be considered when an apparent benign epilepsy has atypical features. MRI should be reviewed for multiple CCMs and genetic testing obtained to avoid missing affected family members even if initially asymptomatic.