The Case of a Twitchy Tongue: An Uncommon Presentation of a Common Childhood Epilepsy

Abstract

A 12-year-old previously healthy boy was evaluated in our neurology clinic for abnormal tongue movements, which were increasing in frequency for over a year. He described having multiple episodes where his tongue would become numb and start twitching. He was unable to speak or swallow and would drool profusely. He maintained awareness during the whole event, which lasted less than 1 minute without postictal changes. Initially, the episodes were nocturnal, interrupting his sleep, but later, they also occurred several times during the day. The patient had a previous work-up, including evaluations by a primary care provider, ear nose throat specialist, and magnetic resonance imaging of the brain, which was normal. A review of the video brought by the mother showed rhythmic clonic twitching of the left hemi-tongue along with subtle left facial droop and profuse drooling (Video 1; available at www.jpeds.com). The primary neurologist was concerned that these movements represented paroxysmal dyskinesia, and the patient was initiated on low dose carbamazepine with no improvement. There was no tongue atrophy or fasciculation to suggest a peripheral cause, and the phenomenology was not suggestive of motor stereotypies. The patient was developing appropriately with a normal neurologic examination and had no evidence of tremors or movement disorders. He was not taking any medications that could explain the symptoms. A routine electroencephalogram (EEG) was then obtained which was normal, so he was admitted for a 48-hour epilepsy monitoring unit (EMU) stay to characterize these episodes because of its stereotypical nature. During the EMU stay, multiple typical events were captured (Video 2; available at www.jpeds.com). Ictal EEG recording confirmed that they were epileptic seizures. Electrographically, these episodes showed a buildup of moderate to high voltage rhythmic theta frequency activity intermixed with sharp wave discharges over the right frontocentral head region (Figure). One of the events evolved into a bilateral tonic-clonic seizure. Between seizures, he was noted to have right central sharp discharges, which markedly increased in frequency with sleep, thus, confirming his diagnosis of benign partial epilepsy of childhood with centrotemporal spikes (BECTS). Carbamazepine was then switched to Oxcarbazepine, and he finally achieved seizure freedom 18 months after the episodes first started. A prolonged 24-hour EEG was repeated after he had remained seizure-free for 2 years, and it was normal. Oxcarbazepine was successfully weaned off without breakthrough seizures or new concerns. BECTS was suspected in our patient because of the typical age of presentation, clinical history of stereotypical nocturnal seizures, which evolves into diurnal seizures of mostly motor symptoms in a developmentally normal child as well as typical sleep activated right central sharp wave on the EEG. Typically, seizures in BECTS arise from the rolandic cortex (frontoparietal opercular region) and are characterized by unilateral facial (lip, mouth, and tongue) clonic movements associated with laryngeal symptoms, speech difficulty, swallowing or chewing movements, and hypersalivation. Consciousness and recollection are fully retained in more than one-half (58%) of these seizures.1Wirrell E.C. Benign epilepsy of childhood with centrotemporal spikes.Epilepsia. 1998; 39: S32-S41Crossref PubMed Scopus (127) Google Scholar Though tongue has a more extensive cortical representation in the rolandic cortex, isolated lingual seizures manifesting as paroxysmal lingual rhythmic movements2Jabbari B. Coker S.B. Paroxysmal, rhythmic lingual movements and chronic epilepsy.Neurology. 1981; 31: 1364-1367Crossref PubMed Google Scholar are a rare epileptic phenomenon that correlated with electrographic changes arising from the right frontocentral region in our case. Focal clonic seizures involving the tongue have been reported in the medical literature3Mizrahi E.M. Kellaway P. Characterization and classification of neonatal seizures.Neurology. 1987; 37: 1837-1844Crossref PubMed Google Scholar yet not commonly associated with childhood epilepsy as a primary presenting type of seizure. This case illustrates the difficulty and delay in diagnosing an unusual presentation of focal seizures (lingual seizures) even when associated with common childhood epilepsy. This seizure can be confused with a paroxysmal movement disorder because of preserved awareness. Even though it is a rare and unique presentation, recognition of such stereotypical movements with a consistent pattern of spread is the key to the seizure diagnosis as the seizure comes from the same focus with similar seizure propagation pathways. Further evaluation to characterize the episodes by obtaining video EEG ensures appropriate diagnosis and prompt treatment. eyJraWQiOiI4ZjUxYWNhY2IzYjhiNjNlNzFlYmIzYWFmYTU5NmZmYyIsImFsZyI6IlJTMjU2In0.eyJzdWIiOiJhNDg1NDkyZmIxY2YyZmFiMTQwMmI1ZGE3NmVkNmZlNSIsImtpZCI6IjhmNTFhY2FjYjNiOGI2M2U3MWViYjNhYWZhNTk2ZmZjIiwiZXhwIjoxNjc5MTM4MjM3fQ.pvsoO3CY7oxABk_LkwzuQ5j-YfDMNwmB16JYm2-S4K0tWbvCH3YrKxIwRAQ5NpogbVoqIpTjFsWJe1ei0LsVopsj-CJAzsgSnB65E8kxNdrLckv6aocS09_4TOwYa86oL4Xg3LaD4GSaRVEHfqzb4NhqZVgpFqz5VsCMcFXmKRVZwmKfo6GyDErvh4csBEp6WYU6rKatstYPb2ijLigHL-h98Vtfpq2LxXw0xs7GvVIV1Ocn-pcJA2AAcjA8rUjJJKqP9rn2k1MxfYVtEnnBEw3ncva5HhazsrN69lCX3LHJQ7NwAct42oqoTBdPpjytBcU_xDxM95rTnQB8NfUl3Q Download .mp4 (7 MB) Help with .mp4 files Video 1eyJraWQiOiI4ZjUxYWNhY2IzYjhiNjNlNzFlYmIzYWFmYTU5NmZmYyIsImFsZyI6IlJTMjU2In0.eyJzdWIiOiI2ZGZmNDdlYjdiMzkwYWU2M2YyOGZhZmNiNDRiMDRhNiIsImtpZCI6IjhmNTFhY2FjYjNiOGI2M2U3MWViYjNhYWZhNTk2ZmZjIiwiZXhwIjoxNjc5MTM4MjM3fQ.GXJX-PHsvuJWm5hflDLZa7K7Lcn1lr8v5zWQlEOARPfu-cRsXU-buR6Cu_JN30bTgXEZUlCvWYyw_z59D-fuZy37lt6rBAcgeJqoCMQ6muuNQPGAifn-Rqxo1WYwa7_ywtFHx8cux7rjj5Cs8q5WKYO0LRJNKy_EdwfxRskoYCwjD97tBDQQxcFhDpkFpVHgJ7sAcOqjb2W0xa69ckVdqLLmGIx813kqtTP9Il0hi0tklfLSr3moipwUePALuItndalTcFYkMCL4ijsLdTF70SaAWT4vdqGC9e_Qjp7oB264Euy8xycym-Ow_6uOrGv-Io6UaFpYAL1QT1UVB2IJlw Download .mp4 (14.96 MB) Help with .mp4 files Video 2