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Speech-induced aphasic seizures in epilepsy caused by LGI1 mutation
Eylert Brodtkorb1, Ralf P Michler, Wenli Gu
1Department of Neurology, St. Olav's Hospital, University Hospital, Trondheim, Norway. eylert.brodtkorb@medisin.ntnu.no
Epilepsia
|June 11, 2005
Summary
This study documents the first video-EEG recorded speech-induced seizure in autosomal dominant lateral temporal lobe epilepsy (ADTLE) linked to an LGI1 mutation. The findings suggest LGI1
Area of Science:
- Neuroscience
- Genetics
- Epileptology
Background:
- Autosomal dominant lateral temporal lobe epilepsy (ADTLE) is a genetic epilepsy syndrome.
- Seizures in ADTLE can be triggered by external stimuli such as sound or speech.
- Mutations in the LGI1 gene are a common cause of ADTLE.
Observation:
- A 23-year-old male patient with LGI1-associated ADTLE experienced speech-induced seizures.
- Seizures were characterized by sensory aphasia, distorted speech perception, and generalized tonic-clonic (GTC) seizures.
- Video-EEG monitoring captured seizure activity with frontotemporal rhythmic 6-Hz activity following speech provocation.
Findings:
- The patient presented with unique seizure semiology involving comprehension difficulties and aphasia upon verbal address.
- Interrogative speech successfully triggered GTC seizures with specific EEG patterns.
- This is the first documented video-EEG recording of a seizure in LGI1-caused ADTLE.
Implications:
- Speech-induced seizures and their specific semiology may serve as a diagnostic marker for LGI1-related ADTLE.
- The LGI1 gene might play a role in the neurophysiologic basis of human speech and language processing.
- Further research into LGI1's function could reveal novel therapeutic targets for epilepsy and language disorders.