Ictal aphasia in LGI1-related autosomal dominant epilepsy with auditory features. Issue 4 (30th March 2022)

• Record Type: Journal Article
• Authors: Moloney, Patrick B; McHugh, John; O'Byrne, James; Llamas, Yudy; Lynch, Tim; McGovern, Eavan
• Is Part Of: Practical neurology. Volume 22:Issue 4(2022)
• Page Start: 317
• Page End: 320
• Publication Date: 2022-03-30
• Subjects: EPILEPSY -- GENETICS -- EEG; Neurology -- Periodicals; 616.8005
• DOI: 10.1136/practneurol-2022-003366
• Languages: English
• ISSNs: 1474-7758

Abstract

Abstract : Autosomal dominant epilepsy with auditory features (OMIM 600512) is characterised by focal seizures with distinctive auditory auras and/or ictal aphasia. We describe a 17-year-old girl with recurrent attacks of ictal aphasia and rare nocturnal convulsions. She had a four-generation paternal family history of epilepsy. Her father and aunt perceived bells ringing at the onset of seizures. Sequence analysis of the leucine-rich glioma-inactivated 1 ( LGI1 ) gene identified a novel heterozygous variant in the proband and her father. LGI1 -related genetic epilepsy has a benign clinical course with a favourable response to anti-seizure medications. Auditory or vertiginous seizures may be mistaken for peripheral audio-vestibular symptoms, while complex auditory ictal symptoms may be misattributed to primary psychiatric disorders. Recognising this distinctive inherited syndrome should prompt targeted analysis of the LGI1 gene.