Other Familial Temporal Lobe Epilepsies

Etiologies: Autosomal dominant inheritance, the penetrance rate is about 60%, and some reports are related to the variation of DEPDC5 gene [1].

Age of onset: seizure onset commonly in adolescent or adult years (median about 25 years).

Seizure characteristics: seizures are usually mild and infrequent, easy to be controlled by antiepileptic drugs, and have focal seizures with temporal lobe involvement characteristics (deja vu, experiential phenomena and hallucinations, which can appear together with autonomic nerve symptoms, fear, light and sound distorted hallucinations, difficult to locate numbness and tingling somatosensory), and some can be extended to bilateral tonic clonic seizures [2-3].

EEG: Background: most are normal. Interictal: it can be normal, and some can show unilateral, mild slow wave or epileptiform discharge limited to the temporal region. Ictal: there are inadequate published records of ictal EEG in this syndrome.

Brain MRI: most of them are basically normal, and some serious cases may have hippocampal atrophy.

Developmental progress: Most of them are basically normal.

References

1. Ishida, S., et al., Mutations of DEPDC5 cause autosomal dominant focal epilepsies. Nat Genet, 2013. 45(5): p. 552-5.
2. Panayiotopoulos.  癫痫综合征及临床治疗.  北京 : 人民卫生出版社, 2012.
3. 刘晓燕.  临床脑电图学. 第2版.  北京 : 人民卫生出版社, 2017.