Landau-Kleffner Syndrome

Also known as acquired  epileptic aphasia

Etiologies: consider genetic correlation (some mutations of GRIN2A gene are found, and others may involve complex polygenic genetic patterns) [1].

Age of onset: onset between the ages of 2 and 8 years (peak 5 to 7 years).

Seizure characteristics: characterized by acute or subacute onset of acquired aphasia in a child with previous normal development and cognition (mainly verbal and auditory aphasia, i.e. unable to understand and express oral language). Seizures can be seen in about 3/4 of children, and there can be many types of seizures, but generalized tonic seizure should be excluded [2-3].

EEG: Background: it can be normal, focal or diffuse slowing. Interictal: about 80% of patients have continuous spike-and-wave in slow sleep (usually in the middle and posterior temporal region, but can also in other parts). Ictal: depending on different seizure types [2-3].

Brain MRI: Most of them are normal.

Developmental progress: It is mainly language impairment typically fluctuates, and some can be basically recovered after remission, and some will residual language impairment (especially if onset is earlier).

Reference

1. Lesca, G., et al., GRIN2A mutations in acquired epileptic aphasia and related childhood focal epilepsies and encephalopathies with speech and language dysfunction.Nat Genet, 2013. 45(9): p. 1061-6.
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.