Landau–Kleffner syndrome

 

Presentation

Subacute onset epileptic encephalopathy between 3 – 8 years age leading to progressive or fluctuating aphasia.

 

A previously healthy child acquires verbal auditory agnosia, unable to understand spoken language or noises; and stop responding verbally, sometimes assumed to be ‘autistic feature’. However, unlike children with ASD, prior language development is normal with no perinatal complications. There may be cognitive or behavioural disturbance with attention difficulties.

 

Seizures occur in most cases, and when present are infrequent, often in sleep and resolve with age.

These may be focal or generalized, atypical absences or atonic seizures.

 

Investigations

Prolonged EEG shows frequent spike discharges frequently during sleep & often over the temporal (speech) areas.

Electrical Status Epilepticus in Sleep (ESES; also called CSWS) is commonly noted, but can be missed in awake EEG.

 

Management

Suppressing seizures especially during sleep offers better prognosis, often treated with Prednisolone or Clobazam.

AEDs that may benefit are Valproate, Ethosuximide, Levetiracetam or their combination.

Avoid Carbamazepine.

Surgery with Multiple subpial transaction considered by tertiary centres.

Seizures and EEG abnormality resolve with age in most cases, but cognitive and language impairment often persists.

Input from Speech Therapy, Psychology and Educational support is helpful.