Landau-Kleffner Syndrome

Synonyms: Acquired epileptic aphasia

An acquired epileptic aphasia disorder in which children who have developed age-appropriate speech then experience language regression with verbal auditory agnosia, abnormal epileptiform activity, behavioral disturbances, and sometimes overt seizures.¹ Landau and Kleffner initially described acquired epileptic aphasia in 1957.² Landau-Kleffner syndrome has three required features:³

• An acquired receptive aphasia
• Temporo-parietal spike-wave discharges in the awake state
• Frequent generalised spike-wave discharges in sleep (electrical status epilepticus in sleep - ESES)

• The syndrome has been thought to be very rare but, with improved diagnostic techniques, the known prevalence may increase significantly.
• Currently, over 200 cases have been described in world literature
• There is a slight increase in incidence in boys
• Most cases do not have a well-defined cause. A few cases appear to have been secondary to low-grade brain tumors, closed head injury, neurocysticercosis and demyelinating disease.

• Onset of aphasia is usually between the ages of 3 and 8 years old
• The syndrome is typically characterised by an abrupt or gradual loss of language ability and inattentiveness to sound (auditory agnosia)
• Receptive language is often severely impaired
• Reading and writing may be preserved
• The child may be completely mute or have severe expressive speech problems
• Seizures occur in most cases and usually present between the ages of 4 and 10 years
• Seizures may be partial, generalised tonic-clonic, absence or myoclonic
• Many affected children have behavioural disturbances, including hyperactivity and decreased attention span, aggression and attacks of rage.

• This is very broad and includes other causes of epilepsy, autism, hyperactivity and reduced attention span, aggressive (e.g. rage attacks), oppositional, or psychotic behaviour and mental retardation. Some patients may appear deaf, autistic or psychotic.
• Head injury, brain neoplasms (e.g. low-Grade astrocytoma), and cerebrovascular thromboembolism and neurocysticercosis may all be associated with acquired aphasia, an epileptiform EEG and seizures.
• Neurodegenerative disorders, especially adrenoleukodystrophy, and acute disseminated encephalomyelitis may also produce a similar presentation.

• MRI is essential to rule out cerebrovascular thromboembolism, brain tumours, demyelination, neurodegenerative disease and central nervous system infections.
• Fluoro-deoxyglucose positron emission tomography reveals decreased metabolism in one or both temporal lobes. Single-photon emission computed tomography of the brain demonstrates decreased perfusion of the left temporal lobe.
• EEG: abnormalities are present in this syndrome but no consensus exists about what constitutes typical abnormalities.
• Brainstem auditory evoked potentials and hearing tests.

• Patients have special educational needs and require speech therapy. Speech therapy, including sign language, and a number of classroom and behavioural interventions are beneficial.¹
• Teaching sign language may help
• Psychotherapy may be indicated
• A ketogenic diet has been recommended but experience is very limited

Drugs

• Anticonvulsant medications have variable success
• As initial therapy, valproic acid or diazepam is often used. Subsequently, other antiepileptic drugs, corticosteroids, or intravenous immunoglobulin therapy are often used. Various corticosteroid regimens including oral prednisone, high doses of intravenous pulse corticosteroids, and ACTH have been reported to be effective.¹

Surgical

Multiple subpial transections (MST) has been used:⁴

• The cortex is sliced in parallel lines in the midtemporal gyrus and perisylvian area in order to prevent the spread of the epileptiform activity without causing cortical dysfunction.
• This treatment is reserved for patients who have not responded to multiple medical therapies, but has been followed in selected cases by a marked improvement in language skills and behaviour.
• There is currently no accepted consensus about suitable candidates for this procedure or evidence for its effectiveness.¹

• The overall long term prognosis for language development is poor.⁵ Between 25% and 50% maintain language function good enough to hold a job and have a normal social life.²
• Patients with an onset of language regression before the age 5 of years may have a worse prognosis and symptoms persisting for more than 1 year are predictive of poor language recovery.²
• Fluctuations in clinical severity and EEG changes are not unusual and short-term remissions may occur.²