Landau-Kleffner Syndrome (LKS)

Rare syndrome of childhood period characterized by aphasic epilepsy of unknown origin resulting in severe language dysfunction (e.g., loss of previously acquired speech and language skills [aphasia]) and auditory agnosia).

Acquired Epileptiform Aphasia; Epileptic Aphasia; Infantile Acquired Aphasia.

Neurologic disorder first described in 1957 under the name of Acquired Epileptic Aphasia.

No data available, but a rare form of epilepsy. Male predominance (2:1).

None.

The cause of LKS is not known. Possible suggested mechanisms include a dysfunctional immune system, exposure to a virus, and brain trauma.

Electroencephalography (EEG) tracings are typical (present even in children with no clinical seizures); there is an epileptiform electrical activity in one but sometimes both temporal lobes, particularly when the child is asleep (“continuous spike waves of sleep” or “electrical status epilepticus of sleep”).

LKS usually starts between the ages of 3 and 8 years and may develop either slowly over months or rapidly overnight. Most children have seizures that are readily controlled by antiepileptic drugs, but some children never have obvious seizures. Children with LKS have a language disorder that affects comprehension and/or understanding. They become unable to understand their own name, to identify environmental sounds such as telephone ringing, and to recognize family and friends or common objects, such as food and clothes. They may appear to be deaf. Some patients lose their speech completely and have behavioral problems (hyperactivity, poor attention, depression, and irritability). Occasionally, they have other neurologic problems, such as loss of bladder and bowel control, and visual disturbances (they can see but are unable to understand what they see). In some children who do not respond to steroids, a new brain surgery procedure called multiple subpial transection may be successful. Some affected children may regain some of their language abilities (over months or years), sometimes with remissions and relapse. Seizures generally disappear by adulthood.

An anesthesiology consultation before elective surgical procedures is highly recommended. Complete evaluation of the epileptic disorder and the pharmacological therapy used to control the disease must be obtained before anesthesia.

The induction of anesthesia must take into consideration the possibility of behavioral problems, communication, and sudden burst of epileptic activities under stress. Use of sedation as premedication must be considered to reduce the risk of epilepsy.

It is important to review the list of anticonvulsant medications used to control the disease and assess their potential interactions with the anesthetic agents. Special consideration must be given to the patient receiving chronic corticosteroid administration when intraoperative steroid supplementation must be given.

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