Landau-Kleffner syndrome
Encyclopedia
Landau–Kleffner syndrome (LKS), also called infantile acquired aphasia, acquired epileptic aphasia or aphasia with convulsive disorder, is a rare, childhood neurological syndrome
Syndrome
In medicine and psychology, a syndrome is the association of several clinically recognizable features, signs , symptoms , phenomena or characteristics that often occur together, so that the presence of one or more features alerts the physician to the possible presence of the others...

.

It is named for William Landau
William Landau
William M. Landau is a professor of neurology at the Washington University School of Medicine in St. Louis. It was within his specialty of stroke and movement disorders that he gained eponymous recognition for the Landau–Kleffner syndrome.-Works published:...

 and Frank Kleffner
Frank Kleffner
Frank R. Kleffner is a professor at Washington University in St. Louis, noted for his contributions in the fields of speech and language pathology and hearing....

, who characterized it in 1957.

Presentation

It is characterized by the sudden or gradual development of aphasia
Aphasia
Aphasia is an impairment of language ability. This class of language disorder ranges from having difficulty remembering words to being completely unable to speak, read, or write....

 (the inability to understand or express language) and an abnormal electroencephalogram
Electroencephalography
Electroencephalography is the recording of electrical activity along the scalp. EEG measures voltage fluctuations resulting from ionic current flows within the neurons of the brain...

 (EEG). LKS affects the parts of the brain that control comprehension and speech (Broca's area
Broca's area
Broca's area is a region of the hominid brain with functions linked to speech production.The production of language has been linked to the Broca’s area since Pierre Paul Broca reported impairments in two patients. They had lost the ability to speak after injury to the posterior inferior frontal...

 and Wernicke's area
Wernicke's area
Wernicke's area is one of the two parts of the cerebral cortex linked since the late nineteenth century to speech . It is involved in the understanding of written and spoken language...

). The disorder usually occurs in children between the ages of 3 and 7 years. Typically, children with LKS develop normally but then lose their language
Language
Language may refer either to the specifically human capacity for acquiring and using complex systems of communication, or to a specific instance of such a system of complex communication...

 skills. While many of the affected individuals have clinical seizures, some only have electrographic seizures, including electrographic status epilepticus of sleep (ESES).

Differential diagnosis

The syndrome can be difficult to diagnose and may be misdiagnosed as autism
Autism
Autism is a disorder of neural development characterized by impaired social interaction and communication, and by restricted and repetitive behavior. These signs all begin before a child is three years old. Autism affects information processing in the brain by altering how nerve cells and their...

, pervasive developmental disorder, hearing impairment, learning disability, auditory/verbal processing disorder, attention deficit disorder
Attention-deficit hyperactivity disorder
Attention deficit hyperactivity disorder is a developmental disorder. It is primarily characterized by "the co-existence of attentional problems and hyperactivity, with each behavior occurring infrequently alone" and symptoms starting before seven years of age.ADHD is the most commonly studied and...

, mental retardation, childhood schizophrenia
Schizophrenia
Schizophrenia is a mental disorder characterized by a disintegration of thought processes and of emotional responsiveness. It most commonly manifests itself as auditory hallucinations, paranoid or bizarre delusions, or disorganized speech and thinking, and it is accompanied by significant social...

, or emotional/behavioral problems.

Treatment

Treatment for LKS usually consists of medications, such as anticonvulsant
Anticonvulsant
The anticonvulsants are a diverse group of pharmaceuticals used in the treatment of epileptic seizures. Anticonvulsants are also increasingly being used in the treatment of bipolar disorder, since many seem to act as mood stabilizers, and in the treatment of neuropathic pain. The goal of an...

s and corticosteroid
Corticosteroid
Corticosteroids are a class of steroid hormones that are produced in the adrenal cortex. Corticosteroids are involved in a wide range of physiologic systems such as stress response, immune response and regulation of inflammation, carbohydrate metabolism, protein catabolism, blood electrolyte...

s (such as prednisone
Prednisone
Prednisone is a synthetic corticosteroid drug that is particularly effective as an immunosuppressant drug. It is used to treat certain inflammatory diseases and some types of cancer, but has significant adverse effects...

), and speech therapy, which should be started early.

A controversial treatment option involves a surgical technique called multiple subpial transection
Multiple subpial transection
In the treatment of epilepsy, if partial seizures originate in areas of the brain that cannot be removed safely, multiple subpial transections are an alternative. The surgeon makes a series of shallow cuts into the brain's cerebral cortex...

 in which multiple incisions are made through the cortex
Cerebral cortex
The cerebral cortex is a sheet of neural tissue that is outermost to the cerebrum of the mammalian brain. It plays a key role in memory, attention, perceptual awareness, thought, language, and consciousness. It is constituted of up to six horizontal layers, each of which has a different...

 of the affected part of the brain
Brain
The brain is the center of the nervous system in all vertebrate and most invertebrate animals—only a few primitive invertebrates such as sponges, jellyfish, sea squirts and starfishes do not have one. It is located in the head, usually close to primary sensory apparatus such as vision, hearing,...

 beneath the pia mater
Pia mater
Pia mater often referred to as simply the pia, is the delicate innermost layer of the meninges, the membranes surrounding the brain and spinal cord. The word finds its roots in Latin, meaning literally "tender mother." The other two meningeal membranes are the dura mater and the arachnoid mater....

, severing the axon
Axon
An axon is a long, slender projection of a nerve cell, or neuron, that conducts electrical impulses away from the neuron's cell body or soma....

al tracts in the subjacent white matter
White matter
White matter is one of the two components of the central nervous system and consists mostly of myelinated axons. White matter tissue of the freshly cut brain appears pinkish white to the naked eye because myelin is composed largely of lipid tissue veined with capillaries. Its white color is due to...

.

Prognosis

The prognosis for children with LKS varies. Some affected children may have a permanent severe language disorder, while others may regain much of their language abilities (although it may take months or years). In some cases, remission and relapse may occur. The prognosis is improved when the onset of the disorder is after age 6 and when speech therapy is started early. Seizures generally disappear by adulthood.

External links

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