Landau Kleffner Syndrome

What is Landau Kleffner Syndrome?

Landau Kleffner Syndrome Is Otherwise known as the infertile acquired aphasia or acquired epileptic aphasia or aphasia with convulsive disorder, Landau Kleffner syndrome is a very rare childhood neurological syndrome. This syndrome is named after the people who characterized it in 1957 by running a diagnosis of six children. These people were William Landau and Frank Kleffner.

Signs & Symptoms

This syndrome is characterized by the following symptoms;

• Sudden or gradual development of aphasia. This is the inability to understand or express any language.
• Abnormal electroencephalogram.

Landau Kleffner syndrome affects some parts of the brain that help in controlling comprehension and speech. The parts of the brain that carry these functions include the Broca’s areas and the Wernicke’s areas.

This disorder mostly occurs in children between the ages of three to seven years.

There is slight male dominance in the diagnosis of the syndrome. The ratio to which this syndrome occurs between male to female is 1.7.

Children with Landau-Kleffner syndrome grow and develop normally, but with time they lose their language skills.

Many of the affected individuals usually have clinical seizure while others experience only electrographic seizures that include electrographic status epilepticus of sleep (ESES).

The first symptom of the language problem is usually auditory verbal agnosia. Verbal agnosia is usually demonstrated in patients through multiple ways that include:

• The inability to recognize any familiar voices and noises.
• The impairment of the ability to lateralize or localize any sound.

Any receptive language is often critically impaired but in some patients impairment in expressive language is very profound. In a study made in cases of Landau Kleffner syndrome, six children were found to have the aphasia. This syndrome usually appear during very critical period of language acquisition in a child’s life therefore some speech production are affected as severely as language comprehension.

The Landau Kleffner syndrome can emerge between the age between eighteen months to thirteen years but the most predominant time of this syndrome emergence is between three and seven years.

Earlier manifestation of the syndrome correlates with very poor language recovery and then there is night seizures that lasts longer than thirty six months.

The Landau Kleffner syndrome has many symptoms but they are all different and lacks uniformity in diagnosis criteria between different cases. In many studies, there have been no follow up on patients tested so there has been no other discovery on the symptoms and the recovery of the syndrome.

Language deterioration in the patients occurs in over a period of weeks or months however, the acute onset of the condition have been reported as well as episodic aphasia.

Seizures that occur during the night are crucial indicators of Landau Kleffner syndrome. The prevalence of the clinical seizure in acquired epileptic aphasia is about seventy to eighty percent. In one third of all the patients suffering from LKS, only a single episode of seizure was recorded. These seizures usually appear between the age of four and eight and they disappear before adulthood at the age of fifteen.

Diagnosis

This syndrome is very difficult to diagnose and it is often misdiagnosed as autism, pervasive development disorder, learning disability, hearing impairment, auditory or verbal processing disorder, childhood schizophrenia, intellectual disability, attention deficit hyperactivity disorder or emotional or behavioural problems.

In many cases of patients that are exhibiting LKS there will show a lot of abnormal brain activity in both the right and the left hemispheres of the brain which occurs frequently when sleeping.

The best way to confirm the presence of LKS is by obtaining overnight sleep EEGs and EEGs in all sleeping stages should be recorded. In some conditions such as demyelination and brain tumors, they can be ruled out with the use of magnetic resonance imaging (MRI).

In cases of Landau Kleffner Syndrome, fluorodeoxyglucose (FDG) and position emission tomography (PET) scanning carried out can show decreased metabolism on one or both the temporal lobes. Hypermetabolism has been reported in case of patients diagnosed with acquired epileptic aphasia.

Landau Kleffner Syndrome do not have a known cause but occasionally the condition maybe induced by other diagnoses such as low-grade brain tumors, closed-head injuries, neurocysticercosis and demyelinating disease. Central Nervous System vasculitis is also associated with the Landau Kleffner syndrome.

Differential Diagnosis

The arrangement below show the extensive differences in acquired epileptic aphasia diagnoses along with Cognitive and Behavioural Regression.

Diagnosis	Deterioration	EEG Patterns
Autistic epileptiform regression	Expressive language, RL, S, verbal and nonverbal communication	Centrotemporal spikes
Autistic regression	Expressive language, RL, S, verbal and nonverbal communication	Normal
Acquired epileptic aphasia	RL, possibly behavioural	Left or right temporal or parietal spikes, possibly ESES
Acquired expressive epileptic aphasia	Expressive language, oromotor apraxia	Centrotemporal spikes
(ESES)	Expressive language, RL, possibly behavioural	ESES
Developmental dysphasia (developmental expressive language disease)	No; lack of expressive language acquisition	Temporal or parietal spikes
Disintegrative epileptiform disorder	Expressive language, RL, S, verbal and nonverbal communication, possibly behavioural	ESES
Stuttering	Expressive language, no verbal-auditory agnosia	Spike-and-wave discharges on the left temporocentral and frontal regions

Treatment

Treating Landau Kleffner Syndrome consist using medications such as anticonvulsants and corticosteroids and speech therapy is also very useful. Some patients show change after using corticosteroids or adrenocorticotropin hormone which has led to believes that inflammation and vasospam may play a major role in cases of acquired epileptic aphasia.

Controversial treatment options usually involve a surgical technique that is called multiple subpial trans-section whereby the incisions are made through the cortex of the infected part of the brain.

Some hospitals have programs that are designed to treat conditions such as Landau Kleffner Syndrome. One of these hospitals is the Children’s Hospital in Boston which is internationally known for its work with children who are non-speaking and those that are severely impaired.

A care team for children with LKS include a neurologist, neuropsychologist and a speech pathologist. Speech therapy is usually done immediately after diagnoses along with other medical treatment that include steroids and anti-epileptic medications. Children with Landau Kleffner Syndrome can be taught sign language, which can help them when communicating.

Prognosis

The prognosis varies in different cases. Some affected children may have permanent severe language disorder while others regain language abilities after some time. The prognosis is improved when the onset of the disorder is after the age of six and when speech therapy is started early.

Short term remissions are not common in Landau Kleffner Syndrome but they may create difficulties when a patients response to different therapeutic modalities. There are a hundred cases reported of Landau Kleffner Syndrome diagnosis in every ten years.

Reference List

1. Landau–Kleffner syndrome. Available at https://en.wikipedia.org/wiki/Landau%E2%80%93Kleffner_syndrome
2. Landau–Kleffner syndrome. Available at http://www.epilepsy.com/learn/types-epilepsy-syndromes/landau-kleffner-syndrome
3. Landau-Kleffner Syndrome (LKS or Infantile Acquired Aphasia). Available at http://www.medicinenet.com/landau-kleffner_syndrome/article.htm
4. Landau-Kleffner Syndrome. Available at https://rarediseases.org/rare-diseases/landau-kleffner-syndrome/
5. Landau-Kleffner Syndrome. Available at http://www.gosh.nhs.uk/medical-information/search-medical-conditions/landau-kleffner-syndrome
6. Landau-Kleffner syndrome. Available at https://rarediseases.info.nih.gov/diseases/6855/landau-kleffner-syndrome