Landau-Kleffner Syndrome Beginning With Stuttering: Case Report Downloaded from . Glauser TA, Morita DA: Encephalopathic epilepsy after infancy, in. Download full-text PDF. Content . case have possessed normal psychomotor development. He. could not patient was accepted as Landau-Kleffner syndrome. We Uso de flunarizina en un caso con síndrome de Landau- Kleffner. N e u ro p e d i at rics, 24, 4 7 -5 0. Síndrome de Landau-Kleffner (afasia adquirida con epilepsia). Etiopatogenia y respuesta al tratamiento anticonvulsivo .
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Landau–Kleffner syndrome (LKS) or acquired epileptic aphasia is a childhood disorder in which acquired aphasia and Epileptic seizures are infrequent and not a prerequisite for Landau–Kleffner syndrome. . Download high-res image ( KB) · Download full-size image. Fig. .. J. AicardiEl síndrome de Landau Kleffner. Downloads surgery, on the Landau-Kleffner syndrome and related disorders, on sensory cortex properties in progressive .. Guevara Campos J, Guevara González L, and Dravet Ch () Sindrome de Landau-. Kleffner. Landau-Kleffner syndrome (LKS) is a rare form of epilepsy. What do the seizures in Landau-Kleffner syndrome look like? Seizures occur in 3.
Children with a good outcome regain ability in spoken language and tend to score within the average range for their age group on formal assessments. However, they often experience subtle difficulties, such as trouble with short-term memory and problems listening when there is background noise.
Those with a moderate outcome have some degree of language difficulty but spoken language will be their main means of communication. Even children with a poor language outcome, who never recover spoken language, may become very proficient at British Sign Language, and go on to lead a full and independent adult life. In general, the longer the active period, the worse the outcome.
Children who develop LKS at an older age and when their language is more mature, tend to do better, as do those who respond promptly to medication. Where children lose skills in addition to language, it is often the problems with social communication that pose the greatest barriers to recovery.
Adjustment and support LKS can be both bewildering and distressing for the child and their family. They are vulnerable to poor self-esteem and low mood. It is important to support the child as much as possible, providing opportunities for them to spend time with their existing friends, but also creating chances for them to find new friends who might experience similar difficulties, perhaps drawn from other children with language, learning or hearing difficulties.
For parents, there is the very painful experience of watching their child lose skills. Many parents report that the behavioural changes in their child, particularly aggression and sleep disturbance, are the hardest parts to manage. Having a child with LKS inevitably affects the whole family.
As well as the demands of caring for their child with LKS, there are also the needs of other siblings to consider, who may also be confused and resentful of the attention paid to their brother or sister. LKS is a rare diagnosis and there is often little knowledge amongst local professionals about the syndrome.
These needs can vary over time and it is important that support can be accessed when windows of opportunity arise. Parents may face a large number of differing views and approaches by professionals, which can be daunting, confusing and exhausting. It is common for parents to feel completely overwhelmed at times. Details of some helpful organisations follow in the next section. Improving clinical care and research We have a number of initiatives to advance knowledge and clinical management of LKS, including a registered database of clinical information and various research projects such as a genetics project.
If you are invited to take part in a research project, you will be given full written information about the study. You can also withdraw from the project at any time without explanation. Call them on ext. The umbrella organisation Contact a Family may be able to put you in touch with another family affected.
Call their helpline on or visit their website. Definitions and explanations The following pages contain definitions and explanations to some commonly used terms.
Child development This is the process by which children change and increase in their abilities in all areas, such as movement, language and social skills, over time. The brain is not fully developed at birth. Generally, children follow a predictable sequence, for example sitting before walking, although at different speeds. For the younger child, development is often assessed by considering skills in different areas, such as gross motor sitting and walking for instance , fine motor including hand usage , vision, language, cognitive ability such as puzzles or problem solving and personal-social skills.
For older and more able children, it is common to concentrate on language and cognitive nonverbal intelligence skills. This does not generally happen, as it requires development at a faster rate than normal. The case for children with LKS is different. These children generally had normal early development and were increasing their skills at the normal rate.
What is actually happening, however, is recovery of their previous developmental path and retrieval of skills that were inaccessible to them in the acute active phase due to the effect of LKS on their brain. Unfortunately this is not always the case in LKS and some children do not recover their lost skills at this time.
At the end of the active phase of LKS, children or adolescents are often left with residual impairments. They then tend to make steady developmental progress. For some, this progress is at an increased rate and their language skills improve and may approach their peers. Others may show new learning, but at a slower rate and continue to have significant difficulty with language. For some young people who have recovered from LKS, there is evidence of continuing progress after the age of 20 which is unusual.
In this case they would benefit from continuing in education and should be encouraged and supported to do this. Regression This is the loss of previously acquired skills, so that the child appears to have returned to an earlier stage in their development.
Loss of skills can sometimes affect one particular skill and not another so the child has an uneven profile. For example a child may still be able to read words, but no longer understand the words they read. This may leave the child with isolated retained skills from their previous abilities, which can mask their lost skills. Pediatric Neurology, 9, 49— Spech therapy in Landau and Kleffner syndrome.
Acquisition and Breakdowm of Language in the Developing Brain pp. Google Scholar Gordon, N. The Canadian Journal of Psychiatry, 34, 77— Acquired aphasia in childhood: the Landau-Kleffner syndrome. Developmental Medicine and Child Neurology, 32, — Epilepsia, 31, — Electrical status epilepticus during slow-wave sleep: A review.
Recurrent aphasia with subclinical bioelectric status epilepticus during sleep. European Journal of Pediatric, , — Relationship of language impairment and seizures in children with verbal auditory agnosia. Google Scholar Landau, W.
The conception and embarassing birth of an eponym. Syndrome of acquired aphasia with convulsive disorder in children. Neurology, 7, — Effect of early corticosteroid therapy for Landau-Kleffner syndrome. Developmental Medicine and Child Neurology, 33, — Acquired childhood aphasia. Outcome 1 year after onset. Archives of Neurology, 47, — Aphasia and epilepsy in childhood.
Acta Neurologica Scandinavica, 56, 46— Landau-Kleffner syndrome: A pharmacologic study of five cases. An aphasic syndrome in children. Canadian Medical Association Journal, , — Landau-Kleffner syndrome-epileptic aphasia in children — possible role of Toxoplasma gondii infection.
Acta Paediatrica Hungarica, 29, — Google Scholar Miller, J. Language behavior in acquired childhood aphasia. Holland Ed. Language Disorders in Children pp.
San Diego: College-Hill Press. Google Scholar Montovani, J. Acquired aphasia with convulsive disorder: Course and prognosis. Neurology, 30, — Clinical outcome in Landau-Kleffner syndrome treated by multiple subpial transection. Epilepsia, 33 Suppl. Google Scholar Mouridsen, S. Neuropediatrics, 24, 47— Landau-Kleffner syndrome EEG topographic studies. Brain and Development, 11, 43— Landau-Kleffner syndrome: Temporal lobe tumor resection results in good outcome.
Pediatric Neurology, 9, — Acquired aphasia the Landau-Kleffner syndrome due to neurocysticercosis.
Epilepsia, 30, — Archives of Neurology, 49, — This is demonstrated in patients in multiple ways including the inability to recognize familiar noises and the impairment of the ability to lateralize or localize sound.
In addition, receptive language is often critically impaired, however in some patients, impairment in expressive language is the most profound. In a study of 77 cases of Landau—Kleffner syndrome, 6 were found to have this type of aphasia. Because this syndrome appears during such a critical period of language acquisition in a child's life, speech production may be affected just as severely as language comprehension.
Generally, earlier manifestation of the disease correlates with poorer language recovery, and with the appearance of night seizures that last for longer than 36 months.
However, acute onset of the condition has also been reported as well as episodic aphasia. Seizures, especially during the night, are a heavily weighted indicator of LKS. In one third of patients, only a single episode of a seizure was recorded.