Request PDF on ResearchGate | On 31, , Luis Lozano González and others Síndrome de Landau-Kleffner (afasia adquirida con epilepsia). 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. Landau-Kleffner Syndrome Beginning With Stuttering: Case Report. Published by : rialadhamssubsca.ml can be found at: Journal of Child Neurology.
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Landau-Kleffner syndrome (LKS) is an age-related epileptic encephalopathy where developmental regression occurs mainly in the language Approximately 1/3 of patients do not have seizures. Emergency guidelines; Français (, pdf). Landau-Kleffner syndrome (LKS), or acquired epileptiform aphasia, is an majority of children with autism who undergo language regression do so before three. A collection of disease information resources and questions answered by our Genetic and Rare Diseases Information Specialists for Landau-Kleffner syndrome.
The former occur prior to language emergence and may have many different causes. The latter can be subdivided into congenital aphasia and acquired aphasia.
Congenital aphasia is caused prenatal or perinatal whereas acquired aphasia always occurs postnatal. Therefore, language acquisition has to be at least partially completed.
The linguistic symptoms can include all parts of language that is phonology, morphology, semantics, syntax and lexicon. Both receptive and productive language skills can be affected and the child may not only have problems with spontaneous speech, but also with alphabetisating and repeating words and sentences. The constitution and severity of the different symptoms vary and depend on individual factors like the cause of the brain lesion.
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Other reasons are infectious diseases e. The prognosis depends on aetiology, the age of the child, the kind of aphasia and the degree and location of lesion, the latter one being the most important factor. He proposes that focal, non-progressive lesions are rarely followed by permanent speech-defects.
Therefore, the prognosis for such cases is comparatively good. Furthermore, he argues that the severest cases are diffuse and progressive lesions, which are mostly followed by permanent speech-defects.
If aphasia starts before puberty, the chance of recover is much better than afterwards.
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Landau and the pedagogue Frank R. Kleffner observed six children who all suffered from acquired aphasia and some kind of epilepsy and found a syndrome they called Syndrome of Acquired Aphasia with Convulsive Disorders in Children.
It is a rare disease about cases up to 11 and in literature it is discussed controversially, thus, a clear definition cannot be taken. Landau-Kleffner Syndrome occurs exclusively in children, the time of onset being between the age of two to thirteen.
Nevertheless, adults may suffer from the consequences of the disease. Furthermore, boys are twice as often contracted by LKS as girls.
Bishop 4 , in an analysis of reported cases up to , argued that the association between age of onset and prognosis suggests LKS is a disorder of higher-level auditory processing.
In a younger child without advanced language development, the effect is devastating, as the normal auditory route leading to acquisition of language is blocked.
In the older child the result will be less severe, since language has been partially learned. An analogous scenario is profound deafness, when Wernicke's area is intact but the sensory input is defective. Profound deafness in a young child has a more profound effect on language development than in an older child. Even the relatively mild and fluctuating hearing loss of otitis media may impair language in a young child in the process of developing language 8.
Children with LKS may not follow precisely an auditory processing disorder model 9. Given the usual age of presentation between 4 and 7 years, the child has not acquired sufficient reading and writing skills; the older child, however, may lose these skills.
Partial retention of writing skills suggests a better prognosis in the reeducation phase The language disorder of LKS has commonalities with autism spectrum disorder.
Communication deficits in autism include abnormal development of spoken language and impaired ability to initiate or sustain conversation.
The autistic child's language is often stereotyped, repetitive, and idiosyncratic, with echolalia and neologisms Confusing the picture is the fact that seizures may occur in autism, and EEG abnormalities are common Furthermore, at least a third of autistic toddlers demonstrate neurodevelopmental regression, involving language, sociability, play, and cognition LKS represents selective loss of language in association with an abnormally paroxysmal EEG, eventually characterized by electrographic status epilepticus of slow-wave sleep ESES.
While there is considerable overlap in the semiology of LKS and autism, some differences emerge. The great majority of children with autism who undergo language regression do so before three years of age 14 , versus a mean age of language regression in LKS of 5—7 years.
As regression in autism occurs early, it usually entails the loss of single words, versus more drastic changes in LKS children who are typically older and have more developed vocabulary and language.
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LKS does not feature the behavioral profile that encompasses the core deficits of autism, i. There is an intricate relationship between LKS, autism, ESES, and developmental dysphasias and the interaction between epileptiform discharges and cognitive dysfunction remains enigmatic The presence of fluctuation in language and behavioral deficits, however, should raise concern regarding an accompanying diagnosis of epilepsy Focal epilepsy can interfere with language.
Aphasic status epilepticus 16 and post-ictal aphasia 17 have been reported.
Children with severe focal epilepsy from static brain lesions involving language cortex may have episodic ictal aphasia or status epilepticus and become permanently aphasic 3. The early stages of LKS, with hyperkinesis and mild verbal auditory agnosia, may be confused with attention deficit hyperactivity disorder ADHD Personality disturbances, aggressiveness, and depression are noted Nonverbal developmental disorders may occur, but operational and intellectual capacities are usually preserved in LKS 9, 19, The differential diagnosis also includes deafness, elective mutism, and acute psychiatric disorders.
In others, infrequent seizures occur between ages 5—10 years. After age 10, only one-fifth of patients continue with sporadic seizures; by age 15, seizures rarely persist. Generalized tonic-clonic seizures, atypical absences, and myoclonic-astatic seizures occur less frequently 3. Complex partial seizures with psychomotor automatisms are rare Tonic seizures are not characteristic 3, The frequency and type of seizures have no influence on prognosis.We report a year-old bipolar patient, wheelchair-bound as a result of lithium toxicity 20 years ago, who experienced worsening tremor for 1 month.
Delays and abnormalities in long-latency cortical evoked responses suggest localization to the posterior temporal regions 6. MRI showed marked atrophy of cerebellum and vermis. LKS does not feature the behavioral profile that encompasses the core deficits of autism, i.
Rio de Janeiro: Revinter; He underwent a rehabilitation program consisting of sensory integration and behavioral modification techniques with an emphasis on verbal and nonverbal forms of communication. Rocha AF.
This auditory agnosia extends to familiar noises including bells, whistles, or a ringing phone. However, others found the opposite.