LANDAU - KLEFFNER SYNDROME; LKS
Victor A. McKusick, OMIM, Johns Hopkins University, August 31, 2005
[for Professionals mainly]
APHASIA, ACQUIRED, WITH CONVULSIVE DISORDER
Landau and Kleffner (1957) described 6 children with acquired aphasia and convulsive disorder. Ansink et al. (1989) found reports of more than 170 children with this condition. Characteristically, Landau - Kleffner syndrome (LKS) affects previously normal children who undergo a regression of receptive and/or expressive language abilities. The regression may be sudden or insidious and is not accompanied by any abnormal neurologic signs, impairment of hearing, or loss of cognitive ability. Males are more frequently affected than females by a ration of 2:1; most children develop behavioral disturbances … Some children are left with such severe auditory agnosia … others recover completely, even after several episodes of aphasia. About one-half of patients are left with severe residual language deficits … all children are seizure - free by age 15 years. All children with LKS, regardless of seizures, have abnormalities on EEG …

ROLANDIC EPILEPSY AND SPEECH DYSPRAXIA
Orest Hurko, OMIM, Johns Hopkins University, March 14, 2007
[for Professionals mainly]
Scheffer et al. (1995) described a syndrome of nocturnal partial seizures involving the face and an are associated with oral dyspraxia and cognitive impairment in a family with 9 affected individuals in 3 generations … all affected individuals had oromotor apraxia with difficulty organizing and coordination movements necessary to product fluent speech but without dysarthria … also mild impairments of perceptive language … The authors suggested that this disorder may be part of a spectrum, the extremes of which are represented by benign rolandic epilepsy, a mild disorder with the more severe Landau - Kleffner or continued spike and wave syndromes …

Friends of Landau - Kleffner Syndrome [UK]
Friends of LK Syndrome
[Support Groups]
FOLKS was established following several years of hard work and dedication by its founder member, Vicki Horsewell. Vicki established the Landau Kleffner UK Parent Support Group with the assistance of the UK national charity … seeks to support families of children with rare conditions by putting them in touch with other similarly affected families … Thanks to all those who have visited these pages and made inquiries for more information. We now have contacts in USA, Norway, Germany, Italy and India as well as UK. Most parents in UK probably do not have on-line facilities, and we are surprised by the number of US inquiries … FOLKS is pleased to announce that the revised and expanded 1999 FOLKS LKS Information Pack has just been published. Copies will be dispatched by post to paid up members of FOLKS starting 11th October 1999, but in the meantime 90% of the Pack is now available for free to everyone, anywhere, online …

What is Landau Kleffner Syndrome? [USA]
Friends of LK Syndrome
[Support Groups]
(Word Deafness Syndrome; (Language Aphasia); (Auditory Aguosin Syndrome); (Epileptic Aphasia)... What is LKS? … is a rare form of childhood epilepsy which results in a severe language disorder … All children with LKS have abnormal electrical activity in one, sometimes both temporal lobes, the area of the brain responsible among other functions for processing language … two-thirds of LKS children have seizures … in most children affects comprehension or understanding … These children may appear to be deaf … ability to speak is often seriously affected … Some children have … "autistic type behaviour" … repetitive play … problems such as loss of bladder and bowel control … maybe mistaken for developmental language disorder, deafness or autism … speech and language therapy and special education are essential … early diagnosis and prompt treatment may improve your child's chance of a good recovery …

Landau - Kleffner Syndrome [USA]
Stephen M. Edelson, Ph.D., Center for the Study of Autism, Salem, Oregon, 1995
[Support Groups]
Landau - Kleffner Syndrome is manifested as a form of aphasia, (loss of language), which usually develops between 3 and 7 years … People with Landau - Kleffner Syndrome have abnormal EEG patterns … involves examining the person's EEG patterns during sleep … often diagnosed in conjunction with autism, is the failure to respond to sounds. Thus, parents may suspect their child of hearing loss ... Some suggested causes have been a dysfunctional immune system ... For additional information, contact: C.A.N.D.L.E. 4414 McCampbell Montgomery, AL 36106.

Aphasia, National Aphasia Association [USA]
National Aphasia Association, October 2007
[Support Groups]
A nonprofit organization that promotes public education, research, rehabilitation and support services to assist people with aphasia and their families …

Epilepsy Foundation
Epilepsy Foundation, 2007
[Support Groups]
We have transformed the "Information and Education" section into the Epilepsy Answer Place - offering you greater depth of information, more topic areas and more links between them …

NIDCD Health Information, Landau - Kleffner Syndrome
NIDCD, National Institutes of Health, August 24, 2007
What is Landau - Kleffner syndrome? ... a childhood disorder … gradual or sudden loss of the ability to understand and use spoken language. All children with LKS have abnormal electrical brain waves that can be documented by an electroencephalogram (EEG), a recording of the electric activity of the brain. Approximately 80 percent of the children with LKS have one or more epileptic seizures that usually occur at night. Behavioral disorders such as hyperactivity, aggressiveness and depression can also accompany this disorder. LKS may also be called infantile acquired aphasia, acquired epileptic aphasia or aphasia with convulsive disorder. This syndrome was first described in 1957 by Dr. William M. Landaua and Dr. Frank R. Kleffner, who identified six children with the disorder … LKS occurs most frequently in normally developing children who are between 3 and 7 years of age. For no apparent reason, these children begin having trouble understanding what is said to them … "word deafness" ... auditory agnosia … Children may also appear to be autistic or developmentally delayed. The inability to understand language eventually affects the child's spoken language, which may progress to a complete loss of the ability to speak (mutism). Children who have learned to read and write before the onset of auditory agnosia can often continue communicating through written language … epileptic seizure that usually occurs at night … The seizures usually stop by the time the child becomes a teenager … The cause of LKS is unknown. Some experts think there is more than one cause for this disorder … There have not been many long - term follow - up studies of children with LKS … it impossible to predict the outcome of this disorder … Generally, the earlier the disorder begins, the poorer the language recovery ...

Landau Kleffner Syndrome
British Epilepsy Association, July 11, 2007
Acquired Epileptic Aphasia in Childhood
The first sign of difficulty usually appears in the form of difficulty with communicating with speech and language. They show not only difficulty understanding what is said to them (a receptive dysphasia) but also has difficulty in putting their thoughts into words (an expressive dysphasis) …

El síndrome de Landau-Kleffner. Presentación de dos casos
Dra. Albia J. Pozo Alonso et al., Rev Cubana Pediatr v.77 n.2 Ciudad de la Habana jabr.-un. 2005
[Spanish]
Se presentan dos niños que reúnen los criterios clínicos y electroencefalográficos del síndrome de Landau-Kleffner. En uno de los pacientes las dificultades para comprender el lenguaje y para la expresión oral espontánea comenzaron a manifestarse a los 6 años, y en el otro caso a los 6 años y 9 meses. Uno de los niños no ha presentado crisis epilépticas hasta el momento actual. En este paciente la tomografía computadorizada por emisión de positrón único (SPECT cerebral) mostró una ligera hipoperfusión temporoparietal posterior bilateral. En el niño que presenta crisis epilépticas; éstas se iniciaron a la edad de 2 años y 6 meses y eran focales simples motoras que se generalizaron secundariamente. Desde hace un año no presenta crisis. Desde el punto de vista clínico y electroencefalográfico, ambos pacientes tuvieron una respuesta favorable al tratamiento con prednisona. En un niño, aunque ha mejorado, persisten las dificultades para la comprensión y la expresión orales. Se concluye que son muy importantes para el diagnóstico de este síndrome la existencia de una afasia adquirida y las descargas observadas en el electroencefalograma. ... Palabras clave: Afasia epiléptica adquirida, agnosia auditiva verbal, encefalopatía epiléptica, síndrome de Landau-Kleffner ...

Landau Kleffner, Síndrome de
Instituto de Investigación de Enfermedades Rares
[Spanish]
Sinónimos: Afasia con Trastorno Convulsivo, Afasia Adquirida con Enfermedad Convulsiva, Afasia Infantil Adquirida, Afasia Adquirida Epiléptica ... Descripción en lenguaje coloquial: El síndrome de Landau Kleffner es una enfermedad neurológica muy rara. Fue descrita por primera vez en 1957, por William Landau y Frank Kleffner, como una afasia (trastorno, defecto o pérdida de la facultad de expresión hablada, escrita o mímica) epiléptica (epilepsia es una enfermedad crónica nerviosa caracterizada por accesos de pérdida súbita del conocimiento, convulsiones y a veces coma) adquirida, de la que la literatura recoge unos 200 casos. ... La clínica suele aparecer entre los 4 y los 7 años. No parecen existir diferencias en uno u otro sexo, ni se han descrito antecedentes familiares significativos. ... Aunque se desconoce la causa de la enfermedad, se postulan diferentes hipótesis patogénicas (mecanismos de producción de enfermedad) para el Síndrome de Landau Kleffner ..

El Síndrome de Landau-Kleffner
University Healthcare, University of Utah
[Spanish]
¿Qué es el síndrome de Landau-Kleffner? ... El síndrome de Landau-Kleffner (también llamado afasia infantil adquirida, afasia epiléptica adquirida, o afasia con desorden convulsivo) es un desorden del lenguaje. Frecuentemente se da en niños con un desarrollo normal, generalmente entre los tres y siete años de edad, y se caracteriza por la pérdida gradual o repentina de la capacidad para usar o comprender el lenguaje hablado ... En un desorden raro, con aproximadamente 160 casos diagnosticados entre 1957, cuando se identificó el primer caso, y 1990 ...

________________________________________________________________________________________________

Last Updated: 2008/3/10

________________________________________________________________________________________________