LANDAU-KLEFFNER
SYNDROME
AGENDA
•
•
•
•
•
•
•
What is Landau-Kleffner Syndrome?
What is Aphasia?
LKS and Autism Spectrum Disorder
Associated issues
Prevalence and treatment(s)
To read
Tips to help with 4 types of communication
• Oral, reading, listening, and writing
• Parent resources and support
• References
WHAT IS LANDAU-KLEFFNER
SYNDROME?
• A very rare form of epilepsy in which children
between the ages of 3 and 7 begin to develop
difficulties in understanding and expressing
language (aphasia)
• Can occur without grand mal seizures but seizures
do often occur throughout the child’s life
• Typically occur at night while child is sleeping
“WHAT IS LKS?” CONTINUED
• A neurological disorder characterized by:
1. Sudden or gradual development of aphasia, and
2. An abnormal electroencephalogram (EEG)
• The onset of LKS occurs between 18-months and
13-years, though the most cases have been noted
to have developed between ages 3 and 7
“WHAT IS LKS?” CONTINUED
• Many children appear to develop normally and
then lose their language skills around the age of 3
(similar onset to ASD)
• They appear to become unresponsive to verbal
address or direction
• As a result, many parents bring their child to an
audiologist because they think that their child may
be losing his/her hearing
AN EXAMPLE OF LKS SPEECH
• Watch the video below and listen to the voice and
speech of this young woman who has LKS
• Reflect on what you are hearing and make note of
challenges this type of speech may provide for the
individual in her life (both in and out of school)
https://www.youtube.com/watch?v=wU1gbC1fIK4
WHAT IS APHASIA?
• A communication impairment that affects all
aspects of language
• Speaking, understanding speech, reading, and writing
• Aphasia is caused by a brain injury to the area of
the brain that processes and creates language
• Often the individual can still speak but they may not
be as clear and concise as before
• Individuals with aphasia often have difficultly
recalling the right word and will replace the
forgotten word with something else
APHASIA SIMULATIONS
• Visit the website listed below and try one simulation
from each category (listening, reading, writing, and
speaking)
• Make notes of your results to discuss with your peers
• Reflect on challenges this may present both in, and
out, of the classroom
http://aphasiacorner.com/aphasia-simulations/whatis-aphasia.html
LKS AND AUTISM SPECTRUM DISORDER
• Due to the similar timeframe and appearance of
the onset of LKS and ASD, many children are
misdiagnosed with the latter
• In both cases, the individuals progress normally in their
development and then suddenly, around age 3, display a
significant regression in their language abilities
• Individuals with LKS also seem non-responsive to verbal
stimuli and appear anti-social, behaviours that are also
common with ASD
• Many children are taken to an audiologist to have their
hearing checked and to have more tests to diagnose the
issue
• Diagnosis for LKS is done by completing EEGs while sleeping
to test for seizures
ASSOCIATED ISSUES
• Executive functioning
• Executive function is the cognitive process that allows us to
plan, organize, adapt, predict outcomes, control our
behaviour, and help us to focus
• These skills often begin to develop around the age on onset
for LKS; as a result, they often to not develop properly and
individuals with LKS can have difficulties in these areas
ranging from mild to severe
• ADD/ADHD often present in children with LKS
ASSOCIATED ISSUES
• Memory
• Due to the limitations in language processing and
acquisition, memory is often significantly impacted in
individuals with LKS
• It is often hard to know if they have forgotten something or
simply never have learned it in the first place
ASSOCIATED ISSUES
• Motor function
• Around 2/3 of children with LKS have motor function
impairment; these difficulties are exacerbated during the
active phase of the condition
• Difficulties often include (but are not limited to): dyspraxia,
tremors, unusual limb postures, weakness and even neglect
on one side of the body (a common feature in stroke
victims as well). These difficulties affect activities ranging
from writing to dressing oneself.
• In some cases, weakness in the facial and neck muscles
can affect the individual’s ability to swallow, controlling
saliva, and speech
ASSOCIATED ISSUES
• Behaviour
• At least ½ of children with LKS experience behavioural
disturbances with aggression being the most common
(issues with executive functioning may play a role here)
• Children can be very emotional and sensitive, often
requiring extra TLC
• Children can become easily frustrated because they
cannot communicate what they wish to communicate and
often others need to guess at the needs of the individual
• Depression and anxiety can occur in some children
PREVALENCE
• 200 diagnosed cases between 1957 (the year it was
identified) and 2002
• Twice as likely to occur in males than females
• Seizures occur in roughly 2/3 of the LKS population
TREATMENT
• Options vary depending on the severity of the issues
• Anticonvulsants medications are administered to those
individuals who suffer from clinical seizures
• Some individuals are put on Ritalin for their ADD/ADHD
comorbidities
• Some individuals required anti-depressants and/or antianxiety medications depending on the severity of their
mood disturbances
• The most common intervention for LKS is speech
therapy
• With early intervention and limited brain damage, some
individuals are able to regain much of their abilities back,
though they will struggle with language for the rest of their
lives
TO READ
• Please read the following:
1. What Every Educator Should Know About LKS
http://trainland.tripod.com/whatever.pdf
2. The case study at the bottom of this webpage (in a blue
text box)
http://leader.pubs.asha.org/article.aspx?articleid=229168
9
3. The case studies in the following article
http://www.audiology.org/sites/default/files/journal/JAAA
_05_02_10.pdf
TIPS TO HELP WITH…
• Oral communication
• Keep paper and pen handy so that the individual can draw
a picture of what they are trying to communicate
• Ask questions that can help narrow down the topic
• Give the individual time to answer or to ask for help
• Encourage individual to point or gesture to something that
could help them express themselves
• If the individual becomes frustrated suggest that they take a
break
• Use picture exchange communication (PECs) to help
individual communicate
TIPS TO HELP WITH…
• Reading
• Use PECs where possible
• Underline or highlight key words in written text
• Provide redundancy
• The repetition within a text may be more helpful to someone
with aphasia because they have more opportunities to find and
understand the meaning
TIPS TO HELP WITH…
• Listening comprehension
• Speak slowly, though naturally, with pauses between
phrases
• Repeat the key words, or, if necessary the entire phrase
• Use gestures when talking
• Write down any key words for individual to reference while
you are talking
• Have pictures available to use to aid the individual’s
understanding
TIPS TO HELP WITH…
• Written communication
• Encourage the individual to write the first letter of the word
and then to draw lines to indicate how many letters remain
(ex. kitty  k
)
• Make a list of frequently used words for the individual to
choose from
• Encourage individual to draw parts of the message that
they cannot write
• Ask the individual to identify features of the missing letters
(ex. “is the letter tall like a ‘t, f, l, h, d, b’?”)
PARENT RESOURCES AND SUPPORT
• Hands and voices – Blog and info contains resources in
multiple languages and is very accessible
http://www.handsandvoices.org/resources/index.htm
• NORD reference guide
Mantovani JF. Landau-Kleffner Syndrome. In: The NORD
Guide to Rare Disorders, Philadelphia, PA: Lippincott,
Williams and Wilkins; 2003:547-8.
http://rarediseases.org/rare-diseases/landau-kleffnersyndrome/
• Kaitlin Atkinson Family Resource Library (CHEO)
http://www.cheo.on.ca/En/epilepsy
REFERENCES
Alpern, C. (2010). Identification and treatment of
Landau-Kleffner syndrome. Retrieved on July 6th
from:
http://leader.pubs.asha.org/article.aspx?articleid=2
291689
Alpern, C. (2008). Identification and treatment of
Landau-Kleffner syndrome: A review and case
study. Communicative Disorders Review, 2, 21– 31.
Chapman, T., Stormont, M., & McCathren, R. (1998). What
every educator should know about Landau-Kleffner
syndrome. Focus on Autism and Other
Developmental Disabilities, 13, 39–44.
REFERENCES
Disabled World. (2010). Landau-Kleffner Syndrome
and Aphasia – Facts and Information. Retrieved
on July 5th, from: http://www.disabledworld.com/health/neurology/lks-aphasia.php
Great Ormond Street Hospital for Children NHS Trust:
Information for Families. (2010). Landau Kleffner
syndrome: abilities, behaviour and education.
GOSH
NHS Trust April 2010.
REFERENCES
Holmes, G. and Fisher, R. (2013). Landau-Kleffner
Syndrome. Epilepsy Foundation. Retrieved on July 5th
from http://www.epilepsy.com/learn/types-epilepsysyndromes/landau-kleffner-syndrome
Loddenkemper, T., and Fernandez, I., (2014). Landau
Kleffner Syndrome. Retrived on July 15th, from:
http://www.orpha.net/consor4.01/www/cgibin/OC_Exp.php?lng=EN&Expert=98818
Mikati, M. A., & Shamseddine, A. N. (2005).
Management of Landau-Kleffner syndrome.
Pediatric Drugs, 7(6), 377–389
REFERENCES
Tharpe, A., and Olson, B. (1994). Landau-Kleffner
Syndrome: Acquired Epileptic Aphasia in
Children. J Am Acad Audiol 5:146-150