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04/20/23
CLASSROOM INSTRUCTION for CHILDREN WITH LANDAU-KLEFFNER SYNDROME
Pat Van Slyke, Ph.D./ CCCSpeech Language PathologistSPEECH PATHOLOGY CONSULTANTS, LLC
[email protected](847) 828-7093
95 Grant St.Crystal Lake, Illinois 60014
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Landau-Kleffner Syndrome (LKS) is:
Rare form of acquired childhood aphasia
Abnormal electroencephalographic (EEG) findings in speech cortex
Continuous spike and wave pattern during sleep
Posterior temporal regions (usually)
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Overt seizures or subclinical Language deterioration after
period of normal cognitive and language development
Onset between 2 1/2 - 6 years of age
Severe auditory verbal agnosia
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Auditory Verbal Agnosia: Rapin et al. (1977) described
this as a severe comprehension disorder due to the disruption of the auditory input system
Not a disruption of the cortical structure
Children with LKS appear to have a progressive deterioration of the auditory response to lang.
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Concomitant characteristics may include attention deficits and behavioral disturbances
First reported by Dr. William Landau and Dr. Frank Kleffner in 1957
Currently 200 + cases reported
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Etiology hypotheses: Inflammatory
process Encephalitis Slow type of
virus Myelin defect
Low erythrocyte zinc content
Toxoplasma Gondii infection
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Current hypothesis from Morrell et al. (1995):
Epileptiform activity develops during critical time period when the basic circuitry for speech is being established
Synaptogenesis is disrupted and inappropriate connections are formed resulting in disrupted language acquisition
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Age of onset is critical: Bishop
(1985):cases found the younger the child at onset, the worse the prognosis for language recovery
Late onset cases have not shown as severe a loss in language, either receptively or expressively
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Treatment: Pharmacological with
anticonvulsants, corticosteroids and/or a combination of the two.
Surgical intervention: Multiple Subpial Transection (MST)
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MST: Developed by Dr. F. Morrell and
Dr. W. Whisler at RPSLMC Severs the “horizontal
intracortical fibers, while preserving the vertical fiber connection of the incoming and outgoing nerve pathways and the penetrating blood vessels” (Morrell et al., 1989)
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Language characteristics: Deterioration
can be rapid, progressive, but not acute as seen in TBI patients
Wide variations
Exacerbation and remission depending on the seizure control
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During recovery language continues to vary with these children. Don’t see a uniform profile
Auditory verbal agnosia: severe deficits in comprehen-sion of language
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Some children lose all verbal language and become mute
Usually respond to sign language
Telegraphic speech
Jargon Good nonverbal
means of communicating
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Recovery dependent on: Frequency of epileptic activity in
brain Duration of the activity Extent to which activity spreads
to other areas of brain Efficacy of the anti-epileptic
drugs
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Recovery in general is slow and gradual over time
Auditory verbal agnosia remains, even after surgery and takes quite some time to resolve
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Classroom performance during recovery:
Myth of being cured Heavy language
demands of the classroom
Academic success Impact of Auditory
Verbal Agnosia
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Classroom methodology reports: Vance (1991) reports using sign
language, daily diary of sequenced pictures for classroom routine, auditory training, and Cued Articulation (developed by Passy, 1990)
Also uses ‘graphic conversations’ with written word of literal word on balloons
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Lea (1979) describes a Color - Pattern Scheme
Worster-Drought (1971) uses reading to teach language
Others describe using written language to by pass the auditory verbal agnosia
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Curriculum modifications:
READING:
Computer program with decoding skills, high visual interest and auditory reinforcement of sounds/symbols
One to one teaching with color coding, letter sign and sound
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Phonemic awareness skills Delete word families and choral
rehearsal Sign language and short picture
stories to increase comprehension
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Modifications: MATHAMATICS:
Teach the concept rather than rote memory with manipulatives
Picture sequence for steps in addition and subtraction
Initiate his own work rather than drill
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General academic modifications: Visual daily lesson plan on desk
i.e. circle, book, pencil, swing, numbers, sandwich, map, X and a bus.
Sign language for all subject areas
Aide should pre-teach one day in advance the content area
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Aide have same materials and pictures on desk as teacher is doing on board and direct J2’s attention to each point
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Used picture symbols and drawing to communicate to teachers to compensate for severe auditory verbal agnosia
Story boards initiated in 2nd grade through 4th for daily scheduling
Used FM trainer in classroom
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Sign language used until 7 months post surgery
Instructional language of classroom modified to meet his functional language level for science and social studies grade level curriculum
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Use of computer decoding programs
Intensive program to teach phonemic awareness skills
Reduced the amount of time 1:1 aide is in classroom as his understanding increased
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Moved from a point system for behavior modification to ‘time to reflect’ to create his own internal self control.
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Curriculum modifications: Four hours of speech-language
therapy per week Twenty hours per week 1:1
teaching per week on academics with aide
Using Discrete trial from ABA
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Data collected on trials weekly and changes in cueing procedures were made
1:1 aide in the classroom full days
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Discussion: Variable learning styles Fluctuation of language
abilities varies significantly, primarily when the seizure activity is not under control
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One child prior to surgery could not even comprehend his/her name; seven months post surgery - functioning at a 7 year linguistic level.
Unusual case, probably because the seizure focus was in the non-dominant hemisphere
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Language skills in the dominant hemisphere were masked by the spread of activity from the right side (non dominant)
Some had greatest period of growth in academic learning 18-24 months post surgery.
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Most continue to show vocabulary and language growth 3-7 years post
Understanding of the oral input in the classroom was difficult for all of these children due to the severe deficit in processing the information
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Important note: None of the children demonstrated regression of language, once the abnormal epileptiform activity was controlled, either through medication or surgery.
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General guidelines: Small
language based classroom
1:1 aide Supportive
teaching environment
Intensive S & L therapy
Sign language as an alternative
Functional approach to communica-tion
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Use of visual aides with pictures, color coding, and drawing
Functional levels of language considered
Computer programs to assist in decoding skills
Closely monitor the interventions
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ACADEMIC SUCCESS