Transcript An Introduction to Catatonia in Autism Spectrum Disorders

Treatment Strategies and Management
Principles for Individuals with Autism who
Develop Catatonia-Like Deterioration
Jan M. Downey, MA, CCC-SLP
Director of Long Island Programs and
Services
Director of Speech Services
Eden II Programs
May 2014
1
Acknowledgements
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Dana Battaglia, my colleague and friend, whose
help and support made this power point possible.
Mary Bainor, my wonderful and dedicated Speech
Coordinator and friend who provided tremendous
support to her “technically challenged” Director.
Piera Interdonati, whose tireless support and
friendship on a daily basis helped to make this
power point possible
Dr. Joanne Gerenser, my supervisor and friend,
whose continuous inspiration and support is
greatly appreciated.
2
Autism
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Neurological disorder that manifests itself within
the first three years of life (Pervasive
Developmental Disorder)
Considered a “spectrum disorder” because
symptoms and severity vary from person to
person
Significantly impairs a person’s abilities
particularly in the areas of language,
communication and social relations
One in every 110 children born today will have
autism
(CDC 2010)3
Catatonia
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Originally described in 1874 by Karl Kahlbaum
as a constellation of motor, affective and vocal
symptoms that can occur at any age
Characterized by abnormalities of movement,
speech and behavior
Currently, the DSM-IV-TR characterizes catatonia as
a specifier for schizophrenia, primary mood
disorders, and mental disorders due to a general
medical condition. It does not recognize catatonia as
a separate disorder.
(L. Wachtel, S. Kahng, D. Dhossche, N. Cascella, I. Reti 2008)
(Kakooza-Mwesige, A., Wachtel, L.E., Dhossche, D.M.
2008)
4
Catatonia and Autism
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Increased recognition of catatonia as a
comorbid syndrome of autism
A limited number of studies suggest
catatonia occurs in 12-17% of
adolescents and young adults with autism
An increasing number of cases of
catatonia in autism have been reported
throughout the world over the last 15
years
(Kakooza-Mwesige, A., Wachtel. L.E., Dhossche, D.M. 2008)
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Assessing Catatonia in Individuals with
Autism
A marked and obvious deterioration in
the following:
• Movement
• Vocalizations
• Pattern of activities
• Self-care
• Practical Skills
(Kakooza-Mwesige, A., Wachtel, L.E., Dhossche, D.M., 2008)
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These Criteria Require at Least Two of
the Five Symptoms
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Motoric Immobility
Excessive Motor Activity
Extreme Negativism
Peculiarities of Voluntary Movement
Echolalia or Echopraxia
Many modern researchers believe that catatonia may
represent a separate neurobiological syndrome.
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Expanded Criteria for Diagnosing
Catatonia in Individuals with Autism
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Slowed movement and verbalizations
Slowed task initiation and completion
Reliance on prompting
Passivity/amotivation
Parkinsonian features
Day-night reversal
Repetitive/Ritualistic behavior
Agitation/Excitement
(Wing and Shah 2008)
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Catatonia Terminology
There is a distinction between:
Catatonic Stupor
&
Catatonia-like deterioration
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Catatonic Stupor
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Sudden onset; motionless, apathetic state;
individuals appear oblivious to outside
stimuli. Not seen frequently in individuals with ASD.
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Akinesia: Absence of movement
Catalepsy: Holding bizarre posture, holding postures
when placed in them; e.g., waxy flexibility
Mutism:
Absence of speech
There can be dramatic recovery with medication

Lorazepam
10
Catatonia-Like Deterioration
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Small, but growing minority
Some parkinsonian features appear
typically during adolescence
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Symptoms are severe enough to interfere with
activities of every day life.
Onset is usually gradual and presentation of
classic stupor features is rare.
Previously classified as Schizophrenia
(Wing & Shah, 2005)
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More on Catatonia-like deterioration
•
•
•
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Chronic condition
Difficult to diagnose
Seen more often in individuals with
ASD
Leads to SEVERE difficulties for
individuals and caregivers
Depending on the severity, nonmedical management (psychological
approach) effective
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Severity of Catatonia
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Severe: Individual is immobile, holds
strange postures and is mute; autonomic
instability and/or fever (blood pressure
problems, heart problems, trouble
breathing and swallowing) may occur.
Moderate: Limited mobility, use of
speech and performing activities of daily
living.
Mild: Less severe form than moderate.
13
Features of Catatonia-like Deterioration
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Marked slowness of movements
Difficulty initiating and completing
movement
Freezing or getting “stuck”, immobile
Decline in self-help skills and independence
May become incontinent
Bizarre gait
Head and trunk twisted
Rigid, stiff, posture
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Identifying Catatonia-Like Deterioration in Individuals
with ASD
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Onset of the deterioration is
characteristically slow; progresses to
extreme obsessive slowing and immobility
Tasks previously mastered (performed
independently) now require assistance;
e.g., ADL skills (showering, eating,
dressing)
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Identifying Catatonia-Like Deterioration in Individuals
with ASD
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Premorbid symptoms are worsened
-
Presentation of classic stupor features is
rare
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Absence of waxy flexibility
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More Features of Catatonia-like
Deterioration
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Walking without arm swinging
Rocking foot to foot
Head bent forward
Arms bent at elbows and wrists
Stereotyped movements of body, limbs
Repetitive attempts to carry out an
action
Inability to stop an action once started
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Features of Catatonia-like Deterioration
Continued
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Facial grimaces
Fixed expression
Fixed empty smile
Fixed gaze
Mouth and tongue movements
Odd finger and hand postures
Turning in circles
(Shah and Wing, 2005)
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More Features of Catatonia-like
Deterioration
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Overactivity
Underactivity
Destructiveness
Self-injury
Violent acts
Sudden bizarre acts
Stripping off clothes
Hypermetamorphosis (an excessive visual
exploration of the environment; excessive
rapid change of ideas)
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How is Catatonia Diagnosed?
“Catatonia is not a diagnosis. Rather it is a descriptive
term for a presentation observed in a wide variety of
disorders” including autism.
(Brasic, J.R., 2009)
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Diagnostic Criteria for Catatonia in
Autism
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Criterion A
Immobility
Drastically decreased speech
or Stupor of at least one day
duration, associated with a least
one of the following: catalepsy,
automatic obedience, or posturing
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Diagnostic Criteria for Catatonia in
Autism
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Criterion B
In the absence of immobility, drastically
decreased speech, or stupor, a marked
increase from baseline, for at least one
week, of at least two of the following:
slowness of movement or speech,
difficulty in initiating movements or
speech unless prompted, freezing during
actions, stereotypy, echophenomena,
catalepsy, automatic obedience,
posturing, negativism, or ambitendency
(Kakooza-Mwesige, A., Wachtel, L.E., Dhossche, D.M 2008)
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Catatonia Rating Scales and Clinical
Assessments
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Currently there are no catatonia rating
scales designed specifically for individuals
with Autism Spectrum Disorder
The BUSH-FRANCIS CATATONIA RATING
SCALE (BFCRS)
DISCO (Diagnostic Interview for Social
and Communication Disorders) contains a
section on catatonic phenomena
23
Differential Diagnosis
Some characteristics of catatonia are also
characteristics of autism, such as posturing,
stereotypic speech, echolalia, stereotypic or
repetitive behaviors, seemingly purposeless
agitation, which could increase the likelihood of
misdiagnoses.
 key issue:
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emergence of “new” symptoms and/or a
“change” in the type and pattern of premorbid
functioning.
(Ghaziuddin et al. 2006)
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Possible Misdiagnoses
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Schizophrenia
Depression
Manic Depression
Mood Disorder
Psychosis
Challenging Behavior
Deliberate non-cooperation,
willfulness, laziness, etc.
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Possible Causes of Catatonia-like
Deterioration
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A neurological problem, however, the
underlying neuropathology is unknown
Weak central coherence
Biological factors; e.g., sickness, pain,
and hormonal changes during puberty
Effects of medication
Autoimmune diseases
Anxiety and Stress
Unknown
(Shah and Wing 2005)
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Rule Out Treatable Causes
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Clinicians must first rule out
treatable causes when presented
with an individual demonstrating
catatonia-like characteristics.
It is necessary to use appropriate
methods of management when a
treatable underlying cause cannot
be identified.
(Brasic, J.R., 2009)
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“Whether a particular disorder is
precipitated or relieved by
psychological factors has no
bearing on whether a
neurological or psychological
paradigm is more appropriate for
understanding it.”
(Rogers, 1992)
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Identifying Individuals Who May be
More Likely to Develop Catatonia in
Adolescence
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Baseline catatonia-Like features in
individuals with autism make them
more susceptible to later developing
catatonic deterioration; e.g., history
of slowed movement, slow to
initiate, slow to respond.
Some researchers suggest that
catatonia-like deterioration is a later
complication of autism.
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Important Considerations
It is important that ALL individuals
working with the student understand the
following:
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The student is not being deliberately
stubborn or willful
The movements (or lack of) are not
under voluntary control
The condition causes severe distress
and frustration for the student
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Important Considerations
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A sensitive and sympathetic approach
should be taken
Catatonia-like deterioration does not
impair cognitive abilities; therefore,
structured activities should be selected
based on the likelihood that they will
motivate the student, and provide
cognitive stimulation
(Shah and Wing 2005)
31
The Effects of Stress
Continuous stressful experiences are
a major precipitating factor in
many individuals who develop
catatonia-like deterioration.
(Shah and Wing, 2005)
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The Stress Factors
*
External factors: e.g., unstructured
environments, loss of routine,
significant life events (death in the
family, moving, divorce, break up of a
relationship, etc.)
* Psychological factors; e.g., experiencing
conflict, pressure, confusion (not
understanding one’s difficulties), or in
higher functioning individuals an
awareness of their limitations and
differences from peers.
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Management Principles
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Psychological Treatment and
Management
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An initial assessment is
recommended to ascertain to what
degree the catatonia-like
deterioration has interfered with the
individual’s every day life
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e.g., activities of daily living, leisure
skills, work/school, etc.
Severity level (severe, mild, moderate)
(National Autistic Society’s Diagnostic Interview for Social and
Communication Disorders…DISCO)
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Management Principles
1.
Identify and reduce stress as much as
possible
A. May involve restructuring the individual’s lifestyle,
B.
C.
D.
E.
environment, daily program
Resolving cognitive/psychological sources of stress
Cognitive/behavioral therapy
Increase motivation and meaningful activities by
providing external goals and stimulation
Programs must be adapted to the individual; e.g.,
appropriate staffing patterns, increased level of
support
36
Management Principles
2.
Understanding the Nature of Catatonia-like
Deterioration
A. This is a neurological complication that can
occur in individuals with ASD
B. Movement effects are not under the
individual’s control
e.g., slowness, difficulty initiating,
episodes of “freezing”, etc.
C. The individual is not engaging in these
behaviors “on purpose”
D. Those affected require a sensitive,
sympathetic, and understanding approach as
the condition must cause them significant
distress and frustration
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Management Principles
3.
Use of Prompts to initiate, continue and
complete an activity
A. Level and type of prompting needed may vary
from day to day
B. The goal of the prompt is to assist the person
in carrying out movements and actions as
smoothly as possible
C. Gestural, followed by physical prompts should
be implemented prior to verbal prompts (as verbal
prompting is more difficult to fade)
D. Verbal prompts can vary from quietly calling
the person’s name to giving instructions specific to
the required task; e.g., “Steven, drink your juice”
E. Prompts may need to be repeated to initiate
and/or continue and complete the task
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Management Principles
F. Individuals will require time to respond to the
verbal prompt
G. Physical prompts should begin with a light touch;
however, if this is not sufficient it should be
increased to gently moving the person in the target
direction
Important note:
•
Parents, teachers, etc., may be concerned about the Individual
becoming prompt dependent or encroaching on the individual’s
right to privacy and dignity
•
however, the possible long term effects of catatonia-like
deterioration on independence and functioning if the condition
progresses make prompting necessary for the individual to
overcome the difficulties in the central control of voluntary
movement, and gradually regain their independence.
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Management Principles
4.
Maintaining and Increasing Activity
A. To significantly reduce the effects of catatonialike deterioration, the individual should be kept
active, mobile and stimulated without placing
additional demands or pressure.
B. Rhythmic activities such as walking, swimming,
bicycling, roller skating, dancing, etc., are very
beneficial
C. Meaningful and enjoyable activities that are not
difficult for the individual to engage in should be
included
D. Activities that require excess physical effort or
are difficult for the individual should be avoided
E. May be helpful for the individual to participate in
small group activities as the momentum of the group
may assist the individual to begin and continue the
activity (1:1 support and guidance may still be
necessary)
(Shah and Wing, 2005)
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Management Principles
5.
Structure and Routine
A. A structured plan of activities (as previously
stated) and a predictable routine are important
for the individual to develop the habit of
participation
B. Rather than new and/or sporadic activities,
habitual actions are much easier
C. Unpredictability and uncertainty increase
stress and may increase freezing and mobility
issues
(Shah and Wing, 2005)
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Management of Specific
Difficulties
The Impact on Speech-Language and
Communication, Eating and Swallowing, and Overall
Daily Living Skills
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Management of Specific Problems
Speech and Communication Problems
•
Individuals who once demonstrated good speech
intelligibility may become somewhat unintelligible,
at times, due to imprecise placement and/or
strength of articulators
- speech therapy to improve production of target
sounds; i.e., placement and strengthening
exercises
- verbal imitation drills of frequently used
words/phrases/sentences targeting those sounds
- target sounds presented in pictures (magazines,
books, etc.), written paragraphs for independent
production
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Management of Specific Problems
Speech and Communication Problems
•
Individuals may take longer to verbally
respond
- when appropriate encourage non-verbal
responses; e.g., thumbs up/down, pointing,
waving, etc.
- Reduce pressure to talk, but talk to the
individual focusing on the current activity
- Target goals to increase fluency and rate
of responding
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Management of Specific Problems
Speech and Communication Problems
•
Difficulty making choices
- Suggest and encourage based on
knowledge of the individual’s likes and
dislikes
- May need to make choices, at times, for
the individual based on knowledge of their
likes and dislikes
- Visual communication systems may be
helpful; e.g., written scripts, pictures
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Problems Associated with Eating,
Drinking and Swallowing
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Dysphagia
- Important to rule out physical abnormalities
- Modified Barium Swallow Study to assess
oropharyngeal swallowing function
Difficulty initiating the swallow
- try to provide a relaxed environment
46
Problems Associated with Eating,
Drinking and Swallowing
Eating Problems
•
Poor motor coordination and movement using
utensils as well as articulators
- use a spoon rather than a fork and knife
- may need to adjust food consistency
- may need to use a bowl rather than a plate
•
Difficulty initiating and completing the movements
- May need to prompt (1. gesture, 2. physical, 3.
verbal) to initiate and continue eating throughout the
meal
- Individual may have to be fed if prompts are not
effective
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Problems Associated with Eating,
Drinking and Swallowing
Drinking
•
Difficulty initiating drinking
- prompt individual to begin drinking (gestural, physical,
verbal)
- straw may be helpful
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Critical Considerations
“Catatonia carries the potential for serious
medical morbidity and mortality.”
(Kakooza-Mwesige, A., Wachtel, L., Dhossche, D., 2008)
Individuals with catatonia-like deterioration may experience:
 Significant weight loss
 Dehydration
 Possible exacerbation of other aspects of their condition if
their eating and nutrition are not closely monitored.
Individuals with malignant catatonia who present with fever,
altered consciousness, stupor, and autonomic instability are
at greater risk and demand immediate treatment.
(Kakooza-Mwesige, A., Wachtel, L., Dhossche, D., 2008)
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Management of Specific Problems
Incontinence
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Regularly scheduled bathroom times
Frequent prompts to use the bathroom
Provide enough time to get to the bathroom
Provide assistance (if necessary) with clothing,
etc.
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Management of Specific Problems
Walking Difficulties
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May be able to walk without stopping when holding
caregiver’s arm
Light physical prompt to initiate or continue walking
Walking in a group sometimes helpful
External stimulus; e.g., walking a dog while holding on
to the leash
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Management of Specific Problems
Fixed Postures
•
Verbally or physically prompt
individual to move
•
Immediately engage in a different
activity
52
Management of Specific Problems
Catatonia-Like Excitement
•
“episodes of uncontrollable, frenzied,
and inappropriate behavior”
•
May be wrongly interpreted as
outbursts of “challenging” behavior
causing teachers, caregivers to look
for “triggers” or “communicative
functions”
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Management of Specific Behaviors
•
•
If the episode is of short duration,
may be best to ensure safety, but
not intervene
Longer lasting episodes may require
intervention; e.g., distract the
individual to something else,
physically lead to different
environment
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Appropriate Environment and Staffing
Patterns
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Individuals with Autism Spectrum Disorder
who develop catatonia-like deterioration
need an intensive program in the right
environment
Depending on the severity the individual
may need 24 hour care in a structured
environment where an organized daily
program can be implemented consistently
A high staff ratio and sufficient trained staff
is essential
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Related Service Providers
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Speech-Language Pathologist
Programs to focus on:
- Increase fluency and response
rate
- Increase speech intelligibility
- Improve feeding and eating skills
- Dysphagia
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Related Service Providers
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Occupational Therapist
- Increase fine motor skills (ability and
speed) to perform various activities of
daily living; e.g., dressing, showering,
toileting, etc.
- Increase ability and speed of school
related tasks; e.g., writing, computer,
unpacking/packing backpack, taking out
lunch or buying lunch, opening/closing
locker.
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Related Service Providers
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Physical Therapist
Physical therapy should target:
- activities to increase initiating
- stretching of muscles that have become
“tight” or “stiff” due to rigid posture and
decreased mobility
- Increasing ability and speed of gross
motor skills needed to perform activities
of daily living; e.g., bending
down/standing up, sitting down/standing
up, reaching for items on a high shelf,
walking up/down stairs, etc.
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Appropriate Environment and Staffing
Patterns
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Access to appropriate stimulating physical
and occupational activities are vital
In the initial stages individuals with
catatonia-like deterioration will require a
full time 1:1 aide (it is preferable that more
than one person become familiar with the
individual to build a rapport and
relationship)
Once the individual begins improving and
becoming more independent the level of
staffing can be systematically decreased
Front loading the increased staff may
decrease the duration that so much support
is needed
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Pharmacological Treatments
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Benzodiazepines (Lorazepam is
usually prescribed, 2mg. 3x per day
and gradually increased until near
baseline is achieved)
Studies have shown Lorazepam
more effective in individuals with
catatonic stupor as opposed to
individuals with catatonic
deterioration
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Electroconvulsive Therapy or
Electroshock Therapy (ECT)
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Electric currents are shot through the brain
causing a grand-mal seizure
Usually administered in a series of treatments;
e.g., 6-12, then maintenance treatment that is
tapered
Highly controversial method of treating severe
depression, mood disorders, schizophrenia, and
catatonia
Some researchers suggest ECT should figure
prominently in the treatment for individuals
with autism who develop severe catatonic
deterioration
(Dhossche et. al 2008)
61
Summary/Conclusions

Recent reports indicate that individuals on the autism
spectrum have an increased incidence of catatonic
symptoms, as well as frank catatonic deterioration
(Wachtel,L., Kahng, S., Dhossche,D., Cascella, N., Reti, I. 2008)
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It is estimated that 12-17% of individuals with autism,
typically between the ages of 15-20 will develop
catatonia-like deterioration
Socially passive individuals as well as those with
“catatonia-like” symptoms at a younger age are at an
increased risk of developing catatonic deterioration in
adolescence
Significantly interferes with every day activities
Presents severe difficulties for the individuals and their
caregivers
Early recognition and proper treatment are essential if
the individual is to improve and possibly recover
62
Clinical Implications
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Catatonia is a later complication of
autistic spectrum disorders which adds
considerably to the burden of caring
Catatonia develops in adolescence in a
small proportion of individuals with
autistic spectrum disorders
Recognition of catatonia in individuals
with autistic spectrum disorders is
necessary in order to institute appropriate
management and care
(Wing, L. Shah, A., 2000)
63
More Questions Than Answers
1.
2.
3.
4.
5.
What is the relationship between catatonia-like
features that are so common in autistic disorders;
e.g., stereotypy, and catatonia-like deterioration?
Is there a subgroup of autism that is an early
expression of catatonia?
Is catatonia found in autism representative of a
separate clinical phenomenon?
Is catatonia-like deterioration a later complication
of autism?
Do autism and catatonia share a common
neuronal dysfunction with differences in age of
onset accounting for the incomplete symptom
overlap?
(Dhossche 2004)
64
Future Research
Future research should include:
• Investigation of the neuropathology
• Identification of the early signs of
vulnerability to exacerbation of catatonic
features
• Role of environmental stress
• Methods of management and treatment
(Wing, L., Shah, A., 2008)
65
Research Highlights
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Currently, The Cody Center @ Stony
Brook University, NY is conducting
research with their patients who have
autism and catatonia-like deterioration
Columbia Presbyterian Hospital, NY is
performing ongoing genetic testing on
individuals on the autism spectrum who
develop catatonia-like deterioration.
The Kennedy Krieger Institute in Maryland
treats individuals with ASD and
Catatonia-Like Deterioration
66
Thank you!!!!!
Jan M. Downey, MA, CCC-SLP
Director of L.I. Programs and Services Eden II/Genesis
Director of Speech Services Eden II/Genesis Programs
[email protected]
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References
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Dhossche, D., Wing, L., Ohta, M., Neumarker, K-J.
(2006). Catatonia in Autism Spectrum Disorders.
International Review of Neurobiology, Vol. 72.
Dhossche, D. (1998). Brief report: Catatonia in
autistic disorders. J. Autism Dev. Disord. 2B, 329-331.
Fink, M. and Taylor, M.A. (2003). “Catatonia: A
Clinician’s Guide to Diagnosis and Treatment.”
Cambridge University Press, Cambridge.
Ghaziuddin, M., Quinlan, P., and Ghaziuddin, N.
(2005). Catatonia in autism: A distinct subtype? J.
Intellec. Disabil. Res. 49, 102-105.
Hare, D.J., and Malone, C. (2004). Catatonia and
autistic spectrum disorders. Autism 8, 183-195.
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