1 Treatment Strategies and Management Principles for Individuals with Autism who Develop Catatonia-Like Deterioration Jan M. Downey, MA, CCC-SLP Director

1

Treatment Strategies and Management Principles for Individuals with Autism who Develop Catatonia-Like Deterioration

Jan M. Downey, MA, CCC-SLPDirector of Long Island Programs and

ServicesDirector of Speech ServicesEden II ProgramsMay 2014

2

Acknowledgements

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.

3

Autism

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)

4

Catatonia

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)

5

Catatonia and Autism

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)

6

Assessing Catatonia in Individuals with Autism

A marked and obvious deterioration inthe following:• Movement• Vocalizations• Pattern of activities• Self-care• Practical Skills

(Kakooza-Mwesige, A., Wachtel, L.E., Dhossche, D.M., 2008)

7

These Criteria Require at Least Two of the Five Symptoms

Motoric Immobility Excessive Motor Activity Extreme Negativism Peculiarities of Voluntary Movement Echolalia or Echopraxia

Many modern researchers believe that catatonia mayrepresent a separate neurobiological syndrome.

8

Expanded Criteria for Diagnosing Catatonia in Individuals with Autism

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)

9

Catatonia Terminology

There is a distinction between: Catatonic Stupor

&Catatonia-like deterioration

10

Catatonic Stupor

Sudden onset; motionless, apathetic state; individuals appear oblivious to outside

stimuli. Not seen frequently in individuals with ASD. 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

11

Catatonia-Like Deterioration

Small, but growing minority Some parkinsonian features appear

typically during adolescence 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)

12

More on Catatonia-like deterioration

• Chronic condition• Difficult to diagnose• Seen more often in individuals with

ASD• Leads to SEVERE difficulties for

individuals and caregivers• Depending on the severity, non-

medical management (psychological approach) effective

13

Severity of Catatonia

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.

14

Features of Catatonia-like Deterioration

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

15

Identifying Catatonia-Like Deterioration in Individuals with ASD

- 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)

16

Identifying Catatonia-Like Deterioration in Individuals with ASD

- Premorbid symptoms are worsened

- Presentation of classic stupor features is rare

- Absence of waxy flexibility

17

More Features of Catatonia-like Deterioration

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

18

Features of Catatonia-like Deterioration Continued

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)

19

More Features of Catatonia-like Deterioration

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)

20

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)

21

Diagnostic Criteria for Catatonia in Autism

Criterion AImmobilityDrastically decreased speech or Stupor of at least one day duration, associated with a least one of the following: catalepsy, automatic obedience, or posturing

22

Diagnostic Criteria for Catatonia in Autism

Criterion BIn 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)

23

Catatonia Rating Scales and Clinical Assessments

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

24

Differential Diagnosis

Some characteristics of catatonia are alsocharacteristics of autism, such as posturing,stereotypic speech, echolalia, stereotypic orrepetitive behaviors, seemingly purposelessagitation, which could increase the likelihood ofmisdiagnoses. key issue:

emergence of “new” symptoms and/or a “change” in the type and pattern of premorbid functioning.

(Ghaziuddin et al. 2006)

25

Possible Misdiagnoses

Schizophrenia Depression Manic Depression Mood Disorder Psychosis Challenging Behavior Deliberate non-cooperation,

willfulness, laziness, etc.

26

Possible Causes of Catatonia-like Deterioration

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)

27

Rule Out Treatable Causes

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)

28

“Whether a particular disorder isprecipitated or relieved bypsychological factors has nobearing on whether aneurological or psychologicalparadigm is more appropriate forunderstanding it.”

(Rogers, 1992)

29

Identifying Individuals Who May be More Likely to Develop Catatonia in Adolescence 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.

30

Important Considerations

It is important that ALL individualsworking with the student understand thefollowing:

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

31

Important Considerations

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)

32

The Effects of Stress

Continuous stressful experiences area major precipitating factor inmany individuals who developcatatonia-like deterioration.

(Shah and Wing, 2005)

33

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.

34

Management Principles

35

Psychological Treatment and Management

An initial assessment is recommended to ascertain to what degree the catatonia-like deterioration has interfered with the individual’s every day life 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)

36


1. Identify and reduce stress as much as possible

A. May involve restructuring the individual’s lifestyle, environment, daily program B. Resolving cognitive/psychological sources of stress C. Cognitive/behavioral therapy D. Increase motivation and meaningful activities by providing external goals and stimulation E. Programs must be adapted to the individual; e.g., appropriate staffing patterns, increased level of support

37


2. Understanding the Nature of Catatonia-like Deterioration

A. This is a neurological complication that can occur in individuals with ASDB. 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

38


3. Use of Prompts to initiate, continue and complete an activity

A. Level and type of prompting needed may vary from day to dayB. 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

39


F. Individuals will require time to respond to the verbal promptG. 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.

40


4. Maintaining and Increasing ActivityA. To significantly reduce the effects of catatonia-like 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 beneficialC. Meaningful and enjoyable activities that are not difficult for the individual to engage in should be includedD. Activities that require excess physical effort or are difficult for the individual should be avoidedE. 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)


41


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 easierC. Unpredictability and uncertainty increase stress and may increase freezing and mobility issues


42

Management of Specific Difficulties

The Impact on Speech-Language and Communication, Eating and Swallowing, and Overall Daily Living Skills

43

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

44


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

45


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

46

Problems Associated with Eating, Drinking and Swallowing

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

47


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

48


Drinking

• Difficulty initiating drinking - prompt individual to begin drinking (gestural, physical,

verbal) - straw may be helpful

49

Critical Considerations

“Catatonia carries the potential for seriousmedical 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 areat greater risk and demand immediate treatment.

(Kakooza-Mwesige, A., Wachtel, L., Dhossche, D., 2008)

50


Incontinence

• 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.

51


Walking Difficulties

• 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

52


Fixed Postures• Verbally or physically prompt

individual to move• Immediately engage in a different activity

53


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”

54

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

55

Appropriate Environment and Staffing Patterns

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

56

Related Service Providers

Speech-Language Pathologist Programs to focus on: - Increase fluency and response rate - Increase speech intelligibility - Improve feeding and eating skills - Dysphagia

57


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.

58


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.

59

Appropriate Environment and Staffing Patterns

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

60

Pharmacological Treatments

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

61

Electroconvulsive Therapy or Electroshock Therapy (ECT)

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)

62

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)

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

63

Clinical Implications

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)

64

More Questions Than Answers

1. What is the relationship between catatonia-like features that are so common in autistic disorders; e.g., stereotypy, and catatonia-like deterioration?

2. Is there a subgroup of autism that is an early expression of catatonia?

3. Is catatonia found in autism representative of a separate clinical phenomenon?

4. Is catatonia-like deterioration a later complication of autism?

5. Do autism and catatonia share a common neuronal dysfunction with differences in age of onset accounting for the incomplete symptom overlap?

(Dhossche 2004)

65

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)

66

Research Highlights

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

67

Thank you!!!!!

Jan M. Downey, MA, CCC-SLPDirector of L.I. Programs and Services Eden II/GenesisDirector of Speech Services Eden II/Genesis [email protected]

68

References

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.

Documents

1 Treatment Strategies and Management Principles for Individuals with Autism who Develop Catatonia-Like Deterioration Jan M. Downey, MA, CCC-SLP Director