Jan M. Downey, MA, CCC-SLP Director of Long Island Programs and Services

1. 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

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 in the 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 may represent 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 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

22. Diagnostic Criteria for Catatonia in Autism • 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)

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 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: • emergence of “new” symptomsand/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 andStress • 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 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)

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 individuals working with the student understand the following: • 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 are a major precipitating factor in many individuals who develop catatonia-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. Management Principles • 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. Management Principles • 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

38. Management Principles • 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

39. 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.

40. Management Principles • Maintaining and Increasing Activity A. 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 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)

41. Management Principles • 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)

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. 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

45. 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

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. 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

48. Problems Associated with Eating, Drinking and Swallowing Drinking • Difficulty initiating drinking - prompt individual to begin drinking (gestural, physical, verbal) - straw may be helpful

49. 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)

50. Management of Specific Problems 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.