Altered Awareness Syndromes

1. Altered Awareness Syndromes Theoretical Basis, Diagnosis, Rehabilitation, Consequences HENRY H. STONNINGTON

2. The five factors influencing an outcome of Functional Autonomy Perceptual Factor Cognitive Factor Motor Factor (upper extremity) Motor Factor (balance) Significant other(s)

3. Reason Perceptual (awareness) factor is vital in rehab outcome Impaired awareness significantly complicates the rehab process. These patients consistently underestimate their impairments, when compared to family members’, clinicians’ ratings, and their performance in neuropsychological tests.

4. Disorders of self awareness The perceptual Factors Theoretical Basis

5. Theoretical Implications (1) Mersulam 1985 Primary motor and Sensory Cortex respond to one type of stimulus (idiotypic) Rest of Cortex “association” 1. “unimodel” – modality specific – association area 2. “heteromodel” – high order-association area

6. Theoretical Implication (2) Heteromodel Association area Frontal lobe, pre-frontal, inferior parietal lobule,superior marginal gyrus, angular gyrus Interface Information External WorldInterior World Sensorimotor cortex Paralimbic areas

7. Theoretical Implications (3)“Syndromes” of impaired awareness • Pre-Frontal: Social judgment, anticipate change, inappropriate social behavior and comments • Inferior Parietal Lobe: self-awareness of body, self image, anosognosia • Superior marginal+angular gyrus+superior temporal lobe: self perception of linguistic output, visual, auditory, memory impairments, misinterpretations leading to paranoid thinking

8. Theoretical Implications (4) • Involvement of Basal Ganglia • Particularly the Putamen, Posterior limb of Internal Capsule, Pulvinar of Thalamus, but also Lentiform and Caudate nuclei. • The frontostriato-pallido-thalamo-frontal neuronal circuit involving the heteromodel association areas

9. ANOSOGNOSIA The inability to be aware of the severity of impairments, believing that everything is intact. This “Unawareness of Impairment” is a Cognitive / Behavioral phenomenon, with a variety of syndromes, related to damage of various heteromodel brain areas, for example: Inferior parietal lobule: Unawareness of hemiplegia, Angular gyrus: aphasic syndromes: unaware of impaired language output.

10. Complete and Incomplete Anosognosia syndromes • Bilateral Cerebral dysfunction in the Heteromodel regions will result in complete syndrome. • Unilateral Cerebral dysfunction will result in partial syndromes. • After unilateral stroke, bilateral dysfunction may be present for short time, and as the bilateral phenomenon clears, the complete anosognosia resolves into incomplete residual unawareness syndromes.

11. NEGLECT • This is different from anosognosia, but sometimes both can be present. If both are present then patient is unaware of, for example, hemineglect • Neglect can be diagnosed particularly with “double sensory stimulation”, touching both arms at the same time, testing both visual fields simultaneously.

12. Particular Unawareness Syndromes

13. Complete Anosognosia In patient with hemiplegia (particularly left hemiplegia, but can occur in right hemiplegia): Denies that hemiplegic side belongs to him/her, says “ Ah that’s Jimmy” or “ that belongs to the guy in the next bed.” That absolute unawareness usually improves as it becomes a partial unawareness syndrome

14. Linguistic Unawareness Syndromes • Aprosodia • Unawareness of language error, inability to self monitor. • Jargon, Wernicke’s aphasia, a fluent aphasia characterized by marked auditory comprehension deficits, babbling with incomprehensive words very fluently. • Reality monitoring: confabulation.

15. Anton’s syndrome • Unable to demonstrate sight: cannot count fingers, discriminate shapes, objects,colors • Pupils react to light • Denies any visual difficulty, confabulates, guesses, makes excuses for errors. • Visual hallucinations • Lesion involves bilateral calcerine cortex, as well as other heteromodel areas.

16. Diagnostics Methods of measuring self-awareness and neglect at the bedside.

17. Physical Examination • History: denials • Observing behavior and denials • Signs: Double sensory stimulation for sensation and visual fields, differentiation from homonymous hemianopia, • testing denials, • Other bed-side tests:

18. Draw a clock with numbers and hands

19. Cancel all E’s and I’s(keep paper straight and quantify misses) HERSIKEzUMINOPENFIKGHEIVZQOPIW MBEZIDVQILMEJYTITSEKIXCEIRYMEK JCINPDE THRINMKEQWRETHIZLFEWIZ IHPWIZNPEKVCDEJMIZXYENPEITRFKI QPESTIKLMEDOPUEAMNIQWTEHTIESX INPESTAKUNOVFKENPIROAEZQPECIT

20. COPY DIAGRAMS

21. THREE DIMENSIONAL DESIGNS Have photograph of blocks And ask patient to copy that design with actual blocks One way of finding Constructional Apraxia If present it will indicate possible difficulties with dressing and other ADLs

22. Skilled Professional Tests • Visual discrimination, figure ground, visual memory, visual synthesis & consistency, • Bells test, a more refined cancellation test, Benton test, Rey complex figure test, • Occupational Therapy perceptual evaluation battery • Aphasia screening test-Halstead-Reitan neuropsychological test battery

23. Behavioral tests for visual neglect Picture scanning, Telephone dialing, Menu reading, Article reading, Time reading, Coin sorting, Sentence copying, Map navigating, Card sorting.

24. Levels of Awareness • Complete Anosognosia • Intellectual Awareness: understanding having difficulty in one specific activity • Emergent Awareness:understanding having difficulty in many circumstances • Anticipatory Awareness: understanding implication of deficit.

25. Management Rehabilitative Therapies

26. Strategies Remediation Strategies Strategies used to regain abilities Compensatory Strategies Strategies used to substitute for lost skills

27. Mobility&Neglect Remediation • Positioning of furniture, • Early correct positioning, and handling, • Controlled transfers, standing up, walking without use of cane or any device. • Lateral transfers over affected hand, looking to affected side, lateral transfer kneeling, always controlled by therapists and nurses, and involving families in techniques.

28. Rehab of neglect, mobility, loss of awareness • Restraining normal side techniques • Full “old fashioned” Proprioceptive- Neuromuscular-Facilitation technique of Kabat/Knott/Voss, ie using a lot of oral and sensory (touching) stimulation • Use of pressure (air splints), taping

29. Motor Memory • The reason Proprioceptive Neuromuscular Facilitation technique is important is: • Memory and Learning involves two systems: Explicit and Implicit. • Explicit means facts, while Implicit (abstract?) involves Perceptual-motor processes. PNF provides Explicit information, attenuating Implicit learning deficits.

30. Kinetic Chain “Closed Kinetic Chain” exercises have become popular in Sport and Musculoskeletal rehabilitation methodology particularly using external loads. Studies have shown this to work. It also can be applied to stroke rehabilitation as it follows the same principles as PNF.

31. BEWARE Although loss of awareness and neglect are most obvious (if looked for) in patients with Left Hemiplegia, it must also be always looked for in Patients with Right Hemiplegia, where it is not uncommon. It always needs to be looked for in all patients who have Brain Injuries or diseases such as brain tumors.

32. VisualCognition Visual Memory Pattern Recognition Scanning Attention Oculomotor Control Visual Fields Visual Acuity

33. Neuro-Rehabilitative Optometry Neuro-optometric rehabilitation is an individualized treatment regimen for visual deficits resulting from physical disabilities, traumatic brain injuries and other neurological insults. Identifying neurological, binocular, motor, perceptual problems, and followed with Orthoptics /Vision Therapies.

34. Neuro-Optometric Therapy The Rehabilitation of Visual / Perceptual / Motor Disorders: Acquired strabismus, diplopia, binocular dysfunction, convergence and/or accommodative paresis/paralysis, oculomotor,visual-spatial dysfunction, visual perceptual, cognitive deficits, Visual field loss, Visual neglect, denial

35. Neuro-Rehabilitative Optometry 2 • Visual Motor Therapy • Visual Perceptual therapy to allow relearning eye-hand coordination providing perceptual information of object size, texture, location, visual discrimination • Neglect/Homonymous hemianopia differ • LATER: Prism, Lenses, Occlusion

36. Remediation of Spatial deficits • Searching for increasingly complex arrays of visual details (figure ground), • Assemble three dimensional figures working through progressive levels of complexity (constructional apraxia), • To improve perception of body schema: name, identify and move neglected body part.

37. Remediation of Visual Spatial Deficits. • Interactive 3-D software (action games, navigation simulators), • In a LEFT hemiplegia, stimulation with TENS, vibration on the LEFT side of neck, and hand as well as pressure and movement appears to activate the contra lateral right hemisphere.

38. Educating family in Visual-Spatial impairments Caretakers are often torn between whether to believe the treatment team or the patient regarding contradictory appraisal of abilities Patient asserts the treatment team is “making a big deal out of nothing”, rallying support of family. Lack of concern of deficits and unawareness of deficits will have profound impact on driving, recreational and vocational pursuits and giving responsibilities to patient.

39. Caretakers It must always be remembered that the counseling and proper management of the caretakers is as much the task of the rehabilitation team, as is the patient/client. There must be early involvement of the caretakers as well as those involved in the community reintegration, such as vocational therapists, and the use of Supportive Employment.

40. CONSEQUENCES LONG TERM OUTCOME

41. Eventual Outcome • “Impaired self-awareness reflects a clear disruption of the integration of thinking and feeling” (Prigatano) • Blame others, become paranoid, and therapists can precipitate a clinical crisis. • There is a positive association between accurate self-awareness and favorable employment outcome ( Sherer ).

42. Further Vocational Facts 30% of patients after a Traumatic Brain Injury resume reasonably productive lifestyles 2 to 4 years after injury. Only 10% remain productive in a 10 – 15 year follow-up. It may well be that this is due to loss of self awareness syndromes, as suggested in the Sherer research studies, but more research is necessary

43. Do we know? Prigatano hypothesized that there may be a possibility that the neural substrate for self-awareness may be the same as the neural substrate for other complex integrative functions that are needed for successful employment outcome. As always in Rehabilitation more research is needed.

44. REFERNCES (1) • Prigatano GP, Disturbances of self-awareness of deficit after traumatic brain injury. IN: Prigatano GP., Schacter DT, eds: Awareness of deficits after brain injury: Clinical and Theoretical Issues, New York N.Y., Oxford University Press,1991 • Prigatano GP: Disorders of self awareness after brain injury, IN: Principles of Neuropsychological Rehabilitation: New York, Oxford, Oxford University Press, 1999 • Mersulam MM: Principles of Behavioral Psychology. F.A. Davis, Philadelphia, 1985

45. REFERNCES (2) • Shaw J. The assessment of Rehabilitation of Visual-Spatial Disorders. IN: Johnstone B., Stonnington HH, eds: Rehabilitation of Neuropsychological Disorders: Psychology Press, Philadelphia, PA, 2001 • Sherer M. et al: Impaired awareness and Employment Outcome after TBI: J. Head Trauma Rehabilitation, 1998: 13(5) 52-61