Altered Awareness Syndromes

Theoretical Basis, Diagnosis, Theoretical Basis, Diagnosis, Rehabilitation, ConsequencesRehabilitation, Consequences

HENRY H. STONNINGTONHENRY H. STONNINGTON

The five factors influencing an outcome of Functional Autonomy

Perceptual FactorPerceptual Factor

Cognitive FactorCognitive Factor

Motor Factor Motor Factor (upper extremity)(upper extremity)

Motor Factor Motor Factor (balance)(balance)

Significant other(s)Significant other(s)

Reason Perceptual (awareness) factor is vital in rehab outcome

Impaired awareness significantly complicates Impaired awareness significantly complicates the rehab process. These patients the rehab process. These patients consistently underestimate their consistently underestimate their impairments, when compared to family impairments, when compared to family members’, clinicians’ ratings, and their members’, clinicians’ ratings, and their performance in neuropsychological tests. performance in neuropsychological tests.

Disorders of self awareness

The perceptual FactorsThe perceptual Factors

Theoretical BasisTheoretical Basis

Theoretical Implications (1)

Mersulam 1985Mersulam 1985Primary motor and Sensory CortexPrimary motor and Sensory Cortex respond respond

to one type of stimulus (idiotypic)to one type of stimulus (idiotypic)Rest of Cortex Rest of Cortex “association”“association” 1. “unimodel” – 1. “unimodel” – modality specificmodality specific – –

association areaassociation area 2. “heteromodel” – 2. “heteromodel” – high orderhigh order-association -association

areaarea

Theoretical Implication (2)

Heteromodel Association areaHeteromodel Association area

Frontal lobe, pre-frontal, inferior parietal lobule,Frontal lobe, pre-frontal, inferior parietal lobule, superior marginal gyrus, angular gyrussuperior marginal gyrus, angular gyrus

Interface InformationInterface Information

External WorldExternal World Interior WorldInterior World

Sensorimotor cortex Paralimbic areasSensorimotor cortex Paralimbic areas

Theoretical Implications (3)“Syndromes” of impaired awareness

Pre-FrontalPre-Frontal: Social judgment, anticipate change, : Social judgment, anticipate change, inappropriate social behavior and commentsinappropriate social behavior and comments

Inferior Parietal LobeInferior Parietal Lobe: self-awareness of body, self : self-awareness of body, self image, anosognosiaimage, anosognosia

Superior marginal+angular gyrus+superior Superior marginal+angular gyrus+superior temporal lobetemporal lobe: self perception of linguistic output, : self perception of linguistic output, visual, auditory, memory impairments, visual, auditory, memory impairments, misinterpretations leading to paranoid thinkingmisinterpretations leading to paranoid thinking

Theoretical Implications (4)

Involvement of Basal GangliaInvolvement of Basal Ganglia Particularly the Putamen, Posterior limb of Particularly the Putamen, Posterior limb of

Internal Capsule, Pulvinar of Thalamus, but Internal Capsule, Pulvinar of Thalamus, but also Lentiform and Caudate nuclei.also Lentiform and Caudate nuclei.

The frontostriato-pallido-thalamo-frontal The frontostriato-pallido-thalamo-frontal neuronal circuit involving the neuronal circuit involving the heteromodel association areasheteromodel association areas

ANOSOGNOSIA

The inability to be aware of the severity of impairments, The inability to be aware of the severity of impairments, believing that everything is intact.believing that everything is intact.

This “Unawareness of Impairment” is a This “Unawareness of Impairment” is a Cognitive / Behavioral phenomenon, with a variety of Cognitive / Behavioral phenomenon, with a variety of

syndromes, related to damage of various heteromodel syndromes, related to damage of various heteromodel brain areas, for example:brain areas, for example:

Inferior parietal lobule: Unawareness of hemiplegia, Inferior parietal lobule: Unawareness of hemiplegia, Angular gyrus: aphasic syndromes: unaware of Angular gyrus: aphasic syndromes: unaware of impaired language output.impaired language output.

Complete and Incomplete Anosognosia syndromes

Bilateral Cerebral dysfunction in the Bilateral Cerebral dysfunction in the Heteromodel regions will result in complete Heteromodel regions will result in complete syndrome.syndrome.

Unilateral Cerebral dysfunction will result in Unilateral Cerebral dysfunction will result in partial syndromes.partial syndromes.

After unilateral stroke, bilateral dysfunction After unilateral stroke, bilateral dysfunction may be present for short time, and as the may be present for short time, and as the bilateral phenomenon clears, the complete bilateral phenomenon clears, the complete anosognosia resolves into incomplete residual anosognosia resolves into incomplete residual unawareness syndromes.unawareness syndromes.

NEGLECT

This is different from anosognosiaThis is different from anosognosia, but , but sometimes both can be present. If both are sometimes both can be present. If both are present then patient is unaware of, for present then patient is unaware of, for example, hemineglectexample, hemineglect

Neglect can be diagnosed particularly with Neglect can be diagnosed particularly with “double sensory stimulation”, touching both “double sensory stimulation”, touching both arms at the same time, testing both visual arms at the same time, testing both visual fields simultaneously. fields simultaneously.

Particular Unawareness Syndromes

Complete Anosognosia

In patient with hemiplegia (particularly left In patient with hemiplegia (particularly left hemiplegia, but can occur in right hemiplegia):hemiplegia, but can occur in right hemiplegia):

Denies that hemiplegic side belongs to him/her, says Denies that hemiplegic side belongs to him/her, says “ Ah that’s Jimmy” or “ that belongs to the guy in “ Ah that’s Jimmy” or “ that belongs to the guy in the next bed.”the next bed.”

That absolute unawareness usually improves as it That absolute unawareness usually improves as it becomes a partial unawareness syndromebecomes a partial unawareness syndrome

Linguistic Unawareness Syndromes

AprosodiaAprosodia Unawareness of language error, inability to Unawareness of language error, inability to

self monitor.self monitor. Jargon, Wernicke’s aphasia, a fluent Jargon, Wernicke’s aphasia, a fluent

aphasia characterized by marked auditory aphasia characterized by marked auditory comprehension deficits, babbling with comprehension deficits, babbling with incomprehensive words very fluently.incomprehensive words very fluently.

Reality monitoring: confabulation.Reality monitoring: confabulation.

Anton’s syndrome

Unable to demonstrate sight: cannot count Unable to demonstrate sight: cannot count fingers, discriminate shapes, objects,colorsfingers, discriminate shapes, objects,colors

Pupils react to lightPupils react to light Denies any visual difficulty, confabulates, Denies any visual difficulty, confabulates,

guesses, makes excuses for errors.guesses, makes excuses for errors. Visual hallucinationsVisual hallucinations Lesion involves bilateral calcerine cortex, Lesion involves bilateral calcerine cortex,

as well as other heteromodel areas.as well as other heteromodel areas.

Diagnostics

Methods of measuring self-Methods of measuring self-awareness and neglect at awareness and neglect at

the bedside.the bedside.

Physical Examination

History: denialsHistory: denials Observing behavior and denialsObserving behavior and denials Signs: Double sensory stimulation for Signs: Double sensory stimulation for

sensation and visual fields, differentiation sensation and visual fields, differentiation from homonymous hemianopia,from homonymous hemianopia,

testing denials,testing denials, Other bed-side tests: Other bed-side tests:

Draw a clock with numbers and hands

Cancel all E’s and I’s(keep paper straight and quantify misses)

HERSIKEHERSIKEzzUMINOPENFIKGHEIVZQOPIWUMINOPENFIKGHEIVZQOPIW

MBEZIDVQILMEJYTITSEKIXCEIRYMEKMBEZIDVQILMEJYTITSEKIXCEIRYMEK

JCINPDE THRINMKEQWRETHIZLFEWIZJCINPDE THRINMKEQWRETHIZLFEWIZ

IHPWIZNPEKVCDEJMIZXYENPEITRFKIIHPWIZNPEKVCDEJMIZXYENPEITRFKI

QPESTIKLMEDOPUEAMNIQWTEHTIESXQPESTIKLMEDOPUEAMNIQWTEHTIESX

INPESTAKUNOVFKENPIROAEZQPECITINPESTAKUNOVFKENPIROAEZQPECIT

COPY DIAGRAMS

THREE DIMENSIONAL DESIGNS

Have photograph of blocksHave photograph of blocks

And ask patient to copy that design with And ask patient to copy that design with actual blocksactual blocks

One way of finding One way of finding Constructional ApraxiaConstructional Apraxia

If present it will indicate possible difficulties If present it will indicate possible difficulties with dressing and other ADLswith dressing and other ADLs

Skilled Professional Tests

Visual discrimination, figure ground, visual Visual discrimination, figure ground, visual memory, visual synthesis & consistency,memory, visual synthesis & consistency,

Bells test, a more refined cancellation test, Bells test, a more refined cancellation test, Benton test, Rey complex figure test,Benton test, Rey complex figure test,

Occupational Therapy perceptual evaluation Occupational Therapy perceptual evaluation batterybattery

Aphasia screening test-Halstead-Reitan Aphasia screening test-Halstead-Reitan neuropsychological test batteryneuropsychological test battery

Behavioral tests for visual neglect

Picture scanning, Telephone dialing,Picture scanning, Telephone dialing,

Menu reading, Article reading,Menu reading, Article reading,

Time reading, Coin sorting, Time reading, Coin sorting,

Sentence copying, Map navigating, Sentence copying, Map navigating,

Card sorting. Card sorting.

Levels of Awareness

CompleteComplete Anosognosia Anosognosia Intellectual AwarenessIntellectual Awareness:: understanding

having difficulty in one specific activity Emergent Awareness:Emergent Awareness: understanding

having difficulty in many circumstances Anticipatory Awareness: Anticipatory Awareness: understanding

implication of deficit.

Management

Rehabilitative TherapiesRehabilitative Therapies

Strategies

Remediation StrategiesRemediation Strategies

Strategies used to Strategies used to regain regain abilitiesabilities

Compensatory StrategiesCompensatory Strategies

Strategies used to Strategies used to substitute substitute for lost skillsfor lost skills

Mobility&Neglect Remediation

Positioning of furniture,Positioning of furniture, Early correct positioning, and handling,Early correct positioning, and handling, ControlledControlled transfers, standing up, walking transfers, standing up, walking

withoutwithout use of cane or any device. use of cane or any device. Lateral transfers over affected hand, looking Lateral transfers over affected hand, looking

to affected side, lateral transfer kneeling, to affected side, lateral transfer kneeling, always controlled by therapists and nurses, always controlled by therapists and nurses, and involving families in techniques.and involving families in techniques.

Rehab of neglect, mobility, loss of awareness

Restraining normal side techniquesRestraining normal side techniques Full “old fashioned” Proprioceptive- Full “old fashioned” Proprioceptive-

Neuromuscular-Facilitation technique of Neuromuscular-Facilitation technique of Kabat/Knott/Voss, ie using a lot of oral and Kabat/Knott/Voss, ie using a lot of oral and sensory (touching) stimulationsensory (touching) stimulation

Use of pressure (air splints), tapingUse of pressure (air splints), taping

Motor Memory

The reason Proprioceptive Neuromuscular The reason Proprioceptive Neuromuscular Facilitation technique is important is:Facilitation technique is important is:

Memory and Learning involves two systems: Memory and Learning involves two systems: Explicit and Implicit.Explicit and Implicit.

Explicit means facts, while Implicit (abstract?) Explicit means facts, while Implicit (abstract?) involves involves Perceptual-motor Perceptual-motor processes. PNF processes. PNF provides provides ExplicitExplicit information, attenuating information, attenuating ImplicitImplicit learning deficits. learning deficits.

Kinetic Chain

““Closed Kinetic Chain” exercises have Closed Kinetic Chain” exercises have become popular in Sport and become popular in Sport and Musculoskeletal rehabilitation methodology Musculoskeletal rehabilitation methodology particularly using external loads. Studies particularly using external loads. Studies have shown this to work.have shown this to work.

It also can be applied to stroke rehabilitation It also can be applied to stroke rehabilitation as it follows the same principles as PNF.as it follows the same principles as PNF.

BEWARE

Although loss of awareness and neglect are Although loss of awareness and neglect are most obvious (if looked for) in patients with most obvious (if looked for) in patients with Left Hemiplegia, it must also be always Left Hemiplegia, it must also be always looked for in Patients with Right looked for in Patients with Right Hemiplegia, where it is not uncommon.Hemiplegia, where it is not uncommon.

It always needs to be looked for in It always needs to be looked for in allall patients patients who have Brain Injuries or diseases such as who have Brain Injuries or diseases such as brain tumors.brain tumors.

Visual Cognition

Visual Memory

Pattern Recognition

Scanning

Attention

Oculomotor Control Visual Fields Visual Acuity

Neuro-Rehabilitative Optometry

Neuro-optometric rehabilitation is an Neuro-optometric rehabilitation is an individualized treatment regimen for individualized treatment regimen for visual deficits resulting from physical visual deficits resulting from physical disabilities, traumatic brain injuries and disabilities, traumatic brain injuries and other neurological insultsother neurological insults..

IdentifyingIdentifying neurological, binocular, motor, neurological, binocular, motor, perceptual problems, and followed withperceptual problems, and followed with

Orthoptics /Vision Therapies.Orthoptics /Vision Therapies.

Neuro-Optometric Therapy

The Rehabilitation of The Rehabilitation of Visual / Perceptual / Motor Disorders: Visual / Perceptual / Motor Disorders: Acquired strabismus, diplopia, binocular Acquired strabismus, diplopia, binocular

dysfunction, convergence and/or dysfunction, convergence and/or accommodative paresis/paralysis,accommodative paresis/paralysis,

oculomotor,oculomotor, visual-spatial dysfunction, visual-spatial dysfunction, visual perceptual, cognitive deficits, visual perceptual, cognitive deficits,

Visual field loss, Visual neglect, denialVisual field loss, Visual neglect, denial

Neuro-Rehabilitative Optometry 2

Visual Motor TherapyVisual Motor Therapy Visual Perceptual therapy to allow Visual Perceptual therapy to allow

relearning eye-hand coordination relearning eye-hand coordination providing perceptual information of providing perceptual information of object size, texture, location, visual object size, texture, location, visual discriminationdiscrimination

Neglect/Homonymous hemianopia differNeglect/Homonymous hemianopia differ LATER: Prism, Lenses, OcclusionLATER: Prism, Lenses, Occlusion

Remediation of Spatial deficits

Searching for increasingly complex arrays Searching for increasingly complex arrays of visual details (figure ground),of visual details (figure ground),

Assemble three dimensional figures Assemble three dimensional figures working through progressive levels of working through progressive levels of complexity (constructional apraxia),complexity (constructional apraxia),

To improve perception of body schema: To improve perception of body schema: name, identify and move neglected body name, identify and move neglected body part.part.

Remediation of Visual Spatial Deficits.

Interactive 3-D software (action games, Interactive 3-D software (action games, navigation simulators),navigation simulators),

In a LEFT hemiplegia, stimulation with In a LEFT hemiplegia, stimulation with TENS, vibration on the LEFT side of neck, TENS, vibration on the LEFT side of neck, and hand as well as pressure and movement and hand as well as pressure and movement appears to activate the contra lateral right appears to activate the contra lateral right hemisphere.hemisphere.

Educating family in Visual-Spatial impairments

Caretakers are often torn between whether to believe Caretakers are often torn between whether to believe the treatment team or the patient regarding the treatment team or the patient regarding contradictory appraisal of abilitiescontradictory appraisal of abilities

Patient asserts the treatment team is “making a big Patient asserts the treatment team is “making a big deal out of nothing”, rallying support of family.deal out of nothing”, rallying support of family.

Lack of concern of deficits and unawareness of Lack of concern of deficits and unawareness of deficits will have profound impact on driving, deficits will have profound impact on driving, recreational and vocational pursuits and giving recreational and vocational pursuits and giving responsibilities to patient.responsibilities to patient.

Caretakers

It must always be remembered that the counseling It must always be remembered that the counseling and proper management of the caretakers is as and proper management of the caretakers is as much the task of the rehabilitation team, as is the much the task of the rehabilitation team, as is the patient/client. patient/client.

There must be There must be earlyearly involvement of the caretakers as involvement of the caretakers as well as those involved in the community well as those involved in the community reintegration, such as vocational therapists, and reintegration, such as vocational therapists, and the use of Supportive Employment.the use of Supportive Employment.

CONSEQUENCES

LONG TERM OUTCOMELONG TERM OUTCOME

Eventual Outcome

““Impaired self-awareness reflects a clear Impaired self-awareness reflects a clear disruption of the integration of thinking and disruption of the integration of thinking and feeling” (Prigatano)feeling” (Prigatano)

Blame others, become paranoid, and Blame others, become paranoid, and therapists can precipitate a clinical crisis.therapists can precipitate a clinical crisis.

There is a positive association between There is a positive association between accurate self-awareness and favorable accurate self-awareness and favorable employment outcome ( Sherer ).employment outcome ( Sherer ).

Further Vocational Facts

30% of patients after a Traumatic Brain Injury 30% of patients after a Traumatic Brain Injury resume reasonably productive lifestyles 2 to 4 resume reasonably productive lifestyles 2 to 4 years after injury.years after injury.

Only 10% remain productive in a 10 – 15 year Only 10% remain productive in a 10 – 15 year follow-up.follow-up.

It may well be that this is due to loss of self It may well be that this is due to loss of self awareness syndromes, as suggested in the Sherer awareness syndromes, as suggested in the Sherer research studies, but more research is necessaryresearch studies, but more research is necessary

Do we know?

Prigatano hypothesized that there may be a Prigatano hypothesized that there may be a possibility that the neural substrate for self-possibility that the neural substrate for self-awareness may be the same as the neural awareness may be the same as the neural substrate for other complex integrative substrate for other complex integrative functions that are needed for successful functions that are needed for successful employment outcome.employment outcome.

As always in Rehabilitation more research is As always in Rehabilitation more research is needed.needed.

REFERNCES (1)

Prigatano GP, Disturbances of self-awareness of Prigatano GP, Disturbances of self-awareness of deficit after traumatic brain injury. IN: Prigatano GP., deficit after traumatic brain injury. IN: Prigatano GP., Schacter DT, eds: Awareness of deficits after brain Schacter DT, eds: Awareness of deficits after brain injury: Clinical and Theoretical Issues, New York injury: Clinical and Theoretical Issues, New York N.Y., Oxford University Press,1991N.Y., Oxford University Press,1991

Prigatano GP: Disorders of self awareness after brain Prigatano GP: Disorders of self awareness after brain injury, IN: Principles of Neuropsychological injury, IN: Principles of Neuropsychological Rehabilitation: New York, Oxford, Oxford University Rehabilitation: New York, Oxford, Oxford University Press, 1999Press, 1999

Mersulam MM: Principles of Behavioral Psychology. Mersulam MM: Principles of Behavioral Psychology. F.A. Davis, Philadelphia, 1985F.A. Davis, Philadelphia, 1985

REFERNCES (2)

Shaw J. The assessment of Rehabilitation of Shaw J. The assessment of Rehabilitation of Visual-Spatial Disorders. IN: Johnstone B., Visual-Spatial Disorders. IN: Johnstone B., Stonnington HH, eds: Rehabilitation of Stonnington HH, eds: Rehabilitation of Neuropsychological Disorders: Psychology Neuropsychological Disorders: Psychology Press, Philadelphia, PA, 2001Press, Philadelphia, PA, 2001

Sherer M. et al: Impaired awareness and Sherer M. et al: Impaired awareness and Employment Outcome after TBI: J. Head Employment Outcome after TBI: J. Head Trauma Rehabilitation, 1998: 13(5) 52-61Trauma Rehabilitation, 1998: 13(5) 52-61