CURRENT ADVANCES IN ASSESSMENT AND MANAGEMENT OF PATIENTS IN LOW LEVEL NEUROLOGICAL STATES THIRD ANNUAL PACIFIC NORTHWES

1. CURRENT ADVANCES IN ASSESSMENT AND MANAGEMENT OF PATIENTS IN LOW LEVEL NEUROLOGICAL STATESTHIRD ANNUAL PACIFIC NORTHWESTBRAIN INJURY CONFERENCE NATHAN D. ZASLER, MD CEO & MEDICAL DIRECTOR, CONCUSSION CARE CENTRE OF VIRGINIA AND TREE OF LIFE SERVICES CLINICAL PROF., DEPT. OF PM&R, VCU CLINICAL ASSOC. PROF., DEPT. OF PM&R, UVA

2. INTRODUCTION • CHALLENGES IN DX. AND TX. • INCONSISTENCY IN NOMENCLATURE USE AND UNDERSTANDING • CONFUSION REGARDING PROGNOSTICATION • GUIDELINE DEVELOPMENT ISSUES • CURRENT RECOMMENDATIONS – ANBICS • RECENT RESEARCH DEVELOPMENTS • FUTURE DIRECTIONS FOR RESEARCH

3. CLARIFICATION OF TERMINOLOGY • COMA • VEGETATIVE STATE • PVS - PERSISTENT VS. PERMANENT • MINIMALLY CONSCIOUS STATE (MCS) • AKINETIC MUTISM • LOCKED IN SYNDROME

4. COMA • STATE OF UNAROUSABLE UNRESPONSIVENESS • TYPICALLY EYES CLOSED - NO SLEEP WAKE CYCLES • DO NOT FOLLOW COMMANDS • NO GOAL DIRECTED BEHAVIOR • NO VERBALIZATION • NO SUSTAINED VISUAL PURSUIT

5. VEGETATIVE STATE • AROUSAL WITHOUT AWARENESS • PERIODS OF EYE OPENING • SUBCORTICAL RESPONSES SEEN • SLEEP WAKE CYCLES PRESENT • DIAGNOSIS ONLY MADE BY SERIAL NEUROBEHAVIORAL EXAM • LIMITS OF ASSESSING INTERNAL AWARENESS

6. MINIMALLY CONSCIOUS STATE • PRIMITIVE NEUROBEHAVIORAL RESPONSES SEEN - SUB-CORTICAL • EVIDENCE OF SOME LEVEL OF AWARENESS TO STIMULI • MUST LOOK AT FREQUENCY AND CONTEXT OF RESPONSES • INCONSISTENT RESPONSES THAT DO NOT REACH THRESHOLD FOR RELIABLE AND/OR CONSISTENT COMMUNICATION • AKINETIC MUTISM - MCS SUBSET

7. AKINETIC MUTISM • MINIMAL DEGREE OF MOVEMENT AND SPEECH • DA SYSTEM INVOLVEMENT • TYPICALLY + EYE OPENING AND TRACKING • PATIENTS TYPICALLY IMPROVE WITH DOPAMNE AGONIST TX.

8. LOCKED IN SYNDROME • AWARENESS RELATIVELY WELL PRESERVED • ANARTHRIA AND QUADRIPLEGIA • VENTRAL PONTINE LESION • VERTICAL EYE MOVEMENTS AND BLINK TYPICALLY PRESERVED • LOWER CRANIAL NERVE AND SWC DYSFUNCTION COMMONLY SEEN

9. TRANSITION FROM COMA TO VEGETATIVE STATE • EYE OPENING • FADING OF DECEREBRATE REACTIONS • RETURN OF SLEEP WAKE CYCLES • EMERGENCE OF SUB-CORTICAL RESPONSES • CONTROVERSY ON: VISUAL TRACKING, DISCRETE MOTOR LOCALIZATION AND EMOTIONAL RESPONSES - VS OR MCS?

10. VS AND MCS • DIFFERENTIAL DIAGNOSTIC ISSUES: ? HIGH RATE OF MISDIAGNOSIS • PROGNOSTICATION ISSUES: EARLY VS. LATE PARAMETERS • PAIN PERCEPTION: WHAT DO WE REALLY KNOW?

11. LANDMARK PUBLICATIONS • AAN POSITION PAPERS - 1989 • AMA COUNCIL REPORT - 1990 • MSTF POSITION PAPER - 1994 • ACRM POSITION PAPER - 1995 • AAN PRACTICE PARAMETER - 1995 • INT. WORKING PARTY - 1996 • ANBICS - IN PROGRESS

12. EMERGENCE FROM VS • MUST DIFFERENTIATE BETWEEN SIGNS THAT ARE PART AND PARCEL OF VS AND SIGNS THAT INDICATE EMERGENT AWARENESS • TIME COURSE FOR EMERGENCE IS VARIABLE BUT GENERALLY CORRELATES WITH LEVEL OF FUNCTIONAL DISABILITY • PERMANENT VEGETATIVE STATE CRITERIA • RECOVERY AFTER “PERMANENCY”

13. PREDICTING OUTCOME IN SEVERE TBI • EARLY PREDICTORS - GCS, IMAGING (S VS. D), MMEPs (INCLUDING LAPs AND ERPs), RISK FACTORS FOR SECONDARY BI, EEG, AGE • LATE PREDICTORS - PRETTY MUCH ALL THE EARLY ONES WITH PARTICULAR EMPHASIS ON SECONDARY BI AND MMEPs. PLUS DURATION OF VS. • MULTIFACTORIAL REGRESSION ANALYSIS FOR OUTCOME PREDICTION • DURATION OF VS MUCH MORE TIED TO LIKELIHOOD OF IMPROVEMENT THAN DURATION OF MCS

14. NEUROREHABILITATIVE CARE FOR VS/MCS • ORTHOTICS AND SEATING • FAMILY EDUCATION AND TRAINING • TREAT NEUROMEDICAL FACTORS MASKING RECOVERY • TREAT NEUROMEDICAL ISSUES ASSOCIATED WITH CONDITION • AVOID IATROGENIC COMPLICATIONS • NUTRITIONAL MANAGMENT • PREVENT MORBIDITY • RESPIRATORY MANAGEMENT

15. ADDRESS POTENTIAL FACTORS MASKING RECOVERY • PTE • LATE INTRACRANIAL PATHOLOGY • PTCH • NEUROENDOCRINE DYSFUNCTION • OCCULT INFECTION • ELECTROLYTE IMBALANCE

16. TREAT NEUROMEDICAL ISSUES SEEN IN LLNS • CENTRAL DYSAUTONOMIA • NHO • ALTERATIONS IN SLEEP WAKE CYCLE • TONAL ALTERATION • RARE SEQUELAE

17. AVOID IATROGENIC COMPLICATIONS • DRUGS • ELECTROLYTE IMBALANCES • UNDER- VS. OVER-STIMULATION

18. NUTRITIONAL MANAGEMENT • ENTERAL FEEDINGS • LONG TERM NUTRITIONAL ISSUES

19. PREVENTION OF MORBIDITY • CONTRACTURES • SKIN BREAKDOWN • INFECTION CONTROL • IMMOBILIZATION • PULMONARY TOILET • DECANNULATE AS POSSIBLE

20. FAMILY EDUCATION AND TRAINING • PURPOSE OF EDUCATION • OPPORTUNITIES TO TRY AND CARE FOR PATIENT AT HOME - SHOULD THEY BE ENCOURAGED/ • SHOULD ALL FAMILIES TAKE ON HOME CARE? WHAT IS OUR RESPONSIBILITY AS CLINICIANS?

21. FUNCTIONAL ASSESSMENT • CRITICAL FOR PROPER BEHAVIORAL TRACKING AND ASSESSMENT OF VS & MCS • VARIOUS BATTERIES AVAILABLE: • DRS SSAM • CNC RLAS • WNSSP CRS • SMART

22. COMA STIMULATION • TRADITIONALLY MEANT TO IMPLY STRUCTURED SENSORY STIMULATION • PHARMACOTHERAPY & NEURAL STIMULATION? • SENSORY REGULATION • SCIENTIFIC EVIDENCE OF BENEFIT

23. PHARMACOTHERAPY FOR VS AND MCS • IN PERSONS IN VS, NO EVIDENCE THAT MEDICATIONS ALTER RATE OF RECOVERY OR EVENTUAL PLATEAU. • IN PERSONS IN MCS, MEDICATIONS MAY HELP AROUSAL AND BRADYKINESIA. • NEURAL RECOVERY FACILITATORS VS. INHIBITORS. • TREATMENT REMAINS VERY MUCH EMPIRICAL AT PRESENT; HOWEVER, BEST EVIDENCE IS FOR PRO-DOPAMINERGIC AGENTS IN FACILITATION OF NEURORECOVERY.

24. NEUROSTIMULATION • DORSAL COLUMN STIMULATION • THALAMIC STIMULATION • PERIPHERAL NERVE (SOMATOSENSORY) STIMULATION

25. VARIABLE IN ERMPs • LENGTH OF STAY • THERAPIST EXPERTISE • PHYSICIAN EXPERTISE • ACCESS TO NEURODIAGNOSTIC FACILITIES • METHODS FOR OUTCOME TRACKING • ADMISSION/DISCHARGE CRITERIA

26. GUIDELINE DEVELOPMENT ISSUES • GENERAL PURPOSE OF PRACTICE GUIDELINES: DEVELOP STRATEGIES FOR PATIENT MANAGEMENT TO ASSIST IN CLINICAL DECISION MAKING • UTILIZES AN EXPLICIT RATHER THAN IMPLICIT APPROACH

27. CLASSIFICATION OF EVIDENCE • CLASS I - BASED ON PROSPECTIVE, RANDOMIZED, CONTROLLED STUDIES • CLASS II - PROSPECTIVE DATA COLLECTION STUDIES AS WELL AS RELIABLE RETROSPECTIVE DATA ANALYSES (COHORT, CASE CONTROL, PREVALENCE AND OBSERVATIONAL STUDIES). • CLASS III - RETROSPECTIVE DATA ANALYSIS (UNCONTROLLED CLINICAL SERIES, DATA BASES, CASE REPORTS & EXPERT OPINION).

28. MORE ON GUIDELINES • STANDARDS ARE BASED ON CLASS I EVIDENCE • PRACTICE GUIDELINES ARE BASED PRIMARILY ON CLASS II EVIDENCE • OPTIONS FOR MANAGEMENT ARE BASED ON CLASS III EVIDENCE • REFLECT: HIGH, MODERATE, LOW CLINICAL CERTAINTY, RESPECTIVELY

29. CURRENT RECOMMENDATIONS • APPROPRIATE AND PREREQUISITE INTERVENTIONS • DECREASE MORBIDITY • MEDICAL MANAGEMENT • SUPPLEMENTAL INTERVENTIONS - ONCE VS IS PERMANENT NO LONGER SUPPORTED: • SENSORY STIMULATION/REGULATION • PHARMACOLOGIC INTERVENTIONS

30. APPROPRIATE AND PREREQUISIT INTERVENTIONS • ROM EXERCISES • POSITIONING PROTOCOLS • BOWEL & BLADDER REGIMENS • DIETARY MANAGEMENT • ADDRESS TONAL ALTERATIONS • MANAGE NHO • MANAGE CENTRAL DYSAUTONOMIA • PROTOCOL FOR DECANNULATION • TREAT REVERSIBLE MEDICAL CONDITIONS • SKIN CARE

31. OTHER RECOMMENDATIONS • PROMOTE ALERTNESS, INCREASE COMMUNICATION ABILITY AND ALLEVIATE PAIN/SUFFERING IN PERSONS IN MCS • ADMINISTRATION/WITHDRAWAL DETERMINATIONS TO BE MADE BY MD IN CONSULTATION WITH FAMILY/GUARDIAN (LIVING WILL ISSUES) • SETTING MUST BE ABLE TO PROVIDE RECOMMENDED TREATMENTS • DIAGNOSIS AND CONSULTATION BY SPECIALIZED M.D.

32. ADMINISTRATION AND WITHDRAWAL ISSUES • MEDICATIONS AND OTHER COMMONLY ORDERED TREATMENTS • SUPPLEMENTAL OXYGEN AND ANTIBIOTICS • COMPLEX ORGAN SUSTAINING TREATMENTS - E.G. DIALYSIS • ADMINISTRATION OF BLOOD PRODUCTS • ARTIFICIAL HYDRATION AND NUTRITION

33. LONG TERM CARE ISSUES • REASSESSMENT SHOULD OCCUR AT 3, 6, & 12 MONTHS AFTER DETERMINATION OF PERMANENCE • ONCE VS IS PERMANENT - DNR ORDER IS APPROPRIATE (MAY BE MADE EARLIER)

34. ISSUES AND CONTROVERSIES • ANALYSIS OF DATA AND LIMITATIONS • LIFE EXPECTANCY • EMERGENCE FROM VS • MCS - A NEW TERM AND PATIENT CATEGORY - LITTLE DATA • GRAY ZONE BETWEEN VS & MCS • CONFLICT RESOLUTION & CROSS DISCIPLINARY CONSENSUS • PAIN AND SUFFERING IN VS AND MCS

35. RECENT RESEARCH DEVELOPMENTS • FUNCTIONAL VS – PATIENTS MAY APPEAR VS BUT ACTUALLY BE MCS • LIKELY ARE WIDE VARIATIONS IN BRAIN METABOLISM IN VS WITH SOME CEREBRAL REGIONS RETAINING PARTIAL FUNCTION • NOCICEPTIVE STIMULI MAY PRODUCE INCREASED BRAIN ACTIVITY IN PRIMARY SOMATOSENSORY CORTEX IN VS – DISASSOCIATED WITH HIGHER ORDER ASSOCIATIVE CORTEX ACTIVATION

36. RECENT RESEARCH DEVELOPMENTS • IN A SUBPOPULATION OF VS PATIENTS, THERE IS PRESERVATION OF THALAMOCORTICAL FEEDBACK CONNECTIONS THAT ALLOW FOR CORTICAL INFORMATION PROCESSING AND MAY EVEN INVOLVE SEMANTIC LEVELS OF PROCESSING • RECOVERY OF CONSCIOUSNESS APPEARS TO BE ASSOCIATED WITH RESTORATION OF CORTICOTHALAMOCORTICAL INTERACTION • SOME MCS PATIENTS MAY RETAIN WIDELY DISTRIBUTED CORTICAL SYSTEMS WITH POTENTIAL FOR COGNITIVE AND SENSORY FUNCTION DESPITE THEIR INABILITY TO FOLLOW SIMPLE COMMANDS OR RELIABLY COMMUNICATE

37. FUTURE DIRECTIONS FOR RESEARCH • INCIDENCE AND PREVALENCE OF VS AND MCS IN TBI • NATURAL HISTORY, RECOVERY COURSE AND LONG TERM OUTCOME • LEVELS OF CERTAINTY ASSOCIATED WITH PREDICTORS OF RECOVERY • UTILITY OF ASSESSMENT METHODS • TREATMENT EFFICACY • IMPACT OF OPTION DISSEMINATION • EXAMINATION OF FAMILY BELIEFS AND RELATION TO OUTCOME/UTILIZATION

38. WATCH FOR: • “BRAIN INJURY MEDICINE: PRINCIPLES AND PRACTICE” • EDITED BY N. ZASLER, D. KATZ AND R. ZAFONTE • CORE TEXTBOOK ON TBI ASSESSMENT AND MANAGEMENT • OVER 60 CHAPTERS WRITTEN BY INTERNATIONAL LEADERS IN THE FIELD • PUBLISHED BY DEMOS PUBLICATIONS - NY, NY • EXPECTED DATE OF PUBLICATION IS EARLY 2006

39. QUESTIONS AND ANSWERS