1. 1. When One Hemisphere Innervates Both Body Sides Combined Sections Meeting 2014 Las Vegas, Nevada, February 3 6, 2014 A Look at Children Status Post Hemispherectomy Dr. Nisha Pagan, PT, DPT, NCS, PCS Whole Hearted Pediatric Physical Therapy Dr. Stella DeBode, PhD Brain Recovery Project Foundation Department of Radiology and Neurosurgery at UCLA
2. 2. Dr. Stella de Bode, PhD Research focus: Manipulating cortical plasticity to help individuals after cerebral hemispherectomy achieve their maximum potential Doctorate in Applied Linguistics and Neuroanatomy from UCLA. Fifteen years of experience working with children post- hemispherectomy Current affiliations: Brain Recovery Project Foundation, a non- profit research foundation and UCLA, Dept of Radiology and Neurosurgery 2
3. 3. Acknowledgements Dr. Stacy Fritz, PT, PhD: Program Director & Associate Professor at University of South Carolina Rehabilitation Examining Differences in Outcomes for Intensive Mobility Training (IMT) Compared to Locomotor Training in Chronic Stroke (PI) Compared IMT effects for individuals with chronic impairments & disabilities from stroke to a therapy of equal dosage and task intensity 3
4. 4. Acknowledgements Dr. Stella DeBode, PhD Dr. Stacy Fritz, PT, PhD Gary Mathern, MD Investigated cortical plasticity and effects utilizing IMT with children with cerebral hemispherectomy 4
5. 5. Dr. Nisha Pagan, PT, DPT, NCS, PCS Working since 2001 with various diagnoses from NY to AK to CA USC/Rancho Los Amigos Neurology Residency (2005) USC Post Professional Doctoral Physical Therapy (2007) Intervention therapist providing repeat bout of IMT (2011) 5
6. 6. Repeat bout of IMT The Effect of Chronic Hemiparesis on the Cortical Motor Maps in Individuals after Cerebral Hemispherectomy UCLA Institutional Review Board (IRB) Intensive Mobility Training (IMT) at Precision Rehab Functional brain images before and after 2 week period 6
7. 7. Acknowledgements Permission to disclose pictures and clinically relevant information for educational purposes was obtained by all patients and families. 7
8. 8. Learning Objectives: What is a cerebral hemispherectomy? Who and why do individuals undergo this procedure? How do patients with only an ipsilateral corticospinal tract (CST) present clinically, in the upper extremity and lower extremity? What is Intensive Mobility Training (IMT)? Review the life span of children and young adults with cerebral hemispherectomy through a case study approach. 8
9. 9. Course Content: Presenter Dr. Stella DeBode, PhD Various etiologies leading to cerebral hemispherectomy The effect of seizures before surgery and brain atrophy after Types of cerebral hemispherectomy (anatomical vs functional) Differences with upper extremity and lower extremity motor innervation Presurgical Organization of corticospinal tract (CST) 9
10. 10. Presenter Nisha Pagan, PT, DPT, NCS, PCS Acute and Chronic Precautions Examination and Clinical Presentation Therapeutic Options UE/LE and Intensive Mobility Training (IMT) Outcome Measures Case examples of different etiologies across the life span utilizing IMT 10
11. 11. Our Audience? Who has worked with an individual with a cerebral hemispherectomy? 11
12. 12. What is Cerebral Hemispherectomy? Cerebral Hemispherectomy is the partial or complete removal and disconnection of one cerebral hemisphere of the brain. Surgical techniques differ in how much tissue is removed and how to perform this removal, but in all techniques the deceased cerebral hemisphere is rendered completely functionally and anatomically disconnected from the remaining good hemisphere. 12
13. 13. What is Cerebral Hemispherectomy? Cerebral Hemispherectomy is the partial or complete removal and/or disconnection of one cerebral hemisphere of the brain. Cerebral means that deep subcortical structures remain intact although some centers are now starting to remove parts of Basal Ganglia. 13
14. 14. Why Cerebral Hemispherectomy? Life-threatening Anti-epileptic drugs, AED, resistant seizures Prevalence: 16-20% of all pediatric seizure-related surgeries in the US (Harvey et al 2008). Seizures, clinical and subclinical, are manifestation of the underlying disorder. In case of hemispherectomy these underlying disorders fall into the following groups: 14
15. 15. Disorders Leading to Cerebral Hemispherectomy Seizures, clinical and subclinical, are manifestation of the underlying disorder. In case of hemispherectomy these underlying disorders fall into the following groups: 1. Developmental (e.g., disorders of neuronal migration) 2. Acquired (e.g. pre- and postnatal stroke) 3. Progressive (e.g. Sturge Weber Syndrome and Rasmussen Encephalitis) 15
16. 16. What is the status of the remaining brain? The remaining hemisphere may be relatively healthy, but there is always a danger of microscopic damage caused by either primary insult (e.g., cortical dysplasia) or seizures that affect both hemispheres or both. Post-surgical complications (e.g., hydrocephalus) may also compromise the remaining brain
17. 17. Example of Anatomical Hemispherectomy 17
18. 18. Example of Functional Hemispherectomy 18
19. 19. Neuroanatomy of an Isolated Cerebral Hemisphere Corticospianal tract, the only conscious motor control tract from the remaining hemisphere now innervates both sides of the body by its ipsi- and contralateral components The sequelae of the isolated ipsilateral CST are not known We will describe existing evidence drawing from population with CP, animal models of neurobiological substrate and functional measures of both sides 19
20. 20. Neuroanatomy of an Isolated Cerebral Hemisphere There is a difference in CST innervation of the Upper and Lower extremities. 20
21. 21. 21 What is the Degree of Functional Impairements after Hemispherectomy, Affected Side? De Bode et al., 2005 The Fugl-Meyer scores by side (paretic & non-paretic) in 12 children post- hemispherectomy (5 years +).
22. 22. What do we know about pre-surgical organization of the corticospinal tract? Case study: 18 y.o. male with right-sided RE, duration 6yrs (de Bode & Davis, 2007) Non-Affected hand Affected foot Non-Affected foot Affected hand 22
23. 23. 23 Motor Innervation from the Remaining Hemisphere, LE De Bode & Fritz, 2007 Similar cortical areas, M1S1 & SMA are involved in moving a paretic leg
24. 24. 24 Motor Innervation from the Remaining Hemisphere, UE De Bode & Fritz, 2007 Remaining hemisphere supporting both hands: Red nonaffected hand Blue affected hand
25. 25. The Effects of Ipsilateral Innervation and Sharing Sensorimotor Brain Representations The effects of ipsilateral corticospinal innervation is known from CP studies and animal models: In a study of UE impairments Staudt et al (2007) found that participants with unilateral innervation of both hands were most impaired and therapy- resistant in comparison to those who had normal bilateral and mixed (drawing from both S1M1) representations of their hand. 25
26. 26. The Effects of Ipsilateral Innervation and Sharing Sensorimotor Brain Representations In other words, when the most severe impairments in CP were associated with brain representations that are identical to the patients after hemispherectomy. Why? The closest answer comes from the animal models 26
27. 27. Animal Models 27 John Martin and colleagues, 2007-12 Bilateral immature Critical refinement period Predominantly contralateral mature Cortex Spinal cord
28. 28. 28 Blockade of motor activity on ONE side Absence of activity- dependent competition Maladaptive laterality pattern and functional outcome similar to CP Martin et al 1999; Friel & Martin, 2005; Martin, 2009 refinement period 85% 15% 55% 45% Animal Models: Sequelae of Hemispherectomy
29. 29. 29 Blockade of motor activity on ONE side Absence of activity- dependent competition Maladaptive laterality pattern and functional outcome similar to CP Martin et al 1999; Friel & Martin, 2005; Martin, 2009 Animal Models: Sequelae of Hemispherectomy Functionally, unilateral blockade of one hemisphere and ipsilateral corticospinal tract claiming the territory of both tracts resulted in severe deficits in animals
30. 30. Putting it All Together 1. Cerebral hemispherectomy often arrests seizures, but results in permanent hemisparesis caused by the removal of S1M1 of the deceased hemisphere and corticospinal tract of the remaining hemisphere forced to support both body sides 2. Functionally, such reorganization results in severe deficits of the UE, less deficits in the LE and the presence of the distal-proximal gradient 3. Motor representations that are now shared by both body sides mean that any therapy aiming at the paretic side would potentially affect the strong side. There is one study (Dijkerman etal, 2008) suggesting that following hemispherectomy subtle deficits are associated with the non-affected side similar to studies in populations with CP. 4. Therapy in this population should be guided by the understanding that, in contrast to CP, functional improvements of the affected side based on recruitment of the contralateral hemisphere is not possible. 30
31. 31. Cerebral Hemispherectomy: Clinical Considerations and Application of IMT
32. 32. Acute Stage Blood loss (developmental > RE and infarct) Level of interaction will by 48 hours due to brain swelling Chemical meningitis is common Restarted on AEDs once taking liquids by mouth (Lam and Mathern (2010) Functional Hemispherectomy at UCLA chapter 27 in Pediatric Epilepsy Surgery: Preoperative Assessment and Surgical Treatment edited by Oguz Cataltepe, George I. Jallo) 32
33. 33. Acute Stage Discharged 7 to 14 days after surgery Older children walking before surgery receive inpatient rehab Neuro-surgeon F/U is 6 months, 12 months and then annually May taper AEDs 3 months after surgery Lam and Mathern (2010) 33
34. 34. Chronic Many report headaches/migraines (Lew, 2013) Cerebral shunt in 32% of patients (Lam & Mathern 2010) Any signs of increased intracranial pressure, immediately order neuro-imaging to R/O Late Acquired Hydrocephalus 34
35. 35. Examination: history Etiology (i.e. developmental vs. acquired) (Van der Kolk 2012) Age of surgery ( improvements LE) distal-proximal gradient Sensory 36
36. 37. Acquired Etiology Status Post Wrist Fusion Note Associated Movements in Unaffected Hand Example 37
37. 38. Sensory Vision: Right or Left homonymous hemianopsia www.Lighthouse.org 38
38. 39. Sensory Light Touch & Proprioception Sensory Integration ?? Low registration Sensory sensitivity Sensory avoiding 39
39. 40. Cognitive Functioning Limited Cognitive Function Decreased planning and problem solving skills Age-appropriate skills 40
40. 41. Therapeutic Goals Family and patient centered Typically to keep up with their peers Promote the best quality of life 41
41. 42. What is the optimal dosage? Tap into the brains amazing ability to reorganize itself Extended, repetitive, meaningful, skilled training Intensity matters--- but what is optimal? 42
42. 43. Why provide Intensive Therapy? Therapists Perspective Parents Perspective Suggests rate of treatment more critical than # of treatments Intermittent intensive physiotherapy in children with cerebral palsy: a pilot study. Trahan and Malouin. Developmental Medicine and Child Neurology (2002) 44: 233-239 43
43. 44. Upper Extremity Therapy HABIT versus CIMT (Gordon et al 2006, 2007, 2008; de Bode 2009) Depends on individual functioning level, neuroanatomy & goal Limited research on therapy in this population Recruitment of the contralateral hemisphere is not possible 44
44. 45. LE training: Intensive Mobility Training (IMT) Repetitive, Task-specific training in a mass practice schedule 45
45. 46. IMT participants: Incomplete spinal cord injury (ISCI) Parkinsons disease Stroke Cerebral hemispherectomy (Fritz et al 2011, DeBode et al 2007) Chronic (6 months) & varying ambulation skills Feasibility of Intensive Mobility Training to Improve Gait, Balance, and Mobility in Persons With Chronic Neurological Conditions: A Case Series. Fritz et al JNPT 2011;35: 17) 46
46. 47. IMT protocol (Fritz et al 2011) Two weeks 10 days of therapy (3hrs a day, total 30 hours) 1/3 of each session in Body Weight Support Training 1/3 interventions aiming at improving balance 1/3 muscle coordination, flexibility, strengthening TIME BASED PROTOCOL 47
47. 48. 50 minutes of BWST. 50 Minutes of Balance Re- training 50 Minutes of Strengthening, ROM and Coordination Activities Take initial BP, HR, Fatigue and Pain Level. Take final BP, HR, Fatigue and Pain Level. Activities are modified based on BP, HR, fatigue, pain, frustration, interest level and performance. Rest breaks given did not total more than 30 minutes of the 3 hour session. 48
48. 49. Activities Activity list compiled and used as a template Always challenge the patient time/distance/height, change support surface, support 49
49. 50. An example of how to utilize IMT: Preparation Gait Training Higher Coordination Skills/Activities 50
50. 51. Treadmill guidelines 1) approach normal temporal parameters of gait 2) maintain upright trunk 3) approximate normal joint kinematics for lower extremity joints 4) avoid excessive weight bearing on the upper extremities Berhman AL & Harkema SJ Locomotor training after human spinal cord injury: a series of case studies. PT 2000; 80:688-700 51
51. 52. Treadmill guidelines BWS used if unable to accomplish independently on TM Maximize bilateral limb loading without knee buckling Manual cues used if unable to generate the stepping motion Berhman AL & Harkema SJ Locomotor training after human spinal cord injury: a series of case studies. PT 2000; 80:688-700 52
52. 53. Treadmill guidelines Once optimal gait kinematics was achieved: 1st BWS was decreased as well as manual assistance Following speed of walking was increased 53
53. 54. IMT Outcome measures Fugl Meyer LE and balance 6 minute walk Timed Up and Go Dynamic Gait Index Berg Balance Scale Step Length Toe in and out Combined Functional Index (CFI) was analyzed 54
54. 55. IMT Results: 19 children status post hemispherectomy Fritz et al 2011 55
55. 56. 56 2006 2013 2013
56. 57. 70 75 80 85 90 PRE 2006 POST 2006 PRE 2012 POST 2012 CFI CFI Changes Associated with Two Therapy IMT AC, 14-20 GM, 9-15 AS, 5-11 LE, 9-16 Drop in scores following 6 yrs w/out therapy DeBode and Pagan, unpublished 57
57. 58. Additional Pediatric Outcome Measures Gross Motor Function (GMFCS - E & R Robert Palisano, Peter Rosenbaum, Doreen Bartlett, Michael Livingston, 2007) Bruininks-Oseretsky Test of Motor Proficiency, 2nd Ed (BOT-2) 58
58. 59. Additional Pediatric Outcome Measures Developmental Exams Bayley Scales of Infant Development (BSID-III) Peabody Developmental Motor Scales, Second Edition (PDMS-2) Developmental Assessment of Young Children, Second Edition (DAYC-2) 59
59. 60. Additional Pediatric Outcome Measures Sensory Profile by Winnie Dunn, PhD, OTR, FAOTA Infant/Toddler School age Adolescent/Adult 60
60. 61. Case Study: Young adult Infarct in utero (left hemiplegia, left hand surgery at birth) Intractable seizures began at 4 years and 2 months old At 6 years old and 3 months received a left hemispherectomy 61
61. 62. Case Study: Young adult 13 years old participated in IMT for the first time in SC with Dr. Fritz Following PT 1x/year 19 years and 9 months old participated in a repeat bout of IMT 62
62. 63. Strengths Motivated to work hard Able to articulate her goals and difficulties Extremely artistic 63
63. 64. Impairments Mental age in Low Average (test-PPVT) BMI ~ 40.2 (obesity) Baseline blood pressure 135/84; HR 112 64
64. 65. Impairments force production throughout R > L ROM/contractures UE > LE Pain in left wrist and back 65
65. 66. Activity Limitations and Participation Level Attends college Difficulty walking across campus Falls a couple times of month Fitness level, balance, pain, fear and depression activity and participation 66
66. 67. Goals Dressing (pulling up pants and zipping up jacket) Motor planning, Balance, UE Control To stand up from the floor Motor planning, UE/LE/trunk strength, balance, ROM 67
67. 68. 68
68. 69. 70 75 80 85 90 PRE 2006 POST 2006 PRE 2012 POST 2012 CFI CFI Changes Associated with Two Therapy IMT AC, 14-20 Drop in scores following 6 yrs w/out therapy DeBode & Pagan, unpublished 69
69. 70. Outcomes Gait Toe out decreased 15 to 6.5 No change in gait velocity Balance Improved static balance (standing tandem, on one leg) Improved dynamic balance (horizontal head turns, stepping around obstacles) Mobility 6 min walk pre: 45.7% normal post: 49.6% normal 70
70. 71. Outcomes Flexibility UE mobility improved no LE ROM changes Pain Pain decreased at back on final assessment 71
71. 72. Bruininks-Oseretsky Test of Motor Proficiency, 2nd Ed (BOT-2) 72
72. 73. Referrals Provided Psychologist and primary care physician Fitness gym within Physical Therapy clinic Primary PT and OT for further therapy Vocational Rehabilitation Cerebral palsy clinic 73
73. 74. A.C 2006 Age 14 Paretic Knee Non-paretic Knee 74
74. 75. Repeat Bout IMT Effects Age 19Paretic Knee Non-paretic Knee 75
75. 76. Follow-up 5 months after IMT California Childrens Services approved 1x/week therapy for 4 visits Followed up with UCLA referral Reported a fall on the stairs on college campus 76
76. 77. Aging with a disability Pain fitness Surgical candidate Gait and mobility changes Functional changes? Requires continued follow up with a team approach 77
77. 78. Case Study: Pre-Teen 4 years old intractable seizures due to RE 4 years and 6 months old had a R hemispherectomy 5 years old participated in IMT in SC with Dr. Fritz 78
78. 79. Case Study: Pre-Teen Monthly to twice a year PT Weaned off of medicine due to persistent headaches 11 years and 6 months old participated in a repeat bout of IMT 79
79. 80. Strengths Cheerful Motivated Cooperative Active Supportive Family 80
80. 81. Impairments Mental age in Low Average (test-PPVT) Leg Length discrepancy found 88 cm on R, 86 on L ROM limitations due at knee extension and ankle df Spasticity at Hamstrings and plantarflexors Proprioception impaired as tested on foot 81
81. 82. Activity Limitations and Participation Level GMFCS Level I Actively plays basketball Big brother to a 6 year old sister Strong Compensational strategies with learned disuse on left side with absent left protective reaction 82
82. 83. Description of therapy LL discrepancy corrected Range of motion Gait re-education Electrical stimulation 83
83. 84. 84
84. 85. 70 75 80 85 90 PRE 2006 POST 2006 PRE 2012 POST 2012 CFI CFI Changes Associated with Two Therapy IMT AS, 5-11 Drop in scores following 6 yrs w/out therapy Pagan and DeBode 85
85. 86. ROM changes Active ankle movement Demonstrated fair understanding of prescribed a HEP 86
86. 87. Pre (age equivalent) Post (age equivalent) Post 6 months Coordination UE 8-8:2/bilateral 5:4- 5:5 UE 11:3-11:5/ bilateral 6:3-6:5 NT Balance 5:10-5:11 16:5-16:11 5-5:1 () Running Speed and agility 5:6-5:7 8:0-8:2 NT Strength 7:0-7:2 11:9-11:11 7:9-7:11 () 87
87. 88. 6 month follow up Primary PT providing monthly consults to monitor a HEP Rapid decline due to Growth spurt? tightness at hamstrings and ankle plantarflexors activity however with gait deviations & compensations 88
88. 89. Are IMT effects lasting? What needs to be modified to maintain results? Should IMT be provided more frequently prior to puberty? Would an of conventional PT vs monitoring suffice? 89
89. 90. Case Study: Toddler intractable seizures due to cortical dysplasia 2 months old 1st surgery left hemispherectomy 15 months old required a 2nd surgery revision 2 hours of PT and 2 hours of OT weekly Nearly 22 months participated in IMT 90
90. 91. Developmental Scores: BSID-III & DAYC Area of Development % Delay Cognition 36% Receptive Communication 36% Expressive Communication 23% Fine Motor 41% Gross Motor 68% Social Emotional 45% Adaptive Development 36% 91
91. 92. Parent Goals Toddler to initiate steps Hand use Assess orthotic and equipment needs 92
92. 93. Description of Therapy STM ROM with sensory play Strengthening included sit to stand and squatting UE play kinesiotaping Education/Equipment needs 93
93. 94. Gait progress 1st week dragging and increase rigidity of feet Progressed to spontaneous stepping over treadmill without harness turning of right foot Total time on treadmill 23 - 34 minutes Speed of treadmill .2-.6 mph Remaining time gait training overground: forward and cruising 94
94. 95. Changes Reaching out with right hand more frequently Sit to stand from 22 cm height with contact guard assist Hamstring length improved (R popliteal angle from 40 to 20) Initiating steps forward over treadmill and over ground with stabilization 95
95. 96. 96
96. 97. Follow Up 7 months after IMT Not creeping or crawling Not transitioning from sitting to quadruped Occasionally pulling to stand Began to cruise independently 3 months after IMT Stands without support for up to 30s Taking about 10 independent steps with coaxing and reassurance 97
97. 98. Toddler case study IMT feasible for toddler with incorporation of sensory play Decrease compensational strategy within all daily activities Trial of equipment such as stander and orthotics Parents reported a faster rate of change with IMT 98
98. 99. Conclusions: Long term disability at risk of function & participation Consider goals, cognitive & sensory processing abilities 99
99. 100. Conclusion Consider: the role of etiology previous length of time exposed to seizures history of therapy (including frequency, dosage and timing throughout lifespan) 100
100. 101. Take home messages More longitudinal and case by case research needs to be collected to determine: the impact of therapy on functional expectations throughout the life span; to determine optimal dosage and how to retain results 101
101. 102. QUESTIONS??? 102
102. 103. Contact information nishapagan@wholeheartedpediatrictherapy.com stella.de.bode@me.com 103
103. 104. References: 1. Cook SW, Nguyen BH, Yudovin S, Shields WD, Vinters HV, Van d Wiele BM, Harrison, RE, Mathern GW. Cerebral hemispherectomy in pediatric patients with epilepsy: comparison of three techniques by pathological substrate in 115 patients Journal of Neurosurgery: Pediatrics, February 2004 / Vol. 100 / No. 2 : Pages 125-141 2. Jonas, R., Nguyen, S., Hu, B., Asarnow, R. F., C., L., Curtiss, S., Shields, W. D.. Cerebral hemispherectomy: hospital course, developmental, language, and motor outcomes. Neurology, 62, 1712- 1721. (2004) 3. Harvey AS, Cross JH, Shinnar S, Mathern BW; ILAE Pediatric Epilepsy Surgery Survey Taskforce, Defining the spectrum of international practice in pediatric epilepsy surgery patients. Epilepsia 2008; 49 (1): 146-155 4. van der Kolk NM, Boshuisen K, van Empelen R, Koudijs SM, Staudt M, van Rijen PC, van Nieuwenhuizen O, Braun KP Etiology-specific differences in motor function after hemispherectomy. Epilepsy Res. 2013 Feb;103(2-3):221-30. doi: 10.1016/j.eplepsyres.2012.08.007. Epub 2012 Sep 5. Staudt M 2007 Reorganization of the developing human brain after early lesions. Dev Med Child Neurol. 2007 Aug;49(8):564. 104
104. 105. References 6. Martin, J. H., Chakrabarty, S., & Friel, K. M. Harnessing activity-dependent plasticity to repair the damaged corticospinal tract in an animal model of cerebral palsy. Developmental Medicine and Child Neurology, 53(3), 9-13. (2011). 7. De Bode, S., Mathern, G. W., Bookheimer, S. & B. Dobkin Locomotor training remodels fMRI sensorimotor cortical activations in children after cerebral hemispherectomy. J of Neurorehabilitation and Neural Repair 21(6): 497-508(2007). 8. Fritz, S. L., Rivers, E., Merlo, A., Mathern, G. W., & de Bode, S. Intensive Mobility Training Post Cerebral Hemispherectomy: Early Surgery Shows Best Improvements. Eur J Phys Rehabil Med, e-pub ahead of print. (2011). 9. De Bode, S., Firestine, A., Mathern, G. & B. Dobkin. Residual Motor Control and Cortical Representations of Function Following Hemispherectomy. Journal of Child Neurology, 1: 78-90 (2005). 10. Lew, Sean M; Matthews, Anne E; Hartman, Adam L; Haranhalli. Posthemispherectomy hydrocephalus: Results of a comprehensive, multiinstitutional review, Neil. Epilepsia54.2 (Feb 2013): 383-389 105