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
Transcript
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. 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. 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. 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. 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. 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. Acknowledgements Permission to disclose pictures and
clinically relevant information for educational purposes was
obtained by all patients and families. 7
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. 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. 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. Our Audience? Who has worked with an individual with a
cerebral hemispherectomy? 11
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. 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. 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. 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. 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. Example of Anatomical Hemispherectomy 17
18. Example of Functional Hemispherectomy 18
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. Neuroanatomy of an Isolated Cerebral Hemisphere There is a
difference in CST innervation of the Upper and Lower extremities.
20
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. 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 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 Motor Innervation from the Remaining Hemisphere, UE De
Bode & Fritz, 2007 Remaining hemisphere supporting both hands:
Red nonaffected hand Blue affected hand
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. 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. Animal Models 27 John Martin and colleagues, 2007-12
Bilateral immature Critical refinement period Predominantly
contralateral mature Cortex Spinal cord
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 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. 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. Cerebral Hemispherectomy: Clinical Considerations and
Application of IMT
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. 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. 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. Examination: history Etiology (i.e. developmental vs.
acquired) (Van der Kolk 2012) Age of surgery ( improvements LE)
distal-proximal gradient Sensory 36
37. Acquired Etiology Status Post Wrist Fusion Note Associated
Movements in Unaffected Hand Example 37
38. Sensory Vision: Right or Left homonymous hemianopsia
www.Lighthouse.org 38
40. Cognitive Functioning Limited Cognitive Function Decreased
planning and problem solving skills Age-appropriate skills 40
41. Therapeutic Goals Family and patient centered Typically to
keep up with their peers Promote the best quality of life 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
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
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
45. LE training: Intensive Mobility Training (IMT) Repetitive,
Task-specific training in a mass practice schedule 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
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
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
49. Activities Activity list compiled and used as a template
Always challenge the patient time/distance/height, change support
surface, support 49
50. An example of how to utilize IMT: Preparation Gait Training
Higher Coordination Skills/Activities 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
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
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
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
55. IMT Results: 19 children status post hemispherectomy Fritz
et al 2011 55
56. 56 2006 2013 2013
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
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
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
60. Additional Pediatric Outcome Measures Sensory Profile by
Winnie Dunn, PhD, OTR, FAOTA Infant/Toddler School age
Adolescent/Adult 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
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
63. Strengths Motivated to work hard Able to articulate her
goals and difficulties Extremely artistic 63
64. Impairments Mental age in Low Average (test-PPVT) BMI ~
40.2 (obesity) Baseline blood pressure 135/84; HR 112 64
65. Impairments force production throughout R > L
ROM/contractures UE > LE Pain in left wrist and back 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
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
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
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
71. Outcomes Flexibility UE mobility improved no LE ROM changes
Pain Pain decreased at back on final assessment 71
72. Bruininks-Oseretsky Test of Motor Proficiency, 2nd Ed
(BOT-2) 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
74. A.C 2006 Age 14 Paretic Knee Non-paretic Knee 74
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
77. Aging with a disability Pain fitness Surgical candidate
Gait and mobility changes Functional changes? Requires continued
follow up with a team approach 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
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
80. Strengths Cheerful Motivated Cooperative Active Supportive
Family 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
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
83. Description of therapy LL discrepancy corrected Range of
motion Gait re-education Electrical stimulation 83
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
86. ROM changes Active ankle movement Demonstrated fair
understanding of prescribed a HEP 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
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
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
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
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
92. Parent Goals Toddler to initiate steps Hand use Assess
orthotic and equipment needs 92
93. Description of Therapy STM ROM with sensory play
Strengthening included sit to stand and squatting UE play
kinesiotaping Education/Equipment needs 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
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
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
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
99. Conclusions: Long term disability at risk of function &
participation Consider goals, cognitive & sensory processing
abilities 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
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
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
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.
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