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1 DEVELOPED BY: AMY MORGAN, PT, ATP COMMON PEDIATRIC NEUROLOGICAL DISORDERS: SEATING AND MOBILITY IMPLICATIONS List three common pediatric neurological diagnoses that are impacted by seating and mobility equipment. Describe two differences when prescribing equipment for a progressive compared to a non-progressive disorder. Identify three reasons for selecting power mobility over a manual wheelchair or ambulatory aide. Differentiate between each of the GMFCS classification levels, and be able to classify a client with Cerebral Palsy into a GMFCS category. 4 AN OVERVIEW OF REHABILITATION STRATEGIES

2 Muscular Dystrophy (MD) Spinal Muscular Atrophy (SMA) 16 Permobil Corp. Progressive skeletal muscle weakness. Onset and severity vary the disease has many forms Duchenne Muscular Dystrophy (DMD) Becker Muscular Dystrophy (BMD) Limb-Girdle Muscular Dystrophy (LGMD) Congenital Muscular Dystrophy (CMD) Fascioscapulohumeral (FSH) Muscular Dystrophy And More! 17 Permobil Corp. DMD: Predominantly male, diagnosis typically between 3-5 y/o. Typically requires wheelchair assistance by 12 y/o. Life expectancy has been increasing due to recent research/advances, but is still rare beyond the 30 s. Muscle tissue breaks down and is replaced with fatty/fibrous tissue (proximal musculature most affected). BMD: gradual weakening of the lower body muscles may eventually need wheelchair assistance in adulthood. Predominantly in males, average onset is 12 y/o. BMD has significantly less impact than DMD on voluntary muscle, but similar affect on the heart muscle. LGMD: Muscle weakness starting from proximal muscles (hips or shoulders). Progression patterns are unclear some lose ability to walk within a few years while others progress slowly. CMD: Global weakness noticed at or near birth which may or may not be accompanied by abnormal tone. May also present with intellectual disabilities, eye deficits or seizures. Many types are slowly progressive. FSHD: Affects both men and women gradual weakening of the upper body muscles usually starting with the facial muscles. Very slow progression and often have normal life span. 18 Permobil Corp. Emery, A. (2002). The muscular dystrophies. The Lancet, 359 (9307),

3 Caused by the absence of dystrophin (protein involved in muscle integrity) Onset between 3-5 yrs of age Rapid progression Usually unable to walk by 12 yrs of age 19 Permobil Corp. In Duchenne Muscular Dystrophy, the attachment of muscle fibers to their surrounding endomysium (extracellular matrix) becomes weakened due to mutations in the dystrophin gene. Figure I: Structure of a muscle. (Image courtesy of the National Cancer Institute) 20 Permobil Corp. During the ambulatory stage, a lightweight manual chair (with solid seat and back for positioning) may be utilized. Families often choose to purchase a power scooter for distance mobility Power Assist Wheels? Smart Drive? Bushby, K.; Finkel, R.; Birnkrant, D.J.; Case, L.E.; Clemens, P.R.; Cripe, L.; et al. (2010). Diagnosis and management of duchenne muscular dystrophy, part 2: implementation of multidisciplinary care. Lancet Neurology, 9, Permobil Corp.

4 Once community ambulation is no longer possible, a power wheelchair is necessary. High incidence of scoliosis, lumbar lordosis, muscle tightness Will need custom seating and positioning components Tilt, Recline, ELRs, and Seat Elevation Standing has also been recommended to increase sitting tolerance and manage progression of contractures. Bushby, K.; Finkel, R.; Birnkrant, D.J.; Case, L.E.; Clemens, P.R.; Cripe, L.; et al. (2010). Diagnosis and management of duchenne muscular dystrophy, part 2: implementation of multidisciplinary care. Lancet Neurology, 9, Permobil Corp. Boys with DMD tend to sit with extreme lumbar lordosis and capital extension Leads to permanent skeletal deformities, pain becomes a huge issue Scoliosis very common often requires surgical intervention due to impact on respiration clinical_genetics.shtml 23 Permobil Corp. Adjustability is KEY to provide comfort/sitting tolerance Swing Away lateral trunk supports Thigh supports necessary to control hip external rotation/abduction Pressure relieving cushion with moderate support Custom molded backrest? Chest strap: Often positioned lower and used to assist with respiration (row-a-boat technique) Armrests positioned in internal rotation to provide appropriate support and reduce leaning... Make sure they are long enough! 24 Permobil Corp.

5 25 y/o with DMD Armrest set low to accommodate custom foam in place armrest Midline mount with HMC Mini JS Drives with 1 finger Egg switch mounted near head to access seat functions Custom foot box mounted to calf pad brackets to make it angle adjustable. Inhibited tilting forward to ensure his feet didn t hit floor Custom molded backrest 25 Permobil Corp. Motor Neuron Disease 1 in 6000 children affected Leg weakness > Arm weakness Proximal > Distal involvement Breathing affected Sensation is not affected Cognitive functions unaffected Children often have above average IQ and are very bright and sociable. 26 Permobil Corp. understandingsma/whatissma/ Chung, B.H.Y.; Wong, V.C.N.; & Ip, P. (2004). Spinal muscular atrophy: survival pattern and functional status. Pediatrics, 114, e Both parents must be carriers Missing Gene SMN1 protein deficiency affects motor neurons Results in muscle weakness and orthopedic deformities 27 Permobil Corp.

6 Determination of SMA type is based upon achievement of developmental milestones. Type I: Most severe diagnosis early. Poor head control, unable to sit or stand without help. Scoliosis and pelvic issues common. Type II: Diagnosis before 2 years of age. Able to sit unsupported. Unable to stand w/o external support and unable to walk. Also may have issues with scoliosis and hip displacement. Type III: Stands or walks independently. Difficulty with ambulation and walking develops as they get older. Not able to tolerate extreme physical activity and may have symptoms of joint pain and overuse. May have tremors. Type IV: Adult onset. Mild-moderate symptoms occur between the second and third decade of life. Normal life expectancy. 28 Permobil Corp. Dependent Device: Requires ability to fully recline and support A LOT of equipment (Vent/BiPAP, Suction machine, Cough Assist, etc.) Power Mobility: Access is often the biggest challenge don t give up! Power Tilt (at minimum!) Recline (power or manual) for sitting tolerance and respiratory management ELRs for positioning and promote circulation May require single switch scanning if other access methods were unsuccessful Dunaway, S.; Montes, J.; O Hagen, J.; Sproule, D.M.; Vivo, D.C.; & Kauffmann, P. (2012). Independent mobility after early introduction of a power wheelchair in spinal muscular atrophy. Journal of Child Neurology, 28(5), Permobil Corp. Power Mobility as early as possible! Power seat functions to promote independence Power wheelchair base to allow independent exploration... Wherever kids go! Playgrounds Home School Etc. Jones, M.A.; McEwen, I.R.; & Hansen, L. (2003). Use of power mobility for a young child with spinal muscular atrophy. Physical Therapy, 83 (3), Permobil Corp.

7 Power seat functions Lateral trunk supports Supportive headrest Lateral swing-away pads Custom modifications? Chest strap Or NOT! 31 Permobil Corp. Vent Tray/O2 Tank Holder Vent/Cough Assist IV pole (feeding) Remote Stop Switch Attendant Control 32 Permobil Corp. Compact Joystick? Lite? Mini Joystick? Micropilot? Microlite Switches? Proximity Sensors? Fiber Optic Switches? 33 Permobil Corp.

8 Began using power mobility at 18 months (Permobil Koala) Has a younger brother with SMA as well Uses a Mini-Proportional joystick with right finger Head supports using lateral swingaway positioning pads and Hensinger neck collar. Lateral trunk supports, chest harness and trunk orthosis (TLSO) Wears AFO s for foot positioning 34 Permobil Corp. When prescribing mobility for an individual with a progressive disorder A snapshot view is not enough Flexible systems are necessary Mounting & Programming Educate family that there will continue to be options for operating PWC as function declines It takes TIME... But it is very rewarding when you succeed! Rolfe, J. (2012). Planning wheelchair service provision in motor neuron disease: implications for service delivery and commissioning. British Journal of Occupational Therapy, 75(5), Permobil Corp. Cerebral Palsy (CP) Brain Injury Traumatic (TBI) Acquired (ABI) Cerebrovascular Accident/Stroke (CVA) Congenital Spinal Cord Injury/Spina Bifida (SCI) 36 Permobil Corp.

9 Neurological disorder that appears in infancy Lack of muscle coordination Symptoms: ataxia, spasticity 37 Permobil Corp. Spastic CP: % of the pts. Based on extremities affected (diplegia, hemiplegia) Athetoid or dyskinetic CP Uncontrolled, writhing movements (10-20 %) Ataxic CP Rare form affects sense of balance and depth perception (5-10%) Mixed forms 38 Permobil Corp. General Headings for Each Level: LEVEL I - Walks without Limitations LEVEL II - Walks with Limitations LEVEL III - Walks Using a Hand-Held Mobility Device LEVEL IV - Self-Mobility with Limitations; May Use Powered Mobility LEVEL V - Transported in a Manual Wheelchair GMFCS Robert Palisano, Peter Rosenbaum, Stephen Walter, Dianne Russell, Ellen Wood, Barbara Galuppi, 1997 CanChild Centre for Childhood Disability Research, McMaster University (Reference: Dev Med Child Neurol 1997;39: ) 39 Permobil Corp.

10 40 Permobil Corp. motorgrowth.canchild.ca/en/gmfcs/resources/gmfcs-er.pdf Describes specific motor performance in each of the 5 GMFCS levels at different age ranges: Birth 2 years 2 years 4 years 4 years 6 years 6 years 12 years 12 years 18 years GMFCS - E & R Robert Palisano, Peter Rosenbaum, Doreen Bartlett, Michael Livingston, 2007 CanChild Centre for Childhood Disability Research, McMaster University 41 Permobil Corp y/o with CP (spastic diplegia; GMFCS level 3) Previous manual wheelchair was difficult to propel and transfer from. Chose LifeStand Helium wheelchair to provide standing capability Functional Benefits: cooking/ access to refrigerator, dressing/ access to hanging clothes, hygiene activities, etc. Medical Benefits: increased ROM, spasticity management, pain/pressure relief, bowel/bladder function 42 Permobil Corp.

11 4 y/o with CP (spastic quadriplegia; GMFCS level 4) First power wheelchair Koala vs. K300 PS JR Family chose Koala due to small size base After market seating Lateral trunk supports Thigh supports Ankle huggers Chest Harness/Pelvic Belt Midline mounted joystick 43 Permobil Corp. Contusions and lacerations- with or without skull fractures- damage can be to any area of the brain Occipital blows are more likely to cause contusions (irregular shape) Can also injure cranial nerves Lacerations of dura and/ or arachnoid may cause CSF to be discharged (rhinorrhea) 44 Permobil Corp. Diffuse axonal injury or shearing- may be one of the most common types of lesions Unequal acceleration, deceleration or rotation of contingent tissues Penetrating objects with high velocities may cause shock wave damage. Severing of axons may be severe enough to result in coma Less severe- causes deficits in memory, concentration, decreased attention span, headaches and sleep disturbance Damage often involves corpus callosum, basal ganglia, brain stem, and cerebellum. Davies, Patricia. Starting Again: Early Rehabilitation After Traumatic Brain Injury or Other Severe Brain Lesion., ISBN Permobil Corp.

12 Mainly due to a lack of oxygen Increased intracranial pressure- due to swelling Cerebral hypoxia or ischemia- ruptured blood vessels Intracranial hemorrhage- cause hypoxia Electrolyte imbalance and acid-base imbalance Infection Seizures Visual Impairments- Cranial nerves III, IV and VI Davies, Patricia. Starting Again: Early Rehabilitation After Traumatic Brain Injury or Other Severe Brain Lesion., ISBN Permobil Corp. Autonomic Nervous system- pulse, temp., and respiratory rates, BP, excessive sweating, salivation and tearing Motor, Functional, Sensory and Perceptual- rigidity (decorticate and decerebrate), abnormal tone, motor deficits, cranial nerve involvement Davies, Patricia. Starting Again: Early Rehabilitation After Traumatic Brain Injury or Other Severe Brain Lesion., ISBN Permobil Corp y/o with Secondary Brain Injury due to Meningitis as an infant Questionable Vision Concern for Safety Awareness/ Impulsivity Required postural supports for stability and positioning Trialed joystick which resulted in veering due to tone and inability to stop Trialed 3 switch head array as well as proximity switches in tray Still working on best access method for 48 success. Permobil Corp.

13 Stroke: a disease of cerebro-vasculature which failure to supply oxygen to brain cells result in their death Ischemic: 80% of stroke Hemorrhagic: 20% of stroke 49 Permobil Corp. Most common form of stroke Cause of injury is tissue anoxia Caused by cessation of cerebral blood flow Embolic Stroke most common ischemic stroke Cardiac sources MI most common Cardiac emboli occlude middle cerebral artery 80% of time 10% posterior cerebral artery Vascular sources Ulcerated plaques in aorta and carotid arteries Paradoxical Unknown 50 Permobil Corp. Obvious findings of cerebral infarction on CT scan. A CT scan of the brain shows a large left middle cerebral infarct, indicated by the hypodensity or dark color (a); the size of the infarct is indicated by the blue color (b). This infarct will be associated with a contralateral hemiplegia, homonymous hemianopsia, and a hemisensory defect, all of which likely will be permanent. 51 Permobil Corp.

14 One in every 2,000 live births each year result in on of the three types of spina bifida. Occulta: the symptom-less form Miningocele: the rarest form Myelomeningocele: Most severe (1 in 1,000 births) 52 Permobil Corp Permobil Corp. Surgery typically takes place 24 to 48 hours after birth* Success rate of about 70% Success being measured by a return in spinal function and the innervation of spinal nerves below the level of the cyst. 90% of children with develop hydrocephalus; which can result in mental retardation. *Newer surgical techniques can be performed in utero. 54 Permobil Corp.

15 Most individuals with spina bifida will have aberrations in the development of the brain Chiari II malformation Brain being positioned further down the spinal cord Brain tissue blocks the normal flow of cerebrospinal fluid After birth, this is treated by the insertion of a shunt, which may be required for life. 55 Permobil Corp. 2 y/o with L4-5 Spina Bifida Participant in infant power mobility research at Univ. of Delaware K450 MX chosen to allow lowering to floor level Independent transfers Crawling > Standing > Walking Also walks with forearm crutches independently for short distances Uses power w/c for longer distances and varying terrains. 56 Permobil Corp. Lynch, A.; Ryu, J.C.; Agrawal, S.; & Galloway, J.C. (2009). Power mobility training for a 7- month-old infant with spina bifida. Pediatric Physical Therapy. 21(4): PEDIATRIC REHABILITATION FOR NEUROLOGICAL INJURIES Pediatric Rehabilitation must focus on increasing independent mobility experiences. Intervention should maximize age appropriate activities and participation. Proper postural supports and multiple positions (sitting, standing, prone, etc.) are recommended. Mobility has a direct impact on brain development, learning, visual/spatial awareness, and socialization. Independent mobility options should be initiated as early as 57 possible Permobil Corp. (Ideally birth 3 years old)

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