Case: Children with Disability

Case J.R. 3 y/o boy Stiffness when crying Tiptoe walking

Maternal and Birth History NSD, full term APGAR score: 5BW: 2.5kg 35y/o G1P1 ( ) Regular pre-natal check-up with O.B. Mother (+) hx of UTI during last trimester, no bleeding Upon delivery, cord was coiled on the neck 3x

Developmental History 6 mos Rolled over 12 mos Sat with support 18 mos Pulled himself up to stand At Present Walks but needs assistance - Can make simple sounds Continues to drool

Pertinent PE Findings Ambulatory with scissoring gait Poor balance Maintains high guard of upper extremity (+) cortical thumb With good head control (+) drooling Good sitting balance (+) tightness of both Achilles tendons (+) clonus

Diagnosis: Cerebral Palsy secondary to perinatal brain injury

What are the pertinent facts in the history that may point or contribute to your working diagnosis?

Salient Features 3y/o boy – stiffness when crying and tiptoe walking Delay in achieving developmental milestones Birth weight: 2.5 kg (LBW) Cord coiled on the neck 3x Ambulatory with scissoring gait Poor balance Maintains high guard of extremities (+) cortical thumb (+) drooling (+) tightness of both achilles tendons (+) clonus

Developmental Milestones Gross Motor FunctionNormal (months) J.R. (months) Rolls Over Sits without support Stands Alone At present (3y/o) walks but needs assistance Can make simple sounds Continues to drool

Cerebral Palsy collection of diverse syndromes characterized by disorders of movement and posture non-progressive static movement disorder (weakness possible) delayed developmental milestones persistence of primitive reflexes increased motor tone/floppy

Cerebral Palsy Etiology brain injury that occurs prenatal, perinatal or postnatal brain dysfunction Incidence 2 per 1000 live births (industrialized countries) M>F 1.33 : 1.00

Cerebral Palsy Factors that Increases Incidence of CP low birth weight <25OO g gestational age <32 wk – most common antecedent of CP maternal factors – mental retardation – seizure disorder – hypothyroidism – 2 or more prior fetal deaths – sibling with motor deficit – 3rd trimester bleeding

Cerebral Palsy Manifestations floppy infant persistent obligatory reflexes

Cerebral Palsy Diagnosis High Risk History Posture - FSP, ESP(flexor, extensor synergy patterns), opisthotonus, scissoring gait = d/t spasticity of hip adductors; straphanger UE Oral Motor - tongue thrust, tonic bite Strabismus Tone (while awake and not struggling) Evolution of Postural Reactions Reflexes - MSRs, clonus, Babinski confirmed by: MRI Other tests: metabolic, biochemical, developmental

Cerebral Palsy Classification by limb affectation: Quadriplegia – all 4 extremities + trunk Diplegia – LE > UE, most common Monoplegia – only 1 limb, either an arm or leg Hemiplegia – involvement on one side of body, including arm and leg

Cerebral Palsy 0No increase in muscle tone 1Slight increase in muscle tone, manifested by a catch and release or by minimal resistance at the end of the range of motion when the affected part(s) is moved in flexion or extension. 2Slight increase in muscle tone, manifested by a catch, followed by minimal resistance throughout the reminder (less than half) of the ROM (range of movement). 3More marked increase in muscle tone through most of the ROM, but affected part(s) easily moved. 4Considerable increase in muscle tone passive, movement difficult. 5Affected part(s) rigid in flexion or extension. by muscle tone: Spastic (70-80%) most common signs of UMN involvement Hypertonic NM condition stemming from damage to corticospinal tract, motor cortex or pyramidal tract/ velocity dependent inc in muscle tone Modified Ashworth scale->

Cerebral Palsy Atonic (10%) Hypotonia and tremors Motor skills like writing, typing, using scissors, balance while walking are affected Visual and/or auditory processing of objects Athetoid (25%) Alternating hyper and hypotonia Trouble holding themselves upright, steady position of sitting or walking Involuntary motions Difficulty getting their hand to a certain spot (e.g. scratching nose, reaching for a cup) May not be able to hold on to objects

Cerebral Palsy Comorbidities Mental retardation (IQ<50) – 31% Active seizures- 21% Mental retardation (IQ <50)+ and not walking- 20% Blindness- 11%

Topographical Classification of Cerebral palsy in our patient Spastic – (+) cortical thub, (+) drooling, (+) clonus Diplegia – ambulatory with scissoring gait, poor balance and maintains high guard of UE, good head control

5. How will you grade the spasticity of the patient? (Modified Ashworth)

Clinical Scale For Spastic Hypertonia MODIFIED ASHWORTH SCALE 0No increase in tone 1 Slight increase in muscle tone manifested by a catch and release or minimal resistance at the end of the ROM when the affected part(s) is moved in flexion or extension 2 Slight increase in muscle tone, manifested by a catch followed by minimal resistance throughout the remainder (less than half) of the ROM 3 More marked increase in muscle tone through most of the ROM, but affected part(s) easily moved 4 Considerable increase in muscle tone, passive movement difficult 5Affected parts rigid in flexion or extension

6. How will you manage the spasticity?

Management Rehabilitation Daily stretching Oral Medications Local Injectables Surgical treatments – Tendon release, Selective dorsal rhizotomy

Management Rehabilitation – individualized stretching and exercise program that can be used at home on a regular basis – Improve ROM and function – Useful component to all other modalities – Braces, walking aids (help reduce impact of spasticity)

Management Oral Medications – Baclofen (Lioresal) – Tizanidine (Zanaflex) – Diazepam (Valium) – Clonazepam (Klonopin) – Dantrolene sodium (Dantrium)

Management Advantages of Oral Medications: – Relax a large number of muscles – Dose is easily adjusted – May be stopped anytime Disadvantages: – Modest effect – Drowsiness, dizziness, weakness – Liver inflammation

Management Local Injections  Phenol black  Intrathecal Baclofen pump  Botox injection

Management Botilinum toxin Protein made by bacteria causing botulinism BTX-A (Botox), BTX-B (Myobloc) Can relax spastic muscle when injected in small quantities Affects only injected muscle 2-3wks to take effect last 3-6mo. Safe and can be injected w/o use of sedatives, however injections maybe uncomfortable

Management Intrathecal Baclofen Therapy (ITB) – Baclofen pump – Surgically implanted – More effective than oral Baclofen – Delivers medication directly to the spinal fluid via catheter – Well tolerated, reversible – Test injection (to determine if patient is good candidate for the Baclofen pump)

7. What will be your immediate rehab goal for this patient?

Goals Obtain optimal function despite residual disability Prevent secondary impairement or complications Prevent or delay contractures Maintain mobility Improve function – Promote motor and developmental skills – Activities necessary to help the child reach his/her full potential

Physical Therapy: Daily ROM Exercises Stretching exercises – Increase motion Progressive resistance exercises – Increase muscle strength

Daily ROM Exercises Strengthening knee extensor muscles helps to improve crouching and stride Postural and motor control training – Should follow the dev’t sequence of noraml children (head and neck  trunk control) Use of age appropriate play and of adaptive toys and games based on the desired exercises

Daily ROM Exercises Hippotherapy – Horse back riding therapy – Improve child’s tone, ROM, strength, coordination, balance – Offers many potential cognitive, physical and emotional benefits

Occupational Therapy Activities of Daily Living – Feeding, dressing, toileting, grooming – Goal: to function as independently as possible w/ w/o use of adaptive equipment

Speech Therapy Cerebral Palsy – Involvement of the face and oropharynx, causing dysphagia, drooling and dysarthria – Speech therapy can be implemented to help control the muscles of the mouth and jaw and improve communication

Encourage se of age appropriate play and of adaptive toys and games based on the desired exercises to achieve child’s cooperation

8. Will he need assistive device?

Assistive Devices Ankle foot orthoses (AFO) – Help improve balance and walking – Hinge AFO more effective and helpful – Reduction in foot pronation and prevent contracture by stretching spastic muscles Splints – Correct spasticity in the hand muscles

Assistive Devices – Devices that help individuals move about more easily and communicate successfully at home at school, or in workplace – Help child with CP to overcome physical and communication limitations

Assistive Devices Postural support or seating systems Open front walkers Quadrapedal canes (lighweight or metal) Gait poles