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: 5 BW: 2.5kg• 35y/o G1P1 (1-0-0-1)• 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 MilestonesGross Motor Function Normal

(months)J.R. (months)

Rolls Over

Sits without support

Stands Alone

2-4.5

5-8

10-14

6

12

18

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 dysfunctionIncidence• 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 PalsyDiagnosis• 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 PalsyClassification 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 Palsy0 No increase in muscle tone1 Slight 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.

2 Slight increase in muscle tone, manifested by a catch, followed by minimal resistance throughout the reminder (less than half) of the ROM (range of movement).

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.

5 Affected 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 HypertoniaMODIFIED ASHWORTH SCALE

0 No increase in tone

1Slight 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

2Slight 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

5 Affected 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