Professional Documents
Culture Documents
47 Cerebral Palsy
2011/08 by R4陳彥榮, 2016/08 by R1賴俐安, 2018/10 by R2紀劭禹, 2020/11 by R1 李宗旻
Definition
I. “Cerebral Palsy (CP) describes a group of permanent disorders of the development of movement
and posture, causing activity limitation, that are attributed to non-progressive disturbances that
occurred in the developing fetal or infant brain. The motor disorders of cerebral palsy are often
accompanied by disturbances of sensation, perception, cognition, communication and behaviour;
by epilepsy; and by secondary musculoskeletal problems.”
II. Exclude other similar diseases
1. Genetic or metabolic disease
2. Peripheral etiology: e.g. neuromuscular or peripheral nerve injury
3. After CNS fully developed: e.g. traumatic injuries or oncologic manifestations
Epidemiology
I. The most common cause of disability in developed countries.
II. Overall prevalence rates have neither increased nor decreased in the past 20 years (2.11 per 1000 live
births)
III. Dramatic increases in children with very low birth weight (less than 1500g) (59.6 per 1000 live births)
and very early gestation (111.8 per 1000 live births).
Etiology/Risk Factors
I. Five major categories of risk factors
II. Large portion have multifactorial etiology from a mixture of known risk factors
III. Some have clear etiology or relationship: e.g.
1. Placental abruption with cardiorespiratory compromise → global hypoxic ischemic injury → spastic
quadriplegia
2. Basal ganglion or thalamic hemorrhage damage → dystonic type
3. Hyperbilirubinemia → athetoid type (近來因Rh incompatibility之血型檢驗而發生率減少許多)
IV. Prematurity: The germinal matrix surrounding the ventricles is at increased risk of ischemic or
hemorrhagic injury caused by the immature vasculature and fluctuations in cerebral blood flow.
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1. Germinal matrix hemorrhage: arterial hemorrhage from fragile capillaries in the watershed zone
next to the lateral ventricles
a. Highest risk for periventricular hemorrhage is between 23 and 32 weeks’ gestation
b. Can be detected early in the clinical course
via fontanelle cerebral high-resolution
ultrasound
2. Periventricular hemorrhagic infarction: of
venous origin, usually asymmetric and located
lateral to the external angle of the lateral ventricle
Classification
I. Topography or distribution of affected limbs
1. Hemiplegic CP
a. Unilateral upper and lower limb
b. Arm is often more affected than leg
c. Common in term and near-term infants
d. Associated with focal brain pathology (e.g.
unilateral strokes, asymmetric periventricular
leukomalacia (PVL), or congenital
malformations), or seizures.
2. Quadriplegic/tetraplegic CP
a. Bilateral upper and lower limb
b. Common in term and near-term infants
c. Associated with diffuse brain pathology (e.g.
cortical, subcortical, and intraventricular lesions)
3. Diplegic CP
a. Bilateral lower extremity
b. Most common in preterm infants 考
c. Associated with PVL.
II. Motor signs
1. Positive motor signs: involuntary increases in
frequency or magnitude of muscle activity
a. Hypertonia: examiner induced abnormally
increased resistance to movement about a joint
b. Hyperkinesia: unwanted, excess movement
2. Negative motor signs: lack of muscle activity or
control and encompass weakness, reduced
selective motor control, ataxia, and apraxia.
III. Standardized terminology (by Surveillance of Cerebral Palsy in Europe (SCPE)):
Unilateral spastic CP, bilateral spastic CP, dystonic CP, choreoathetoid CP, ataxic CP, non-classifiable
IV. Recognition clinical findings
1. multifaceted classification system: disturbances of sensation, perception, cognition, communication,
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and behavior.
2. Four components: motor abnormalities, accompanying impairments, anatomic and
neuroimaging findings, and causation and timing.
V. Functional classifications
上肢的功能 日常溝通
行動獨立
自主容易
行動上基本獨立但受部分限制
速度稍慢
行動需要手持輔具
需要協助
自行行動受限,可能使用”電動”的行動裝置 環境需要調整
行動依靠輪椅
b. Quantifies typical performance in gross motor tasks with mobility aids into 5 levels in 5 age
groups (before 2 y/o, 2~4 y/o, 4~6 y/o, 6~12 y/o, 12~18 y/o)
c. Used to develop motor development curves for prognosis
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activity and participation (d), environmental factors (e), and personal factors
4. Each domain has multiple levels associated: chapter number (first digit), second level categories (2
digits), third level categories (1 digit), fourth level categories (1 digit), and qualifiers (1 digit).
History
I. Basic medical history
II. Birth history and developmental history (when and how a milestone was achieved)
1. Different patterns of achieving the milestone
e.g. Crawling at 9 months, but different patterns:
a. Commando crawling: the child pulls their body across the floor with their arms
b. Bunny hopping: using flexion at the hip and knees but no reciprocal motions below the waist
c. increased muscle tone in the legs
2. Regression of milestones after attainment: neurodegenerative process → indicate a metabolic or
genetic disease workup
III. Family history of blood clots or stroke
IV. Any possible risk factors
V. Functional history: assist in determining treatment and life goals, include:
1. Mobility in the home and community setting
2. Participation in activities of daily living and community activities
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3. Persistence of primitive reflexes: a common finding (Table 47-6)
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b. Intellectual disability
c. Loss of ROM and hyperreflexia in all affected limbs
d. Persistent primitive reflexes
III. Abnormal tones
1. Hypotonia
a. Often a precursor to spasticity or athetosis
b. Prolonged period of hypotonia warrants further evaluation for a neuromuscular disorder (e.g.
congenital myopathy or spinal muscular atrophy)
c. Persistent hypotonia is often associated with genetic or metabolic syndromes
d. Only a small percentage of children are considered hypotonic CP
2. Spasticity (spastic type): 75% of CP, most common 考
a. Extension pattern: hip adduction, knee extension, and ankle plantar flexion (scissoring)
- More prominent in weight-bearing or vertical suspension.
- Special movement patterns: combat crawling pattern (difficult in reciprocal crawling, often
used in CP with lower limb extensor tone, yet is also used in “normally” developing children),
bunny hopping, knee walking, toe walking
- Severe extensor tone can exhibit opisthotonic posture with hyperextension of neck and trunk.
b. Flexion pattern: shoulder abduction, elbow/wrist/finger flexion, wrist pronation, thumb adduction
- More pronounced when stimulated or engaged in an activity resulting in a “high-guard” position
- Abnormal fisting of the hand can be the only manifestation in mildly affected children.
3. Dystonia (dystonic type)
a. Dyskinetic tone abnormality with alternating tone or co-contraction in the antagonist and agonist
→ twisting and repetitive movements and abnormal postures; often seen during rest
b. Frequently coexist with spasticity in CP
c. Compared with spasticity:
- Greater degree of co-contraction
- Increased resistance to external motion at slower velocities
- Greater impairment in muscle strength
- Less predictable surgical outcome
4. Athetosis (athetoid type): involuntary constant rotatory or writhing movement of distal extremities
5. Ataxia
a. Warrant further investigation into a degenerative process of the cerebellum.
b. Differential diagnosis: e.g. progressive spinocerebellar diseases (often present with a
combination of ataxia and spasticity), Friedreich ataxia and ataxia telangiectasia.
IV. Diagnostic algorithm (American Academy of Neurology)
1. Clinical presentation → workup → treatment plan
2. Examples:
a. Prenatal stroke causing hemiplegic pattern → coagulation workup and neuroimaging for brain
malformation
(Coagulation abnormalities are found frequently in these children and include factor V Leiden
mutation, protein C or S deficiency, elevated lipoprotein-a levels, the presence of anticardiolipin
or antiphospholipid antibodies, and G20210A mutation of prothrombin.)
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b. Periventricular leukomalacia → ultrasound evaluation
c. Suspected CP without determined etiology → routine imaging (preferably with MRI over CT)
Functional Prognosis
I. Prognosis for ambulation 考
1. Positive predictors
- Independent sitting before 2 y/o
- Less than 3 primitive reflexes by 18~24 m/o
- Able to crawl on hands and knees by 1.5 to 2.5 years (Delisa)
2. Negative predictors
- Not able to sitting independently before 4 y/o
- Not able to walk before 8 y/o (大黃)
3. Over 50% of all children with CP will ambulate (80-90% diplegia, 50% quadriplegia, 75% dyskinesia)
II. Prediction of longitudinal gross motor function
1. E.g. motor development curves based on an individual’s GMFCS level (Rosenbaum et al, 2012)
2. GMFCS levels I and V tended to remain, but GMFCS levels II, III, and IV tended to need
reclassification
Medical Management
I. Newer consensus definition of CP: a greater emphasis on recognizing associated impairments and
functional limitations as well as frequently cooccurring medical conditions aside from the primary
abnormalities in movement and posture.
II. Function, participation, and quality of life are often influenced by these comorbidities.
III. More severe the motor disorder or higher GMFCS level, the greater the likelihood the child will have
more numerous or more severe medical comorbidities; yet 2 exceptions:
1. Pain occurring in all levels of disability
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2. Behavior disorders seen more commonly in milder motor disability
IV. Feeding, Growth and Nutrition
1. Dysphagia is common in patients with CP
2. Impaired oropharyngeal strength and coordination
→ malnutrition (limited intake of key nutrients and endocrine abnormalities) and aspiration
→ risk for growth disturbances, increased infection rates, skin breakdown, osteopenia
→ ultimately decreased life expectancy
3. Management
a. Formal swallow study (indications: impaired oropharyngeal skills, wet vocal quality, increased
congestion during feeding, suspected silent aspiration, no protective cough)
b. Nutritional counseling, supplemental caloric and fluid intake
c. Increased time and aggressive positioning for feeding
d. Placement of gastrostomy tube: many parents resisted
4. Oral hygiene may be compromised, and children may have excessive sialorrhea, which can
contribute to aspiration events and cause secondary skin irritation
5. Endocrine 考
a. Hormone assays in children with CP have identified growth hormone deficiency.
b. Children with moderate to severe CP have been found to lack the dramatic pubertal growth
spurt that accounts for a significant amount of adult height.
c. Children with CP often experience early adrenarche that lasts longer than their peers.
d. Additional work has suggested that pubertal girls with CP, even when adjusting for nutritional
status, have decreased insulin-like growth factor-1 and growth hormone levels.
V. Pulmonary
1. Lung disease is an important cause of morbidity and mortality in CP.
2. Repeated aspiration events, infections, decreased mucociliary clearance, kyphoscoliosis, and airway
obstruction→cumulative injury to lung architecture
3. Aspiration 考
a. Modified feeding consistencies
b. Sialorrhea control with anticholinergic medications or botulinum toxin
c. Treatment of reflux
d. Chest physiotherapy and bronchodilators: assist mucociliary clearance
e. Gastrostomy with or without fundoplication
f. Immunization: e.g. Influenza, pneumococcus
g. Antibiotics
4. Upper airway obstruction: continuous positive airway pressure +/- surgery
5. Lower airway obstruction: bronchodilators and pressure
6. Sleep-disordered breathing:
a. Investigation of sleep apnea: history of snoring, irregular breathing patterns, daytime somnolence
b. Central/peripheral/both apnea: e.g. microaspiration and cough, altered light perception,
medication side effects
7. Hypoventilation: frequently in neuromuscular weakness
a. Noninvasive ventilator support
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b. External aids of secretion mobilization and cough generation
8. Scoliosis surgery may improve restrictive lung disease
VI. Neurologic Issues
1. Seizures 考
a. Most frequent in quadriparetic or hemiparetic presentation
b. Total 43% (hemiparetic 30%, quadriparetic 50~94%, diplegia or ataxic 16-27%)
c. Hyperkinetic movement disorders: may require prolonged video EEG monitoring for D/D
d. Medication use may be associated with cognitive dulling or other side effects such as anorexia
e. Weaning of seizure medication: depend on severity, and period of free of seizures (2 years)
2. Intellectual disability
a. 50% estimated to have cognitive impairment (約有 25%在學習方面是正常或優異的)
b. Nonverbal status and severe intellectual disability more common in quadriparetic presentation
c. Differential diagnosis:
e.g. Severe dysarthria with preservation of intellectual abilities: esp. basal ganglia injury and
dyskinesias
e.g. Cognitive dysfunction, hearing or vision limitations or dysarthria for communication
difficulties
d. Screening for impaired attention and executive functioning deficits, visual perceptual deficits,
mood disorders (anxiety and depression)
3. Vision deficits 考
a. Prematurity → retinal damage and myopia
b. Occipital lobe pathology → cortical visual impairment (CVI)
- CVI is now the most common cause of acquired bilateral visual impairment in childhood in
developed countries.
- The overall prognosis for significant improvement in visual function with CVI is poor.
c. Strabismus 斜視→ amblyopia 弱視 (約 20~60%腦性麻痺兒有斜視。六個月後仍有症狀應矯正。)
4. Hearing deficits
a. Etiologies: conductive vs. sensorineural
b. Dyskinetic CP by kernicterus →sensorineural hearing loss
c. Risk factors: very low birth weight, kernicterus, neonatal meningitis (e.g. TORCH =
Toxoplasmosis, Rubella, Cytomegalovirus, Herpes), severe hypoxic-ischemic insults, ototoxic
medications.
5. Speech and language disorders
a. Incidence: 38% of CP with articulation disorders and impaired speech intelligibility.
b. Anarthric or dysarthric speech: bilateral corticobulbar dysfunction or lesions to cortical speech-
language centers → oral-motor dysfunction
c. Many have preserved receptive language despite being nonverbal or markedly dysarthric.
d. Marked motor and speech impairment with relative preservation of intellect is the hallmark of
athetoid CP secondary to subcortical basal ganglia lesions.
e. Alternative means: yes-no/picture boards, adaptive switches, or augmentative devices.
VII. Genitourinary
1. Voiding dysfunction: impaired processing of sensory feedback and also incoordination of muscular
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functions (bladder contraction and sphincter relaxation)→ detrusor sphincter dyssynergia
2. Incontinence: difficulty and delayed in full continence
3. Urinary retention → increase chances of UTI → if high intravesical pressure → vesicoureteral reflux
→ hydronephrosis and pelviectasis (腎盂擴張)
a. Anticholinergic treatments for dystonia or sialorrhea may trigger urinary retention
b. Clean intermittent catheterization: required in chronic urinary retention with urinary stasis
4. Renal stone: Levetiracetam (Keppra) for epilepsy have an increased risk of renal calculi formation.
VIII. Gastrointestinal
1. Gastroesophageal reflux disease (GERD) and constipation may exacerbate hypertonicity
2. Refractory GERD may have dysmotility with delayed gastric empting and esophagitis
IX. Musculoskeletal pain and osteopenia
1. Musculoskeletal pain
a. Less mobile children (e.g. GMFCS IV or V) → increased risk for contracture and bony deformity
→pain
b. Common sites: hips, spine, knee, and foot and ankle
complex
c. Associated with patella alta, spondylolysis with
spondylolisthesis, and degenerative hip
conditions with aging
*Patella alta: high-riding patella, defined by Insall-Salvati
ratio (= patellar tendon length / patellar length, on a lateral
knee x-ray or sagittal MRI) over 1.2 (or 1.5)
*Patella baja: <0.8
2. Osteopenia 考
a. Risk factors for osteoporosis: decreased weight bearing, use of anticonvulsants, malnutrition,
and decreased sunlight exposure
b. Supplementation with calcium and vitamin D: routinely used to improve bone density, but
unclear effects on prevention of fragility fractures
c. Bisphosphonates: effective in improving BMD but with risk → reserved for fragility fracture
d. Increased weight-bearing via passive standing: failed to demonstrate a positive effect on BMD
and the risk for pathologic fracture
Therapeutic Management
I. A rising paradigm shift in the treatment of CP: motor impairments → each individual’s function
1. WHO ICF’s recommendation: focused on the individual’s level of activity and participation
2. Ultimate goal: encourage the child’s maximal potential in motor, cognitive, and social realms.
II. Childhood Disabilities and Education (in America)
1. Special education laws
2. Early intervention programs: government-sponsored programs to evaluate children at risk or with
delays between ages of 0 and 36 months → therapies, nutrition monitoring, care coordination,
audiovisual and social work
3. Public school system: after 36 months of age
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4. Individualized education program: classroom accommodations and associated information provided
to the children and the family
III. Therapy Interventions
1. General concepts
a. Many forms of therapies with currently minimal evidence
b. Combination of therapies
c. Family and child included in home setting
d. Various setting and frequency of therapy
e.g. School-based outpatient models (1~2 times a week)
e.g. “Pulse” or “episode” therapy through camps or day rehabilitation programs
2. Stretching
a. Goal: reduce the risk of contracture development
b. Sustained stretching: positioning devices, orthoses, and serial casting
c. 2017 Cochrane systematic review found high-quality evidence: stretch did not have clinically
important effects on joint mobility in individuals with neurologic conditions. (However, no study
followed patients longer than 7 months, and no conclusive evidence if stretching affects
quality of life or pain.)
d. A SR examining the effects of casting, either alone or in combination with botulinum toxin
type A (BoNT-A), on equinus in children with CP revealed no strong and consistent evidence
that combining casting and BoNT-A is superior to using either intervention alone.
3. Strengthening
a. No negative effects of increased spasticity or reduced ROM.
b. Increased participation and improved self-esteem
c. Cochrane systematic review(2017): all exercise interventions for CP strengthening is safe, and
weak evidence showing no improvement in gait speed, gross motor function, participation,
or quality of life.
4. Aerobic exercise
a. Improved physiologic measures of aerobic fitness without adverse effects (increased spasticity
or musculoskeletal trauma).
b. Improvements in aerobic fitness noted with 45 minutes four times a week of “high-intensity”
activities (such as wheelchair sports, swimming, matt exercises, or cycling). The benefit is not
maintained if the activity is not maintained.
5. Constraint-Induced Movement Therapy (CIMT)
a. For hemiparesis to improve motor function in the affected upper limb (患肢已有部分功能後再使
用此療法效果較好)
b. Unaffected limb is restrained with a removable cast, typically for 3 weeks. And the child
undergoes intensive, structured therapy in addition to daily activities and play.
c. Medium beneficial effect in arm function and activity level in the ICF-CY model
d. Home CIMT better than clinic- or camp-based settings
6. Functional Electrical Stimulation (FES):
a. Functional electrical stimulation (FES): NMES of sufficient intensity to elicit muscle contraction
and applied during a functional activity
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b. Threshold electrical stimulation (TES): low-intensity, subthreshold electrical stimulation, to
increased blood flow and muscle growth applied during sleep
c. Generally limited evidence. More evidence to support NMES and FES than TES
7. Transcranial Magnetic and Direct Current Stimulation(TMS and DCS)
a. Both are experimental interventions showing potential benefit in both children and adults with CP.
b. tDCS alone or in combination with standard therapies: improved proprioception, mobility,
body sway and balance, gait distance and velocity, and spasticity.
c. Focused on upper limb function in hemiparetic CP(Canadian Occupational Performance
Measure)
d. tDCS + CIMT :current ongoing studies.
8. Robotic and Partial Body Weight Support Treadmill Training (PBWSTT)
a. Repetitive and task-specific approach to facilitate stepping, locomotion and a more normalized
gait pattern
b. Setting: overhead harness system for body weight support + treadmill + walking facilitation by a
therapist/robot
c. Currently limited evidence in CP children
d. Despite reported improvements in gross motor function, functional status, walking performance,
and gait parameters, statistical significance was not reached in most of the studies.
9. Neuromotor therapy approaches (Table 47-8)
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c. Improving breath support, and optimizing the head and trunk position to facilitate a safe swallow,
feeding and communication
3. Specialized seating devices
a. Provide upright seated posture (e.g. wheelchair when a child outgrows strollers or additional
support needed): sitting on the floor, sitting on the toilet, feeding, bathing,and incorporation in a
mobility device.
b. Facilitate interaction with the environment
c. Minimize deformity or postural abnormalities
d. Special considerations:
e.g. Wheelchair base or stroller(嬰兒推車) base
e.g. Power mobility: at least 18 months old, with fair motor control, no visual deficits, and good
cognition
4. Gait trainer, walker, or crutches
a. For children with adequate head and trunk support
b. Facilitate gait training and provide external support and improve upright posture
c. Gait trainer: a wheeled walker with a sling seat and various support, allowing the patient to
propel the device without having a coordinated, reciprocal gait pattern
5. Mobile mechanical lift or overhead lift device
a. For dependent child and adult
b. Facilitate transfers and floor recovery
6. Augmentative communication: computers, switch devices, signboards, similar adaptive equipment.
7. Splinting and orthoses
a. Manage spastic but flexible dynamic deformities
b. Tailored to age, deformity, motor control and tolerance. E.g.:
- Lower limb orthoses: considerations including gait deviation, ankle-foot alignment, ROM and
tone
- Ankle-foot orthoses: decrease the energy cost of walking, improve gait parameters of stride
length and velocity.
V. Management of Hypertonia
1. Hypertonia
a. The most prevalent comorbidities of CP that affect function
b. Can be focal or generalized; affecting limbs and trunk
c. Can lead to secondary musculoskeletal complications (e.g. joint contractures and dysplasia) and
source of pain
d. Most common forms:
-Spasticity: velocity-dependent resistance
-Dystonia: more dynamic movement disorder, which involves an involuntary alteration in pattern
of muscle activation during voluntary movement; may fluctuate in presence and severity over
time
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2. Oral medications
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- Dosage and dilution guidelines: established by Allergan and Ipsen (pharmaceutical companies
that manufacture Botox and Dysport, respectively) for particular muscles; depends on age,
weight, severity of hypertonia, muscle localization
- Period of clinically useful relaxation: usually 12 to 16 weeks
- Injections spaced a minimum of 3 months apart: concern of neutralizing antibody formation
- Adverse events: rare (injection site pain, fatigue, excessive weakness; dysphagia and urinary
incontinence)
- Main indication: hypertonia hindering function (all functional GMFCS levels) + if left untreated
will become a fixed (rather than dynamic) contracture.
- Ideally start at a young age when gait patterns and motor function are still flexible→allowing for
gross motor function learning including strengthening of antagonist muscle groups during the
time window of tone reduction.
- effectiveness for dynamic equinus: BoNT-A injections + serial casting: mixed results
c. Phenol 考
- Mechanism: proteolytically causing neurolysis (主要作用於神經本身)
- Localization: electrical stimulation +/- ultrasound; sedation required for nerve isolation
- Commonly injected nerves: musculocutaneous, obturator, and sciatic nerve;
- Adverse effect: e.g. injecting other nerves have higher risk of dysesthesias
- Inexpensive than botulinum toxin
- Effective for 6 ~18 months
4. Intrathecal baclofen therapy
a. Often used to generalized spasticity or generalized moderate-to-severe dystonia
b. Indications: tone that is thought to interfere with function or the ability to provide care; modified
Ashworth scores >3; and definable goals for spasticity reduction
c. Programmable pump placed subfascially in abdominal wall, connecting to a catheter tunneled
from the side of the pump and inserted in intrathecal space at a desired spinal level
d. Dose: micrograms (compared to oral: milligrams) → smaller dose, reduced side effect;
screening lumbar bolus dose can be used to evaluate responsiveness
e. Pump replacement: every 5 to 7 years owing to battery life.
f. Effects: reduces hypertonicity and the effect continues long term.(positioning, ADLs, transfers,
sleep, oral motor skills, hand use, and comfort)
g. Potential (acute) complications: infections, cerebrospinal fluid leaks, catheter problems
(disconnection, migration, or kinking), urinary retention
h. Chronic complications: constipation and weight gain
i. Abrupt withdrawal: a medical emergency
- Increased tone, spasms, diaphoresis, agitation, and pruritus.
- If untreated, can progress to rhabdomyolysis and multisystem failure.
- Treatment: high doses of oral baclofen or intrathecal baclofen via lumbar drain,
benzodiazepines and cyproheptadine (for pruritus)
5. Deep brain stimulation
a. Programmable pulse generator implanted subcutaneously in the infraclavicular region
+ a stereotactic (立體定位) implantation of electrodes into the basal ganglia
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b. Treatment of choice in primary dystonias, or generalized secondary dystonia in CP (typically
quadriplegics with GMFCS levels IV to V)
c. For those who do not respond adequately to ITB, DBS should be considered. The response is
less than that seen in primary dystonia.
6. Selective dorsal rhizotomy 考
a. Surgical technique: single or multilevel laminectomies exposing the L2–S2 nerve roots and
selectively cutting a percentage of the dorsal rootlets (sensory nerve) with abnormal response
with the aid of electrophysiologic monitoring
b. Ideal candidates: 考
- Age 3-8 years old
- Diplegic CP, predominantly spastic tone (typically GMFCS levels I to III)
- Little upper limb involvement
- Minimal contractures
- Sufficient underlying strength, good selective motor control
- Fair cognitive and social capacity
c. Positive preoperative functional predictors:
- The ability to rise from a squatted position with minimal support
- The ability to crawl on hands and knees or tall kneel for a younger child
d. Positive outcomes: reduced spasticity, increased range of motion, improved gait pattern and
kinematics, increased speed, and decreased oxygen cost, making gait more efficient
- SDR done at early age: reduce bony deformity, decreasing the need for orthopedic surgery
- Some degree of lower limb weakness can be unmasked postoperatively by reducing the
spasticity, making intensive physical therapy a necessity
e. A study (136 patients retrospectively) : spasticity decreased substantially at the major muscle
groups—hip flexors, adductors, hamstrings, and rectus femoris; less reduction in the plantar
flexor group, which may be functionally beneficial for joint stabilization and power production.
f. Long-term outcomes 5 and 20 years after SDR : improvement in the Gross Motor Function
Measure and gait pattern.
g. Long-term complications: infrequent (sensory dysfunction, bladder or bowel dysfunction, or back
pain)
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7. Combined dorsal and ventral rhizotomies: performed in a few children with severe spasticity and
dystonia who were not candidates for ITB.(加上 Ventral roots prevents the recruitment of muscles into
dystonic muscle activation.)
Orthopedic Management
I. General concepts
1. Surgeries designed to improve ambulation: preferred in GMFCS levels I to III
Surgeries aimed toward improving care and comfort: in GMFCS levels IV to V
2. Indications for orthopedic procedures:
a. Presence of contractures that hinder function or interfere hygiene
b. Subluxation/dislocation of joints (most commonly the hips)
c. Rotational problems or lever (槓桿) arm dysfunction, causing gait problems
3. Basic 4 major types of orthopedic surgeries in CP:
a. Muscle releases and lengthening
b. Tendon transfers
c. Osteotomies 截骨術
d. Arthrodesis 關節固定術
4. Timing of surgical intervention: determined by
a. CNS maturation
b. Ambulation potential
c. The rate which the deformity is developing
d. “Graham has suggested delaying any orthopedic surgical intervention until ages 7 to 9 years
because of a high risk of recurrence unless there is evidence of hip subluxation. Before this age
the focus should be therapies along with tone management.”
5. Orthopedic surgery has NO effect on motor control, strength or muscle tone
→The brief reduction in hypertonia : direct result of the temporary decrease in muscle tension that
involves the Golgi tendon apparatus and the muscle spindle.
→ Further comprehensive team to address tone management pre-surgery and post-surgery
II. Hips: hip displacement
1. Prevalence: estimated that 1/3 children with CP
2. Highest risk: in nonambulatory population GMFCS levels IV-V (approximately 60% not walking by
the age of 5 years are likely to develop hip subluxation, with the greatest risk in those with severe
neurologic involvement.)
3. Pathophysiology:
a. Strong hip adductors / flexors (iliopsoas) + weak hip abductors / extensors
→ head of the femur to gradually migrate out of the socket
b. Lack of weight bearing (nonambulatory children): axial weight-bearing helps deepen the
acetabulum and induces a progressive varus angle of the femoral neck with respect to the shaft
→ acetabular dysplasia and coxa valga
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*Coxa valga:
Angle formed between the neck of the femur
and its shaft (caput-collum-diaphyseal
(CCD) angle or the femoral angle of
inclination) is increased beyond >140º.
Normal is between 125-135º in adults, but
can be 20-25º greater at birth and 10º
greater in children. When the angle is
<120º, this is considered coxa vara
4. Soft tissue surgeries: combination of hip adductor tenotomy, psoas recession, iliopsoas tenotomy,
or medial hamstring lengthening after hip subluxatation
5. Bony surgeries
a. Periacetabular osteotomies: improve coverage of the femoral head
b. Proximal varus and external rotation osteotomies of the femur: address the angular and
rotational deformities
c. Guided growth with transphyseal screw to create a temporary medial hemiepiphysiodesis for
control hip migration: effective in controlling progressive hip subluxation and prevented major
reconstructive surgery in the majority of patients
6. Predictors: 誰需要密切追蹤甚至開刀? 考
a. Children with CP who walked 10 steps alone by 30 months of age → did not require treatment
of their hips by 5 years of age
b. Severity of motor disability:
- At 18 months of age → not predictive
- By 24 to 30 months of age → predictive → children with bilateral CP should have a
radiograph of the hip at age 30 months or earlier
c. Radiology:
- Migration percentage greater than 33% → likely to need further treatment
- Progression of the migration percentage by more than 7% per annum → careful monitoring
- Acetabular index over 30 degrees → likely to need further treatment
- consensus for potential hip migration in children with CP published in 2011 and with guidelines
18
in 2015
7. Goals
a. For ambulatory CP: contained and stable hip → preserved ambulation
b. For nonambulatory CP: prevention of dislocation, maintenance of sitting balance, facilitation of
hygiene, prevention of pain, prevention of skin breakdown, and prevention of progressive
scoliosis
8. Pain
a. The relationship between hip pain and subluxation or dislocation of one or both hips is highly
variable; the relationship between radiographic and clinical findings remains elusive.
b. Treatment for established pain attributed to hip dislocation:
-Intraarticular steroid injections: temporary relief
-Salvage procedures: proximal femoral resection, osteotomies to redirect the femoral head, and
hip arthroplasty
9. Management
a. regularly screened for the presence and progression of neuromuscular hip dysplasia
b. follow Australian Hip Surveillance Guidelines : standard of care
c. monitoring the acetabular index and migration percentage are the most effective parameters to
determine timing of surgical intervention
III. Lower Limb(考很多次)
1. Primary impairments include hypertonia, poor balance, poor selective motor control, and weakness.
2. Delays in gross motor skills acquisition and atypical gait patterns lead to further deformity.
3. Biarticular muscles are more commonly contracted (e.g., psoas, rectus femoris, hamstrings, and
gastrocnemii) than the monoarticular muscles
4. Diplegic sagittal gait pattern classification: 考
to define gait pathology, potential causes and treatment strategies
(Rodda JM, Graham HK, Carson L, et al: Sagittal gait patterns in spastic diplegia, J Bone Joint Surg Br 86:251-258, 2004.)
19
a. Group I: true equinus
- The ankle is in equinus (limited in dorsiflexion).
- The knee extends fully or goes into mild recurvatum (excessive extension of tibiofemoral joint).
- The hip extends fully and the pelvis is within the normal range or tilted anteriorly.
- Orthotic: hinged AFO or anterior leaf spring AFO → restricts excessive plantar flexion and
aids movement into dorsiflexion
b. Group II: Jump gait (most common gait in young children)
- The ankle is in equinus, particularly in late stance.
- The knee and hip are excessively flexed in early stance and then extend to a variable degree in
late stance, but never reach full extension.
- The pelvis is either within the normal range or tilted anteriorly.
- Orthotic: similar to group I.
c. Group III: Apparent equinus
- The ankle has a normal range.
- The knee and hip are excessively flexed throughout stance.
- The pelvis is normal or tilted anteriorly.
- Orthotic: solid AFO → assist the plantargrade alignment of the tibia with the foot during the
ankle rocker during the stance phase
d. Group IV: Crouch gait (most common gait in older children)
- The ankle is excessively dorsiflexed throughout stance.
- The knee and hip are excessively flexed.
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- The pelvis is in the normal range or tilted posteriorly.
- Orthotic: GRAFO (ground-reaction AFO) 考→ restricts excessive dorsiflexion during the ankle
rocker and directs the ground reaction force in front of the knee throughout stance (via anterior
pre-tibial shell to increase the proximal lever arm) so that knee extension is gained and the
plantar flexion knee extension couple is restored
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b. E.g. Stiff knee gait or overactive rectus femoris
6. Contracture managements
a. Tone management: recommended before orthopedic intervention, especially at a young age
b. Orthopedic surgeries: lengthening and transfer → more effective if integrated into a single-
event multilevel surgery (SEMLS, correction of all orthopedic deformities in one session)
c. Guided growth : management of leg length discrepancies, genu valgum, and knee flexion
contractures. Use transphyseal screw(temporary hemiepiphysiodesis to force the bone to grow
in one direction or not at all)-> prevent the need for later major bony orthopedic interventions
d. Goals:
- For ambulatory CP: gait without restriction (for ambulatory CP)
- For nonambulatory CP: contain hips (psoas and adductors) without including bony surgery;
facilitating sitting (hamstrings) and perineal care (adductors)
7. Abnormal long-bone torsions (lever arm dysfunction, LAD)
a. E.g. femoral anteversion and tibial torsion (less common, developed later)
b. If untreated → produce further malalignment of bone and unwanted gait compensations
8. Foot and ankles: goal of obtaining a plantigrade, brace-able foot and stable base of support for
standing and gait.
IV. Upper Limb
1. Management of spasticity and orthopedic intervention similar to lower limb
2. Pattern of joint contractures: flexor synergy → shoulder internal rotation, elbow flexion, forearm
pronation, wrist and finger flexion, thumb-in-palm deformity, and swan neck deformities
3. Common functional impairments: deficiency in sensation, pinch, grasp-release, and reach
4. Ideal candidate for surgical intervention: motivated child with volitional use of the hand, spasticity
without fixed contractures, and reasonable sensory function including stereognosis, proprioception,
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and light touch.
5. Patients with significant athetoid movements and dystonia do not benefit from upper limb surgery
given the unpredictability of the outcomes
6. Operation interventions: (Table 47-11)
V. Spine: scoliosis 考
1. Incidence: estimated as 21% to 76%
2. Severity: directly correlates with the degree of total body involvement (and level of mental
retardation) and inversely correlates with functional and ambulatory status and GMFCS level
3. Scoliotic curves: 2 groups
a. Group 1: Curves are single thoracic or double thoracic and lumbar curves with level pelvis,
typically associated with ambulatory patients (GMFCS II to III)
b. Group 2: Curves are long thoracolumbar or C-shaped curves with associated pelvic obliquity,
typically associated with nonambulatory patients
4. Natural history: onset occurs from 3 to 10 years of age with rapid progression during the adolescent
growth spurt
5. Evaluations:
a. Physical examination: performed every 6 to 12 months
b. Radiograph: obtained if a curve is detected
6. Managements:
a. Observation: flexible curves less than 40 degrees
b. Bracing: very limited role in decreasing curve progression
c. Spinal instrumentation and fusion: most considered because of significant curve progression,
loss of sitting balance, and comfortable function
- Delayed in children with flexible deformities until skeletal maturity
- High surgical complications
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Complementary and Alternative Medicine
24
考古題 *小兒復健主要在第 25~33 題
109 年
B 034 下列有關腦性麻痺及其治療之相關敘述,何者錯誤?
A.腦性麻痺是指兒童腦部尚未成熟之前受到非進行性的傷害,造成永久性動作與姿勢障礙症候群
B.嬰幼兒的動作發展是軀幹由頭至腰,四肢由遠端至近端,手部發展由橈端至尺端,故腦性麻痺復健也須遵
照神經發展循序漸進
C.常用的復健治療法包含 Bobath、Vojta、引導式療法;其他療法包含 Rood 療法、治療性電刺激、多派別
技術應用及侷限誘發療法;馬術治療是另類輔助療法之一
D. Vojta 治療是讓患童在一定的姿勢下針對 trigger area 給予刺激,誘發出反射性爬行及反射性翻身,藉由
反覆加強刺激使正常運動模式得到記憶及加強,進而達到治療目的
E.腦性麻痺兒童若 2 歲以前能獨自坐穩,將來有 9 成以上可獨自行走,而痙攣性半邊麻痺型幾乎都可獨立行
走與生活自理
C037 一位 12 歲大的腦性麻痺病人可不用輔具行走,但室外行走仍稍有限制,只有長距離移動時需要使用輪
椅,根據粗動作功能分類系統(gross motor function classification system,GMFCS),該病人是屬於下列那
一級?假設其成長過程中功能等級並未改 變,往前推估其 2 歲時的能力表現應該會是如何呢?
A.GMFCS 第 1 級/ 2 歲時可以獨立行走
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B.GMFCS 第 2 級/ 2 歲時可以翻身
C.GMFCS 第 2 級/ 2 歲時可用雙手支撐維持坐姿
D.GMFCS 第 2 級/ 2 歲時可以維持獨立坐姿
E.GMFCS 第 3 級/ 2 歲時可以獨立行走
12 歲可步行但稍受限->屬於第 2 級
2 歲時可自行維持坐姿,但雙手操作物品時可能有平衡困難
GMFCS 繁體中文版
https://canchild.ca/system/tenon/assets/attachments/000/000/082/original/GMFCS-ER_Translation-
Traditional-Chinese.pdf
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108 年
C 030.某腦性麻痺病人在室內行走需用助行器,室外短距離路程可自己手推輪椅,需要他人協助準備才能用
手操作物件,和熟悉的人溝通可有效的表達,但和不熟悉的人溝通會有困難。請問其 Gross Motor
Function Classification System(GMFCS)、Manual Ability Classification System(MACS)以及
Communication Function Classification System(CFCS)分別在下列何種 level?
(A) Ⅱ,Ⅱ,Ⅱ
(B) Ⅱ,Ⅱ,Ⅲ
(C) Ⅲ,Ⅲ,Ⅲ
(D) Ⅲ,Ⅱ,Ⅱ
(E) Ⅳ,Ⅱ,Ⅱ
E 031. 下列有關成人腦性麻痺患者的敘述何者錯誤?
(A) 由於醫療照護的進步,90%的腦性麻痺患者可以活到成人
(B) 很容易有肌肉骨骼系統的提早退化
(C) 成人患者容易有腰背及腿部疼痛,要給予正確的診斷及治療
(D) 他們的平均餘命(life expectancy)與智力呈正比
(E) 他們的平均餘命(life expectancy)與運動能力呈反比
大黃:Ch.21 p393.
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(A) 90% 的腦性麻痺患者可以活到成人。
(B) 現肌肉骨悟系統提早老化是常有的事
(C) 成人患者則有 30~67% 都有腰背及腿部疼痛 。 需要給予正確診斷及治療巧
(D)腦性麻痺者的壽命(life expectancy) 與智力及運動能力成正比。高功能患者可能與正常人
壽命相等,但低功能患者則因活動力及進步能力較差,平均比常人壽命縮短 11 年。因此,他們在不同嚴重度
患者之間壽命的差距會高達 40 年
C 033.下列那一種狀況的腦性麻痺兒童將來可獨立行走的機會最高?
(A) 在三歲時可以獨立坐穩
(B) 在兩歲時存在三種原始反射
(C) 在兩歲半的時候可用四點著地的姿勢爬行(crawl on hands and knees)
(D) 雙邊麻痺痙攣型的兒童在兩歲左右可從仰臥姿勢翻身至俯臥姿勢
(E) 四肢麻痺痙攣型的腦性麻痺兒童在痙攣出現之前有過一段低張力的時期
107 年
E 029. 發現下列臨床徵象時,要懷疑個案可能有腦性麻痺,但何者除外?
(A)出生時全身低張力,2 個月時就可以用 opisthotonus posture 翻身
(B)3 個月大時有 Rossolimo reflex
(C)8 個月大的嬰兒慣用右手
(D)爬行時呈現 bunny hopping 姿勢
(E)8 個月大有 parachute response
(A) Opisthotonus posture: a state of severe hyperextension and spasticity in which an individual's head,
neck and spinal column enter into a complete "bridging" or "arching" position. -> severe cerebral palsy,
traumatic brain injury, tetanus
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(B) primitive reflexes ->controlled at the level of the brainstem and spinal cord. 當 CNS
成熟後就會去整合和抑制 primitive reflex(大概出生 3-6 個月後會陸續消失,若 CNS 有
問題就會持續存在) Rossolimo reflex is one of a number of Babinski-like responses
(C) Children begin to develop hand dominance around 2-3 years and this should be well
developed by age 5 and fully developed by age 6.
(D) Bunny hopping: Impaired ability to coordinate reciprocal lower limb movement->
compensatory crawling strategy of simultaneous hip flexion (ie,“bunny-hopping”)
(E) Primitive reflexes 約在 2 個月大左右開始被 posture response 給取代 (通常持續終身):用以促進幼兒的平
衡和保護動作的發展(ex:parachute reaction, which appears at 6 months and never disappears)
A 032. 下列有關使用肉毒桿菌毒素治療腦性麻痺肌肉痙攣的敘述,何者錯誤?
(A)注射當天就有最佳效果
(B)療效可維持 3-6 個月
(C)不會產生感覺異常
(D)一般不需在麻醉下進行
(E) 藥物是作用在 neuromuscular junction
(A) 約幾天後開始有效
The period of clinically useful relaxation is usually 12 to 16 weeks, and it is recommended that injections be
spaced a minimum of 3 months apart because of concern for neutralizing antibody formation.
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106 年
相關指標需要密集監控
(A) Migration percentage > 33%
(B) migration percentage 每年增加> 7%
(C) Acetabular index>30 度
105 年
E 027. 下列何者與腦性麻痺(CP)患者能否走路的預後無關?
(A) Clinical type of CP
(B) Independent sitting before 2 years old
(C) Crawl on hands and knees by 1.5 to 2.5 years
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(D) Persistence of 3 or more primitive reflexes at 18 to 24 months
(E) Gender
C 033. 關於腦性麻痺兒童痙攣之處理,下列敘述何者正確?
(A) 肉毒桿菌注射後會造成感覺異常
(B) 肉毒桿菌注射後張力立刻降低
(C) Phenol 主要作用於神經本身(nerve)
(D) 肉毒桿菌注射後會造成 smoky urine *smoky urine: 意指顏色偏深的尿液(i.e. cloudy/cola-colored urine)
(E) 選擇性背神經根主要切斷運動神經
104 年
A 29. 一位 5 歲雙邊痙攣型腦性麻痺(spastic diplegia)兒童,以 modified ashworth scale 評估肌肉張力,
其 gastrocnemius 為 2 分,soleus 為 1 分, hamstring 為 1 分,hip adductor 為 0 分,上肢肌肉為 0 分,
請問下列何種治療最適合?
(A) 肉毒桿菌毒素注射
(B) 口服抗痙攣藥物
(C) 椎管內 baclofen 注射手術(intrathecal baclofen pump)
(D) 骨科手術
(E) 選擇性背神經根切除術
B 30. 腦性麻痺因 hamstring spasticity 導致的 crouch gait,若無嚴重的 knee flexion contracture,最適
合穿戴:
(A) Hinged AFO (B) Floor reaction AFO (C) Posterior leaf spring AFO
(D) Supramalleolar AFO (E) Foot orthosis
31
(E)跟其他 idiopathic scoliosis 比起來,curve 達四十度的 CP 病人即使骨骼成熟了,scoliosis 仍是可能繼續
progression.因此不一定需要等到骨頭成熟或成長停止才手術.
A 198. 一位腦麻痺病人由兒童成長為成人後,在復健照顧上最重要的是強調:
(A) 功能獨立
(B) 肢體攣縮的校正
(C) 髖關節脫臼之外科手術
(D) 脊柱的校正
(E) 教育
D 199. 以下關於腦性麻痺患者的髖關節疾病敘述何者正確?
(A) 通常腦性麻痺病童如有髖關節脫位,從出生時的髖關節解剖構造,就可以發現有明顯脫位
(B) 髖關節脫位(hip displacement)的機率隨著 GMFCS level 增加而下降
(C) X 光檢查髖關節 Reimer’s migration percentage 若是<30%是篩檢腦性麻痺患者的髖關節問題的最簡單方
法
(D) 下肢不對稱的肌肉張力與痙攣被認為是造成髖關節脫位的重要原因
(E) 髖關節逐漸脫位的過程中,病童最常會因疼痛就醫
D 200. 關於腦性麻痺各種變形及外科處理的敘述,以下何者錯誤?
(A) 術前應就病患的年紀、就醫的動機、詳細評估其智力、感覺功能及運動功能
(B) Crouch gait 患者若有 knee extensor insufficiency 合併 patellar alta 可以考慮進行 Distal femoral extension
osteotomy 及 patellar tendon advancement 來矯正
(C) 若前臂僵直在旋前姿勢(pronation)且手腕伸展能力不佳時,可考慮施行旋前圓肌轉移手術(pronator
transfer)
(D) 病患出現 crouch gait 經常代表 Hamstring 長度太長,需要進行 hamstring tightening procedure
(E) 若合併 scoliosis 及 hip dysplasia,則手術處理的順序,可能需依病患年紀而有不同考量
(B) crouch gait 問題的 CP 病人接受了 Distal(supracondylar)femoral extension osteotomy and patellar tendon
advancement 後,改善了 knee function in gait , improving knee extensor strength, reducing knee pain and
improving function.
(C) Forearm pronation and wrist flexion contracture can be a disability for daily living care and 2-hand function
in cerebral palsy (CP) children. improved the functional outcome in wrist extension and decreased the
forearm pronation.
(D) hamstring, psoas, and gastrocnemius tightness cause tightness
(E) they differ somewhat in their presentation and treatment approach. These include spasticity and the
various orthopedic conditions of contractures, scoliosis and hip dysplasias, bowel and bladder incontinence,
decubitus prevention, and gastroesophogeal reflux.
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103 年
B 027. 腦性麻痺患者使用 intrathecal baclofen pump 治療,下列敘述何者錯誤?
(A) 適用於 generalized dystonia
(B) 適用於 focal spasticity
(C) Sedative effect 較口服 baclofen 低
(D) 費用昂貴
(E) 可能有慢性便秘及體重增加問題
102 年
33
101 年
34