Cerebral Palsy: Causes and Signs

• Cerebral palsy (CP) is a group of disorders affecting movement and posture development, leading to activity limitations.
• It involves non-progressive disturbances in the developing fetal or infant brain.
• Motor disorders often coexist with disturbances in sensation, cognition, communication, perception, behavior, and/or seizures.

Causes of CP:

• Genetic abnormalities
• Hypoxia (lack of oxygen)
• Maternal infection
• Maternal drug/alcohol abuse
• Prolonged labor
• Fetal stroke
• Traumatic brain injury
• Placental abnormalities
• Infant infection
• Prematurity

Signs of CP:

• Slow motor development causes difficulty with movement
• Unable to sit independently at 9 months
• Abnormal muscle tone
• Atypical or asymmetrical posture or movement
• Retention of primitive reflexes
• Involuntary movements
• Poor head control
• Difficulty feeding
• Fisted hands after 4 months, where hands should begin to open and move
• Hand preference before 12 months may indicate hemiplegia if exhibited too early

Diagnostic tools:

• MRI at term age can validate motor signs
• Hammersmith Infant Neurological Examination is uses a neurological exam for high-risk infants between 2-24 months tested for: cranial nerves, posture, movements, tone, and reflexes. It also identifies children at risk of CP relative to the type and severity of CP
• General Movement Assessment is appropriate for children at 20 weeks corrected age at risk for delays and predictive of CP by three months post-term. It involves video assessment for the absence or atypical general movements of infants in supine
• A lack of "fidgety movements" at three months of age can lead to suspicion of CP.
• Developmental Assessment of Young Children, a comprehensive norm-referenced test (birth to 5 years) with decreased scores between 6 and 12 months, may be indicate CP.

Classification of CP by Type:

• Spastic CP is caused by damage to the cerebral cortex with stiff muscles(spasticity).
• Ataxic CP is caused by damage to the cerebellum with poor balance and coordination (ataxia).
• Dyskinetic CP is caused by damage to the basal ganglia characterized by uncontrolled movements
• Athetoid
• Dystonic
• Hypotonic

Classification of CP by Location:

• Monoplegia/monoparesis affects one limb, usually an arm
• Hemiplegia/hemiplegic affects one side of the body, including the leg, trunk, and arm
• Diplegia/diparesis affects both legs and potentially the upper limbs
• Quadriplegia/quadriparesis affects all four limbs and the trunk

GMFCS (Gross Motor Function Classification System) levels:

• Level 1: Able to walk independently on various surfaces, climb stairs without railing, run, and jump; limitations in speed, balance, and coordination
• Level 2: Able to walk independently in most settings and climb stairs with a railing; limitations in speed, balance, coordination, endurance, and potentially need mobility aids for longer distances with limited running and jumping ability.
• Level 3: Able to walk indoors with mobility aids and stairs with railing under supervision; limitations in speed, balance, coordination, endurance, and reliance on wheelchair mobility for longer distances
• Level 4: May use power chair independently and walk short distances indoors with assistance; limitations in dependent manual wheelchair transport in all settings
• Level 5: Dependent on manual wheelchair with full support for posture and limited arm and leg movement
• Patients may vary in their classification based on different therapies like occupational (OT) and speech therapy, which specialize on hand and speech respectively.

Gross Motor Function Measure (GMFM):

• GMFM is a standardized assessment tool measuring gross motor function over time in children.
• It includes 5 dimensions: lying/rolling, sitting, crawling/kneeling, standing, walking/running/jumping.

Omtario Motor Growth Curves

• Patterns of gross motor development in a large cohort of children with CP who were followed for several years and tested using GMFM.
• Predictive curves for gross motor function based on GMFCS levels.
• Vertical lines on the growth curves represent the age a child is predicted to reach 90% of their potential motor development.
• Red dots indicate a child achieving 50% likelihood of completing specific motor tasks.
• D = walking down four steps reciprocally without hand support.
• C = walking forward ten steps unsupported.
• B =sitting unsupported (with hands free) for three seconds.
• A = lifting and maintaining the head in a vertical trunk position with trunk support in sitting for three seconds.

CP primary impairments: (different severities based on the type they have)

• Impaired posture
• Impaired motor control (gross, fine, oral)
• Impaired coordination
• Atypical muscle tone
• Impaired balance

CP secondary impairments:

• Oral motor dysfunction
• Speed disorder
• Feeding disorder
• Drooling
• Intellectual disability
• Learning disability
• Seizure disorder
• Pain
• Sleep disturbances
• Behavioral/emotional difficulty
• Drooling
• Constipation/incontinence
• Hearing impairment
• Visual impairment
• Orthopaedic abnormalities
• Joint contractures
• Hip subluxation/dislocation
• Scoliosis
• Foot deformities

Contracture prevention:

• No evidence to support passive, manual stretching to increase ROM in children with CP
• Incorporate high-intensity, active ROM activities
• Spasticity management
• Prolonged stretches

Other interventions for managing CP include:

• Orthotics
• Equipment like standers
• Serial casts for mild cases, with or without Botox
• Surgery for severe cases
• Isolated tendon/muscle release
• Single-event multilevel surgery

Hips in patients with CP:

• Risk factors for subluxation/dislocation
• Positioning such as hip flexion, adduction, internal rotation leading to superior posterior subluxation, acetabular dysplasia, and hip dislocation
• Femoral head-shaft angle where a larger angle of inclination (coxa valga) increases risk
• Higher GMFCS levels (primarily levels 4 and 5)

Effects of hip subluxation/dislocation:

• Pain
• Asymmetrical positioning of hips/pelvis/spine
• Leg length discrepancy

Treatment for hip subluxation/dislocation:

• Prevention including postural management and weight bearing
• Motor training
• Spasticity management via Botox, oral baclofen, or intrathecal baclofen and selective dorsal rhizotomy (SDR)
• Surgery for higher levels

Guidelines for hip surveillance:

• Guidelines from the American Academy of Cerebral Palsy and Developmental Medicine indicate frequency of hip checks and imaging based on GMFCS levels, more frequent if higher

Neuromuscular scoliosis in individuals with CP:

• Incidence increases with higher GMFCS level
• 50% of children with GMFCS levels 4 and 5 develop severe scoliosis
• Typically occurs before 10 years old and progresses beyond skeletal maturity
• Primarily caused by spasticity, muscle weakness, and poor muscle control
• Bracing is often ineffective as surgical is correction is recommended with curves greater than 50 degrees

Prevention of foot deformities:

• Equinas deformity includes plantarflexion
• Equinoplanovalgus deformity consists of plantarflexion, eversion, and abduction
• Conservative interventions include serial casting and AFOs with or without Botox for equinas
• Operative interventions such as TAL (tendo-Achilles lengthening for equinas) or calcaneal osteotomy

PT intervention for CP:

• Focus on function, participation, and quality of life
• Prevent joint contractures
• Position for optimal spine and hip alignment
• Bracing and equipment to promote alignment and function
• Improve fitness for cardiovascular health and weight-bearing to promote bone growth
• Motor interventions

Motor interventions include:

• Task-specific interventions
• Active, self-initiated movements
• High intensity and frequent
• Address the goals of the child
• Mobility training
• Treadmill training
• Strengthening
• Hand function
• Constraint-induced movement therapy
• Bimanual training
• Action observation training
• Neuroplasticity is dependent on motivation, attention, and repetition
• Intervention planning, consider the child's priority impairments
• Utilize multiple interventions to improve outcomes
• Considerations include current GMFCS level and Ontario Growth Curves
• What is the child's expected level of gross motor abilities?
• What will the child need to maximize function and participation?
• Consider bracing, equipment, and focus of PT treatment.

Spina Bifida Information

• Spina bifida, also known as myelodysplasia, involves the defective development of the spine and spinal cord, typically in the lower segments.
• It's caused by the failure of the neural tube to close early in gestation.
• It affects the musculoskeletal, neurological, and urinary systems

Types of spina bifida:

• Spina bifida occulta involves a closed lesion with non-fusion of the vertebral spinous process, intact spinal cord and meninges, and no neurological consequences
• Spina bifida with meningocele includes a failure of fusion of vertebrae with protruding meninges and sac of CSF through the defect, intact spinal cord within the spinal canal, and potential for slight problems depending on sac size
• Spina bifida with myelomeningocele (MM) involves protruding meninges, CSF, and spinal cord through the vertebral defect

Additional Information Relevant to Spina Bifida

• Spina bifida with myeloschisis, spinal cord is fully exposed.
• Conditions associating with spina bifida:
• Hydrocephalus is excessive accumulation of CSF in the ventricles leading to swelling of the head and often requiring a ventriculoperitoneal shunt.
• This shunt takes CSF from the ventricles and into the abdomen.
• Chiari II malformation is when the brainstem and cerebellum move caudally through the foramen magnum due to malformation of neurological tissues and may lead to breathing changes, swallowing difficulty, and upper extremity weakness Primary impairments of spinal cord abnormalities
• Paralysis and/or weakness
• Loss of sensation
• Loss of bowel and bladder
• Hypotonia or hypertonia
• Secondary orthopaedic impairments of spinal cord abnormalities due to motor and tone deficits
• Joint contractures secondary to intrauterine positioning and muscle imbalances
• Hip dislocation/subluxation is of secondary importance due to muscle pulls and weight-bearing forming the hips
• Bony mal-alignment like scoliosis can result from impaired motor functioning, where bracing will no longer fix and a club foot may develop, continuing to worsen

Additional Considerations for Individuals with Spinda Bifida

• Lack feet working at all or only working in one action (e.g., just DF)
• Associated comorbidities with spinal cord abnormalities:
• Neurogenic bladder (or not functioning at all)
• Learning disability or intellectual disability
• Hydrocephalus or malformation of the spinal cord which might cause issues with brain functioning
• Skin integrity issues due to not repositioning
• Osteoporosis/ Osteopenia from lack of weight bearing
• Obesity from decreased activity Individuals with spina bifida require a large medical team:
• Neurology/ neurosurgeon focuses on the close lesion
• Orthopaedics focus on potential contractures
• Urology ensures holding urine does not back up causing damage to the kidneys
• Endocrinology focuses on obesity and bone density
• Neuropsychology focuses on cognitive issues
• Dietician focuses on a increased chance of obesity
• Classification for spina bifida is determined by:
• Motor level: lowest intact, functional neuromuscular segment
• Dermatome testing: sensory may or may not correlate with motor level
• Motor and sensory testing which may differ between L and R sides
• Skip lesions, potentially creating intact and impaired sections
• Infant classification, observe spontaneous antigravity movements during palpating as good indicator of strength and functional level
• General ambulation status definitions
• Normal: independent without an assistive device
• Community: independent gait outdoors with or without assistive devices or orthosis, may use wheelchair for longer distances
• Household: indoor gait with orthoses and/or assistive devices, wheelchair for outdoor mobility
• Nonfunctional: walks only in therapeutic settings for bone health, strengthening (exercise or PT)
• Non-ambulatory: uses a wheelchair for mobility at all times

Spina Bifida Functional Abilities

• High lumbar L1-L2 causes hip flexion and adduction
• Mid lumbar L3-L4 causes knee extension
• Low lumbar L4-L5 causes hip abduction, knee flexion, ankle DF and inversion and toe extension
• Sacral causes hip extension, ankle PF and eversion alongside toe flexion and is more common
• Thoracic causes the abdominals, paraspinals, and Quadratus lumborum to contract causing hip flexion, abd, and ER, as well as knee flexion, ankle PF and a hyperlordotic postural deviation
• Reciprocating gait orthosis (RGO): Trunk hip knee ankle foot orthosis (THKAFO)
• Sacral: Ankle foot orthosis (AFO)
• Parapodium is a standing assist device which is usually a standing frame without hand use for thoracic and high lumbar levels, but can be unlocked to sit
• Hip knee ankle foot orthosis (HKAFO) , Supramalleolar orthosis (SMO), Foot orthosis (FO)

Red Flags for Individuals with Spina Bifida:

• Shunt malfunctions include: headache, irritability, nausea, fever, vision changes, speech changes, balance changes, also a kink for backup, as a result of CSF buildup in the brain, increasing pressure. Refer to PCP and neurosurgeon

Examination and Prognosis Factors of Individuals With Spinal Bifidia

• Requires continual referral with a desire to be free-moving from caudal and spinal scar tissue.
• Examination of patients involves foot position/alignment changes and functional goals.
• Interventions focus on spinal bifida levels while including family support, independence and participation.