Pathology Flashcards
Describe cerebral palsy
- Non-progressive CNS disorder of posture & movement resulting from prenatal, perinatal, or postnatal (to 5 yrs) damage with multiple etiologies
- Not a specific disease state but a constellation of symptoms
What are some causes of cerebral palsy
- Lack of oxygen
- Difficult delivery
- TBI
- Infection
Clinical signs of cerebral palsy
- Interference of primitive & pathological reflexes
- Lack or distortion of righting & equilibrium reactions
- Alterations in muscle tone
- Sensory motor dysfunction due to sensory deprivation
What are the different types of CP
- Hypotonic: low tone, floppy (total body)
- Athetoid: fluctuating tone (total body)
- Ataxic: low tone, tremors, & incoordination (total body)
- Mixed can be more rigid tone/spastic
- High tone w/selective body involvement (spastic)
- Hemiplegia, diplegia, quadriplegia (spastic)
Gross motor function classification system lvl 1 for CP
- Children walk indoors and outdoors, and climb stairs without limitations. Children perform gross motor skills including running and jumping
but speed, balance, and coordination are reduced
Gross motor function classification system lvl 2 for CP
- Children walk indoors and outdoors, and climb stairs holding onto a railing but experience limitations walking on uneven surfaces and inclines, and walking in crowds or confined spaces. Children have at best only minimal ability to perform gross motor skills such as running and jumping
Gross motor function classification system lvl 3 for CP
- Children walk indoors or outdoors on a level surface with an assistive mobility device. Children may climb stairs holding onto a railing. Depending on upper limb function, children propel a wheelchair manually or are transported when traveling for long distances or outdoors on uneven terrain
Gross motor function classification system lvl 4 for CP
- Children may maintain levels of function achieved before age 6 or rely more on wheeled mobility at home, school, and in the community. Children may achieve self-mobility using a power wheelchair
Gross motor function classification system lvl 5 for CP
- Physical impairments restrict voluntary control of movement and the ability to maintain antigravity head and trunk postures. All areas of motor function are limited. Functional limitations in sitting and standing are not fully compensated for through the use of adaptive equipment and assistive technology. At level V, children have no means of independent mobility and are transported. Some children achieve self-mobility using a power wheelchair with extensive adaptations
Associated problems with CP
- Seizure disorder
- Mental retardation
- Communication disorders
- Orthopedic dysfunction: poor ankle/foot alignment, contractures, decreased ROM, Scoliosis, hip subluxation/dislocation, femoral ante version/antetorsion, tibial torsion
General PT treatment for CP
- Focus on handling to improve function, gross motor skills, facilitating righting, & equilibrium reactions, ROM, strengthening, balance, coordination, gait, positioning, assess DME, AT, & orthotic needs
What is spina bifida & myelomeningocele
- Myelomeningocele: Spinal defect where the dorsal vertebrae don’t fuse; spinal cord protrudes through the hole in spinal column; sensory/motor deficits below level of lesion
- Neural tube defect at 3-4 wks gestation neuro pore fails to close; can be associated with decreased maternal folic acid level & detected by alpha fetoprotein on testing
Associated problems with spina bifida & myelomeningocele
- Hydrocephalus
- Hip subluxation/dislocation
- Seizure disorders
- Club foot
- Urological difficulties
- Tethered cord syndrome
- Cognitive & visual motor difficulties
- Scoliosis with higher level lesions
General PT treatment for spina bifida & myelomeningocele
- ROM
- Strengthening
- Functional skills (transitions & transfers)
- Use of assistive devices
- Orthotics
- Adaptive equipment
Describe arthrogryposis multiplex congenita
- Congenital condition characterized by non-progressive joint contractures present at birth
- Contractures at shoulders, elbows, hips, & knees
- Typical posturing shoulder adduction with IR, hip abduction with ER
- Sensation is intact
- No cognitive impairments
Associated problems with arthrogryposis multiplex congenita
- Decreased ROM, fixed joint contractures
- Muscle atrophy
- Scoliosis
- Hip dislocation
General treatment for arthogryposis multiplex congenita
- Functional activities, gait
- Positioning, ROM
- Splints, casts, bracing
- DME, AT
- Surgical correction
What is juvenile idiopathic arthritis
- Rheumatic disease characterized by inflammation of connective tissue/joints
- Most common pediatric rheumatic disease
- May have systemic involvement (liver, heart)
Associated problems with juvenile idiopathic arthritis
- Chronic joint pain
- Hot & swollen joints
- Decreased ROM
General treatment for juvenile idiopathic arthritis
- Medication to decrease inflammation (NSAIDs)
- Exercises & activities to achieve goals
- DME, AT as needed
Define neuromuscular disorders
- Pathology of any part of the motor unit from the anterior horn cell to the muscle itself
Define muscular dystrophy
- A myopathy with mutation in genetic coding for the protein dystrophin (muscle is abnormal)
Define spinal muscular atrophy
- Affects the anterior horn cell (lower motor neuron) causing progressive muscle weakness
Describe Duchenne Muscular Dystrophy (Pseudohypertrophic)
- Most common myopathy in children
- Affects males x-linked inheritance from mother
- AVOID excessive fatigue and eccentric contractions
Describe Infantile Spinal Muscular Atrophy (Werdnig-Hoffman disease) SMA type 1
- Genetic disorder (autosomal recessive chrom. #5)
- Affects ant. horn cell, hypotonia
- Dx in first 3 months of life, life expectancy 3 years
- Failure to thrive, feeding problems, pulmonary infections, respiratory failure
Describe Chronic Wernig-Hoffman intermediate SMA type 2
- Dx at 3-6 months
- May live to adulthood
- Vulnerable to pulmonary infections
Symptoms of dystrophies & atrophies
- Progressive muscle weakness, wasting
- Decreased endurance
- Decreased ROM
- Decreased function
- Sensation OK: In Muscular Dystrophy cardiac muscle
involvement, In Spinal Mucular Atrophy decreased DTR’s
Problems associated with neuromuscular disorders
- Scoliosis
- Compromised respiratory function
- Joint contractures
- Poor weight control
- Emotional trauma
General treatment for neuromuscular disorders
- ROM/Positioning
- Strengthening (avoid fatigue)
- Functional mobility
- Pool therapy
- DME AT as needed
Describe the early phase of DUCHENNE MUSCULAR DYSTROPHY
- From Dx (2-6 yo) to onset of
marked functional loss (6-10yo) - Weakness: trunk, shoulder girdle, deltoids, biceps, gluteals, quads, anterior tibialis, and peroneals
- Tightness: calf muscles (decreased DF), hip flexors (decreased ext), IT band (decreased hip ADD)
Describe the transitional phase of DUCHENNE MUSCULAR DYSTROPHY
- Onset of marked functional weakness & postural adjustments (may begin b/w 6-12yrs)
- Gait is slowed, labored, foot drop, Tredelenburg, & difficulty stair climbing
- Posture: anterior pelvic tilt, wide base, shoulders extended back
Describe the non-ambulatory phase of DUCHENNE MUSCULAR DYSTROPHY
- Child uses WC 90% of time (begins ~10-15yrs)
- PT goals: prevent contractures, orthotics, respiratory exercises, & AD
Attention deficit hyperactivity disorder (ADHD) is characterized by
- Inattention, impulsivity, hyperactivity
- Learning disorders
- Etiology unknown
- More common in boys
- Can have ADD without hyperactivity
- Most referrals made between ages of 8-10
- May have mild GM delay, incoordination, sensory integration issues, behavioral issues
- Medication may help (Ritalin, Adderol)
- May have sensory integration dysfunction
General treatment for ADHD & sensory integration dysfunction/dyspraxia
- Sensory integration techniques (tactile)
- Gross motor, motor planning activities, organizational skills
Define Down syndrome (Trisomy 21)
- A genetic mutation of the 21st chromosome, often associated with advanced maternal age
Symptoms of Down syndrome (Trisomy 21)
- Generalized hypotonia
- Ligamentous laxity
- Mental retardation
- Foot deformities pronation, pes planus
- Hip subluxation
- ** high incidence of atlantoaxial dislocation due to lax ligaments may need C1-C2 fusion check cervical x-ray avoid neck flexion, tumbling activities
Generally describe mental impairments
- Intellectual (cognitive & language) ability below average from birth due to abnormal development
- Intelligence is determined both genetically & environmentally
- May be genetic defect (trisomy 21) or due to drugs, fetal alcohol syndrome, CNS bleed, prematurity, meningitis, TBI
- Difficulty learning
- Poor social skills
Autism is a syndrome of early childhood characterized by
- Abnormal social relationships
- Language delay (echolalia)
- Ritualistic behavior (structured)
- Cognitive delay
- Etiology unknown, attention is overfocused
General treatment for Autism
- Sensory integration activities (provide tactile, vestibular, & proprioceptive input)
- Gross motor skills & motor planning activities
Fragile X is a defect in one gene on the X chromosome with neurological symptoms of
- Mental impairment varies LD to severe
- Attention deficit and hyperactivity
- Anxiety and unstable mood Autistic-like behaviors
- Hyperextensible joints, hypotonia
- Morphologic features: Long face, large ears, flat feet
- Emotional & behavioral problems are common
- Tx similar to Autism
Describe developmental coordination disorder
- Motor coordination below expected level for child’s age & intelligence which interferes with ADLs, not due to a general medical condition
- Developmental dyspraxia; poor motor planning
- Clumsy child syndrome
- Visuomotor problems
Describe Leukemia in pediatrics
- Most common form of CA in children
- Malialignant disease of the blood, originates in the bone marrow
- Tx: general conditioning, orthotics
Describe brain tumors in pediatrics
- Malignancy in brain tissue
- Treated surgically through resection
- PT Tx: address sensory motor sequelae
Describe bone tumors in pediatrics
- Malignancy in the epiphyses of the long bones
- Tx: surgical amputation
- PT Tx: prosthetics, gait
Side effects of Vincristine intrathecal chemotherapy treatment for acute lymphoblastic leukemia
- Neutropenia (neutrophil count <500) with increased risk of infection
- Induced peripheral neuropathy causes weakness in DFs & intrinsic muscles of hands & feet
PT treatment of acute lymphoblastic leukemia
- Orthotics
- Stretching
- Age appropriate strengthening & gross motor developmental activities
Define Rett Syndrome
- Progressive loss of cognitive, communication and motor skills and autistic behaviors in females (X-linked genetic disorder)
Describe Osteogenesis Imperfecta
- “Fragile bones” inherited through autosomal dominant trait
- PT Tx: limit weight bearing, no resistive ex, AROM & active strengthening, mobility, DME orthotics
Define Mitochondrial disorders
- Disruption of mitochrondria in cytoplasm, affected genes are structurally altered, results in defective energy production
Define brachial plexus injuries
- Compression or traction injury to plexus commonly associated with a difficult birth or shoulder trauma severity ranging from
sheath swelling to total avulsion
Describe the differences between Orb’s palsy and Klumpke palsy
- Erb: C5/C6 nerve roots; shoulder girdle, & biceps weakness
- Klumpke: C7/C8/T1 nerve roots; distal weakness
Define anencephaly/microcephaly
- Decreased head size, or lack of formation of a portion of the brain, usually associated with profound retardation
Define hip dysplasia
- Subluxation of hip with poor alignment of femoral head & acetabulum associated with breech position
Define club foot
- Congenital foot deformity with PF, inversion, & forefoot adduction contractures
Define patent ductus arteriosus
- Failure to close after birth, L to R shunt allows blood flow from aorta to pulmonary artery
Define ventricular septal defect
- L to R shunt allows blood flow b/w ventricles, surgical patching required
Define asthma
- Lung disease with airway obstruction & inflammation which is usually reversible with exercise and/or use of inhaler
Define respiratory distress syndrome (RDS)
- Hyaline membrane disease seen in 20% of all premature children
- Cause is lack of pulmonary surfactant causing alveolar collapse & increased chance of anoxia
Define cystic fibrosis
- Inherited disease of the exocrine glands which affects respiratory & digestive systems
- Fibrocyctic disease of the pancreas
- Thick mucous production leads to frequent pneumonia
- Tx: general conditioning, postural drainage
What are the 4 stages of pressure ulcers
- 1: skin reddened
- 2: raw, open sore into dermis
- 3: ulcer descends into subcutaneous fat layer
- 4: ulcer descends down into muscle & often to the bone
Define Stevens Johnson Syndrome
- Severe form of toxic epidermal necrolysis
- Exfoliating skin disease
- May be linked to viral illness or drug interaction
Define epidermolysis bullosa
- 4 types of skin disease
- Decrease ROM, loss of fingers & toes
- Multiple surgeries & skin grafts required
Describe general tonic-clonic versus absence seizures
- Tonic-clonic (gran mal): total body stiffening followed by jerking then lethargy
- Absence (petit mal): brief LOC, staring
Precautions should a seizure occur
- Clear the area to avoid injury
– Don’t put anything in the mouth, (choking)
– Place child in sidelying (prevent aspiration)
– Time the seizure, (over 15 min. is emergency)
– Notify parents, teacher
– Avoid flashing lights/unevenly lit rooms with photo
seizures
Notify caregiver if signs & symptoms are observed for shunt
- Fever
– Decreased appetite
– Red, tender warm around shunt site (typically behind one ear)
– Lethargy
– Seizures
– Projectile vomiting
– Irritability
– Restlessness
– Headaches
– Change in behavior
– Eyes crossing
