· PALSY (CEREBRAL): Encompassing a group of non-progressive (meaning the brain damage does not worsen), non-contagious diseases that cause physical disability in human development.
EPIDEMIOLOGY: Incidence: 2/1000 live births. M>F 1.33X. CP is the second-most expensive developmental disability to manage over the course of a person's lifetime (second to mental retardation), with an average lifetime cost per person of USD $921,000 (in 2003 dollars).
PATHOPHYSIOLOGY: Some causes of CP: 1) Before birth: Asphyxia, hypoxia of the brain, birth trauma, premature birth, and certain infections in the mother during and before birth such as strep infections, CNS infections, trauma, consecutive hematomas, placenta abruptio and multiple birth. Between 40% and 50% of all children who develop cerebral palsy were born prematurely 2) After birth: Toxins, severe jaundice, lead poisoning, physical brain injury, shaken baby syndrome, incidents involving hypoxia to the brain (e.g. near drowning), and encephalitis or meningitis.
PRESENTATION: CP symptomatology is very diverse. Symptoms: Abnormal muscle tone, posture (i.e. slouching), reflexes, or motor development and coordination. There can be joint and bone deformities and contractures (permanently fixed, tight muscles and joints). The classical symptoms are spasticity, spasms, other involuntary movements (e.g. facial gestures), unsteady gait, problems with balance, arthritis, and/or soft tissue findings consisting largely of decreased muscle mass. Scissor walking (where the knees come in and cross) and toe walking are common among people with CP who are able to walk. Common comorbidities: Mental retardation (IQ < 50 in 31%), Seizures (21%), unable to walking (20%), Blindness (11%).
TYPES: CP is divided into four major classifications based on the different movement impairments, which reflect the area of brain damaged. The four major classifications are: SAM → S-spastic, A-athetoid/Dyskinetic, A-ataxic, M-mixed.
S _______________, _____________, ______________, _____________, ______________
A ______________, ______________, ______________, ______________, _____________
M ______________, ______________, ______________, ______________, _____________
SPASTIC: Most common type (70% to 80% of all cases of CP).
Ø PATHOPHYSIOLOGY: Patients’ neuromuscular condition stemming from damage to the corticospinal tract, motor cortex, or pyramidal tract. These patient are characteristically hypertonic.
Ø SUBTYPES (classified by topography dependent on the region of the body affected): Hemiplegia, Bilateral hemiplegia, Diplegia, Quadriplegia.
v HEMIPLEGIA: One side being affected. Upper extremities (UE) affected greater than the lower extremities (LE). Injury to the left side of the brain will cause a right sided deficit.
v BILATERAL HEMIPLEGIA: Bilateral. UE affected more than LE.
v DIPLEGIA: Bilateral. LE affected more than UE. Most diplegics eventually walk, but typically with a characteristic crouched gait. Other commom features: toe walking, flexed knees, hip problems (dislocations), myopia, and strabismus (occur 75% of spastic diplegics due to weakness of the extraocular muscle). In many cases the IQ of a person with spastic diplegia is normal.
v QUADRIPLEGIA: Whole body affected. All four limbs affected equally. Features: hemiparetic tremors (unilateral uncontrollable shaking of the limbs that impairs normal movement), fluid buildup (diuretics and steroids are administered to decrease any buildup of fluid in the spine that is caused by leakage from dead cells), frequent UTI, hardened feces (important to monitor because it can cause high blood pressure and autonomic dysreflexia. Blockage of tubes inserted into the body to drain or enter fluids also needs to be monitored to prevent autonomic dysreflexia in quadriplegia, which can arise from the overreaction of the nervous system and can result in high blood pressure, heart attacks, and strokes.
ATAXIC CP: These patients have hypotonia and tremors. Features: Impaired balance (i.e. walking) and motor skills (i.e. writing, typing, or using scissors), and difficulty with visual and/or auditory processing.
Ø PATHOPHYSIOLOGY: Damage to the cerebellum..
ATHETOID/DYSKINETIC: Mixed muscle tone (sometimes hypertonia and sometimes hypotonia). Features: Trouble holding self in an upright position (for sitting or walking), and involuntary motions.
Ø PATHOPHYSIOLOGY: Damage occurs to the extrapyramidal motor system &/or pyramidal tract and to the basal ganglia.
TREATMENT: There is no cure for CP, but various forms of therapy can help a person with the disorder to function and live more effectively. In general, the earlier treatment begins the better chance children have of overcoming developmental disabilities or learning new ways to accomplish the tasks that challenge them. Treatment may include one or more of the following:
Ø Physical/Occupational therapy: Improved gait and volitional movement, maintain muscle tone, bone structure, prevent dislocation of the joints. Orthotic devices such as ankle-foot orthoses (AFOs) are often prescribed to minimise gait irregularities.
Ø Speech therapy: Often starts before a child begins school and continues throughout the school years. It helps patient breathe; talk clearly; and bite, chew and swallow food. Non-speaking people with CP are often successful availing themselves of augmentative and alternative communication systems such as Blissymbols.
Ø Drugs to control seizures, alleviate pain, or relax muscle spasms (e.g. benzodiazepienes, intrathecal baclofen).
Ø Surgery: Usually involves one or a combination of: Loosening tight muscles and releasing fixed joints, most often performed on the hips, knees, hamstrings, and ankles.
Ø Other: Nutritional counseling, massage therapy.