CP and GBS Flashcards
What is cerebral palsy
non-progressive central motor dysfunction affecting muscle tone, posture, movement
abnormalities of developing fetal/infantile brain (perinatal or post natal)
define muscle tone
strength and tension of muscles
define hypotonia
decreased muscle tone
define hypertonia
increased tightness of muscle tone and reduced capacity to stretch
define paresis
weakness
define plegia
paralysis
define monoplegia
paralysis of one limb
define hemiparesis or hemiplegia
paralysis or weakness of one side
define diplegia
paralysis of both legs
define quadriplegia
paralysis of all limbs
Etiology of CP
multifactorial
- no identifiable cause
- can be due to hypoxic event
- damage to developing brain
What are some risk factors of CP
- preterm babies with low birth weight
- perinatal hypoxia
- congenital abnormalities
- genetics
- stroke or hemorrhage in perinatal period
- intrauterine infection
- kernicterus (high bilirubin)
Epidemiology of CP
- most common motor disability in childhood
- 2 per 1000 children
- risk among preterm low birth weight
What are the subtypes of CP
signs and symptoms more clear after 18-24 mos
- spastic (MC)
- dyskinetic
- ataxic
Describe the positive and negative signs of spastic CP
What are the subtypes of spastic CP
Spastic hemiplegia/diplegia/quadriplegia
What happens in dyskinetic CP
-damage to the basal ganglia
What are the hallmarks of dyskinetic CP
- involuntary movement
- usually no contractures
- choreoathetosis
- dystonia
Define choreoathetosis
chorea consisting of rapid irregular contractions of individual muscles and athetosis consisting of slow smooth writhing movements
Where is the lesion in spastic CP
corticospinal tract or motor cortex
Where is the lesion in ataxic CP
cerebellum
Describe the hallmarks of ataxic CP
- rare
- motor and language delayed
- may improve with time
What are some of the associated symptoms of CP
- pain
- intellectual disability
- SLP disorders
- epilepsy
- visual impairment
- hip displacement
what are the clinical features of CP
- nonprogressive but functional ability continue to evolve
- neurobehavioral signs
- developmental reflexes
- motor tone and posture issues
What are some of the developmental reflex issues in CP
- tonic labyrinthine reflex
- tonic neck reflex
- galant
What kind of gait is this
scissoring gate
What kind of gait is this
toe walking
What are some of the early signs of CP
- early hand dominance
- delayed motor milestones
- primitive reflexes
- scissored legs below 6 mos
- floppiness
- stiffness
How is CP diagnosed
clinical, no test to confirm or exclude
- typically get a transcranial and/or MRI
- screen for thrombophilia if MRI shows evidence of cerebral infarction
- screen for associated comorbidities
- metabolic and genetic testing
Describe the treatment and management of CP
- functional assessments: gross motor function classification or Modified Ashworth scale
What meds can be used to treat spasticity in CP
- botulinum toxin
- oral antispastics (Diazepam, Baclofen)
- intrathecal baclofen pump
- DBS or other surgical tx
Motor milestones
- 4 mos = rollover
- 6 mos = sitting
- 1 year = walking
What other treatments may be involved for associated issues with CP
- ortho
- PT/OT
- gastrostomy feeding
- ACh meds for drooling
- psychosocial support
CP prognosis
- most survive to adulthood
- walking early correlated with better IQ outcomes
- poor walking prognosis if head control not achieved by 20 mos
- complications: hip disorders, aspiration pneumonia
Define Guillain-Barre syndrome
acute, monoplastic, paralyzing illness provoked by preceding infection
Etiology of GBS
immune response to infection that cross reacts with PNS because of molecular mimicry
- demyelinating
- MC infection = campylobacter jejuni, also EBV, HIV, covid, Zika
- can be triggered by immunization, surgery, trauma, bone marrow transplant
What is the general time to illness presentation following viral infection (also Covid)
5-16 days
Epidemiology of GBS
1-2 per 100,000 world wide
- age, M>F
Clinical presentation of Guillain-Barre Syndrome
- progressive symmetric muscle weakness with absent/decreased deep tendon reflexes
- few days-week onset
- starts in legs (10% in face/arms)
- parasthesias in distal extremities
- back pain
- progressive syx for 2 weeks
What are the 3 major variants of GBS
- AIDP (acute inflammatory demyelinating polyradiculoneuropathy)(US, Europe)
- MFS (Miller fisher syndrome)(Japan)
- AMAN/AMSAN (acute motor sensory/axonal neuropathy) (US)
How is GBS diagnosed
- clinical
- lumbar puncture: elevated CSF protein, normal WBC
- electrodiagnostics
- spinal MRI: nerve root enhancement
Treatment of GBS
- live threatening! hospital
- supportive care
- DVT prophylaxis
- repositioning
- bowel/bladder care
- pain: gabapentin, carbamazepine
Describe the autonomic dysfunction of GBS
- dysfunction of nerves that regulate HR, BP, sweating
- source of mortality
- occurs in 70%
- tachy, hypo/hypertensive, urinary retention, orthostatic hypotension
Describe the association of vaccines and GBS precautions
- usually no contraindication for routine vax
- may need avoidance of particular immunizations if received within 6 weeks of onset of GBS
GBS prognosis
- full motor recovery after 1 year in 60%, severe motor problems can persist
- less than 7% die despite ICU
- complications: resp distress, sepsis, PE, cardiac arrest
