LOTS to read --ABP Content Specs #9: Motor Disabilities Flashcards
(31 cards)
Spasticitiy?
-hypertonia elicited when muscles are rapidly and passively extended across a joint
-velocity-dependent increase in tone
-“catch and release” quality
-spasticity isn’t elicited when stretched slowly
S/s of spastic CP?
-spasticity
-hyperreflexia
-clonus
-positive Babinski reflex (extensor plantar response)
-diminished voluntary muscle control and strength
-often global hypotonia in early phase; have hypotonia in neck and trunk while spastic in extremities
-lesions in brain are large and extend beyond just the periventricular areas
Associated features of quadriplegic spastic CP?
-central hypotonia, spasticity in extremities
-seizures
-ID
-nonverbal
-difficulty feeding/swallow
-breathing issues
-contractures
*most severe type of spastic CP
*think term infants w/ history of profound HIE and extreme premature infants with grade IV bilateral IVH with subsequent PVL
Diplegic spastic CP?
-spasticity in all four limbs BUT lower extremities much more affected than upper extremities
-lesions in brain usually in periventricular white matter
-better outcomes than spastic quadriplegia
-not as common to have seizures or other medical issues
-good prognosis for cognitive skills
-might be able to ambulate if able to sit by themselves at age 2 years
*most often due to central brain bleed (e.g. IVH in premature infant with resultant PVL)
Hemiplegic spastic CP?
-motor issues on ONE side of body
-about 1/3 of CP cases
-brain: unilateral motor cortex and responsible for contralateral signs
-usually arm more than leg affected; mostly fine motor issues
-decorticate posturing of affected arm is often seen
-most kids can walk but some gait abnormalities (internal rotation of leg, toe walking on affected side)
-no effect on cognition
Dyskinetic CP (extrapyramidal)?
-involving areas of outside of motor pyramidal tracts
-occurs due to brain regions that regulate motor coordination and tone (e.g. cerebellum, basal ganglia, thalamus)
s/s of dyskinetic CP?
-variable tone
-hypertonia is reduced in times of sleep or relaxation
-motor coordination issues
-involuntary movements: seen in shoreoathetoid form of dyskinetic CP
chorea: quick, jerking movements
-athetosis: slow, writhing movements
-arms, trunk, and face mostly involved in abnormal movements
Peripheral nerve damage= neuropathy
-etiologies?
-due to nerve damage/injury, medications, systematic diseases like:
diabetes, vitamin deficiencies (Vitamin B1, B12, copper, or vitamin E and Vitamin B6 deficiency due to TB treatment with isoniazid)
-get motor sxs like weakness, impair coordination, sensory (tingle/numbness)
Meds for spasticity?
-oral meds:
1. baclofen, benzo (gaba agonists)
2. dantroline (calcium release inhibitor)
3. clonidine, tizanidine (alpha-2 adrenergic agonists)
4. gabapentin (increase gaba in brain)
side effects for meds for spasticity?
sedation, drowsy, weakness
-low evidence for oral meds
abrupt withdrawal of baclofen: pruritus, increase in spasticity, hallucinations, confusion, seizures
botox injections for spasticity in CP?
-used for neuromuscular blockade
-easy to admin, low risk sode effects, rapid onset of action
-interferes with relase of acetylcholine at NMJ
-lasts up to 3 months
-reduce spasticity and functional improvement in both upper and lower extremities
-also intrathecal injection of baclofen; gaba agonist with site of action at spinal cord
-given by continuous infusion pump
GMFCS (gross motor function classication system) for CP?
Level 1: walks without restrictions, limitations in advanced skills only
Level 2: walks without assistive devices, limited outdoors/community mobility
Level 3: walks with assistive mobility devices; limited outdoors/community mobility
Level 4: self-mobility with limitations; transported in wheelchair or use power mobility in outdoors/community
Level 5: self mobility is self limited, even with use of assistive technology
What is myelodysplasia?
-a developmental anomaly of the spinal cord
-high prevalence of hydrocephalus and Chiari malformation in children with myelodysplasia
hydrocephalus: excess build up of CSF in brain
a chiari malformation can block the normal flow of fluid and lead to obstructive or non-communicating hydrocephalus
hydrocephalus can occur with any type of chiari malformation but mostly with Type II
What is chiari type 1?
-when lower part of cerebellum (cerebellar tonsils) extend into foramen magnum
-usually only spinal cord passes through this opening and not the brain
-might not cause any sxs; most common type
What is chiari type II?
-more severe than type I
-usually start in childhood
-life-threatening complications
-called classic CM
-both the cerebellum and brain stem protrudes through foramen magnum
-usually accompanied by myleomeningocele; usualy get partial or complete paralysis of area below spinal opening
-usually see multiple ventricular anomalies (4th ventricle is small, aqueduct is small, third ventricle gives shark-tooth deformity, lateral ventricle can be normal or severely deformed/hydrocephalic)
What is chiari type III?
-most severe form of CM
-cerebellum and brain stem stick out or herniate through abnl opening in back of skull
-appears in infancy and life-threatening
-can have severe mental and physical delays, seizures
What is chiari Type 4?
incomplete or underdeveloped cerebellum (cerebellar hypoplasia)
-rare
-cerebellum is in normal position but parts of it was missing
What is chiari type 0?
-usually have syringomelia despite lack of cerebellar tonsil herniation
-have minimal or no herniation of cerebellar tonsils through foramen magnum
-get occipital HA
What prenatal meds increase risk of spina bifida?
valproic acid, carabamazepine, isotretinoin, methotrexate, folid acid antagonists, excess Vitamin A, retinoic acid
What are muscular dystrophies?
-inherited group of progressive myopathic disorders resulting from defects in a number of genes requires for normal muscle function
-primary sxs: muscle weakness
-9 major forms of muscular dystrophy
-
Duchenne muscular dystrophy feature?
X-linked recessive disorder affect skeletal nad cardiac muscle
-affects dystrophin (a glycoprotein structure of muscle sarcolemma)
-membrane instability leads to muscle damage; get inflammation and lead to muscle damage, necrosis and fibrosis
Progressive nature of Duchenne muscular dystrophy in boys?
-muscle affected at birth; see sxs of proximal muscle weakness at age 3-5 years
-proximal muscles happen first
-walk later than siblings at age 18 months
-toe walking common
-short stature
-awkard running, jumping, hopping or impossible
-develop lumbar lordosis and Trendelenburg fait
-fall often and difficulty getting up
-to raise themselves up will need to get into knee-elbow position and “climb up their legs”; doing Gower maneuver
-use wheelchair by age 8-12 years
-in mid-teens will lost ability to feed and care for themselves
-decreased lung function at age 9 -11 years
-calf muscle pseudohypertrophy
Behavioral/developmen issues in Duchenne muscular dystrophy?
-motor and lanugage delay
-1/4 of boys have Autism
-learning disabilities in 50%
-no progressive worsening of cognitive skills
-more risk for anxiety, ADHD, and OCD
Becker’s muscular dystrophy (BMD)?
-X-linked muscular dystrophy disorder
-due to mutation in DMD gene
-milder course
-manifests later in life; loss of ambulation in 30-40’s
-average cognition
-elevated serum CK levels 5x/normal
-will eventually have dilated cardiomyopathy
