neuromuscular

Question 1

differences in kids

Answer 1

immature, gross and fine motor development over first 2yrs of life, age specific milestones, intrinsic and extrinsic factors affect development, primitive reflexes at birth, protective reflexes over first yr of life.

Question 2

nursing interventions

Answer 2

promote skin integrity like frequent repositioning and assess skin under braces, maintain ventilation like diaphragm weakness and clear secretions, maintain nutrition with high cal meals and assess chewing and swallowing ability, prevent falls, emotional support

Question 3

cerebral palsy

Answer 3

nonprogressive, complex, permanent disorder. congenital or aquired. can be spastic, dyskinetic, ataxic, mixed. affects muscle tone and ability to move.

Question 4

sx of cerebral palsy

Answer 4

increased or decreased muscle tone, gross and fine motor delays, feeding difficulties, seizures, joint deformities. tx is supportive care with physical therapy.

Question 5

duchenne and becker

Answer 5

inherited muscular dystrophy, muscle wasting and progressive muscle weakness due to muscle fiber degeneration. affects dystrophin gene. sx are muscle weakness, toe walking, failure to meet motor milestones, gower sign, lordotic posture

Question 6

duchenne

Answer 6

absent or nonfunctional dystrophin gene, <males, onset age 3-5yrs, fast progression, nonambulatory age 12, life expectency mid 20’s, cardiomyopathy follows skeletal progression

Question 7

becker

Answer 7

partially functional dystrophin gene, less common, onset age 12yrs, slow progression, nonambulatory age by 27yrs, life expectancy is 40’s, cardiomyopathy present before skeletal sx.

Question 8

tx of duchenne or becker

Answer 8

glucocorticoids can delay loss of ambulation and preserver lung function. can cause osteoporosis and nutritional deficiencys.

Question 9

spinal muscular atrophy

Answer 9

loss of motor function through all muscles due to mutation in survival motor neurons. type one is most severe and see signs in infancy. genetic. degeneration of motor neurons in anterior horn cells of spinal cord.

Question 10

sx of spinal muscular atrophy

Answer 10

progressive symmetrical weakness, atrophy of proximal muscles leading to premature death. muscle weakness, hypotonia, rr compromise, inadequate weight gain, scoliosis, contractures

Question 11

spinal muscular atrophy tx

Answer 11

exercise, stretching, braces, orthotics, chest physiotherapy or noninvasive ventilation for pulmonary problems, scoliosis tx, enteral feedings, beta 2 adrenergic for breathing, rx for gi reflux, prophylactic abx, zolgensma IV to replace mutated SMN1 gene, spinraza in spinal cord for SMN protein production, evrysdi oral for SMN protein production.

Question 12

guillain barre

Answer 12

demyelination of nerve. triggered by bacterial or viral infection, autoimmune response resulting in attack of peripheral nervous system and deymeylination of peripheral nerves.

Question 13

sx of guillain barre

Answer 13

ascending hypotonia, numbness, pain, paresthesia, decreased deep tendon reflexes, wekaness, progresses bilaterally up from feet to chin. develops over days to 4wks.

Question 14

guillain barre tx

Answer 14

intubation and rr support if diaphragm impaired, IVIG for 5 days, plasmapheresis, pain mx, physical therapy, good nutrition, peripheral nerve and strength will return post tx.