2. Neuromuscular Disorders

Question 1

cerebal palsy

Answer 1

chronic disorders of posture and movement caused by non progressive CNS lesions sustained before 2 years old

resulting in delayed motor development, evolving CNS signs, learning disability and epilepsy

Question 2

cerebal palsy: causes

Answer 2

genetic problems

brain malformation

intrauterine infection

prematurity

intra cranial haemorrhage

hypoxia during birth

meningits

labour

idiopathic

Question 3

cerebal palsy: most common expression

Answer 3

spastic - 80%

injury to motor cortex, upper motor neurones or corticospinal tract resulting in weakness and spasticity

Question 4

cerebal palsy: ataxic

Answer 4

affects cerebellum

reduces coordination and balance

Question 5

cerebal palsy: athetoid

Answer 5

affects extrapyramidal motor system, pyramidal tract and basal ganglia

causes uncontrolled writhing motion, sudden changes in tone and difficulties controlling speech

Question 6

cerebal palsy: monogenic

Answer 6

affects one limb

Question 7

cerebal palsy:hemiplegic

Answer 7

affecting one ipsilateral upper and lower limb - most common

Question 8

cerebal palsy: diplegic

Answer 8

both legs only

Question 9

cerebal palsy: quadriplegic

Answer 9

all 4 limbs usually with learning disabilities

Question 10

cerebal palsy: developmental milestones

Answer 10

• may be delayed in children
• ATNR reflex persists past 6 months

Question 11

cerebal palsy: MSK problems

Answer 11

joint contracture, scoliois and hip dislocations

Question 12

cerebal palsy: non surgical treatment

Answer 12

physio

splintage to prevent contracture

baclofen and botox injection for spastic muscles

Question 13

cerebal palsy: surgical treatment

Answer 13

hip excision or replacement

surgical release of joint contracture

correction of severe scoliosis

joint fusion

tendon transfer

selective dorsal rhizotomy can help some cases of spasticity

Question 14

spasticity

Answer 14

• muscle continuously contracted
• spasticity is thought to be caused by an excessive increase of excitatory signals from sensory nerves without inhibition by GABA.

Question 15

joint/muscle contracture

Answer 15

permanent shortening due to prolonged hypertonic spasticity

Question 16

spina bifida:

Answer 16

congeital disorder where 2 halves of the pos vertebral arch fail to fuse

Question 17

mildest form of spina bifida

Answer 17

spina bifida occulta

Question 18

spina bifida occulta

Answer 18

may be no associated problems, but some can develop tethering of the spinal cord and roots which can cause a high arched foot (pes cavus) and clawing of the toes

neurological symptoms are not common but can occur at any age

Question 19

spina bifida occulta - tell tale sign

Answer 19

dimple/hair tuft overlying defect

Question 20

spina bifida: severe form

Answer 20

spina bifida cystica

Question 21

two types of spina bifida cystica

Answer 21

meningocele and myelomeningocele

Question 22

meningocele

Answer 22

contents of vertebral canal herniate through the defect with herniation of meninges alone

Question 23

myelomeningocele

Answer 23

contents of vertebral canal herniate through the defect with herniation of the spinal cord/cauda equina

neurological defect

Question 24

what is spina bifida cystica associated with in the brain

Answer 24

hydrocephalus -excess CSF around the brain raising intracranial pressure

Question 25

how may hydrocephalus be treated

Answer 25

shunt from the ventricles of the brain to either RA, pleural cavity or peritoneal cavity

Question 26

spina bifida - MSK features

Answer 26

spinal deformity and hip dislocation common contracture can occur

Question 27

polio what is it and where does the infection affect

Answer 27

(poliomyelitis) viral infection that affects motor anterior horn cells resulting in lower motor neuron deficit

Question 28

how common is polio now

Answer 28

vaccination has eradicated new cases in modern healthcare systems however older patients with long term effects from childhood cases are sometimes encountered

Question 29

how does polio present

Answer 29

flu prodrome for 48h pre-paralytic stage \<50% progress to paralytic stage - usually affecting muscles of one limb joint deformities and growth defects can occur with shortening of the limb

Question 30

how are deformities (eg of foot and ankle) and flail joints treated in polio

Answer 30

arthrodesis - surgical immobilisation of the joints by fusion of the bones

Question 31

how is residual paralysis in polio treated

Answer 31

splintage - caliper, orthotics