2. Neuromuscular Disorders Flashcards
cerebal palsy
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
cerebal palsy: causes
genetic problems
brain malformation
intrauterine infection
prematurity
intra cranial haemorrhage
hypoxia during birth
meningits
labour
idiopathic
cerebal palsy: most common expression
spastic - 80%
injury to motor cortex, upper motor neurones or corticospinal tract resulting in weakness and spasticity
cerebal palsy: ataxic
affects cerebellum
reduces coordination and balance
cerebal palsy: athetoid
affects extrapyramidal motor system, pyramidal tract and basal ganglia
causes uncontrolled writhing motion, sudden changes in tone and difficulties controlling speech
cerebal palsy: monogenic
affects one limb
cerebal palsy:hemiplegic
affecting one ipsilateral upper and lower limb - most common
cerebal palsy: diplegic
both legs only
cerebal palsy: quadriplegic
all 4 limbs usually with learning disabilities
cerebal palsy: developmental milestones
- may be delayed in children
- ATNR reflex persists past 6 months
cerebal palsy: MSK problems
joint contracture, scoliois and hip dislocations
cerebal palsy: non surgical treatment
physio
splintage to prevent contracture
baclofen and botox injection for spastic muscles
cerebal palsy: surgical treatment
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
spasticity
- muscle continuously contracted
- spasticity is thought to be caused by an excessive increase of excitatory signals from sensory nerves without inhibition by GABA.
joint/muscle contracture
permanent shortening due to prolonged hypertonic spasticity
spina bifida:
congeital disorder where 2 halves of the pos vertebral arch fail to fuse
mildest form of spina bifida
spina bifida occulta
spina bifida occulta
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
spina bifida occulta - tell tale sign
dimple/hair tuft overlying defect
spina bifida: severe form
spina bifida cystica
two types of spina bifida cystica
meningocele and myelomeningocele
meningocele
contents of vertebral canal herniate through the defect with herniation of meninges alone
myelomeningocele
contents of vertebral canal herniate through the defect with herniation of the spinal cord/cauda equina
neurological defect
what is spina bifida cystica associated with in the brain
hydrocephalus -excess CSF around the brain raising intracranial pressure
how may hydrocephalus be treated
shunt from the ventricles of the brain to either RA, pleural cavity or peritoneal cavity
spina bifida - MSK features
spinal deformity and hip dislocation common
contracture can occur
polio
what is it and where does the infection affect
(poliomyelitis)
viral infection that affects motor anterior horn cells resulting in lower motor neuron deficit
how common is polio now
vaccination has eradicated new cases in modern healthcare systems however older patients with long term effects from childhood cases are sometimes encountered
how does polio present
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
how are deformities (eg of foot and ankle) and flail joints treated in polio
arthrodesis - surgical immobilisation of the joints by fusion of the bones
how is residual paralysis in polio treated
splintage - caliper, orthotics
