Neuromuscular Disorders Flashcards
what is cerebral palsy
neuromuscular disorder with onset before 2-3 years of age due to an insult to the immature brain before, during or after birth
what MSK problems can cerebral palsy cause
joint contractures, scoliosis and hip dislocation
what is spastic cerebral palsy (80% of cases)
injury to the motor cortex, upper motor neurones or corticospinal tract resulting in weakness and spasticity which may worsen as child grows
what is ataxic cerebral palsy
effects cerebellum reduces co-ordination and balance
what is athetoid cerebral palsy
affecting the extrapyramidal motor system, the pyramidal tract and basal ganglia results in uncontrolled writhing motion, sudden changes in tone and difficulty controlling speech
what do the different kinds look like and what area of brain do they effect
what causes cerebral palsy
genetic problems brain malformation intrauterine infection in early pregnancy prematurity intra-cranial haemorrhage hypoxia during birth meningitis often there is no identifiable causes
what are the symptoms of cerebral palsy
variable = range from mild symptoms (one limb) to severe (whole body involvement with profound learning difficulties)
what are non surgical treatment options for cerebral palsy
physiotherapy and splintage to prevent contractures baclofen (injection intrathecally in subarachnoid space) reduces spasticity botox injection into spastic muscles
what are surgical treatment options in cerebral palsy
hip excision or replacement to treat dislocation and subsequent problem with wheelchair sitting release of joint contractures scoliosis correction joint fusions tendon transfers some cases can be helped by spinal procedure of selective dorsal rhizotomy which involves sectioning of overfiring motor nerve rootlets chosen by EMG but benefit not known
what is spina bifida
congenital disorder where the two halves of posterior vertebral arch fail to fuse, probably in first six weeks of gestation
where are the two forms of spinal bifida
mild = spinal bifida occult more severe = spinal bifida cystic
what is spina bifida occult
may be ok but can develop tethering of spinal cord and roots which can cause pes cavus and clawing of toes neurological symptoms occur at any age have dimple or tuft of hair in the skin overlying the defect
what is spina bifida cystic
content of vertebral canal herniate through defect with either herniation of the meninges alone (a meningocele) or with the spinal cord or cauda equina (myeleomeningocle)
what is more serious out of meningocele or myeleomeningocle
meningocele not usually associated with neurological sequelae but myeleomeningocle usually has neurological defect below the lesion and most never function independently
what may spina bifida cystic be associated with
hydrocephalus (excess CSF around brain raising intracranial pressure) and degree of disability depends on spinal level affected
what do different types of spinal bifida look like
what is treatment of spinal bifida cystic
defect is usually closed in 48 hours of birth hydrocephalus may be treated with a shunt from the ventricles of brain to either the RA, pleural cavity or peritoneal cavity at few weeks age
what is other management of spinal bifida
ortho = scoliosis correction, reduction and containment procedures from hip, contraction release and correction of foot derformities avoidance of pressure sores and good food care important
what is polio
viral infection which affects motor anterior horn cells in the spinal cord or brain stem resulting in a lower motor neurone deficit
why is polio not really important anymore
because vaccination has eradicated it however old patients with long term effects of childhood polio often encountered
what are symptoms of polio
virus enters GI tract with flu like illness and variable degree of paralysis affecting usually 1 limb by 2-3 days
what can occur as a result of polio
recovery of muscle weakness but residual paralysis can occur joint deformities and growth defects can occur with shortening of limb sensation preserved
how is polio treated
residual paralysis = splintage short leg = shoe raise or lengthening of short side / shortening of long side tendon transfer may improve function whilst some deformities esp of foot or ankle can be treated with arthrodesis to provide better platform for walking
