Neuromuscular Disorders

Question 1

what is cerebral palsy

Answer 1

neuromuscular disorder with onset before 2-3 years of age due to an insult to the immature brain before, during or after birth

Question 2

what MSK problems can cerebral palsy cause

Answer 2

joint contractures, scoliosis and hip dislocation

Question 3

what is spastic cerebral palsy (80% of cases)

Answer 3

injury to the motor cortex, upper motor neurones or corticospinal tract resulting in weakness and spasticity which may worsen as child grows

Question 4

what is ataxic cerebral palsy

Answer 4

effects cerebellum reduces co-ordination and balance

Question 5

what is athetoid cerebral palsy

Answer 5

affecting the extrapyramidal motor system, the pyramidal tract and basal ganglia results in uncontrolled writhing motion, sudden changes in tone and difficulty controlling speech

Question 6

what do the different kinds look like and what area of brain do they effect

Answer 6

Question 7

what causes cerebral palsy

Answer 7

genetic problems brain malformation intrauterine infection in early pregnancy prematurity intra-cranial haemorrhage hypoxia during birth meningitis often there is no identifiable causes

Question 8

what are the symptoms of cerebral palsy

Answer 8

variable = range from mild symptoms (one limb) to severe (whole body involvement with profound learning difficulties)

Question 9

what are non surgical treatment options for cerebral palsy

Answer 9

physiotherapy and splintage to prevent contractures baclofen (injection intrathecally in subarachnoid space) reduces spasticity botox injection into spastic muscles

Question 10

what are surgical treatment options in cerebral palsy

Answer 10

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

Question 11

what is spina bifida

Answer 11

congenital disorder where the two halves of posterior vertebral arch fail to fuse, probably in first six weeks of gestation

Question 12

where are the two forms of spinal bifida

Answer 12

mild = spinal bifida occult more severe = spinal bifida cystic

Question 13

what is spina bifida occult

Answer 13

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

Question 14

what is spina bifida cystic

Answer 14

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)

Question 15

what is more serious out of meningocele or myeleomeningocle

Answer 15

meningocele not usually associated with neurological sequelae but myeleomeningocle usually has neurological defect below the lesion and most never function independently

Question 16

what may spina bifida cystic be associated with

Answer 16

hydrocephalus (excess CSF around brain raising intracranial pressure) and degree of disability depends on spinal level affected

Question 17

what do different types of spinal bifida look like

Answer 17

Question 18

what is treatment of spinal bifida cystic

Answer 18

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

Question 19

what is other management of spinal bifida

Answer 19

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

Question 20

what is polio

Answer 20

viral infection which affects motor anterior horn cells in the spinal cord or brain stem resulting in a lower motor neurone deficit

Question 21

why is polio not really important anymore

Answer 21

because vaccination has eradicated it however old patients with long term effects of childhood polio often encountered

Question 22

what are symptoms of polio

Answer 22

virus enters GI tract with flu like illness and variable degree of paralysis affecting usually 1 limb by 2-3 days

Question 23

what can occur as a result of polio

Answer 23

recovery of muscle weakness but residual paralysis can occur joint deformities and growth defects can occur with shortening of limb sensation preserved

Question 24

how is polio treated

Answer 24

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