neuromuscular Disorders

Question 1

neuromuscular disorders aetiology

Answer 1

Due to abnormal or deficient motor neurone signals to skeletal muscle

Can develop due to
defect in
- brain
-spinal cord
-peripheral nerves
-NMJ
-Muscle 

Any defect which causes abnormal or deficient motor neurone signals to muscle

Question 2

Upper Motor neurone disease characteristics

Answer 2

Weakness
Spasticity
Hypereflexia

Extensor Plantar Response

• Using a blunt object to firmly stroke down sole of the foot
• normal response is flexion off toes
• Upper motor neurone disease extension of toes

Question 3

Lower motor. neurone disease characteristics

Answer 3

Weakness
Reduced tone
Areflexia
- hyporeflexia (reduced or absent responses)

Question 4

Cerebral Palsy

Answer 4

neuromuscular disorder caused by an insult to the immature brain before, during or after birth

Question 5

Cerebral palsy Onset

Answer 5

usually before aged 2-3

Question 6

Cerebral Palsy Aetiology

Answer 6

Genetic
brain malformation
Intra-uterine infection in early pregnancy
Prematurity
Intra-cranial haemorrhage
Hypoxia during birth 
meningitis

Question 7

Cerebral Palsy types (4)

Answer 7

Spastic
Ataxic
Athetoid
Hemiplegic

Question 8

Spastic Cerebral Palsy

Answer 8

Most common

Due to insult to motor cortex, UMNs or corticospinal tract

Question 9

Ataxic Cerebral Palsy

Answer 9

Due to insult to cerebellum

Decreased coordination and balance

Question 10

Athetoid Cerebral Palsy

Answer 10

Uncontrolled writhing motion

Sudden changes in tone

Difficulties controlling speech

Affects the extrapyramidal motor system, pyramidal tract and basal ganglia

Question 11

Hemiplegic Cerebral Palsy

Answer 11

Most common in regards to limbs affect

Affect ipsilateral upper and lower limbs

Question 12

Cerebral Palsy can lead to

Answer 12

Joint contractures
Scoliosis
hip dislocations

Question 13

Cerebral Palsy treatment

Answer 13

Physiotherapy

Splintage
- prevent contractures

Intrathecal Baclofen Injections
- Reduce spasticity

Botox injection into spastic muscles

Selective dorsal rhizotomy
- Surgical procedure involving sectioning over-firing motor nerve rootlets which are chosen by intra-operative EMG

Question 14

Spina Bifida

Answer 14

Congenital disorder where two halves of the vertebral arch fail to join

Question 15

Spina Bifida Types (2)

Answer 15

Spina Bifida Occulta

Spina Bifida Cystica

Question 16

Spina Bifida occulta

Answer 16

Mildest form

May have no associated problems

Spinal cord and roots may tether to vertebral defects causing pet caves and clawing of toes

Neurological issues can develop at any age

Dimple or tuft of hair in the skin overlying the defect may present

Question 17

Spina Bifida Cystica

Answer 17

Most severe form

Contents of vertebral canal can herniate through the defect

• Meningocele (can be the meninges alone)
• myelomeningocele (can include spinal cord and caudal equine. Neurological defects. below lesion are associated0

Question 18

Spina Bifida Associations.

Answer 18

Hydrocephalus

- Excess CSF around the brain causing raised intracranial pressure

Question 19

Spina Bifida Treatment

Answer 19

Defect closed within 48 hours of birth

Hydrocephalus treated with shunt from brain to either right atrium, pleural cavity or peritoneal cavity

Question 20

Polymyelitis (Polio)

Answer 20

Viral infection which affects motor anterior horn cells which can cause lower motor neurone issues

Question 21

Polymyelitis aetiology

Answer 21

Poliovirus

-Enters body through GI tract

Question 22

Polymyelitis Symptoms

Answer 22

Flu-like illness
Limb paralysis 
joint deformities
Growth Defects 
Shortening of limbs

Question 23

poliomyelitis Treatment

Answer 23

Fusion (arthrodesis) for joint deformities

Shoe raise for leg length discrepancy