CP Long Case

Question 1

Spastic diplegia most common aetiology?

Answer 1

Periventricular leukomalacia secondary to prematurity/IVH

Question 2

Spastic hemiplegia most common aetiology?

Answer 2

Neonatal stroke
Placental infarction
Tight nuchal cord

Question 3

RFs for PVL/resultant spastic diplegic CP?

Answer 3

Chorioamnionitis
Very low birthweight or <32/40
Coag abnormalities, hypoxia, neonatal transport, CPR, mechanical ventilation
Intrapartum asphyxia

Protective:
Antenatal steroid therapy, term infant

Question 4

CP ddx/mimics?

Answer 4

Metabolic
 - Glutaric acidaemia type 1 mimics dyskinetic CP, can be treated with low protein diet and riboflavin & L-carnitine supps
Muscular dystrophies
 - Usually degenerate with time
Mitochondrial disorders
 - Developmental regression 
Malformation syndromes
 - Miller-Dieker lissencephaly or Rett

Question 5

GMFCS?

Answer 5

1 ambulatory in all settings
2 walks without his but has limitations in community setting
3 walks with aids
4 requires wheelchair or adult assist
5 fully dependant for mobility

Question 6

MACS (manual ability classification)?

Answer 6

1 handles objects with ease
2 Handles most objects, some limitations with speed
3 Handles with difficulty, needs set up assist or modelling
4 limited handling only with mods
5 cannot handle objects, severely limited

Question 7

CFCS (communication function classification)?

Answer 7

1 effective receptive and expressive with all
2 effective but slow expressive or receptive
3 effective expressive and receptive only with familiar
4 inconsistent expressive and receptive with familiar
5 seldom effective expressive/receptive even with familiar

Question 8

CP comorbidities from most to least common?

Answer 8

Intellectual impairment
Epilepsy
Speech & language disorders - more common in children with R hemiplegia due to left hemisphere pathology
Ophthalmological deficits
Hearing deficit

Question 9

Most important dev milestones for assessment of CP development?

Answer 9

Rolling
Sit with, then without, support
Crawling
Cruising

Question 10

Investigations relevant to CP?

Answer 10

MRI B - gross malformations, cortical dysplasia (ink 12-20 wks gest), PVL (injury 24-34 weeks gest), watershed atrophy (intrapartum hypoxia), degenerative disorders such as leukodystrophy
TORCH screen
Urine metabolic screen
Chromosome array
LP for exclusion of glut 1 transport deficiency in dyskinetic CP (responsive to keto diet)

Question 11

General paed/nursing care issues to fall back on in CP long case?

Answer 11

Bowels & bladder - incontinence management, constipation management, UTIs
Feeding - PO/NG/PEG, nutritional ax, growth ax
Skin care - pressure sores
Aspiration risk in setting of feeds & saliva

Question 12

CP management - PT/OT/ortho

Answer 12

EARLY INTERVENTION important in maximising physical potential & facilitating ADL capacity
PT/OT ax for therapeutic home prescription exercises built into activities
OT ax for home mods/equipment
Orthoses for contractures/splinting for normal muscle development
Gait lab multidisciplinary team (must be mobile and >4yrs old) ?specific target for intervention to improve efficiency of movement

Question 13

Spasticity management in CP?

Answer 13

Localised/segmental hypertonicity without fixed deformity - Botox
Baclofen - PO or IT
Consider selective dorsal rhizotomy

Question 14

Gastrointestinal management in CP?

Answer 14

GIT mobility - slow gastric emptying (erythromicin, continuous feeds), constipation (aperients, toileting habits)
GORD
Aspiration - consider fundoplication/PEJ

Question 15

Resp management in CP?

Answer 15

Aspiration pneumonia - speech ax/swallow study, positioning, feed thickness, fundo, PEJ
Sleep disordered breathing - overnight oximetry/polysomnogram
Upper airway obstruction/OSA - CPAP/BiPAP, T&As
Restrictive lung disease/scoliosis

Question 16

Sialorrhoea management?

Answer 16

Anterior - mouth breathing corrected w T&As, bibs, encouraging swallowing
Posterior - noted aspiration risk
Anticholinergics (glyco) SEs - urinary retention, blurred vision, headache/dizziness
Transplant or excision of salivary glands

Question 17

Dysarthria/communication management?

Answer 17

Speech therapy
Communication boards
Signing
Facial expressions/eye contact/vocalisations/other movements for communication

Question 18

Sleep management?

Answer 18

OSA - T&As, CPAP/BiPAP

Abnormal sleep pattern - melatonin

Question 19

Walking prognosis in CP?

Answer 19

If hemiplegic or diplegic will usually walk, dyskinetic CP harder to predict.
If can sit independently by 2 can usually walk
If cannot sit by 4yrs unlikely to walk