Paediatric syndromes

Question 1

What is cerebral palsy?

Answer 1

• non progressive defect/lesion in immature brain
• occurs inutero, perinatally or within 2-3 years postnatal
• produces sensory and motor deficits evident in early infancy
• Lesion causing cerebral palsy in non progressive, but resultant deformities can be progressive

Question 2

What are the classifications of CP?

Answer 2

• Spastic, most common
• Dyskinetic
• Ataxic
• Mixed

Question 3

What is usually present with spastic CP?

Answer 3

• hypertonia
• rigidity
• tendon reflexes exaggerated
• clonus

Question 4

What is the multi D intervention?

Answer 4

• paediatrician
• Orthopod
• OT
• Physio
• Exercise physiologist
• Pod/orthotist

Question 5

What is the prognosis of

CP?

Answer 5

predictors of life expectancy
-lack of mobility
-eating and drinking difficulty
Predictors of ambylation
-sitting by 2 years
type other than quad
-tonic neck reflexes
-achievement by 8 years

Question 6

What is CMT?

Answer 6

• degeneration of nerve fibers leading to muscle weakness and nephropathy
• variable presentations

Question 7

CMT type 1

Answer 7

• most common form

- abnormality of the myelin sheath

Question 8

Signs and symptoms of CMT?

Answer 8

• fatigue
• tripping or clumsiness
• lack of agility
• circulation and sensory dysfunction LL
• muscle atrophy
• per cavus, lateral ankle instability
• scoliosis/kyphosis
• hammertoes

Question 9

what is the prognosis of CMT?

Answer 9

• not life limting but progressive
• some may develop tremor
• management of symptoms require a multi D approach

Question 10

What is muscular dystrophy?

Answer 10

• abnormalities in muscle biopsy
• 30 different types
• Duchenne is most severe

Question 11

What is the progression of symptoms in duchenne muscular dystrophy?

Answer 11

-symptoms begin 12-18 months
-weakness of pelvic areas then shoulders
-gowers sign
-increased base of gait
-AFO/leg braces needed by 9 years of age
lift threatening due to weakness of cardiac system

Question 12

When to consider MD or DMD?

Answer 12

• Delay or regression of walking/running
• Family history
• Abnormal reflexes
• Gowers Sign

Question 13

What is the management of MD and DMD?

Answer 13

• medication
• physcial activity
• Steroids
• Physiotherapy
• Surgery
• AFO
• mobility aids