Nervous system

Question 1

What is the most common form of cerebral palsy and what are its features?

Answer 1

Spastic (~70%).
Affects limbs, increased tone (clasp-knife), brisk reflexes, extensor plantar responses.
Can be hemiplegic (one side affected), diplegic (one half e.g. lower body affected), quadriplegic or monoplegic

Question 2

What is cerebral palsy?

Answer 2

The permanent disorder of motor or posture development caused by non-progressive insult to the developing brain. (the insult can occur, antenatally/intrapartum/postnatally).

Question 3

What is the name of the classification system for severity of gross motor impairment?

Answer 3

Gross Motor Function Classification System (GMFCS)
Class 1 (Mildest) to class 5 (most pronounced)

Question 4

What are the types of cerebral palsy?

Answer 4

Spastic - most common
Athetoid - hyperkinesia, “stormy movement”
Ataxic - loss of orderly muscle co-ordination
Mixed

Question 5

How is CP managed?

Answer 5

MDT approach. Splints, mobility aids.
Medical: Baclofen can help with the spasticity (beware withdrawal Sx from baclofen). Botox - paralyse muscles.
Orthopaedic surgery: Selective dorsal rhizotomy

Question 6

Between what ages do febrile fits most commonly occur?

Answer 6

6 months - 6 years

Question 7

Roughly what percentage of children get febrile fits?

Answer 7

3%

Question 8

What features of a febrile fit make it atypical?

Answer 8

Longer than 15 mins
More than one in the same illness
Focal neurology

Question 9

What is the recurrence rate of simple febrile convulsions?

Answer 9

~33%

Question 10

What are some risk factors for developing epilepsy?

Answer 10

Atypical febrile seizure, abnormal/focal neurology or neurodevelopment, positive family history

Question 11

What is the risk of developing epilepsy following a simple febrile convulsion with no risk factors?

Answer 11

1% (same as background risk)

Question 12

What is the risk of developing epilepsy following a febrile convulsion with one risk factors?

Answer 12

2%

Question 13

What is the risk of developing epilepsy following a febrile convulsion with two or more risk factors?

Answer 13

10%

Question 14

What can trigger a typical absence seizure?

Answer 14

Hyperventilation

Question 15

What are the features of juvenile myoclonic epilepsy?

Answer 15

Onset 8-26 years. More common in females. Get tonic-clonic seizures provoked by sleep deprivation, absence seizures, early morning myoclonic jerks of upper limbs. Flashing lights triggers attack in 40%.

Question 16

What is the treatment of juvenile myoclonic epilepsy?

Answer 16

Lifelong anti-epileptic medication.

Question 17

What are the features of benign epilepsy with centro-temporal spikes aka Benign Rolandic epilepsy?

Answer 17

Onset 3-12 years. Peak 9 years. Get unilateral facial parasthesia with ipsilateral facial motor seizure. No LOC but often unable to speak and salivation. Last 1-2 mins. Seizures often at night.
Usually self-resolves by 13

Question 18

What triad is seen in West Syndrome?

Answer 18

Seizures (often infantile spasms), motor regression, Typical EEG pattern (hypsarrhythmia).

Question 19

What is the treatment for West syndrome?

Answer 19

Steroids, vigabatrin, ACTH

Question 20

What percentage of children with West syndrome have developmental delay and persistent seizures?

Answer 20

50%

Question 21

What age group tends to be affected by infantile spasms seen in West syndrome?

Answer 21

1-6 month olds

Question 22

What are the features of tension-type headache?

Answer 22

Tight-band like pain, symmetrical. Gradual onset. Related to stress. May feel tense in muscles around back of head and neck

Question 23

What are the features of migraine without aura?

Answer 23

Lasts 1-72 hours. Commonly bilateral but may be unilateral. Pulsatile. GI Sx may co-occur. May be aggravated by physical activity and may be photo- and phonophobia

Question 24

What are the features of migraine with aura?

Answer 24

Frequent presence of premonitory symptoms. Usually lasts a few hours.

Question 25

What are some red flag features of a headache?

Answer 25

Worse when lying down or with coughing/straining. Headache wakes the child. Associated confusion or persistent N+V. Change in personality/behaviour/educational performance

Question 26

What is plagiocephaly?

Answer 26

Parallelogram shaped head due to pressure on the skull. Improves with age and mobility

Question 27

What is the inheritance pattern on Duchenne’s Muscular Dystrophy?

Answer 27

X-linked recessive disorder.

Abnormal/absent dystrophin protein

Question 28

What condition is Gower’s sign seen in?

Answer 28

Duchenne’s Muscular Dystrophy

Question 29

By what age do boys with DMD tend to no longer be ambulant due to progressive muscular atrophy?

Answer 29

10-14

Question 30

What are the forms of spina bifida?

Answer 30

Spina bifida occulta - overlying skin lesion (tuft of hair/lipoma/birthmark/small dermal sinus) in lumbar region, there may be underlying cord tethering which with growth may cause bladder dysfunction
Meningomyelocele (most common and severe form) - paralysed legs with sensory loss below level of lesion. Hip dislocation and leg deformities common. Hydrocephalus. Neuropathic bladder and bowel
Meningocele - Rare and less serious. Swelling over back is covered by skin and contains no neural tissue