Neurology - CP/Epilepsy

Question 1

What are the causes of cerebral palsy?

Answer 1

80% in antenatal origin due to vascular occlusion/cortical migration disorders/structural maldevelopment of the brain during gestation.
10% due to hypoxic ischaemic injury during delivery
10% are postnatal origin (preterm infants esp vulnerable - IVH, encephalopathy etc)

Question 2

What are the different types of cerebral palsy?

Answer 2

spastic cerebral palsy (90%)
dyskinetic cerebral palsy (6%)
ataxic (hypotonic) cerebral palsy (4%)

Question 3

Describe spastic CP

Answer 3

limb tone is increased due to UMN damage
associated with brisk reflexes
presents early

Question 4

What are the three categories of spastic CP?

Answer 4

Hemiplegia - unilateral involvement of the arm and leg involvement, may be due to neonatal stroke

Quadriplegia - all four limbs affected, often severely, poor head control and low central tone. Associated with seizures, microcephaly

Diplegia - affects legs > arms, likely to be preterm

Question 5

What is dyskinetic CP?

Answer 5

Choreic movements
Athetosis - slow writing distal movements
Dystonia - twisting appearance
Extrapyramidal system affected, used to be due to rhesus disease of the newborn

Question 6

What is ataxic CP?

Answer 6

Mostly genetic, may be acquired brain injury in cerebellum

• Trunk and limb hypotonia
• delayed motor development
• intention tremor

Question 7

What is craniosynostosis?

Answer 7

early fusion of cranial sutures - may cause raised ICP

Question 8

What are the features of CP?

Answer 8

• abnormal limb/trunk posture and tone, delayed motor milestones
• feeding difficulties
• abnormal gait
• asymmetric hand function before 1 year
• primitive reflexes persist

Question 9

What is cerebral palsy?

Answer 9

Abnormality of movement and posture, limiting activities, due to non-progressive disturbances in developing brain

May see disturbance of cognition, communication, perception, sensation, seizure, musculoskeletal problems

Affects 2/1000 live births

Question 10

How are children with CP treated?

Answer 10

Physiotherapy - to prevent muscle weakness and to prevent muscles getting stuck in a rigid position

Drugs - Diazepam (muscle relaxant but lots of SEs), botox injectionscan be used, finally baclofen intrathecal therapy can be used

Orthopaedic surgery - used to lengthen muscles which are causing problems

Question 11

What is epilepsy?

Answer 11

• incidence 0.05%
• recurrent unprovoked seizures associated with abnormal, excessive or synchronous neuronal activity
• mostly idiopathic causes

Question 12

What are generalised seizures?

Answer 12

• arise from both hemispheres
• loss of consciousness
• no warning
• symmetrical seizures
  bilateral synchronous EEG discharge

Question 13

What are the different types of generalized seizures?

Answer 13

absence 
myoclonic 
tonic 
tonic-clonic 
atonic

Question 14

What is an absence seizure?

Answer 14

• transient loss of consciousness with an abrupt onset and termination
• flickering eyelids, altered muscle tone
• typical = petit mal
• hyperventilation may proceed seizure

Question 15

What is a myoclonic seizure?

Answer 15

brief repetitive jerking limbs, neck or trunk

physiological appearance - hiccoughs or Stage II sleep

Question 16

What is a tonic seizure?

Answer 16

generalised increase in tone

Question 17

What is a tonic clonic seizure?

Answer 17

• rhythmic muscle group contraction after tonic phase
• may fall while rigid tonic, become cyanosed
• clonic = jerking of limbs
• cyanosis, irregular breathing, possible tongue biting and incontinence
• may last seconds to minutes
• followed by LoC or deep sleep

Question 18

What is an atonic seizure?

Answer 18

• often combined with myoclonic jerk and transient loss of muscle tone
• sudden fall, or drop of head

Question 19

What are focalised seizures?

Answer 19

• onset limited to one cerebral hemisphere
• relatively small group of dysfunctional neurones
• may see an aura dependent on origin
• may see change in consciousness

Question 20

What happened during a frontal focal seizure?

Answer 20

motor phenomena e.g. Jacksonian march, clonus, atony

Question 21

What happened during a temporal focal seizure?

Answer 21

auditory or sensory phenomena, autosomatisms, déjà vu

Question 22

What happened during a occipital focal seizure?

Answer 22

positive or negative visual phenomena

Question 23

What happened during a parietal focal seizure?

Answer 23

contralateral altered sensation (dysaethesia)

Question 24

What investigations should be carried out when epilepsy is suspected?

Answer 24

EEG - unless seizure is captured, an EEG only supports diagnosis

MRI/CT - assess brain structure if there are neurological signs between seizures or if focal seizures

Other - functional scans (PET/SPECT scan), metabolic and genetic studies

Question 25

What is the first and second line treatment for tonic clonic seizures?

Answer 25

1 - valproate, carbamazepine

2 - lamotrigine

Question 26

What is the first and second line treatment for absence seizures?

Answer 26

1 - valproate, ethosuximide

2 - lamotrigine

Question 27

What is the first and second line treatment for myoclonic seizures?

Answer 27

1 - valproate

2 - lamotrigine

Question 28

What is the first and second line treatment for focal seizures?

Answer 28

1 - carbamazepine, valproate

2 - topiramate

Question 29

What are the principles of epileptic treatment?

Answer 29

• anti-epileptic drugs can be used for treatment
• not all seizures require AED
• all AEDs have unwanted SEs
• Children with prolonged seizures are given rescue therapy (rectal/buccal diazepam)
• AED discontinued is seizure free for 2 years

Question 30

What are the SEs of valporate?

Answer 30

weight gain
hair loss
rarely liver failure
** teratogenic **

Question 31

What are the SEs of carbamazepine?

Answer 31

rash 
neutropenia
hyponatraemia 
ataxia 
liver enzyme induction

Question 32

What are the SEs of lamotrigine?

Answer 32

rash

Question 33

What are the SEs of ethosuximide?

Answer 33

nausea and vomiting

Question 34

What are the SEs of benzodiazepines

Answer 34

sedation

tolerance to effect and increased secretions

Question 35

What is SUDEP?

Answer 35

sudden unexpected death in epilepsy
most common in young adults who have generalised tonic-clonic seizures
RFs - poor seizure control and seizures occurring in sleep

Question 36

What are febrile convulsions?

Answer 36

Short, generalized epileptic seizure
With fever or systemic illness e.g. URTI
Otherwise normal
Do not cause brain damage

Question 37

What are the ddx for febrile convulsions?

Answer 37

epilepsy triggered by fever
CNS infection 
Rigors 
Syncope
Tetany 
Occulo-gyric crisis

Question 38

How common is a febrile convulsion?

Answer 38

aged 6 months to 5 years
often FHx
Common (3%)
30% recur

Question 39

What first aid should you tell parents about if there child has had a febrile convulsion?

Answer 39

recovery position
soft surface away from anything that could cause injury
stay with the child
call for help if lasts longer than 5 mins