Child health- Neurological conditions

Question 1

define anoxic seizures

Answer 1

a type of syncope

– clinical or electroclinical event resulting from
cessation of energy to most metabolically active cortical neurons

Question 2

define syncope

Answer 2

= abrupt cessation of delivery of energy substrates to
cerebral cortex
* Reduction in perfusion, oxygenation or both

Question 3

describe the characteristics of anoxic seizures

Answer 3

Characteristics
* Onset precipitated by noxious stimulus
* Pallor
* Floppy -> stiff
* Jerks
* May bite tongue
* May be incontinent of urine
* Relatively rapid recovery

Question 4

define epilepsy and give diagnostic criteria

Answer 4

disease of the brain defined by any of the
following conditions:

1. At least two unprovoked seizures occurring more
   than 24 hours apart.
2. One unprovoked seizure and a probability of further
   seizures similar to the general recurrence risk after
   two unprovoked seizures (approximately 75% or
   more).
3. At least two seizures in a setting of reflex epilepsy.

Question 5

how common is epilepsy

Answer 5

1:200 - 1:100

evidence in reduction of incidence in UK, due to more accurate diagnosis avoiding misdiagnosis

Question 6

what are the seizure types in epilepsy

Answer 6

focal
motor, sensory, autonomic, psychic +/- automatisms +/- Generalized tonic–clonic seizure

generalised
-absence ± clonic/atonic/tonic/automatism
-atypical absence
-myoclonic
-clonic, tonic, tonic clonic
-atonic

unclassifiable

Question 7

describe how focal seizures present

Answer 7

Manifestations depend on the area of the brain affected
* Twitching, jerking or posturing if arise in the motor areas of the brain
* Visual phenomena if arise in the occipital lobes
* Abnormal smell or experiential symptoms if arise in temporal lobes

Question 8

describe how generalised seizures may present

Answer 8

*Absence – may include features of increase or loss of muscle tone,
jerks, etc
* Myoclonic
* Tonic, clonic, tonic-clonic
* Atonic

Question 9

aetiological diagnosis of epilepsy

Answer 9

imaging eg MRI

genetics

pathology

Question 10

which genes are linked to familial epilepsies eg benign familial neonatal convulsions

Answer 10

KCNQ2, KCNQ3

Question 11

which specific genetic mechanisms are linked to Dravet

Answer 11

SCN1A mutation in Dravet AND in GEFS+

Question 12

which genetic conditions are characterised by the presence of epilepsy

Answer 12

ARX
PDCH19
ring chromosome 20

Question 13

which genetic conditions are complicated by epilepsy

Answer 13

Rett syndrome (MECP2, CDLK5 on the X chromosome)

Angelman syndrome

Question 14

management options for epilepsy

Answer 14

drug therapy

surgery

service delivery

co-morbidity management

Question 15

describe drug therapy in epilepsy management

Answer 15

• ~70% of people with epilepsy will achieve seizure control with a
  single appropriate drug
• 10 - 20% will achieve reasonable control with 2 (or more) drugs
• 10 - 20% will not have adequate seizure control

Question 16

principles of drug therapy in epilepsy

Answer 16

single drug is desirable

careful attention to side effects

tailoring of drug therapy to epilepsy syndrome

drugs suppress- they do not cure

Question 17

define refractory epilepsy

Answer 17

continued epileptic seizures despite a trial of two
appropriate anti-epileptic drugs given to tolerance

Question 18

what can look like refractory epilepsy?

Answer 18

medical issues which are unresponsive to AED

wrong choice of AED

mis diagnosis of ES

inappropriate drug dosage

patient related issues eg. non compliance
lifestyle issues

Question 19

what is clinical management for refractory epilepsy?

Answer 19

reval hx and investx

review prev drug therapy

confirm compliance

THEN

consider next drug options

consider referral to paediatric neurologist
-possible surgery?

Question 20

what are the education complications associated with epilepsy?

Answer 20

50% of children achieve below level expected by IQ
- attentional difficulties
- specific learning difficulties

1/6 have frank learning difficulties

Question 21

what are causes of behavioural problems in epilepsy?

Answer 21

neurobiological
-non epileptic factors
- effects of tx
- direct epileptic mechanisms
- effects of epileptic activity on the brain

external factors
- stigma
- restrictions due to seizure unpredictability

Question 22

treatment for behavioural issues in epileptic children

Answer 22

careful assessment of possible causes of behaviour difficulties in each child

education of child/family

attention to family interactions

formal psychiatric intervention

Question 23

what are the aims of treatment in epilepsy

Answer 23

maximise seizure control

active Tx for interictal abnormalities in Landau Kleffner

avoidance of known AED precipitants

low threshold for AED changes

Avoiding polypharmacy

role of early surgery