Child health- Neurological conditions Flashcards
define anoxic seizures
a type of syncope
– clinical or electroclinical event resulting from
cessation of energy to most metabolically active cortical neurons
define syncope
= abrupt cessation of delivery of energy substrates to
cerebral cortex
* Reduction in perfusion, oxygenation or both
describe the characteristics of anoxic seizures
Characteristics
* Onset precipitated by noxious stimulus
* Pallor
* Floppy -> stiff
* Jerks
* May bite tongue
* May be incontinent of urine
* Relatively rapid recovery
define epilepsy and give diagnostic criteria
disease of the brain defined by any of the
following conditions:
- At least two unprovoked seizures occurring more
than 24 hours apart. - One unprovoked seizure and a probability of further
seizures similar to the general recurrence risk after
two unprovoked seizures (approximately 75% or
more). - At least two seizures in a setting of reflex epilepsy.
how common is epilepsy
1:200 - 1:100
evidence in reduction of incidence in UK, due to more accurate diagnosis avoiding misdiagnosis
what are the seizure types in epilepsy
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
describe how focal seizures present
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
describe how generalised seizures may present
*Absence – may include features of increase or loss of muscle tone,
jerks, etc
* Myoclonic
* Tonic, clonic, tonic-clonic
* Atonic
aetiological diagnosis of epilepsy
imaging eg MRI
genetics
pathology
which genes are linked to familial epilepsies eg benign familial neonatal convulsions
KCNQ2, KCNQ3
which specific genetic mechanisms are linked to Dravet
SCN1A mutation in Dravet AND in GEFS+
which genetic conditions are characterised by the presence of epilepsy
ARX
PDCH19
ring chromosome 20
which genetic conditions are complicated by epilepsy
Rett syndrome (MECP2, CDLK5 on the X chromosome)
Angelman syndrome
management options for epilepsy
drug therapy
surgery
service delivery
co-morbidity management
describe drug therapy in epilepsy management
- ~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
principles of drug therapy in epilepsy
single drug is desirable
careful attention to side effects
tailoring of drug therapy to epilepsy syndrome
drugs suppress- they do not cure
define refractory epilepsy
continued epileptic seizures despite a trial of two
appropriate anti-epileptic drugs given to tolerance
what can look like refractory epilepsy?
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
what is clinical management for refractory epilepsy?
reval hx and investx
review prev drug therapy
confirm compliance
THEN
consider next drug options
consider referral to paediatric neurologist
-possible surgery?
what are the education complications associated with epilepsy?
50% of children achieve below level expected by IQ
- attentional difficulties
- specific learning difficulties
1/6 have frank learning difficulties
what are causes of behavioural problems in epilepsy?
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
treatment for behavioural issues in epileptic children
careful assessment of possible causes of behaviour difficulties in each child
education of child/family
attention to family interactions
formal psychiatric intervention
what are the aims of treatment in epilepsy
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