1: Neurology + Development - Down's Syndrome, Cerebral Palsy, Status Epilepticus, Febrile Convlusions, Seizures, Seizure Disorders Flashcards
1
Q
What is downs syndrome
A
Where there are three copies of chromosome 21 (Trisomy 21)
2
Q
What is the most common chromosomal aberration
A
Downs Syndrome
3
Q
What is the main risk factor for Down’s syndrome
A
Maternal Age
4
Q
What is the risk of DS at maternal age 20y
A
1:1500
5
Q
What is the risk of DS at maternal age 30y
A
1:800
6
Q
What is the risk of DS at maternal age 40y
A
1:100
7
Q
What is the risk of DS at maternal age 45y
A
1:50
8
Q
What is the risk of DS at maternal age 50y
A
1:6
9
Q
What is the most common cause of trisomy 21
A
Meiosis Non-Dysfunction
10
Q
What is non-dysfunction
A
Failure of homologous chromosomes to separate during meiosis
11
Q
Where does the majority of non-dysfunction occur
A
During meiosis I
12
Q
What is the risk of recurrence in future pregnancies if DS is due to non-dysjunction
A
<1:100 if <35
13
Q
What is a balanced robertsonian translocation
A
- Where the long arm of chromosome 14 combines with long arm of chromosome 21. And, short-arm is deleted
- Individuals possess only 45 chromosomes but have all the genetic material to give a normal phenotype
14
Q
What is an unbalanced translocation
A
- Where individuals inherited one ‘balanced translocated’ chromosome and one normal chromosome
- This gives three copies of chromosome 46XY ± 21 ± t(21:14)
15
Q
What % of DS is due to unbalanced translocation
A
5
16
Q
What is mosaicism
A
Where there are two cell lines present. In DS, will be one normal cell line and one DS cell line. Phenotype depends on balance.
17
Q
What are two risk factors for DS
A
- Maternal age
- First-degree relative with DS
18
Q
How will a foetus with DS present on 10-13+6W scan
A
Raised nuchal translucency
19
Q
What are the 4 heart structural abnormalities seen in DS and their frequency
A
- AVSD (40%)
- VSD (30%)
- TOF (5%)
- Patent ductus arteriosus (5%)
20
Q
What are the 2 GI tract abnormalities seen on anomaly scan in foetus with DS
A
- Hyperechogenic bowel
- Duodenal atresia
21
Q
What are the craniofacial dysmorphic features of a child with DS
A
Eyes:
Upward slanting
Large epicanthic folds
Brushfield spots
Nose:
Broad nose
Ears: Small round low-set ears
Mouth: small oral cavity, high arched palate
22
Q
What are brushfield spots
A
aggregation of connective tissue in the peripheral iris
23
Q
In the extremities, what are 5 features of DS
A
- Single palmar crease
- Sandal gap
- Increase risk inguinal hernia
- Increase risk umbilical hernia
- Atlanto-axial instability
24
Q
What is the sandal gap
A
Increased gap between big toe and second toe
25
What are two GI tract features seen in neonates with DS
Hirschsprungs
| Imperforate anus
26
What is the GU features seen in neonates with DS
Cryptochidism
27
What are the aesthetic features of an older child with DS
Short stature
| Obese
28
What are cognitive features of a child with DS
Lower IQ
29
What are the haematological symptoms of a child with DS
ALL
30
What are the endocrine features in DS
Hypothyroidism
31
What are the GU features in an adult with DS
Subfertility in females
| Infertility in males
32
What are the respiratory features of someone with DS
- Recurrent infections and hearing impairment: glue ear
| - OSA
33
What are the long-term neurological symptoms of someone with DS
Increased risk of pre-mature Alzheimer's disease
34
What are the neonatal developmental features of a baby with DS
- Hypotonia
| - Developmental delay
35
What are the two main types of screening test used for DS
1. Combined Test
| 2. Quadruple test
36
When is the combined test performed
10-13W+6
37
When is the quadruple test performed
15-20W
38
What are the four components of the combined test
1. PAPP-A
2. Nuchal Translucency
3. serum bHCG
4. Maternal Age
39
What do low levels of PAPP-A reflect
Poor Placentation
40
What is a low level of PAPP-A
<0.4
41
If a monochorionic multiple pregnancy how is the risk of DS calculated
Per pregnancy
42
If a dichorionic multiple pregnancy how is the risk of DS calculate
Per foetus
43
Explain what will happen at the end of screening
Mum is given a score of 'high risk >1:150' or low risk '<1:150'.
44
What are the two non-invasive pre-natal testing methods for DS
- Amniocentesis
| - Chorionic Villus Sampling
45
What is SAFE
Checking karyotype of foetal blood cells in maternal blood
46
If SAFE test is positive what does this mean
It is still a screening test and would indicate need for diagnostic test
47
Who is the IONA test offered to for free
If high-risk of DS. More than 1:150.
48
If IONA test is positive what does this mean
It is still a screening test and would indicate need for diagnostic test
49
What are the two methods of diagnostic testing for DS
1. Chorionic Villus Sampling
| 2. Amniocentesis
50
What gestation can chorionic villus sampling be performed
First trimester.
| 10-13W
51
Explain the procedure of CVS
Needle placed trans-abdominally to take trans-placental sample
52
What is performed on sample from CVS
- Karyotype (2d)
| - FISH (3W)
53
What is the advantage of CVS
Within termination limits
54
What is the disadvantage of CVS
- 2%. miscarriage risk
- Infection risk
- Placental mosaicism
55
When can amniocentesis be performed
Second trimster 15-20W
56
Explain the procedure for amniocentesis
Needle placed through abdomen to take sample from baby.
57
What is performed on amniocentesis
- Karyotype (3W)
| - FISH and PCR (3d)
58
What is the advantage of amniocentesis
Miscarriage rate 1% - lower than CVS
59
What is the disadvantage of amniocentesis
Miscarriage rate (1%)
60
What 4 post-natal tests re recommended for DS baby
1. Karyotype
2. ECHO
3. Hip US
4. Audiology
61
What is the average prognosis of DS
55y
62
What do the majority of people with DS develop
early-onset Alzhiemer's disease
63
What is the aim of treatment for DS
Supportive. No curative treatment.
64
What should be offered to DS for management
- Paediatrician follow-up
| - Physio to improve tone
65
What follow-up is recommended in DS patients
- Annual TFTs
| - Audiology and Ophthalmology every 1-2Y
66
What is cerebral palsy
Chronic neurodevelopment disorder that presents with abnormalities in movement and posture that start early and continue through life
67
How can the aetiology of cerebral palsy be divided
Cerebral palsy is caused by repeated brain insult prior to 2-years. Its aetiology can be divided into antenatal, perinatal and postnatal.
68
What percentage of cerebral palsy is antenatal in origin
80%
69
What are three causes of antenatal cerebral palsy
1. TORCH Infections
2. Cerebral malformation
3. Placental Insufficiency
70
What are the TORCH infections
```
Toxoplasmosis
'Other'
Rubella
CMV
Herpes
```
71
What percentage of cerebral palsy is peri-natal in origin
10%
72
What causes peri-natal cerebral palsy
Birth asphyxia - resulting in hypoxic-ischaemic injury and encephalopathy
73
What percentage of cerebral palsy is post-natal in origin
10%
74
What causes post-natal cerebral palsy
- IV haemorrhage
- Trauma
- Kernicterus
- Meningitis
75
What are two risk factors for cerebral palsy
Low birth weight
| Premature!
76
In which population is the incidence of cerebral palsy higher
Those born 22-27W
77
How is cerebral palsy categorised
It is categorised by the type of movements present into two main categories:
1. Spastic CP
2. Non-Spastic CP
78
What are the two categories of cerebral palsy
1. Spastic Cerebral Palsy
| 2. Non-Spastic Cerebral Palsy
79
What percentage of cerebral palsy is spastic
70%
80
What percentage of cerebral palsy is non-spastic
30%
81
What are the two-types of non-spastic cerebral palsy
1. Ataxic
| 2. Dyskinetic
82
What is dyskinetic non-spastic cerebral palsy
Presence of involuntary movements. Including choreoarthroid movements and dystonia
83
How does ataxic cerebral palsy present
Intention tremor
Poor balance
Poor co-ordination
84
How will individuals with spastic cerebral palsy present in general
- Hypotonic at birth until 6-12 months when they become spastic
85
what reflexes may remain in spastic cerebral palsy
Primitive reflexes remain
| Upgoing Plantar Reflex
86
what type of gait may be present in cerebral palsy
- Tip toe walking
| - Scissoring gait due to weakness in abductors
87
What are three complications of spastic cerebral palsy
1. Hip dislocation
2. Scoliosis
3. Hearing and visual impairment
88
What are the three types of spastic cerebral palsy
1. Hemiplegic
2. Quadriplegic
3. Diplegic
89
What is hemiplegic CP
Weakness in arm and leg on the same side
90
Are the arms of legs affected more in hemiplegic CP
Arms
91
How will the arms appear in hemiplegic spastic CP
- Hands in fists
- Pronated
- Hand preference before 12m
92
What is a classic indictor of hemiplegic CP and why
Hand preference before 12m. In hemiplegic CP, one side will be weak causing the infant to use the other side giving hand preference
93
How will legs present in hemiplegic CP
Tip toe walk
94
What is diplegic CP
When all four limbs are affected
95
Are legs or arms affected more in diplegic CP
Legs
96
What is quadriplegic CP
When arms and legs are affected
97
How will quadriplegic CP present early-on
Poor head control!!
98
What conditions is quadriplegic CP associated with
- Microcephaly
- Intellectual impairment
- Epilepsy
99
Explain abnormal involuntary movements in non-spastic CP
Worsen with stress and improve with sleep
100
What percentage of CP is dyskinetic
10%
101
How will tone present in dyskinetic CP
fluctuating tone leading to abnormal movements
102
What are three types of movement in dyskinetic CP
- Dystonia
- Chorea
- Athetosis
103
What is chorea
Non-Repetitive, sudden movements
104
What is dystonia
Contraction of agonist and antagonist muscles of the proximal limb
105
What is athetosis
Contraction of agonist and antagonist muscles of the distal limb
106
What percentage of CP is ataxic hypotonic
10%
107
How does ataxic hypotonic CP present
```
Ataxia
Intention tremor
Hypotonia
Poor balance
Un co-ordination
```
108
What are 7 problems associated with CP
- Epilepsy
- Intellectual disability
- Developmental motor delay
- ADHD
- Squint
- Feeding difficultly
- Joint contractures
109
Which type of CP is more associated with intellectual disability
Spastic
110
Why will children with CP struggle with feeding
Struggle with oro-motor co-ordination leading to gagging
111
What causes spastic CP
Damage to pyramidal tract. Damages GABAergic neurons, leading to hypertonia.
112
What causes dyskinetic CP
Damage to basal ganglia which controls initiation of movement. Disinhibiting movement
113
What causes ataxic CP
Damage to cerebellum - resulting in ataxia.
114
What is the term cerebral palsy used to describe
CP is used to describe the disability not the cause - therefore need to find underlying neurological insult.
115
What causes cerebral palsy
repetitive neurological insult before 2-years. Therefore investigation focuses on finding sources of insult
116
What is first-step in investigating CP
Detailed history to try and identify episodes of birth asphyxia and HIE
117
What imaging is used to investigate cause of CP in neonates
Cranial US
118
What can cranial US identify
HIE and structural abnormalities
119
What imaging is used in older children to identify CP
MRI
120
Explain aims of treatment in CP
Treatment is not curative - but aims to relieve symptoms
121
What is used to manage posture and movement in CP
Posture + Movement:
- Exercises
- Orthoses
- Wheelchair
- Management of spasticity
122
What can be used to treat spasticity in CP
- Botox of gastrocnemius = prevents toe walking
- Oral diazepam
- Oral/Intrathecal Baclofen
123
What is used to manage communication in CP
SALT referral
124
What is used to manage congnition in CP
Tailored Educational Program
125
Why is CP classified as non-progressive
As it does not get worse
126
Define status epileptics
- Seizure persisting beyond 30-minutes
| - Or, recurrent seizures with no return to baseline between
127
What are 5 causes of status epileptics
1. SOL
2. Epilepsy
3. Metabolite disturbances
4. CNS Infection
5. Febrile convulsion
6. Head Injury
128
What is a risk factor for status epilepticus
Seizures persisting beyond 5 minutes
129
Why is status epileptics protocol started after 5-minutes
Seizures beyond 5 minutes have a significant risk of transforming into status epilepticus. Therefore risk is avoided by introducing treatment early.
130
How can status epilepticus be divided
- Convulsive
| - Non-Convulsive
131
How is non-convulsive status epilepticus identified
EEG
132
What investigations are performed for status epilepticus
- Blood Glucose
- U+Es
- CRP/ESR
- FBC
- Toxicology screen
- CT
133
When should a CT scan be considered
Focal neurological signs or symptoms. Head trauma
134
How should status epilepticus be managed in general
A-E approach (Emergency)
135
How is status epilepticus managed in 0-5m
- ACTION - for seizure management.
- Call For Help
- IV Access
- 100% Oxygen
- Monitor glucose
136
How is status epilepticus managed in 5-15m
IV Lorazepam OR Rectal Diazepam
137
When is IV lorazepam given
If IV access obtained
138
What should be done at 15-20m if individual is still in status
Repeat dose of IV lorazepam or rectal diazepam (up to maximum of 4mg for lorazepam. Or, 10 for diazepam)
139
What is maximum dose of IV lorazepam
4mg
140
What is maximum dose of rectal diazepam
10mg
141
What medication is given 15-35 minutes if still in status
IV phenytoin
142
If individual is normally on phenytoin, but still not responding to lorazepam or diazepam, what should be given as an alternative
IV Phenobarbitone
143
If status epilepticus has not responded to lorazepam or second-line what is it called
Refractory
144
What should be done for refractory seizures
Contact ITU for admission
1. Intubated
2. Thiopentone given
3. IV midazolam
EEG monitoring
145
What is thiopentone
Muscle relaxant
146
What is IV midazolam used for
Terminate seizure activity
147
Explain medications given in order for status epileptics
1. IV lorazepam or Rectal diazepam
2. Repeat dose IV lorazepam or rectal diazepam
3. IV phenytoin (or If regularly on phenytoin, IV phenobarbitone)
148
What is the risk following status epilepticus in a child
High risk of entering respiratory arrest following status
149
What should be done to prevent children going into respiratory arrest following status epilepticus
- Oxygen
- Observe SpO2
- Recovery position
150
When may a LP be performed if required following a status
Wait for GCS > 9
151
What is a febrile convulsion
Single generalised tonic-clonic seizure lasting less than 15-minutes associated with a temperature >38.
152
In which age group do febrile convulsions occur
6m -5 years.
153
What is peak age for febrile convulsions
12-18 months
154
What are 5 risk factors for febrile convulsions
```
1. HHV6 Infection
(Roseola Infantum)
2. Influenza virus
3. MMR vaccine
4. FH
5. Rapid rise in temperature
```
155
What are the three categories of febrile convulsions
- Simple Febrile Convulsion
- Complex Febrile Convulsion
- Convulsive Status Epilepticus
156
What are the 4 criteria for a simple febrile convulsion
- < 15m
- Generalised Tonic-Clonic
- Quick return to baseline
- No recurrence in 24h
157
What are three criteria for a complex febrile seizure
- Focal
- <15 minutes
- May recur in 24h
158
What is febrile status epileptics
Seizure persisting longer than 30-minutes
159
What are the three names of theories for aetiology of febrile convulsions
1. Fever rises body temperature
2. Fever causes hyperventilation
3. Cytokine theory
160
Explain fever rises core body T theory
Fever increases core body T - increasing neuronal excitability
161
Explain fever causes hyperventilation theory
Fever increases RR, this causes respiratory alkalosis. Respiratory alkalosis leads to increased excitability of neurons
162
Explain fever, cytokine theory
Cytokines act on NMDA receptors increase excitability
163
Explain investigations for febrile convulsions
If simple, febrile convulsions may not need to be investigated
164
What investigations may be performed in febrile convulsions
```
FBC
CRP
Blood Glucose
U+E
Urinalysis
Urine Culture
LP
EEG
```
165
When should children with febrile convulsions be admitted to paediatrics
1. First presentation of febrile convulsion
2. Complex febrile convulsions
3. Under 18m with febrile convulsion
166
What is the three criteria for complex febrile convulsions
1. <15m
2. Focal neurological signs and symptoms
3. May recurrent in 24h
167
What is first-line management for febrile convulsions
ACTION management
168
What is the ACTION approach
```
Assess situation
Call for help
Time
Identity
Over - once finished put in recovery position
Never restrain their head
```
169
If a simple febrile convulsion lasting more than 5 minutes what should be done
Benzodiezapine rescues medication
170
What is given as benzodiezapine rescue medication
Buccal midazolam
| Or, rectal diazepam
171
What dose of buccal midazolam is given if under 11 months
2.5mg
172
What dose of buccal midazolam is given if 1-4 years
5mg
173
What dose of buccal midazolam is given if 5-9 years
7.5mg
174
What is an alternative to buccal midazolam for febrile convulsions
Rectal diazepam
175
Aside from midazolam/diazepam what may be given in febrile convulsions
Anti-Paretics
176
What are criteria for admitting children with febrile convulsions
1. <18m
2. Complex febrile convulsion
3. First presentation
177
Discuss long-term management of febrile convulsions
At start of fever parent's may given paracetamol of wet flannel to cool the child.
Teach parents how to use benzodiezapine rescue pack
178
What have anti-pyretics NOT been shown to do
reduce recurrence of febrile convulsions
179
What is the risk of another seizure in febrile convulsions
1 in 3
180
Explain link between febrile convulsions and epilepsy
There are three risk factors for epilepsy:
1. FH
2. Complex febrile convulsion
3. Background neurodevelopment issue
- If not factors = 2.5% risk
- Multiple factors = 50% risk
181
Define a seizure
Irregular electrical activity due to hyper-activity of neurons
182
What defines a neonate
28d
183
What are four causes of seizure in neonate
1. Peri-natal Infection
2. Peri-natal asphyxia
3. Metabolite abnormalities
4. Congenital Syndromes
184
What is the most common trigger of seizures in infants
Febrile convulsions
185
What age are febrile convulsions most common
12-18m
186
What are 5 causes of seizures in infants
1. Malformaiton
2. TBI
3. Metabolic
4. Fever
5. Infection
187
What are three causes of seizures in adolescents
1. TBI
2. Illicit substance
3. Encephalitis
188
What are 4 causes of seizure in young adults
1. Alcohol withdrawal
2. Illicit substances
3. TBI
4. Brain Tumours
189
What are the two main broad categories of seizures
1. Generalised
| 2. Focal
190
What is a generalised seizure
Electrical seizure activity originates in bilateral networks
191
What is used to classify seizure type
International League Against Epilepsy
192
In ILAE classification, how are seizures first classified
1. Generalised
| 2. Focal
193
If a focal seizure how are they first classified
1. Awareness Impaired
| 2. Awareness Un-Impaired
194
How are focal seizures classified following awareness impaired/unimpaired
Motor
| Non-Motor
195
What can focal seizures become
Focal Seizures can progress to bilateral tonic-clonic seizures
196
How are generalised seizures classified
1. Motor = tonic clonic
| 2. Non-Motor = absence
197
What is a generalised motor seizure called
Tonic Clonic Seizure
198
What is a generalised non-motor seizure called
Absence Seizure
199
How are 'other' seizures classified
Into motor and non-motor
200
What are four types of generalised seizure
1. Tonic-Clonic
2. Myoclonic
3. Atonic
4. Absence
201
Explain clinical presentation of Tonic Clonic Seizures
- Pro-drome
- Individual first goes stiff and then limbs move symmetrically, bilaterally giving a rhythmic appearance.
- Individual may have tongue biting and incontinence during the seizure
202
What age group are myoclonic seizures more common
1-4 years
203
How do myoclonic seizures present
- LOC
| - individual is thrown to the ground where they start violently jerking
204
What is best medication to manage myoclonic seizures
Sodium Valproate
205
How does atonic seizures present
Sudden loss of tone - causes person to fall to the ground
206
In which age-group is absence epilepsy more common
Childhood
207
What is absence epilepsy
Brief loss of consciousness for 5-20 seconds. Eyes may roll up. Individual is unaware
208
What are focal seizures
More commonly due to focal structural damage - occur on one hemisphere. Anatomical location indicates seizure symptoms.
209
What are the two types of focal seizure
1. Consciousness unimpaired = simple partial seizure
| 2. Consciousness impaired = complex partial seizure
210
If a simple partial seizure where may it have arisen
- Frontal Lobe
- Parietal Lobe
- Occipital Lobe
211
How will a frontal focal seizure present
- Jacksonian March: Individual starts with twitching of the hand progresses to twitching of the face
- Head/Facial movements
- Todd's paralysis
212
How will occipital focal seizure present
Flashers and Floaters in Vision
213
How will focal seizure of the parietal lobe present
Parasthesia
214
Where do complex focal seizures originate
Temporal Lobe
215
What % of focal seizures occur in temporal lobe
70-80%
216
What are 6 features of temporal lobe seizures
- Aura
- Deja Vu
- Jamais Vu
- Automatisms (Lip Smacking)
- Behavioural arrest
- Impaired consciousness
217
Child presents with lip smacking, where has the seizure likely originated
Temporal Lobe
218
What age does West Syndrome Occur
4-6 months
219
What is West Syndrome also referred to as
Infantile spasms
220
What gender is West Syndrome more common
Males
221
How does West Syndrome present
Head nodding followed by arm jerks for 3-30s
222
What is head nodding in West syndrome called
Salaam attack
223
What is a reflex-anoxic attack
Paraoxysmal self-limiting asystole triggered by pain or fear
224
How will a child present in reflex-anoxic attacks (6 Features)
- Pale
- Hypotonic
- Rigid
- Upward eye deviation
- Clonic
- Urinary incontinence
225
What investigation should be ordered if a reflex-anoxic attack is suspected
Vagal excitation tests during EEG and ECG monitoring
226
What age does panayiotopolous syndrome occur
5-years
227
What is panayiotopoulos syndrome
Benign Focal Seizure Disorder affecting ~5years. Occurs at night, Autonomic symptoms predominate, with eye deviation and vomiting. Seizures can last 30 minutes. Self-resolves within 2 years
228
Explain age-dependent epileptic encephalopathy
Starts with Otahara syndrome (Tonic spasms) Progresses to West Syndrome then Lenox-Gustalt.
These are age-specific syndrome reactions to a non-specific exogenous source
229
How does Rolandic epilepsy present
Parasthesia of unilateral face worse on waking
230
What is Rolandic epilepsy also known as
Benign epilepsy with centrotemporal spikes
231
What is associated with west syndrome
Low IQ (70%)
232
What is the management of west syndrome
1. Prednisolone
| 2. Vigabatrin
233
What are bedside investigations following seizure
- Neurological Exam
| - Cardiac Exam
234
What are blood investigations for seizure
- FBC (Infection)
- U+E (Metabolic)
- Serum Calcium
- Blood Glucose (Hypoglycaemia)
- Blood Gas ( Hypoxic)
Blood and Urine MC+S
235
What imaging may be ordered following paediatric seizure
MRI
ECG
EEG
236
When are three indications for MRI following a seizure
1. Focal Seizure
2. Children who develop epilepsy before age 2
3. Seizures continue despite first-line treatment
237
When is an ECG performed following a seizure
All seizures
238
When is an EEG performed following a seizure
To SUPPORT diagnosis of epilepsy following second-seizure
239
What conservative management is offered for seizures
Advice:
- Avoid triggers
- Avoid dangerous situations such as bathing or swimming alone
240
What is used to manage epilepsy
anti-epileptic drugs
241
When should AEDs be used
following a second-seizure
242
What population should NOT be offered sodium valproate and why
young girls of reproductive age (from 8 years) due to high teratogenicity
243
What is first-line for focal seizures
lamotrigine or carbamezapine
244
How can you remember first-line for focal seizures
foCaL = has a C for carbamezapine and L for lamotrigine
245
What is first line for generalised TC seizures
Sodium valproate.
246
If a young female with generalised tonic-clonic seizures what medication should be given
Lamotrigine
247
How are absence seizures managed
```
Boys = Sodium Valproate
Girls = Ethosuximide
```
248
How are myoclonic seizures managed
```
Boys = Sodum Valproate
Girls = Levetiracetam
```
249
What are the two types of generalised epilepsy syndrome
Idiopathic epilepsy syndrome
| Symptomatic epilepsy syndrome
250
Explain idiopathic epilepsy syndromes
Most common (25%). Children are generally healthy
251
Explain symptomatic/cryptogenic epilepsy syndromes
structural or metabolic abnormality. Cryptogenic means seizures of unknown origin. Generally this group respond poorly to anti-epileptics. Associated with developmental delay, cognitive impairment or motor impairment
252
What age does childhood absence epilepsy syndrome occur
6-7 years
253
Which gender is childhood absence epilepsy syndrome more common
Female
254
How will children appear in childhood absence epilepsy syndrome
Absence seizures where children may have lip smacking, eye fluttering
255
How will EEG appear in childhood absence epilepsy syndrome
3Hz spikes
256
What is used to treat childhood absence epilepsy syndrome
Ethosuximide
257
What age is juvenile myoclonic epilepsy syndrome more common
12-20
258
What is juvenile myoclonic epilepsy syndrome also referred to as
Janz Syndrome
259
What can cause west syndrome
- Hypoxic- Ischaemic Injury
| - Perinatal infection
260
How will West Syndrome present
Sudden spasms involving flexion of the head (Salaam attack) and extension of the arms
261
What is used to manage West Syndrome
- Prednisolone
| - Vigabatrin
262
What is west syndrome associated with
Neurodevelopment delay
263
What age does Lennox Gastaut Syndrome occur
3-5 years
264
Which gender is Lennox-Gastaut more common in
Males
265
What do 50% of children with Lennox - Gastaut have
West Syndrome
266
How will Lennox-Gastaut present clinically
- Frequent episodes of status epilepticus
| - Atypical absence seizures
267
How will Lennox-Gastaut present on EEG
Slow-spikes
268
What is used to manage Lennox-Gastaut
Sodium Valproate
| Ketogenic diet
269
What is Lennox Gastaut associated with
Neurodevelopment delay
270
What gene is mutated in Dravet syndrome
SC1A
