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Paeds Exam Cram > Neurology > Flashcards

Neurology Flashcards

1
Q

Biceps reflex

A

C5, C6, musculocutaneous nerve

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2
Q

Triceps reflex

A

C7, C8, radial nerve

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3
Q

Wrist reflex

A

C5, C6, radial nerve

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4
Q

Finger reflex

A

C8, ulnar nerve

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5
Q

Knee reflex

A

L3, L4, femoral (3, 4, kick the door)

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6
Q

Ankle reflex

A

S1, S2, tibial

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7
Q

UMN

A 
Hypertonia
Brisk reflexes
Mild-mod weakness
Muscle bulk normal
No fasciculation
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8
Q

LMN

A 
Hypotonia
Reduced/absent reflexes
Severe weakness
Atrophic muscles
Fasciculation
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9
Q

CNI

A

Olfactory

Sensory - nose

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10
Q

CNII

A

Optic

Sensory - eye, afferent pupillary defect, visual fields, fundoscopy

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11
Q

CNIII

A

Occulomotor
Motor - all muscles of the eye except superior oblique
Pupillary light reaction

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12
Q

CNIV

A

Troclear

Motor - superior oblique SO4 eye in

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13
Q

CNV

A

Trigeminal
Sensory - face
Motor - muscles of mastication

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14
Q

CNVI

A

Abducens

Motor - lateral rectus LR6 eye out

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15
Q

CNVII

A

Facial

Motor - muscles of the face, taste anterior 2/3

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16
Q

CNVIII

A

Vestibulocochlear

Sensory - inner ear

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17
Q

CNIX

A

Glossopharyngeal

Motor - pharyngeal musculature - swallowing, taste posterior 1/3 tongue

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18
Q

CNX

A

Vagus
Motor - heart, lungs, bronchi,GIT
Sensory - heart lungs, trachea, larynx, pharynx, GIT, external ear

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19
Q

CNXI

A

Accessory

Motor - sternocleidomastoid, trapezius

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20
Q

CNXII

A

Hypoglossal

Motor - muscles of the tongue

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21
Q

Cavernous sinus

A

III occulomotor
IV trochlear
V trigeminal
IV abducens

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22
Q

Myotomes - C4

A

Shrug shoulders

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23
Q

Myotomes - C5

A

Shoulder abduction

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24
Q

Myotomes - C6

A

Elbow flexion, wrist extension

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25
Q

Myotomes - C7

A

Elbow extension, wrist flexion

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26
Q

Myotomes - C8

A

Thumb extension, finger flexion

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27
Q

Myotomes - T1

A

Finger intrinsics (abduction/adduction)

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28
Q

Myotomes - L2

A

Hip flexion

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29
Q

Myotomes - L3

A

Knee extension

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30
Q

Myotomes - L4

A

Ankle dorsiflexion, ankle inversion

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31
Q

Myotomes - L5

A

Big toe dorsiflexion

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32
Q

Myotomes - L5, S1

A

Ankle eversion

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33
Q

Myotomes - S1, S2

A

Plantar flexion, knee flexion, hip extension

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34
Q

Dermatomes - C2

A

Skull cap

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35
Q

Dermatomes - C3

A

Neck collar

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36
Q

Dermatomes - C5

A

Shoulder

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37
Q

Dermatomes - C6

A

Lateral forearm + thumb

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38
Q

Dermatomes - C7

A

Middle finger

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39
Q

Dermatomes - C8

A

Little finger

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40
Q

Dermatomes - T1

A

Medial elbow

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41
Q

Dermatomes - T4

A

Nipple line

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42
Q

Dermatomes - T10

A

Umbilicus

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43
Q

Dermatomes - T12

A

Suprapubic

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44
Q

Dermatomes - L1

A

Inguinal

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45
Q

Dermatomes - L2

A

Anterior thigh (pockets)

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46
Q

Dermatomes - L4

A

Anterior thigh, knee, medial lower leg

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47
Q

Dermatomes - L5

A

Lateral lower leg, dorm of foot, plantar surface of fott

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48
Q

Dermatomes - S1

A

Posterior leg, side of foot

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49
Q

Dermatomes - S2

A

Posterior leg

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50
Q

Dermatomes - S3

A

Sitting area

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51
Q

Dermatomes - S4, S5

A

Around anus

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52
Q

6th nerve palsy CN6

A

Affected eye turned in
If can straighten eye on cover test NOT a 6th nerve palsy
(*tumour - medulloblastoma, brainstem glioma, raised ICP, meningitis, post infection)

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53
Q

Facial nerve palsy UMN CN7

A

Bottom half of contralateral face
Loss of taste anterior 2/3 tongue
(arterial hypertension, infection, raised ICP)

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54
Q

Bell’s Palsy LMN CN7

A 
Whole ipsilateral face
Taste anterior 2/3 tongue
Can't close eye
Flattening of forehead creases
(EBV, HSV, Ramsay Hunt)
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55
Q

Musculocutaneous Nerve Palsy C5/C6

A

Can’t flex elbow
Loss of sensation lateral forearm
Weak biceps reflex
(fractured neck of humerus)

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56
Q

Axillary Nerve Palsy (C5/C6)

A

Can’t flex elbow
Can’t abduct/rotate shoulder
Loss of sensation lateral arm posterior shoulder
(shoulder dislocation, crutches)

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57
Q

Erb’s Palsy (C5/C6/C7)

A

Can’t flex elbow
Can’t abduct shoulder “waiter’s tip”
(birth trauma, backpack)

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58
Q

Klumpke’s Palsy C8/T1

A

Claw hand
Can get Horner’s also
(birth trauma/underarm injury)

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59
Q

Radial Nerve Palsy C7/C8

A 
Wrist drop
Can't extend elbow/fingers
Numbness dorsal (posterior hand)
Numbness anatomical snuff box
Weak triceps reflex
(hummers fracture)
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60
Q

Median Nerve Palsy C6-T1

A 
Weak opposition thumb + finger
Weak wrist flexion
Sensation palmar surface of lateral 3 digits
Thenar eminence wasting
Absent finger jerk reflex
(supracondylar fracture, carpal tunnel)
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61
Q

Ulnar Nerve Palsy C8/T1

A

Finger abduction/adduction (paper between thumb and second digit)
Sensory 4th and 5th fingers
Intrinsic muscles wasted
(trauma)

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62
Q

Long thoracic nerve palsy C5-C7)

A

Winged scapula

backpack, cardiac surgery

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63
Q

Common perineal nerve palsy L4-S1

A

Foot drop with intact reflexes
Can’t walk on heels
(trauma/compression)

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64
Q

Sciatic nerve L4-S2

A

Foot drop with absent reflexes
Loss of sensation lateral lower leg
(spinal, trauma)

65
Q

Tibial nerve palsy

A

Can’t walk on Toes
Reduced sensation sole of foot
(trauma/compression)

66
Q

Femoral nerve palsy

A

Weak knee extension
Hip flexion
Absent knee jerk
Reduced sensation inner leg and thigh

67
Q

Horners (sympathetic trunk)

A

Ptosis, mitosis (constriction)
Anhidrosis (dry skin)
Think neuroblastoma, lymphoma at lateral neck/peck of the lung
Brachial plexus trauma

68
Q

Polymicrogyria

A

Overfolding
Seizures, ID/Dev delay

PMG + normocephaly - Tubulin genes
PMG + microcephaly - WDR62, CMV
PMG + macrocephaly - mTOR pathways

69
Q

Schiezencephaly

A

Gap from ventricle to cortex

70
Q

Lissencephaly

A

Smooth brain, lack of gyri (due to defective neuronal migration)

LIS1

  • Ch17 mutation
  • More common
  • Posterior brain worse - agyria
  • (Miller Decker if LIS1 deleted - more severe)

DCX

  • X linked
  • Males
  • Most severe
  • Anterior brain worse - styria

Cobblestone

  • AR
  • Irregular cortex + muscle dystrophy
  • High CK
71
Q

Subcortical band heterotopia

A 
DCX mutation
Mostly females
Disorder of neuronal migration
Grey/white/grey
Seizures
ID
Developmental delay
72
Q

Periventricular nodular heterotopia

A

Nodules along ventricle
Males die in utero
Seizures, normal/mild ID

73
Q

Cortical dysplasia

A

Gyral irregularities
mTOR pathyway
Associated TS
Seziures

74
Q

Chiari malformation

A

Type 1 most common, foramen magnum, can block CSF

Type 2 associated with myelomeningocele

75
Q

Agenesis corpus callosum

A

Most common congenital
85% normal
Worse if other abnormality

76
Q

Dandy Walker

A

Agenesis corpus callosum, hydrocephalus

77
Q

Adrenoleukodystrophy

A

X linked
5-10yo
MRI: symmetrical posterior white matter changes
Measure VLCFA ratio
Seizures, gait disturbance, spastic quadriparesis, swallowing difficulty
Adrenal insufficiency ATCH excess -> brown gums

78
Q

Metachromatic leukodystrophy

A

12-18mo
Unable to walk - ataxia, reduced reflexes, spasticity, developmental regression, seizures
MRI: anterior + posterior bilateral symmetrical white matter changes

79
Q

Krabbe

A

AR
Fatal, death by 2
Seizures, deaf, blind

80
Q

Leigh

A 
Mitochondrial
Infantile seizures, hypotonia
Deteriorate with viral illness
MRI: white patches with holes in brain
Increased CSF and blood lactate
81
Q

DMD

A

X linked
Dystrophin gene deletion/point mutation -> non functioning protein (BMD partially functioning)
Gonadal mosaicism
Carrier females mild weakness, cardiomyopathy, mild high CK
Presents 3-7yo, motor delay, proximal myopathy, towers positive, calf hypertrophy, dilated cardiomyopathy
Dx: DNA analysis, PCR
Muscle biopsy gold standard if PCR neg CK >15000

82
Q

BMD

A

X linked recessive
Dystrophin mutation -> reduced or partially functioning protein
3-10yo, can walk until 14, cardio=myopathy less common, cardiac conduction abnormalities worse
Dx: DNA/PCR, muscle biopsy, CK

83
Q

Myotonic dystrophy

A 
AD
Triplet repeat disorder
Congenital - Birth-10yo
Type 1 - 10-30yo
Fish mouth, high arched palate
Progressive weakness, distal muscle wasting *different to other myopathies*, slurred speech, myotonia - can't relax after contraction e.g. fist/handshake (check parent's handshake), dilated cardiomyopathy, cataracts
84
Q

Congenital myotonic dystrophy

A

Poor foetal movements
Polyhydramnios
Won’t breath from birth

85
Q

SMA

A 
5q11
Anterior horn cell degeneration
Regression, proximal myopathy fasciculations, no reflexes
Hypotonia with facial sparing
CK normal

Type 1: most severe, never sit, frog posture, wrist drop
Type 2: never stand, can suck and swallow
Type 3: presents later, shoulder girdle problems

86
Q

Central Core Myopathy

A

Facial trunk weakness
Associated with malignant hyperthermia
CK normal
NADH stain

87
Q

Congenital Myopathy

A

Hypotonia
From Birth
Demaline

88
Q

Fairy disease

A

Hereditary motor-sensory neuropathy
Telangiectasia
Blood group AB or B worse

89
Q

Lesch Nyan

A 
Purine metabolism
Extrapyramidal signs
Self mutilation ~2yo (biting lips), low IQ
High uric acid urine + blood
Allopurinol
90
Q

Retts

A 
X linked dominant (boys die)
Girls affected
MECP2 gene, mostly sporadic
Normal 6 mo then regression - language*, hand movements, gait
Hand wringing, stereotyped movements
Seizures, ataxia
91
Q

Pompe

A 
Profound hypotonia with facial sparing
Areflexia
Big tongue, big heart, big liver
High CK
Enzyme replacement
92
Q

ADEM

A

Destroys myeline
Flair MRI
IVIG

93
Q

Cerebral abscess

A

RF congenital heart disease
Staph/strep
Fever, seizures, headaches
CT ring enhancing lesion with contrast

94
Q

Post infection cerebellits

A

2/52 after varicella/virus
Well
Rapid onset ataxia, nystagmus
Normal strength, reflexes

95
Q

Charcot Marie Tooth

A 
Foot drop, areflexia, deformity
Peripheral neuropathy (tone, reflexes, sensation)
CMT1A
Normal CK
No Rx
96
Q

Freidrich Ataxia

A

Poor tone/reflexes
Cardiomyopathy
DM

97
Q

Dermatomyositis

A

5-10yo
Symmetrical muscle weakness, heliotrope rash, grotton papules, malar rash, nailed capillary changes
Normal/high CK, ANA + ESR normal

98
Q

Polymositis

A

Inflammatory myopathy
Proximal weakness then progresses
No rash

99
Q

Langerhans cell histiocytosis

A

Ping pong skull osteolytic bone lesions on skull
Cutaneous lesions
Recurrent sinopulmonary infecitons

100
Q

Optic neuritis

A

Rapid decline vision loss one eye
Pain on movement
Caused by MS, post viral
Steroids if known MS

101
Q

Guillan Barre

A 
Immune mediated polyneuropathy
Campylobacter, mycoplasma
Reflexes go first, weakness, motor > sensory ascending hrs - days
High CSF protein
Rx: IVIG, plasmapheresis (not steroids)
102
Q

Miller Fischer

A

Like GBS

Areflexia, ataxia, ophthalmoplegia

103
Q

Botulism

A

3/52-6/12

Hypotonia, loss of reflexes, dilated pupils (bilateral CN dysfunction)

104
Q

NF1

A

Neurocutaneous
AD/50% sporadic

Order of appearance:

  • Cafe au last spots >6
  • Plexiform neurofibromas
  • Axillary freckling (childhood)
  • Lisch nodules: harmatomas in iris (childhood)
  • Optic gliomas
  • Neurofibromas (appear later)
  • Skeletal - cortical thinning or sphenoid dysplasia
  • Family history (first degree relative)

Associates: scoliosis, ADHD, seizures, Wilms, Moya Moya, hypertension, CoA, pectus excavatum

105
Q

NF2

A

Neurocutaneous

AD
Presents later
Bilateral acoustic neuromas
Hearing loss, ataxia
Complication: spinal tumour
106
Q

Tuberous sclerosis

A

Neurocutaneous

2/3 sporadic
TSC1 Harmatin, TSC2 Tuberin (severe)
>3 Ash leif macules, seizures (infantile spasms), low IQ
In order:
- Ash leaf
- Forehead plaque
- Sebaceous adenoma (acne like modules on face)
- Shagreen patch
- Subungual
- Periungal fibromas

Other: cardiac rhabdomyoma, cortical tubers, giant cell astrocytoma, retinal haematoma

CXR, baseline ECHO, renal US, MRI

107
Q

Ataxia Telangiectasia

A

Neurocutaneous

AR
ATM gene, high AFT
Ataxia/nystagmus/oculomotor apraxia, telangiectasia
IgA, B+T cell defects - recurrent sinopulmonary infections, lymphoma

108
Q

Sturge Weber

A

Neurocutaneous

Sporadic
Port wine stain on face + seizures, glaucoma, developmental delay

109
Q

Proximal weakness

110
Q

Distal weakness

A

Neuropathy

111
Q

Central scotoma (visual fields)

112
Q

Toddler self mutilating

A

Think Lesch Nyan (high uric acid, allorpurinol)

113
Q

Fasciulations

114
Q

Infantile spasms, cutaneous

115
Q

Symmetrical weakness + absent reflexes

116
Q

Girl with regression, speech, hand movements

117
Q

Benign Rolandic Seizures

A

Benign childhood epilepsy with centrotemporal spikes (BCECTS)
4-10yo
Most common M>F
On awakening
Simple partial (unilateral, face) can become secondarily generalised
Precipitated by lack of sleep
EEG: central temporal spike and wave (waves only in temporal leads)
No treatment
Resolve my adolescence

118
Q

Absence seizures

A 
4-9yo
F>M
15% FHx
95% resolve by adolescence
Sudden motor cessation, no aura, can have >100/day
Precipitated by hyperventilation
Disrupts learning
3Hz spike and wave (waves in all leads)
Ethosuxamide childhood
Valproate/lamotrigine for late onset
NOT carbamazepine - can bring on GTCS
119
Q

Juvenile myoclonic epilepsy (Janz)

A

Adolescence, FHx 50%
Myoclonic/GTCS
Early morning jerking or clumsiness
Precipitated by sleep deprivation, alcohol, photo stimulation
Unlikely to grow out of
EEG 4-6Hz poly spike wave sleep deprived
Tx: valproate, leviteracitam, lamotrigine (teenage girls)
Avoid carbamazepine, phenytoin - makes GTCS worse

120
Q

Generalised epilepsy with febrile seizures

121
Q

Dravet syndrome

A 
SCN1A*
Severe myoclonic epilepsy of infancy
Triggered by fevers/vaccinations
Developmental delay
Refractory
Ketogenic
Poor outcome
122
Q

Ottahara syndrome

A

ARX
Early infantile epileptic encephalopathy
<3mo
EEG - burst suppression

123
Q

West syndrome

A

Infantile spasm
ARX boys
CDKLS girls
4-8mo, 2/3 have underlying neurology (HIE, PVL, TS)
Idiopathic - developmental delay after onset seizures
Symptomatic - developmental delay before onset seizures
Flexor spasms head trunk limbs, extension of arms
1-2 sec, 20-30 bursts
Reduced social interaciton
Development slows then regresses
Hypsarrythmia on EEG (completely chaotic)
Rx: prednisolone, vigabatrin**, clonazepam

124
Q

Myoclonic atonic (drop attacks)

A 
GLUT1/SCN1A
Jerk then drop
Triggered by photosensitivity
Tx: valproate, steroids
NOT carbamazepine, phenytoin, vigabatrin
125
Q

Lennox-Gastaut syndrome

A 
3-5yo
Intractable
Multiple seizure types (drop attack, tonic, absence)
Developmental regression/arrest, behaviour disorder
Hx: infantile spasms
EEG: slow spike and wave
Poor prognosis
Tx: valproate, lamotrigine, vigabatrin
126
Q

Landau-Kleffner syndrome

A 
2-9yo
Infrequent seizures (nocturnal)
Aphasia/speech disturbance
Behavioural disturbance
MRI normal
Tx: steroids, IVIG, ketogenic diet
127
Q

Occipital epilepsy

A

Younger: unresponsive, eye deviation, vomiting
Older: visual disturbance, hallucinations
EEG: occipital discharge suppression by fixation
Resolves no Tx

128
Q

Temporal lobe epilepsy

A

Late childhood
Hippocampal sclerosis
Associated with prolonged febrile convulsions
Complex partial, secondarily generalised
Precipiated by sleep deprivation, hormonal changes
Tx: phenytoin, carbamazepine, leviteracitam
Lobectomy if hippocampal sclerosis can cure 90%

129
Q

Epilepsy treatment

General rules

A

Focal: carbamazepine
Generalised: valproate (carbamazepine worsens)

130
Q

Sodium valproate

A

Generalised tonic clonic
Absence
Myoclonic
Partial

131
Q

Carbamazepine

132
Q

Clonazepam

A

Infantile spasms second line

133
Q

Ethosuximide

134
Q

Leviteracitam

A

Second line in GTCS, Janz

135
Q

Phenytoin

A

Status epilepticus, temporal lobe epilepsy

136
Q

Vigabatrin

A

Infantile spasms, adjunctive therapy for poorly controlled seizures

137
Q

Partial seizures

A

Carbamazepine

138
Q

Benign Rolandic

A

No treatment, resolves by adolesnce

139
Q

Absence

A

Ethosuxamide childhood
Valproate, lamotrigine for late onset
NOT carbamazepine - can bring on GTCS

140
Q

GTCS

A

Valproate
Leviteracitam
Carbamazepine, lamotrigine, topiramate, phenytoin, phenobarbitone

141
Q

Juvenile Myoclonic Epilepsy (Janz)

A

Valproate
Leviteracitam, lamotrigine (teenage girls)
Avoid carbamazepine, phenytoin - makes GTCS worse

142
Q

West syndrome (infantile spasm)

A

Prednisolone
Vigabatrin**
Clonazepam

143
Q

Myoclonic atonic (drop attacks)

A

Valproate
Steroids
NOT carbamazepine, phenytoin, vigabatrin (can worse)

144
Q

Lennox-Gastaut syndrome

A

Valproate

Lamotriginem Vigabatrin

145
Q

Landau-Kleffner syndrome

A

Steroids, IVIG, ketogenic diet

146
Q

Dravet

A

Refractory

Ketogenic diet

147
Q

Temporal lobe epilepsy

A

Phenytoin, carbamazepine, leviteracitam

Lobectomy

148
Q

Side effects

A

In asians check HLAB1502 prior to starting phenytoin/carbamazepine (risk SJS)

149
Q

Valproate

A

Na channel blocker

Weight gain
Nausea
Vomiting
Hair loss
Pancreaittis
Hepatoxicity
Teratogenic 5%
*inhibitor*
150
Q

Carbamazepine/oxcarbazepine

A

Na channel blocker

Rash SJS
Neutropaenia
Low Na
Ataxia
Liver enzyme induciton
Nausea
Vomiting
Drowsiness
*inducer*

Worsens absence, JME - GTCS

151
Q

Vigabatrin

A

Restricted peripheral visual fields
Nigh vision loss
Sedation
Weight gain

152
Q

Lamotrigine

A 
Hypersensitivity
Blurred vision
Diplopia
Nausea
Dizziness
Tremour
Rash SJS (increased levels with valproate, decreased levels with carbamazepine)
153
Q

Ethosuxamide

A 
Nausea
Vomiting
Drowsiness
Rash
Bone marrow suppression
154
Q

Topiramate

A 
Drowsiness
Withdrawal
Weight loss
Renal stones
Glaucoma
155
Q

Gabapentin

A

Insomnia
Ataxia
Excreted unchanged in urine

156
Q

Levetiracetam

A 
Sedation
Dizziness
Depression
Behavioural disturbance
Thrombocytopaenia
157
Q

Benzodiazepines (clonazepam, diazepam)

A

Sedation
Tolerance
Increased secretions

158
Q

Phenytoin

A 
Hirsutism
Gum hypertrophy
Hepatitis
Rash
SJS
*inducer*

Paeds Exam Cram (19 decks)

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