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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
Myotomes - C7
Elbow extension, wrist flexion
26
Myotomes - C8
Thumb extension, finger flexion
27
Myotomes - T1
Finger intrinsics (abduction/adduction)
28
Myotomes - L2
Hip flexion
29
Myotomes - L3
Knee extension
30
Myotomes - L4
Ankle dorsiflexion, ankle inversion
31
Myotomes - L5
Big toe dorsiflexion
32
Myotomes - L5, S1
Ankle eversion
33
Myotomes - S1, S2
Plantar flexion, knee flexion, hip extension
34
Dermatomes - C2
Skull cap
35
Dermatomes - C3
Neck collar
36
Dermatomes - C5
Shoulder
37
Dermatomes - C6
Lateral forearm + thumb
38
Dermatomes - C7
Middle finger
39
Dermatomes - C8
Little finger
40
Dermatomes - T1
Medial elbow
41
Dermatomes - T4
Nipple line
42
Dermatomes - T10
Umbilicus
43
Dermatomes - T12
Suprapubic
44
Dermatomes - L1
Inguinal
45
Dermatomes - L2
Anterior thigh (pockets)
46
Dermatomes - L4
Anterior thigh, knee, medial lower leg
47
Dermatomes - L5
Lateral lower leg, dorm of foot, plantar surface of fott
48
Dermatomes - S1
Posterior leg, side of foot
49
Dermatomes - S2
Posterior leg
50
Dermatomes - S3
Sitting area
51
Dermatomes - S4, S5
Around anus
52
6th nerve palsy CN6
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)
53
Facial nerve palsy UMN CN7
Bottom half of contralateral face Loss of taste anterior 2/3 tongue (*arterial hypertension*, infection, raised ICP)
54
Bell's Palsy LMN CN7
``` Whole ipsilateral face Taste anterior 2/3 tongue Can't close eye Flattening of forehead creases (EBV, HSV, Ramsay Hunt) ```
55
Musculocutaneous Nerve Palsy C5/C6
Can't flex elbow Loss of sensation lateral forearm Weak biceps reflex (fractured neck of humerus)
56
Axillary Nerve Palsy (C5/C6)
Can't flex elbow Can't abduct/rotate shoulder Loss of sensation lateral arm posterior shoulder (shoulder dislocation, crutches)
57
Erb's Palsy (C5/C6/C7)
Can't flex elbow Can't abduct shoulder "waiter's tip" (birth trauma, backpack)
58
Klumpke's Palsy C8/T1
Claw hand Can get Horner's also (birth trauma/underarm injury)
59
Radial Nerve Palsy C7/C8
``` Wrist drop Can't extend elbow/fingers Numbness dorsal (posterior hand) Numbness anatomical snuff box Weak triceps reflex (hummers fracture) ```
60
Median Nerve Palsy C6-T1
``` 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) ```
61
Ulnar Nerve Palsy C8/T1
Finger abduction/adduction (paper between thumb and second digit) Sensory 4th and 5th fingers Intrinsic muscles wasted (trauma)
62
Long thoracic nerve palsy C5-C7)
Winged scapula | backpack, cardiac surgery
63
Common perineal nerve palsy L4-S1
Foot drop with intact reflexes Can't walk on heels (trauma/compression)
64
Sciatic nerve L4-S2
Foot drop with absent reflexes Loss of sensation lateral lower leg (spinal, trauma)
65
Tibial nerve palsy
Can't walk on Toes Reduced sensation sole of foot (trauma/compression)
66
Femoral nerve palsy
Weak knee extension Hip flexion Absent knee jerk Reduced sensation inner leg and thigh
67
Horners (sympathetic trunk)
Ptosis, mitosis (constriction) Anhidrosis (dry skin) Think neuroblastoma, lymphoma at lateral neck/peck of the lung Brachial plexus trauma
68
Polymicrogyria
Overfolding Seizures, ID/Dev delay PMG + normocephaly - Tubulin genes PMG + microcephaly - WDR62, CMV PMG + macrocephaly - mTOR pathways
69
Schiezencephaly
Gap from ventricle to cortex
70
Lissencephaly
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
Subcortical band heterotopia
``` DCX mutation Mostly females Disorder of neuronal migration Grey/white/grey Seizures ID Developmental delay ```
72
Periventricular nodular heterotopia
Nodules along ventricle Males die in utero Seizures, normal/mild ID
73
Cortical dysplasia
Gyral irregularities mTOR pathyway Associated TS Seziures
74
Chiari malformation
Type 1 most common, foramen magnum, can block CSF | Type 2 associated with myelomeningocele
75
Agenesis corpus callosum
Most common congenital 85% normal Worse if other abnormality
76
Dandy Walker
Agenesis corpus callosum, hydrocephalus
77
Adrenoleukodystrophy
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
Metachromatic leukodystrophy
12-18mo Unable to walk - ataxia, reduced reflexes, spasticity, developmental regression, seizures MRI: anterior + posterior bilateral symmetrical white matter changes
79
Krabbe
AR Fatal, death by 2 Seizures, deaf, blind
80
Leigh
``` Mitochondrial Infantile seizures, hypotonia Deteriorate with viral illness MRI: white patches with holes in brain Increased CSF and blood lactate ```
81
DMD
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
BMD
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
Myotonic dystrophy
``` 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
Congenital myotonic dystrophy
Poor foetal movements Polyhydramnios Won't breath from birth
85
SMA
``` 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
Central Core Myopathy
Facial trunk weakness Associated with malignant hyperthermia CK normal NADH stain
87
Congenital Myopathy
Hypotonia From Birth Demaline
88
Fairy disease
Hereditary motor-sensory neuropathy Telangiectasia Blood group AB or B worse
89
Lesch Nyan
``` Purine metabolism Extrapyramidal signs Self mutilation ~2yo (biting lips), low IQ High uric acid urine + blood Allopurinol ```
90
Retts
``` 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
Pompe
``` Profound hypotonia with facial sparing Areflexia Big tongue, big heart, big liver High CK Enzyme replacement ```
92
ADEM
Destroys myeline Flair MRI IVIG
93
Cerebral abscess
RF congenital heart disease Staph/strep Fever, seizures, headaches CT ring enhancing lesion with contrast
94
Post infection cerebellits
2/52 after varicella/virus Well Rapid onset ataxia, nystagmus Normal strength, reflexes
95
Charcot Marie Tooth
``` Foot drop, areflexia, deformity Peripheral neuropathy (tone, reflexes, sensation) CMT1A Normal CK No Rx ```
96
Freidrich Ataxia
Poor tone/reflexes Cardiomyopathy DM
97
Dermatomyositis
5-10yo Symmetrical muscle weakness, heliotrope rash, grotton papules, malar rash, nailed capillary changes Normal/high CK, ANA + ESR normal
98
Polymositis
Inflammatory myopathy Proximal weakness then progresses No rash
99
Langerhans cell histiocytosis
Ping pong skull osteolytic bone lesions on skull Cutaneous lesions Recurrent sinopulmonary infecitons
100
Optic neuritis
Rapid decline vision loss one eye Pain on movement Caused by MS, post viral Steroids if known MS
101
Guillan Barre
``` Immune mediated polyneuropathy Campylobacter, mycoplasma Reflexes go first, weakness, motor > sensory ascending hrs - days High CSF protein Rx: IVIG, plasmapheresis (not steroids) ```
102
Miller Fischer
Like GBS | Areflexia, ataxia, ophthalmoplegia
103
Botulism
3/52-6/12 | Hypotonia, loss of reflexes, dilated pupils (bilateral CN dysfunction)
104
NF1
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
NF2
Neurocutaneous ``` AD Presents later Bilateral acoustic neuromas Hearing loss, ataxia Complication: spinal tumour ```
106
Tuberous sclerosis
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
Ataxia Telangiectasia
Neurocutaneous AR ATM gene, high AFT Ataxia/nystagmus/oculomotor apraxia, telangiectasia IgA, B+T cell defects - recurrent sinopulmonary infections, lymphoma
108
Sturge Weber
Neurocutaneous Sporadic Port wine stain on face + seizures, glaucoma, developmental delay
109
Proximal weakness
Myopathy
110
Distal weakness
Neuropathy
111
Central scotoma (visual fields)
Think MS
112
Toddler self mutilating
Think Lesch Nyan (high uric acid, allorpurinol)
113
Fasciulations
SMA
114
Infantile spasms, cutaneous
TS
115
Symmetrical weakness + absent reflexes
GBS
116
Girl with regression, speech, hand movements
Rett
117
Benign Rolandic Seizures
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
Absence seizures
``` 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
Juvenile myoclonic epilepsy (Janz)
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
Generalised epilepsy with febrile seizures
SCN1A
121
Dravet syndrome
``` SCN1A* Severe myoclonic epilepsy of infancy Triggered by fevers/vaccinations Developmental delay Refractory Ketogenic Poor outcome ```
122
Ottahara syndrome
ARX Early infantile epileptic encephalopathy <3mo EEG - burst suppression
123
West syndrome
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
Myoclonic atonic (drop attacks)
``` GLUT1/SCN1A Jerk then drop Triggered by photosensitivity Tx: valproate, steroids NOT carbamazepine, phenytoin, vigabatrin ```
125
Lennox-Gastaut syndrome
``` 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
Landau-Kleffner syndrome
``` 2-9yo Infrequent seizures (nocturnal) Aphasia/speech disturbance Behavioural disturbance MRI normal Tx: steroids, IVIG, ketogenic diet ```
127
Occipital epilepsy
Younger: unresponsive, eye deviation, vomiting Older: visual disturbance, hallucinations EEG: occipital discharge suppression by fixation Resolves no Tx
128
Temporal lobe epilepsy
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
Epilepsy treatment | General rules
Focal: carbamazepine Generalised: valproate (carbamazepine worsens)
130
Sodium valproate
Generalised tonic clonic Absence Myoclonic Partial
131
Carbamazepine
Partial
132
Clonazepam
Infantile spasms second line
133
Ethosuximide
Absence
134
Leviteracitam
Second line in GTCS, Janz
135
Phenytoin
Status epilepticus, temporal lobe epilepsy
136
Vigabatrin
Infantile spasms, adjunctive therapy for poorly controlled seizures
137
Partial seizures
Carbamazepine
138
Benign Rolandic
No treatment, resolves by adolesnce
139
Absence
Ethosuxamide childhood Valproate, lamotrigine for late onset NOT carbamazepine - can bring on GTCS
140
GTCS
Valproate Leviteracitam Carbamazepine, lamotrigine, topiramate, phenytoin, phenobarbitone
141
Juvenile Myoclonic Epilepsy (Janz)
Valproate Leviteracitam, lamotrigine (teenage girls) Avoid carbamazepine, phenytoin - makes GTCS worse
142
West syndrome (infantile spasm)
Prednisolone Vigabatrin** Clonazepam
143
Myoclonic atonic (drop attacks)
Valproate Steroids NOT carbamazepine, phenytoin, vigabatrin (can worse)
144
Lennox-Gastaut syndrome
Valproate | Lamotriginem Vigabatrin
145
Landau-Kleffner syndrome
Steroids, IVIG, ketogenic diet
146
Dravet
Refractory | Ketogenic diet
147
Temporal lobe epilepsy
Phenytoin, carbamazepine, leviteracitam | Lobectomy
148
Side effects
In asians check HLAB1502 prior to starting phenytoin/carbamazepine (risk SJS)
149
Valproate
Na channel blocker ``` Weight gain Nausea Vomiting Hair loss Pancreaittis Hepatoxicity Teratogenic 5% *inhibitor* ```
150
Carbamazepine/oxcarbazepine
Na channel blocker ``` Rash SJS Neutropaenia Low Na Ataxia Liver enzyme induciton Nausea Vomiting Drowsiness *inducer* ``` Worsens absence, JME - GTCS
151
Vigabatrin
Restricted peripheral visual fields Nigh vision loss Sedation Weight gain
152
Lamotrigine
``` Hypersensitivity Blurred vision Diplopia Nausea Dizziness Tremour Rash SJS (increased levels with valproate, decreased levels with carbamazepine) ```
153
Ethosuxamide
``` Nausea Vomiting Drowsiness Rash Bone marrow suppression ```
154
Topiramate
``` Drowsiness Withdrawal Weight loss Renal stones Glaucoma ```
155
Gabapentin
Insomnia Ataxia Excreted unchanged in urine
156
Levetiracetam
``` Sedation Dizziness Depression Behavioural disturbance Thrombocytopaenia ```
157
Benzodiazepines (clonazepam, diazepam)
Sedation Tolerance Increased secretions
158
Phenytoin
``` Hirsutism Gum hypertrophy Hepatitis Rash SJS *inducer* ```

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