Neurology / Neurodevelopment

Question 1

Main excitatory neurotransmitter

Answer 1

Glutamate

Question 2

Main inhibitor neurotransmitter

Answer 2

GABA

Question 3

Which spinal tract travels in the dorsal column and where does it cross midline?

Answer 3

Fine touch, vibration, proprioception
Crosses at the medualla

Question 4

What is sensed by the spinothalamic tract and where does it cross midline?

Answer 4

Pain and temp
Crosses immediately at SC –> thalamus

Question 5

What spinal tract is in the ventral horn and where does it cross the midline?

Answer 5

Motor tract
Crosses at level of medulla

Question 6

What is Brown-Sequard syndrome and how does it present?

Answer 6

Hemi-section of SC
- Ipsilateral loss of fine touch, proprioception and motor
- Contralateral pain and temperature

Question 7

Features of Charot-Marie-Tooth Syndrome

Answer 7

• Dominant
• Progressive damage to peripheral nerves - demyelination
• Peripheral weakness
• Hammer toes
• High arch feet
• Legs like upside down champagne bottles
• Reduced sensation
• Reduced nerve condution

Question 8

Features of Rett Syndrome

Answer 8

Mostly girls
Mutation MECP2 gene
- Microcephaly
- GI disorder
- Seizures

Question 9

What do nerve conduction studies test for?

Answer 9

Axonal degeneration = reduced amplitude
Demyelination = reduced velocity

Question 10

Pathophysiology and features of MS

Answer 10

Rare in childhood
AI –> demyelination –> lesions in white matter –> demyelinating plaques
Relapsing / remitting neurological Sx
Ix: LP - oligoclonal bands, raised IgG. Evoked potentials.

Question 11

ADEM - Pathophysiology, presentation, Ix and Tx

Answer 11

Immune mediated response to infection / vaccination –> monophasic acute demyelination in brain (white matter) and SC
Presents - Abrupt, fever, headache, drowsiness, coma, seizures, paresis, CN palsies
Ix: MRI, CSF mildly increased lymphocytes / protein
Tx: Support hydration, IV methylpred

Question 12

Pathophysiology of migraines

Answer 12

• Genetic component
• NO - vasodilation
• Cortical spreading depression
• Raised seratonin / dopamine

Question 13

Pathophysiology acute migraine treatment

Answer 13

• Triptans = 5-HT agonists –> block neurotransmission and reduced neuropeptide release
• Dopamine antagonists eg prochlorperazine

Question 14

Pathophysiology of migraine prophylaxis

Answer 14

• Pizotogen >5y = seratonin/ histamine antagonist
• Propranolol >2y = beta-1 receptor antagonist, reduced noradrenergic action, altered GABA firing

Question 15

Features and management of idiopathic intracranial HTN

Answer 15

• More in overweight teen girls
• Presents: Headache, nausea, visual disturbance, papilloedema
• Normal brain imaging, raised LP opening pressure
• Tx: Therapeutic LP, acetazolamide (reduced CSF production) +/- furosemide. If not effective, CSF shunt

Question 16

Presentation of raised ICP

Answer 16

• Headache - more mornings / cough/ micturition
• Vomiting esp morning
• Focal neurological Sx
• Cushing’s sign - low HR, high BP, irregular resps
• Fluctuating mental state

Question 17

Management raised ICP

Answer 17

• Close monitoring Co2/ glucose/ electrolytes/ temp in normal range
• Elevate bed 30 degrees, increased jugular flow
• Analgesia / sedation - reduced O2 demand
• Seizure prophylaxis
• Bolus 3% NaCl / mannitol
• Fluid restriction
• Surgical decompression

Question 18

Causes of ischaemic stroke in children

Answer 18

• Perinatal - MCA - thrombotic from placenta
• Cardiogenic - CHD, arrhythmia, cardiomyopathy, infective endocarditis
• Thromboembolic eg vasculitis
• Haem - sickle cell, coagulation disorder

Question 19

AEDs for generalised tonic clonic seizures

Answer 19

1st line: Sodium valproate
2nd line: Lamotrigine, levetiracetam

Question 20

AEDs for tonic / atonic seizures

Answer 20

1st Line: Sodium Valproate
2nd Line: Lamotrigine

Question 21

AEDs for absence seizures

Answer 21

1st line: Ethosuximide, sodium valproate
2nd line: Lamotrigine

Question 22

AEDs for myoclonic seizures

Answer 22

1st line: Sodium valproate
2nd line: Levetiracetam, topiramate

Question 23

AEDs for Focal seizures

Answer 23

1st line: Carbamazepine, lamotrigine

Question 24

AEDs for Infantile spasms

Answer 24

Vigabatrin or Prednisolone
Vgabatrin only if Tuberous Sclerosis

Question 25

Presentation radial nerve palsy

Answer 25

C5-8, T1 Causes: humerus / radial fracture, "saturday night palsy" Sensory: post. arm, radial distribution of dorsum hand Motor: Weak elbow extension, absent triceps / supinator reflex, weak wrist / thumb / MCP extension

Question 26

Presentation of median nerve palsy

Answer 26

C5-8, T1 Causes: Humerus fracture, carpal tunnel Sensory: palmar thenar side Motor - weak grip, weak forearm pronation. "Hand of benediction"

Question 27

Presentation of ulnar nerve palsy

Answer 27

C8 / T1 Causes: Supra-condylar fracture, compression cubital tunnel Sensory: Hypothenar eminence Motor: Claw hand, weak flexion, intrinsic muscles

Question 28

Presentation of Erb's palsy

Answer 28

C5-8, T1. Brachial plexus injury "Tip the waiter" Causes: shoulder dystocia Sensation - loss over seargent's patch Motor - limp, adducted, internally rotated shoulder, extended elbow, pronated wrist

Question 29

Presentation of CNIII palsy

Answer 29

"Down and out" Ptosis Dilated pupil, absent pupillary reflex

Question 30

Presentation of CNIV palsy

Answer 30

Double vision, improved by tilting head to opposite side --> difficulty reading booking / going down stairs Prob with superior oblique

Question 31

Presentation of CNVI palsy

Answer 31

Eye inwards Prob with lateral rectus

Question 32

Presentation of CN VII

Answer 32

Bell's palsy Weak muscles of facial expression Bell's= UMN --> forehead sparing Horner syndrome = SNS via CN VII - Ptosis, miosis and anhidrosis

Question 33

Presentation of myasthenia gravis

Answer 33

Weakness of skeletal muscles Fatiguability on exertion Bulbar muscle weakness and weakness head extension / flexion ACh receptor antibody

Question 34

Treatment of myasthenia gravis

Answer 34

Thymectomy Immune modulating agents