Neurology

Question 1

what is Gower sign and how to conduct it

Answer 1

Lay child supine and ask child to stand

- shows proximal muscle weakness - first turn prone then “walk up” legs with the hands

Question 2

types and causes of tremors

Answer 2

• essential (initiation and maintenance of posture): AD inheritance or thyrotoxicosis, phaeochromocytoma, wilson
• intention (end of mvt): wilson
• static (at rest, disappears with mvt): wilson, parkinson, huntington

Question 3

what is myoclonus

- causes

Answer 3

• Brief, sudden muscle contractions

* Seen in seizure disorders, metabolic disorders, brain infections, brain injury and degenerative conditions

Question 4

what is chorea

- causes

Answer 4

• Random rapid movements
• Seen most commonly in cerebral palsy, Sydenham’s chorea, Wilson’s and Huntington’s disease
• Due to damage to the corpus striatum

Question 5

what is athetosis

- causes

Answer 5

• Slow writhing movements
• Seen in cerebral palsy and Wilson’s disease
• Due to damage to the putamen

Question 6

what is dystonia

- causes

Answer 6

• Sustained disturbed muscle contraction causing abnormal posturing
• Seen with certain drugs (anticonvulsants), trauma, infections, and vascular, metabolic and degenerative pathologies

Question 7

what is hemiballismus

- causes

Answer 7

• Random gross proximal limb flailing

* Due to contralateral subthalamic brain damage

Question 8

what is tics

- causes

Answer 8

• Spasmodic stereotypic involuntary repetitive movements, typically of the facial movements
• Gilles de la Tourette is an inherited form
Associated vocal tics, obsessive-compulsive disorder and attention deficit hyperactivity disorder

Question 9

how does CN 4 palsy present

Answer 9

• dipopia when looking down and in

- difficulty when walking downstairs

Question 10

signs of cerebellar dysfunction

Answer 10

Truncal ataxia, dysarthric speech, horizontal nystagmus, intention tremor, dysdiadokinesia, dysmetria

Question 11

secondary causes of headache

Answer 11

• trauma
• vascular malformation
• non vasc intracranial malformation
• substances and withdrawal
• infection
• metabolic disorder
• facial pain (cranium, neck, eyes, ears, nose, sinus, teeth, mouth)
• cranial neuralgia

Question 12

triad of hypertensive encephalopathy

Answer 12

• headache
• seizures
• visual impairment

Question 13

cushing triad

Answer 13

raised ICP

• hypertension
• bradycardia
• irregular breathing

seen in the terminal stages of acute head injury and may indicate imminent brain herniation

Question 14

warning signs for headache

Answer 14

	First and worst headache
	Signs and symptoms of raised intracranial pressure
	Aggravation by valsalva maneouvre
	Abnormal neurological findings
	Papilloedema
	Chronic progressive headache, with change in behaviour and deteriorating academic performance
	Early morning vomiting
	Headache waking up patient from sleep
	Immunosuppressed patients
	Neurocutaneous syndromes
	Age < 4 years

Question 15

what is seen on CT for headaches from sinusitis

and what is the tx

Answer 15

 Total opacification, mucosal thickening and air-fluid levels are seen on CT/MRI
 Treat with prolonged antibiotics, short-term decongestants and surgical drainage of sinuses – rarely

Question 16

Precipitiating causes of migraines

Answer 16

anxiety, fatigue, mild head trauma, stress, exercise, excitement, travel, menses, some dietary factors and illness.

Question 17

what are childhood periodic syndromes

Answer 17

early life expression of genes later expressed as migraine

• cyclical vomitting syndrome
• abdominal migraine
• benign paroxysmal vertigo of childhood
• benign paroxysmal torticolis

Question 18

Mgx of migraine

Answer 18

Non pharm
	Reassurance
	Elimination of trigger factors
	Stress relief/stress management
	Regular diet
	Sufficient sleep
	Biofeedback
	Relaxation techniques
	Counseling

Pharm - abortive
 Single analgesic – NSAIDs, ergotamine compounds, triptans
 Adjunctive – anti-emetics

Pharm - prophylactic
	Cyproheptadine 
	Beta-blockers – propranolol
	Valproate
	Amitriptyline
	Flunarizine

Question 19

When to consider migraine prophylaxis

Answer 19

Considered if headaches occur more than four times per month and require substantial medication for relief.

Question 20

Medical problems associated with seizures

Answer 20

 Neurocutaneous syndromes (NF-1, TS, S-W)
 Cerebral palsy
 Neurodegenerative syndromes (Rett’s, Lennox-Gastaut)
 Past hx of organic brain disease: brain tumour, head trauma, meningitis/encephalitis/brain abscess
 Inborn errors of metabolism

Question 21

What is epilepsy

Answer 21

Epilepsy is recurrent seizures (>2 more than 24 hours apart) unrelated to fever or acute cerebral insult

Question 22

causes of seizures

Answer 22

• Birth or gestational insults
• Congenital malformations
• CNS infections
• Metabolic disorders
• Trauma and bleeding
• Degenerative diseases
• Neoplasms
• Toxins

Question 23

what are some epilepsy syndromes you know

Answer 23

• infantile spasms
• lennox-gastaut syndrome
• childhood absence epilepsy
• juvenile myoclonic epilepsy
• benign rolandic epilepsy

Question 24

Infantile spasm

• age range
• EEG features
• associations
• tx

Answer 24

4-6 months onset, boys>girls
EEG: hypsarrhythmias

associated with: tuberous sclerosis; metabolic disease, birth injury e.g. IVH, HIE; postnatal injury e.g. trauma, infections

tx: vigabatrin or steroids

Question 25

What is west syndrome

Answer 25

triad of: (i) infantile spasms; (ii) hypsarrhythmias; and (iii) mental retardation

Question 26

What is Lennox -gastaut syndrome

Answer 26

electroclinical syndrome: - multiple seizure types - EEG showing slow spike and wave

Question 27

Childhood absence epilepsy - onset age - triggers - tx

Answer 27

onset 3-10 years, girls>boys induced by hyperventilation, emotion, hunger tx: valproate, carbamazepine, lamotrigine

Question 28

What is juvenile myoclonic epilepsy - onset - EEG - tx

Answer 28

12-16 years, girls>boys myoclonic jerks, worse in the morning, no loss of consciousness EEG: shows normal background, 4-6 per sec irregular polyspike and wave discharge pattern. Photosensitivity is a feature Tx: valproate, lamotrigine

Question 29

Benign rolandic epilepsy - prognosis - onset - features - EEG - tx

Answer 29

GOOD prognosis, will spontaneously resolve in mid teens onset 2-14 years features: drooling, abnormal sensations in the mouth; secondary generalisations. 75% occur in sleep, 25% occur on waking EEG: repetitive spike focus in the Rolandic area (centrotemporal) Tx: may not need treatment due to spontaneous resolution; carbamazepine may help

Question 30

First aid advice for seizure

Answer 30

(i) Lie the child on the side. (ii) Do not put things in the child’s mouth. (iii) Move away any nearby objects to avoid hurting himself or herself. (iv) Have a time plan: if a seizure lasts longer than 5mins, call an ambulance.

Question 31

SE of - carbamazepine - valproate

Answer 31

(i) Carbamazepine – neutropaenia (screen FBC), nystagmus, ataxia (ii) Valproate – thrombocytopaenia (screen FBC), hepatitis (screen LFT)

Question 32

Causes of status epilepticus

Answer 32

 Sudden withdrawal of anticonvulsants in a known epileptic  Bad epileptic syndrome e.g. West, Lennox-Gastaut Sudden withdrawal of anticonvulsants  Acute infection (CNS or systemic)  Acute insult to the brain  Brain tumour

Question 33

Medications for status epilepticus

Answer 33

IV glucose and IV thiamine if hypoglycaemic IV benzodiazepine (diazepam or lorazepam): 0.25mg/kg at 1mg/min up to 10mg (up to 0.3mg/kg) OR Rectal valium if no IV access (<10kg –2.5mg; >10kg – 5mg) IV Phenytoin 20mg/kg at 1mg/kg/min (up to 30mg/kg) IV phenobarbital 20mg/kg if still persist: GA/ barbiturate coma with EEG monitoring

Question 34

Complications of status epilepticus

Answer 34

```  Tissue anoxia – cerebral anoxia  Brain oedema  Circulatory shock with renal failure  Respiratory failure  Acute rhabdomyolysis  Severe acidosis  Hyperpyrexia  Permanent neurological damage (20-50%)  Death (8-33%) ```