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Royal Super 4th Year Brain > Neurology > Flashcards

Neurology Flashcards

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1
Q

First line treatment of infantile spasms

A

Vigabatrin (in Canada)
ACTH (in USA)
Prednisolone (used at ACH)

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2
Q

Best treatment for infantile spasms in patient with tuberous sclerosis

A

Vigabatrin

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3
Q

Most important side effect of vigabatrin

A

Retinal toxicity

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4
Q

Investigations to consider for frequent sleepwalking and/or safety concerns

A

PSG

Ferritin

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5
Q

If safety/QOL concerns with sleepwalking in absence of underlying trigger, what treatment to consider

A

Benzo

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6
Q

Two types of breath holding spells and what investigation to consider

A

Cyanotic and pallor

Serum ferritin

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7
Q

CSF abnormality in anti-NMDA receptor encephalitis

A

Lymphocytic pleocytosis

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8
Q

First line treatment for pituitary adenoma

A

Dopamine agonist

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9
Q

VPA side effects (x3)

A

Hepatoxicity
Thrombocytopenia
Pancreatitis

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10
Q

You must look for _____ in all presentations of opsoclonus-myoclonus syndrome. What investigation would you start with first?

A

Neuroblastoma

Abdo ultrasound

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11
Q

What gene mutation are you looking for in Rett Syndrome?

A

MECP2 gene mutation

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12
Q

Newborn with arm paralysis and Horner Syndrome

A

Total brachial plexus palsy

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13
Q

Duchenne Muscular Dystrophy - x2 important physical exam findings

A

+Gower’s sign

Calf pseudo-hypertrophy

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14
Q

Duchenne Muscular Dystrophy

a) age of onset
b) age of loss of ambulation

A

a) 2 years old

b) 12 years old

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15
Q

Difference between DMD and BMD from a pathophysiology perspective?

A

DMD - complete absence of dystrophin

BMD - inactivation and decreased production of dystrophin

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16
Q

Systemic manifestations of DMD?

A 
CNS: Intellectual impairment
CVS: Cardiomyopathy
RESP: Impaired pulmonary function
GI: Decreased gastric motility
MSK: Fractures, progressive scoliosis
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17
Q

Classic GI infection leading to Guillain Barre Syndrome as well as the first line treatment

A

Campylobacter jejuni

IVIG

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18
Q

What medication can you give to patients with behavioural side effects secondary to Keppra?

A

Pyridoxine (vitamin B6)

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19
Q

Major risk factors for recurrence of febrile seizures

A

Age <1 years, fever duration <24 hours, fever 38-39deg

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20
Q

Erb’s Palsy - distribution

A

C5-C6 injury

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21
Q

Klumpke’s paralysis - distribution

A

C7-T1 injury

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22
Q

EEG finding for Benign Rolandic Epilepsy

A

Centrotemporal spikes

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23
Q

Causes of thunderclap headache

A
  • Vascular: SAH, CVST, stroke, RCVS
  • Structural: third ventricular colloid cyst, 3/4th ventricle tumour
  • Infectious: meningitis, encephalitis
  • Primary headache
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24
Q

What type of epilepsy would you think about in a child with febrile status epilepticus?

A

Dravet Syndrome

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25
Q

Criteria for a Complex Febrile Seizure

A
  • Age <6 months or >5 years
  • 15+ minute duration
  • Focal
  • Multiple in 24h
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26
Q

What criteria should you use for Sydenhams?

A

Jones Criteria

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27
Q

Major Jones criteria

A 
Carditis
Arthritis
Chorea
Erythema marginatum
Subcutaneous nodules
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28
Q

What is the diagnosis if a child with GAS infection followed by OCD and tics?

A

Sydenhams

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29
Q

Genetic transmission of Huntington’s

A

Autosomal dominant

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30
Q

Difference between OCD and tics and stereotypies?

A

OCD - or else something bad is going to happen
Tic - feel an urge
Stereotypies - occur when they are excited

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31
Q

Criteria for Tourette Syndrome

A
  • Multiple motor
  • At least 1 vocal tic
  • Occurs for >1 year
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32
Q

What should you screen for when adolescent comes in with tics?

A

Substances (Substance induced tic disorder)

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33
Q

What are the first line medications for tics (motor/vocal)? What class of meds would you consider next?

A

Clonidine, guanfacine

Then anti-psychotics

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34
Q

Ataxia telangiectasia - what two things can you see in addition to ataxia and derm

A

Tumors + sinopulmonary infections

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35
Q

Most common cause of stroke in neonates

A

Embolic - placenta, cardiac (PFO, other CHD), umbilical catheter

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36
Q

Three most common causes of childhood ischemic stroke

A
  • Arteriopathy (Sickle cell, arterial dissection, moya moya)
  • Cardiac disease (CHD)
  • Thrombophilia
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37
Q

Two most common causes of childhood hemorrhagic stroke

A
  • Structural (aneurysm, AVM, tumor)

- Hematologic (hemophilia)

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38
Q

Primary + secondary prevention for SCD related strokes

A

Primary - hydroxyurea

Secondary - transfusions

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39
Q

Secondary causes of Moya Moya

A

Sickle cell, TB meningitis, cranial radiation, NF1, Turner Syndrome, T21, CHD, PHACE, Alagille syndrome

40
Q

Migraine criteria

A

A. At least 5 attacks fulfilling B-D within last year
B. Headache attacks lasting 1-72 hours - when untreated
C. At least 2 characteristics: a. Unilateral location (for children can be bilateral), b. Pulsating quality, c. Moderate to severe intensity, d. Aggravation by or causing avoidance of routine physical activity
D. During headache at least one: a. Nausea and/or vomiting, b. Photo AND phonophobia
E. Not better explained by another diagnosis

41
Q

x2 most common causes of ataxia in a healthy child

A

Drug ingestion

Acute post-infectious cerebellitis

42
Q

Normal LP opening pressure (by age range) and when to be concerned for intracranial HTN

A
  • Normal: Neonate (8-10), 1m-4y (1-10), 8y-adol (10-20)

- Concerning: >25

43
Q

Ddx for toe walking (x7)

A
  • CP
  • Congenital shortening of Achilles
  • Spinal dysraphism
  • Equinovarus deformity
  • Intraspinal + filum terminale tumor
  • Hereditary/acquired polyneuropathy
  • Muscular dystrophy
44
Q

Definition of status epilepticus

A

= continuous generalized tonic-clonic seizure activity with LOC for longer than 30 minutes
= OR >2 discrete seizures without return to baseline mental status

45
Q

Pre-hospital medication given for seizure lasting >5 minutes

A

IN midaz 0.2 mg/kg (max 5mg/nare)

46
Q

In-hospital first-line medication for seizure + when to move on to second-line

A
  • First-line: IV midaz/loraz 0.1 mg/kg (max 4-5mg)
  • Next: Can given 1st line again 5 min later. Once at least 2 doses of benzo’s have been given (including pre-hospital) then move on to 2nd line
47
Q

Second line options for status epilepticus (including dosing)

A
  • Fospheny 20mg/kg
  • Phenytoin 20mg/kg
  • Keppra 60mg/kg
  • Phenobarb 20mg/kg
48
Q

Next steps to consider when giving second line options for status epilepticus

A
  • Can given a second dose of 2nd line - do NOT mix pheny with fospheny
  • If 2 or more doses of second line, then consult specialty services and consider alternative airway
  • Consider dose of pyroxidine 100mg IV for infants <18 months
49
Q

Presentation of increased ICP in infants vs children

A
  • Infants = macrocephaly, splayed sutures, apnea, bradycardia, desats, sundowning, bulging fontanel, irritable, shrill cry, FTT
  • Children = AM/night-time headache, emesis, aLOC, anorexia, papilledema, CN 3 + 6 palsy (diplopia), personality difference
50
Q

Idiopathic intracranial HTN - symptoms, exam, and investigations

A
  • Symptoms: HA, fatigue, N/V, anorexia, stiff neck, diplopia
  • Exam: papilledema, 3rd + 6th nerve palsy, visual field constriction
  • Ix: normal CT head, elevated opening pressure, normal CSF profile
51
Q

Idiopathic intracranial HTN - etiologies

A
  • 90% idiopathic
  • Drugs: tetracycline, nitrofurantoin, CC’s, excess vitamin A
  • Endo: obesity, hyperthyroidism, Cushing syndrome, hypoparathyroidism
  • Thrombosis: of dural venous sinuses from head trauma, AOM, mastoiditis, obstruction of jugular veins in SVC syndrome
52
Q

What AED can make absence seizures worse?

A

-Carbazepine

53
Q

What AEDs (x3) to consider for absence seizures?

A

Ethosuximide, VPA, lamotrigine

54
Q

Prognosis for absence epilepsy

A

75% will have a complete remission by adolescence

55
Q

What is the most common epilepsy syndrome?

A

Benign epilepsy with centro-temporal spikes (Rolandic)

56
Q

x2 AED’s not to use for primary generalized epilepsy?

A
  • Carbazepine (will worsen)

- Phenytoin (could worsen)

57
Q

Why do we not use phenytoin or fosphenytoin as a routine anticonvulsant?

A

Gum hypertrophy

58
Q

Red flags for a secondary headache

A
  • Thunderclap
  • Occipital/valsalva = Chiari I
  • Wakening in AM, papilledema, diplopia = inc ICP, intracranial hypertension
  • With standing = spontaneous intracranial hypotension
  • Recent head injury
  • Known coagulopathy/thrombophilia
  • New headache + risk of thrombosis (SLE, nephrotic syndrome)
  • Pituitary symptoms = short stature+obesity, new bed wetting, vision loss
59
Q

Characteristics of a medication overuse headache

A
  • Daily or near daily (>15 days per month)
  • Present upon wakening
  • Relieved by medication
  • Recurs later in the day
60
Q

How much medicine is required to lead to medication overuse headaches?

A
  • NSAIDs or tylenol >15x/month for at least 3 months

- Triptans >10x/month for at least 3 months

61
Q

Most common type of Spinal Muscular Atrophy?

A

Type 1

62
Q

Best gross motor developmental milestones achieved by each type of SMA

A
  • Type 1: Never sit
  • Type 2: Sit but never walk
  • Type 3: Can walk
63
Q

Typical timeline for symptom onset amongst SMA types

A
  • Type 1: <6 months
  • Type 2: 6-18 months
  • Type 3: >18 months
64
Q

How many SMN2 copy numbers for each SMA type?

A
  • Type 1: 2
  • Type 2: 3
  • Type 3: 4
65
Q

What to think of for hammer toes

A

Charcot Marie Tooth

66
Q

Presentation for Charcot Marie Tooth

A
  • Delayed GM milestones
  • Tight heel cords (clumsy, toe walker)
  • Hypotonia
  • Weakness
  • Self-mutilation
67
Q

Exam findings for Charcot Marie Tooth

A
  • Abnormal gait + clumsiness
  • Foot deformities (pes cavus, hammer toes)
  • Weakness
  • Hypotonia
  • Foot drop
  • Evidence of self-mutilation
68
Q

First line testing for Charcot Marie Tooth

A

Genetic testing

69
Q

Difference between muscular dystrophy and congenital myopathy?

A
  • Dystrophy = problem with muscle fibre integrity = high CK

- Myopathy = abnormal function of muscle = normal CK

70
Q

Who is cognitively normal (a) congenital myotonic dystrophy or (b) congenital myopathy?

A

B

71
Q

Muscle disorder with anticipation

A

Congenital myotonic dystrophy

72
Q

3 types of congenital myopathies and the main features

A
  1. Nemaline rod: bulbar dysfunction
  2. Central core: malignant hyperthermia
  3. Centronuclear: eye + face weakness
73
Q

adenomatous sebaceum - what dx and other name?

A

TSC

Angiofibromas

74
Q

What intracranial finding is spastic diplegia associated with?

A

Bilateral PVL

75
Q

What GA timeframe does PVL occur in?

A

24-32 weeks

76
Q

x2 unique physical exam features for myotonic dystrophy?

A
  • grip myotonia = clenched fist, then try to relax but takes several seconds to do so
  • percussion myotonia = to thenar prominence will cause flexion of hand
77
Q

What to be concerned about if patient having seizures, hypertension, oral facial movements, emotional lability/confusion?

A

Anti-NMDA encephalitis

-Orofacial dyskinesias

78
Q

What does dopamine agonist do for pituitary adenoma?

A

Decrease prolactin

Decrease size of adenoma

79
Q

Criteria for low risk BRUE

A
  • > 2 months old
  • Not less than 32 weeks GA
  • Not more than one event
  • No CPR
  • Event <1 min
  • No concerns on history or exam
80
Q

What investigation is important to do for neuroblastoma?

A

Urine VMA + HVA

81
Q

Dx for myoclonus (+/- generalized involvement) typically occurring in the morning?

A

Juvenile Myclonic Epilepsy

82
Q

What kind of neurological presentation is botulism + what scary presentation typically happens first?

A
  • Symmetric, descending, flaccid paralysis

- Begins with CN involvement with bulbar palsies = poor feeding, poor suck, drooling, and weak cry

83
Q

What should you give for a patient with Guillain Barre and what should you definitely not give?

A
  • Give IVIG

- Do NOT give steroids

84
Q

What kind of neurological presentation is Guillain Barre?

A

-Areflexic, ascending, flaccid paralysis

85
Q

What is the most specific indicator for neonatal seizures?

A

Eye movements

86
Q

What would be on the differential for a neuromuscular blockade type presentation similar to myasthenia gravis in a 6-12 month old?

A

Botulinism

87
Q

What happens to pupils in botulinism?

A

Mydriasis - poor reactivity

88
Q

Risk factors for recurrence for a child with first presentation of a febrile seizure - Major + Minor

A

Major:

  • Age <1yr
  • Fever duration <24hr
  • Fever 38-39 degrees

Minor:

  • Family history of febrile sz or epilepsy
  • Complex febrile sz
  • Day care
  • Male
  • Low serum sodium
89
Q

What reflex is intact for a child with Erb’s palsy?

A

Grasp reflex

90
Q

When do we get worried about a sacral dimple and consider U/S?

A
  • Multiple dimples
  • Diameter >5mm
  • > 2.5cm above anus
  • Dimple outside sacrococcygeal region
  • Cannot see the bottom
  • Leaking fluid, hair, or colour change
91
Q

In terms of etiology, what do you think about hemiplegia CP vs spastic diplegia CP?

A

(a) perinatal insult

(b) prematurity, PVL

92
Q

When does regression of development occur for patients with adrenoleukodystrophy?

A

~4 years of age

93
Q

Shape for intracranial bleeds - (a) epidural and (b) subdural

A

(a) lens

(b) crescent

94
Q

x2 treatments for GBS - +1 tx NOT to use

A

IVIG
Exchange transfusion
NOT steroids

95
Q

Bells palsy tx - with doses

A

pred 2mg/kg x5 days then taper

96
Q

Basilar migraine sx

A
  • Diplopia
  • Vertigo
  • Tinnitus
  • Bilateral paresthesias
  • Dysarthria

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