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Neurology Flashcards

1
Q

Breath holding spells

  • features
  • types
  • tx
A
  • 6-18 months olds
  • last 10-60 seconds
    1. pallid = limp and pallor after an injury (vagal nerve mediated)
    2. cyanotic = angry, apnea, cyanosis, crying (hyperventilation mediated)
    Tx = reassure and consider iron supplement
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2
Q

Infantile spasm

  • age of onset
  • features
  • EEG
A
  • peak onset 4-7 months old (95% < 12 mo)
  • 1 sec (clusters), neck flex, arms extends
    1/3 = no underlying cause
    EEG: hypsarrhtyhmia
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3
Q

Infantile spasm

  • tx
  • side effects
A
  1. vigabatrin - retinal toxicity (esp for TSc)

2. ACTH/prednisolone (wt gain, HTN, irritability, adrenal suppression)

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4
Q

Benign myoclonus of infancy

- features

A
  • peak < 2 yrs
  • flexion of trunk and head lasting 1-2 sec or “vibratory flexion of neck”
  • normal EEG, normal exam
  • spontaneous remission by 5 yrs
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5
Q

Benign sleep myoclonus of infancy

- features

A

< 10 months old (most term to 3 months)
= discreet limb jerks during sleep only
EEG is normal

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6
Q

Childhood absence epilepsy

- features

A

4-10 yrs (peak 5-7 yrs)

  • blank stare 5-30 seconds
  • 75% complete remission by adolescence
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7
Q

Absence epilepsy

  • EEG
  • treatment
A

EEG: 3Hz spike and wave
Tx: ethosuxamide, valproic acid (or lamotrigine)

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8
Q

Benign rolandic epilepsy

- features

A
  • range 1-14 yrs (peak 7-10)
  • most common epilepsy syndrome 15-25%
  • seizures usually 2-3 minutes
  • nocturnal, focal, face, tongue
  • may spread to GTC seizure
  • most outgrow during puberty
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9
Q

Benign rolandic epilepsy

  • EEG
  • Tx
A

EEG: bilateral centrotemporal sharp waves
Tx: many do not require treatment
- if required: keppra, carbamazepine

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10
Q

Idiosyncratic reactions to AEDs:

- keppra

A
  • aggression/rage (20%)

- suicidality (rare)

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11
Q

Idiosyncratic reactions to AEDs:

- carbamazepine

A
  • rash, hepatitis, anemia
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12
Q

Idiosyncratic reactions to AEDs:

- topiramate

A
  • kidney stones

- weight loss

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13
Q

Idiosyncratic reactions to AEDs:

- lamotrigine

A
  • rash (5%)
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14
Q

Idiosyncratic reactions to AEDs:

- VPA

A
  • weight gain
  • hepatitis
  • pancreatitis
  • thrombocytopenia
  • rash
  • hair loss
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15
Q

Phenytoin side effects

A

= gum hypertrophy

  • don’t mix with dextrose (crystallizes)
  • may use for status
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16
Q

First line focal epilepsy meds

A
  • keppra

- carbamazepine/ oxcarbamazepine

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17
Q

First line generalized epilepsy meds

A
  • keppra
  • lamotrigine
  • VPA
  • topiramate
  • ethosuximide (absence only)
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18
Q

Migraine diagnostic criteria

A

A. at least 5 attacks
B. headache 1-72hr
C.2+ of:
- unilateral or bilateral (not occipital)
- pulsing
- mod-to-severe
- worse with activity
D. 1+ of: nausea, photophobia AND phonophobia
E. cannot be explained by another disorder

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19
Q

Headache red flags

A
  • thunderclap (sentinel bleed)
  • occipital (chiari 1 malformation)
  • pituitary sx
  • H/A awakening from sleep
  • focal neuro deficits + papilledema
  • head injury and/or coagulopathy
  • new headache and risk of thrombosis
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20
Q

Motor neuron disease

e. g. spinal muscular atrophy
- muscles
- reflexes
- bulbar involvement

A
  • muscle wasting (proximal>distal)
  • early areflexia
  • +/- bulbar swallowing weakness
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21
Q

Peripheral nerve disease e.g. charcot-marie tooth

  • muscles
  • reflexes
  • other
A
  • muscle wasting (distal > proximal)
  • early areflexia
  • sensory loss, painless injury, sulf-mutilation
  • foot defomities
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22
Q

Muscular disease e.g. muscular dystrophy

  • muscles
  • reflexes
  • bulbar
  • CK
A
  • pseudohypertrophy (wasting less prominent)
  • late areflexia
  • myopathic facies
    +/- bulbar/swallowing weakness
  • elevated CK
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23
Q

SMA genetics

A
  • AR
  • 95% have a deletion
  • < 5% have other gene issue
  • SMN1gene
24
Q

Charcot-Marie-tooth disease

- features

A

Infant: delayed GM milestone, “toe-walker”, clumsy gait, hypotonia, weakness, self-mutilation
Children: abN gait and clumsiness, foot deformities (pes cavus, hammertoes) , weakness and foot drop

25
Q

Congenital myopathies vs. muscular dystrophies

A

Myopathies: normal CK, gene mutation impairing contractile unit e.g. nemaline rod myopathy
Dystrophies: gene mutation impairing muscle fiber integrity/stability, high CK

26
Q

Congenital myopathies features

A
  • e.g. nemaline rod myopathy
  • cognitively normal
  • phenotypic variability
  • muscle weakness is non-progressive
  • may show very slow gain in strength
  • diagnosis by genetic testing
27
Q

Duchenne Muscular dystrophy

- features

A

X-linked

  • progressive, proximal muscle weakness
  • sx onset ~4-6 yo
  • steroids prolong ambulation, preserve resp function, slow scoliosis
  • life expectancy mid to late 20s
28
Q

Tic disorder timeline for dx

A
  • 12 months!

provisional is < 12 months

29
Q

Risk factor for seizure relapse after discontinuation of medication

A
  • abN EEG before discontinuation
  • remote, structural epilepsy
  • older age of epilepsy onset
  • longer duration of epilepsy
  • presence of multiple seizure types
  • need to use more than 1 AED
30
Q

Botulism classic triad

A
  1. acute onset of symmetric, flaccid, descending paralysis
  2. clear sensorium
  3. no fever
31
Q

Tx botulism

A
  • human botulism immune globulin given IV
  • supportive care
  • avoid complications
32
Q

Bells palsy manifestations

A
  • paretic upper and lower portions of face
  • corner of mouth droops
  • unable to close eye on involved side
    +/- taste on anterior 2/3 of tongue is lost
33
Q

Bells palsy

  • tx
  • prognosis
A

tx: pred x 1 week then 1 week taper
- + acyclovir/ valacyclovir to be considered
>85% recover sponateneously
10% have mild facial weakness
5% = severe, permanent

34
Q

conversion disorder management

A
  • cognitive behavioural interventions
  • rehabilitation model
  • outpatient setting with appropriate mental health follow up
  • intensive psych tx if profound and pervasive
35
Q

Afebrile Seizure risk of recurrence

A

~ 30% go on to develop later epilepsy

36
Q

Febrile seizure features

A
  • age 6 to 60 months
  • 38C or higher
  • NOT related to metabolic or CNS infection
  • in ABSENCE of prior afebrile seizures
37
Q

Simple vs. complex febrile seizures

A

Simple: Generalized, last a max of 15 minutes, NOT recurrent within a 24hr period
Complex: recurs, prolonged, focal

38
Q

Febrile seizure recurrence

A
  • 30% with first febrile seizure
  • 50% after 2+ episodes
  • 50% for infants < 1 yr
39
Q

Guillain Barre Syndrome

- presentation

A
  • ascending flaccid paralysis
  • often 2-4 weeks after an infection
  • begin with paresthesias
40
Q

GBS CSF findings

A
  • elevated protein

- normal WBC count

41
Q

GBS treatment

A
  • plasmapheresis
  • IVIG
  • close monitoring and supportive care
    need for assisted intubation if vital capacity <20mL/kg, max inspiratory pressures 0 to -30
42
Q

Migraine with brainstem aura

- brainstem sx

A
  • dysarthria
  • vertigo
  • tinnitus
  • hypacusis
  • diplopia
  • ataxia
  • decreased LOC
43
Q

Complications of untreated IIH

A
  • optic atrophy

- blindness

44
Q

Investigations for myasthenia gravis

A

Clinical test: admin of short acting cholinesterase inhibitor e.g. neostigmine showing decreased fatigability
Labs:
- anti-AChR antibodies
- other autoimmune markers
- TSH, fT4, ANA, CK (normal)
- CXR
EMG: decremental response to repetitive nerve stim

45
Q

Infants born to myasthenic mothers

- features

A
  • grow out of it as abN antibodies disappear
  • poor suck, resp insufficiency, hypotonia, weakness
  • fetal akinesia –> arthrogryposis
46
Q

Recommended normal sleep amounts

  • newborn (0-2mo)
  • infant (2-12mo)
  • toddler
  • preschool
  • middle childhood
  • teen
A 
Newborn: 10-19hrs
Infant: 12-13hr
Toddler: 11-13hr
Preschool/middle childhood: 9-11
Adolescence: 9hrs
47
Q

Night terrors features

A
  • most often age 2-7yrs
  • screams, terrified, thrashing
  • difficult to arouse
  • occurs a few hrs after going to sleep
48
Q

Partial arousal parasomnias

A

e. g. sleepwalking, night terrors, sleep talking, confusional arousals
- first 3rd of night when slow wave sleep predominates
- aggravated by caffeine, sleep restriction

49
Q

Genetics

  • SMA
  • DMD
  • Myotonic dystrophy
  • congenital muscular dystrophy
A
  1. SMA: AR
  2. DMD: X-linked
    3: Myotonic dystrophy: AD, CTG trinucleotide repeat expansion
  3. mostly AR
50
Q

tone, reflexes and CK

  1. SMA
  2. DMD
  3. Myotonic dystrophy
  4. congenital muscular dystrophy
A
  1. SMA: severe hypotonia, absent reflexes, CK normal-100s
  2. DMD: progressive weakness prox>distal, reflexes begin to decrease, CK>10,000
  3. MyoD: distal muscle, mild facial wasting, reflexes preserved, CK normal -100s
  4. Cong MD: contractures, diffuse hypotonia, decreased or absent reflexes, CK 100-1000s
51
Q

optic neuritis and MS

A
  • presenting feature of MS in 15-20% of pts

- occurs in 50% at some time during the course of illness

52
Q

Retina findings:

  1. ROP
  2. Tay-Sachs
  3. abusive head trauma
  4. Chorioretinitis
A
  1. ROP: proliferation of tortuous retinal blood vessels
  2. T-S: cherry red spot
  3. Retinal hemorrhages
  4. atrophic scarring demarcated by pigmentation
53
Q

Tic disorder requirements

A

A. single or multiple vocal or motor (or both for Tourettes)
B. more than 1 year
C. onset before age 18 yrs
D. no other cause

if < 12 mo = provisional tic disorder

54
Q

Management for tics

A
  1. psychoeducation
  2. behavioural intervention: habit reversal therapy, classroom accomodations
  3. pharm: if severe impairment in QoL: alpha agonists, SSRI, antipsychotics e.g. haloperidol
55
Q

Acute myelitis vs. GBS

  • motor findings
  • sensory findings
  • CN findings
A

Acute myelitis: paraparesis or quadraparesis, sensory at a spinal cord level, NO CN findings
GBS: LE weaker than UE, sensory loss LE > UE, extra-ocular muscle palsies or facial weakness

56
Q

Acute myelitis vs. GBS

  • autonomic findings
  • MRI findings
  • CSF
A

Acute myelitis: early loss of bowel and bladder, +MRI findings, CSF pleocytosis and/or increased IgG index
GBS: autonomic dysfunction of CV system, MRI normal, elevated protein in absence of CSF pleocytosis

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