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Neurology Flashcards

(56 cards)

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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

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

Charcot-Marie-tooth disease

- features

Study These Flashcards
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
Congenital myopathies vs. muscular dystrophies
Myopathies: normal CK, gene mutation impairing contractile unit e.g. nemaline rod myopathy Dystrophies: gene mutation impairing muscle fiber integrity/stability, high CK
26
Congenital myopathies features
- 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
Duchenne Muscular dystrophy | - features
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
Tic disorder timeline for dx
- 12 months! | provisional is < 12 months
29
Risk factor for seizure relapse after discontinuation of medication
- 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
Botulism classic triad
1. acute onset of symmetric, flaccid, descending paralysis 2. clear sensorium 3. no fever
31
Tx botulism
- human botulism immune globulin given IV - supportive care - avoid complications
32
Bells palsy manifestations
- 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
Bells palsy - tx - prognosis
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
conversion disorder management
- cognitive behavioural interventions - rehabilitation model - outpatient setting with appropriate mental health follow up - intensive psych tx if profound and pervasive
35
Afebrile Seizure risk of recurrence
~ 30% go on to develop later epilepsy
36
Febrile seizure features
- age 6 to 60 months - 38C or higher - NOT related to metabolic or CNS infection - in ABSENCE of prior afebrile seizures
37
Simple vs. complex febrile seizures
Simple: Generalized, last a max of 15 minutes, NOT recurrent within a 24hr period Complex: recurs, prolonged, focal
38
Febrile seizure recurrence
- 30% with first febrile seizure - 50% after 2+ episodes - 50% for infants < 1 yr
39
Guillain Barre Syndrome | - presentation
- ascending flaccid paralysis - often 2-4 weeks after an infection - begin with paresthesias
40
GBS CSF findings
- elevated protein | - normal WBC count
41
GBS treatment
- plasmapheresis - IVIG - close monitoring and supportive care need for assisted intubation if vital capacity <20mL/kg, max inspiratory pressures 0 to -30
42
Migraine with brainstem aura | - brainstem sx
- dysarthria - vertigo - tinnitus - hypacusis - diplopia - ataxia - decreased LOC
43
Complications of untreated IIH
- optic atrophy | - blindness
44
Investigations for myasthenia gravis
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
Infants born to myasthenic mothers | - features
- grow out of it as abN antibodies disappear - poor suck, resp insufficiency, hypotonia, weakness - fetal akinesia --> arthrogryposis
46
Recommended normal sleep amounts - newborn (0-2mo) - infant (2-12mo) - toddler - preschool - middle childhood - teen
``` Newborn: 10-19hrs Infant: 12-13hr Toddler: 11-13hr Preschool/middle childhood: 9-11 Adolescence: 9hrs ```
47
Night terrors features
- most often age 2-7yrs - screams, terrified, thrashing - difficult to arouse - occurs a few hrs after going to sleep
48
Partial arousal parasomnias
e. g. sleepwalking, night terrors, sleep talking, confusional arousals - first 3rd of night when slow wave sleep predominates - aggravated by caffeine, sleep restriction
49
Genetics - SMA - DMD - Myotonic dystrophy - congenital muscular dystrophy
1. SMA: AR 2. DMD: X-linked 3: Myotonic dystrophy: AD, CTG trinucleotide repeat expansion 4. mostly AR
50
tone, reflexes and CK 1. SMA 2. DMD 3. Myotonic dystrophy 4. congenital muscular dystrophy
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
optic neuritis and MS
- presenting feature of MS in 15-20% of pts | - occurs in 50% at some time during the course of illness
52
Retina findings: 1. ROP 2. Tay-Sachs 3. abusive head trauma 4. Chorioretinitis
1. ROP: proliferation of tortuous retinal blood vessels 2. T-S: cherry red spot 3. Retinal hemorrhages 4. atrophic scarring demarcated by pigmentation
53
Tic disorder requirements
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
Management for tics
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
Acute myelitis vs. GBS - motor findings - sensory findings - CN findings
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
Acute myelitis vs. GBS - autonomic findings - MRI findings - CSF
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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