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
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
3
Q
Infantile spasm
- tx
- side effects
A
- vigabatrin - retinal toxicity (esp for TSc)
2. ACTH/prednisolone (wt gain, HTN, irritability, adrenal suppression)
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
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
6
Q
Childhood absence epilepsy
- features
A
4-10 yrs (peak 5-7 yrs)
- blank stare 5-30 seconds
- 75% complete remission by adolescence
7
Q
Absence epilepsy
- EEG
- treatment
A
EEG: 3Hz spike and wave
Tx: ethosuxamide, valproic acid (or lamotrigine)
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
9
Q
Benign rolandic epilepsy
- EEG
- Tx
A
EEG: bilateral centrotemporal sharp waves
Tx: many do not require treatment
- if required: keppra, carbamazepine
10
Q
Idiosyncratic reactions to AEDs:
- keppra
A
- aggression/rage (20%)
- suicidality (rare)
11
Q
Idiosyncratic reactions to AEDs:
- carbamazepine
A
- rash, hepatitis, anemia
12
Q
Idiosyncratic reactions to AEDs:
- topiramate
A
- kidney stones
- weight loss
13
Q
Idiosyncratic reactions to AEDs:
- lamotrigine
A
- rash (5%)
14
Q
Idiosyncratic reactions to AEDs:
- VPA
A
- weight gain
- hepatitis
- pancreatitis
- thrombocytopenia
- rash
- hair loss
15
Q
Phenytoin side effects
A
= gum hypertrophy
- don’t mix with dextrose (crystallizes)
- may use for status
16
Q
First line focal epilepsy meds
A
- keppra
- carbamazepine/ oxcarbamazepine
17
Q
First line generalized epilepsy meds
A
- keppra
- lamotrigine
- VPA
- topiramate
- ethosuximide (absence only)
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
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
20
Q
Motor neuron disease
e. g. spinal muscular atrophy
- muscles
- reflexes
- bulbar involvement
A
- muscle wasting (proximal>distal)
- early areflexia
- +/- bulbar swallowing weakness
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
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
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
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
