Neuro Flashcards

1
Q

What are the 3 different spinal cord tracts and how to test them?

A

Dorsal (posterior) column (sensory)
○ Proprioception, vibration, light touch
○ Ipsilateral findings
○ Testing: position of fingers and toes, vibration using tuning fork

Spinothalamic (sensory)
○ Pain, temperature, touch
○ Injuries show as contralateral findings for pain and temperature
○ Testing: pinprick sensation, temperature

Corticospinal tract (motor)
○ Controls motor function on same side
○ Ipsilateral findings
Testing: abnormal motor movements on same side of lesion

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2
Q

Lower motor neuron findings

A

Bulk: atrophy, fasciculations (muscle fibers twitching)
Tone: flaccid
Pattern: root/plexus/nerve distribution
Reflexes: absent/diminished, toes down
Root, nerve

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3
Q

Upper motor neuron findings

A

Bulk: normal
Tone: spastic (tight muscles)
Pattern: extensors affected more in arms, flexors more in legs
Reflexes: brisk (intense), up-going toes (Babinski reflex)
Cortex to spinal cord

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4
Q

opening pressure 90%ile ULN and 10%ile LLN

A

28cm H2O high

11.5 cm low

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5
Q

contraindications to LP

A

suspected mass lesion (esp posterior fossa)
suspected spinal cord lesion
signs of impending herniation
critical illness
skin infection over the site
Plt <20

*routine head imaging not needed unless signs of optic disc edema or focal signs suggesting mass lesion

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6
Q

Normal CSF

A

up to 5 WBC (up to 15 in newborns)

polymorphonuclear cells are always abnormal in a child (may have 1-2 in neonate)

xanthochromia suggests subarachnoid hemorrhage (can occur in hyperbilirubinemia, carotenaemia, or high CSF protein)

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7
Q

early signs of stroke on CT <24hr

A

CT isnt great for acute infarcts due to changes not being apparent in first 24 hr
subtle signs:
- sulcal effacement
- blurring of gray-white matter junction
- hyperdense MCA sign

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8
Q

brachial plexus injuries

A

C3-C4
-C3,C4,C5 keeps the diaphragm alive

C5-C6
-Biceps
-Erb’s palsy
-Pronate, retained grip strength

C7-C8
-Klumpke palsy
-Claw hand (can’t properly flex or abduct the fingers)

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9
Q

Ramsay hunt syndrome

A

idiopathic bell’s palsy (herpes zoster)

  • steroids + acyclovir
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10
Q

Upper limb myotome testing

A

Upper memory tool - start at 5 - shoulders (5), bicep curl (6), tricep extension (7), fist (8), fingers spread (T1)

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11
Q

Upper limb nerve root testing

A

Nerve testing - circle is median, thumbs up is rad, fingers splayed ulnar

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12
Q

An infant presents with roving eye movements, jerking of the extremities, and ataxia. What is the cause?

A

opsoclonus myoclonus
associated with neuroblastoma
Workup with Urine HMA/VMA or MIBG Scan

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13
Q

Cerebellitis

A

self limiting process usually following viral infection (ex. varicella, coxsackie, echovirus)

Gait disturbance

Associated with nystagmus, slurred speech, vomiting, irritability, dysarthria, headache, ataxia, dysdiadochokinesia

Differentiate from encephalitis by: no fever, no nuchal rigidity, no seizures, normal WBC and protein on CSF

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14
Q

Guillain Barre treatment

A

IVIG

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15
Q

Guillain Barre CSF finding

A

cytoalbuminologic dissociation
- increased protein
- normal to low WBC

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16
Q

early hand preference

A

typically a missed stroke causing spastic hemiplegia

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17
Q

dyskinetic CP

A

dystonia, chorea, athetosis
may not present until 2 yrs
usually associated with perinatal distress of kernicterus

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18
Q

DMD inheritance

A

x linked recessive (out of frame deletion)

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19
Q

You are seeing a 5-year-old boy who is having difficulty walking now after he previously walked at 12 months. His development has otherwise been normal. You notice that he has very well-defined calves. What test would be most in keeping with your suspected diagnosis?

A

duchenne muscular dystrophy
do a CK
X linked recessive

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20
Q

SeLECTS/BECTS epilepsy treatment

A

Focal seizures with motor symptoms involving the face - numbness, twitching, hypersalivation, guttural vocalizations, speech arrest
Most occur at night or on awakening
No impairment of awareness
Can progress to GTC

NO treatment needed
remits by age 15

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21
Q

Teenager wakes up and has messed up face and drooling and then it resolves

-Dx? Tx?

A

SELECTS seizures/Benign Rolandic Epilepsy

NO TREATMENT NEEDED -> unless impairing sleep and school performance then consider carbamazepine

Self resolves by 15yrs old

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22
Q

child presenting with dropping objects in the morning

A

juvenile myoclonic epilepsy

  • treat with VPA > lamotrigine > keppra
  • life long treatment
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23
Q

Side effect of ethosuximide

A

agranulocytosis

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24
Q

side effect phenytoin

A

gingival hyperplasia
ataxia
SJS

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25
Q

workup indications for complex febrile seizures

A

Consider LP, EEG, Neuroimaging if:
- Questionable hx of fever
- Focal seizure
- Hx of devel delay
- Abnormal neuro exam (dysmorphic, focal neuro, neurocutaneous findings)

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26
Q

Newborn baby born with hypotonia. History of decreased fetal movements. Normal facial movements. Alert. Weak extremities and low tone. Absent reflexes. What is the most likely diagnosis?

A

SMA

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27
Q

SMA inheritance

A

autosomal recessive

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28
Q

Best test for myasthenia gravis

A

EMG (decremental response to repetitive stimulation)

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29
Q

tuberous sclerosis inheritance

A

autosomal dominant

30
Q

4yo male with multiple hypomelanotic macules, rough raised lesion with orange peel consistency in the lumbar area, 3 cafe’ au lait spots and retinal hamartoma. What are they most at risk for?

A

cardiac rhabdomyoma
in tuberous sclerosis

31
Q

Major features of tuberous sclerosis

A

ASHLEAF (for major criteria)
- Ashleaf spots (hypopigmentation)
- Shagreen patches (orange peel consistency, usually in the lumbosacral region)
- Heart rhabdomyosarcoma
- Lung hamartomas
- Epilepsy from cortical tubers/dysplasias
- Angiomyolipoma in kidneys
- Facial angiofibromas

+ Multiple retinal nodular hamartomas
+ Astrocytomas

32
Q

tuberous sclerosis screening and surveillance

A

brain MR q1-3yr
optho q1 yr
echo q 1-3 yr
renal imaging

33
Q

adrenoleukodystrophy diagnosis

A

serum very long chain fatty acids

x linked disorder

34
Q

5 year old male who suddenly stops talking. Previously otherwise well. He recently had a generalized seizure and EEG during sleep shows epiletiform activity. What is the most likely diagnosis?

A

landau kleffner
- normal language until 3-7yrs

35
Q

diagnosis tourettes

A

2 motor tics and 1 vocal tic for 1 year

36
Q

anti NMDA encephalitis

A
  • often behavioural for months (anxiety, mood, sleep disturbance, psychosis)
  • later develop seizures and dystonia and autonomic dysfxn (htn, tachycardia)
  • dx: CSF antibodies
  • can be triggered by HSV encephalitis or ovarian teratomas
37
Q

anti GABA encephalitis

A
  • presents with refractory seizures
  • MRI: Multifocal cortical subcortical FLAIR/T2 hyperintensities
38
Q

Acute Disseminated Encephalomyelitis (ADEM) encephalitis

A
  • anti mog
  • post infectious, rapidly progressive encephalitis
  • seizures, motor deficits, ataxia, visual impairments
    MR: large hazy abnormalities on T2/flair
39
Q

NMO encephalitis

A
  • anti-aquaporin
  • affects optic nerve and spinal cord
  • high risk relapse
  • immunotherapy long term
40
Q

infantile spasm treatment

A

steroids or vigabatrin

41
Q

4 year old girl with 3 GTC seizures in past week. Several days of emotional lability and confusion. In ED, mild tachycardia and hypertension. Abnormal (writhing) mouth/facial movements.
Which diagnostic test will best determine the diagnosis?

A

CSF auto antibodies for autoimmune encephalitis

42
Q

17 yo girl with 8 month history of amenorrhea and headaches. Intermittent bothersome galactorrhea. High prolactin on bloodwork. MRI showed adenoma 5mm x7mm. What is the best treatment option?

A

dopamine agonist

43
Q

Teenage kid with new behavioral concerns, anxiety, depression. On examination has hepatomegaly and intentional tremor. Bloodwork shows DAT negative hemolytic anemia. What will you recommend?

A

Wilson disease
restrict dietary intake of copper (liver, shellfish, nuts, chocolate)

44
Q

keiser flischer rings

A

wilsons disease

45
Q

wilsons disease presentation

A
  • liver disease with high liver enzymes, hepatomegaly
  • psychiatric disturbance
  • motor symptoms (ataxia, tremor, seizures)
  • KF rings
  • renal tubular dysfunction
  • DAT negative hemolytic anemia
46
Q

low risk BRUE criteria

A

Age > 60 days
If premature, gestational age >/= 32 weeks and CGA >/= 45 wks
First episode of BRUE
Duration < 1 min
No CPR required by trained medical provider
No concerning features on history or physical

47
Q

Rett syndrome

A

x linked dominant (but boys die so just in girls)
MECP2
regression, acquired microcephaly, ataxia, wringing hands, autistic behaviours

48
Q

In patients with TS, MRI brains are required every ____yrs to monitor for ______

A

1-3 years
giant cell astrocytomas

49
Q

Second line medications in status epilepticus?

A

Fospheny OR Pheny 20mg/kg (do not give both, do not use if concern for overdose)

Keppra 60mg/kg (good choice if concerned about resp depression)

VPA 30mg/kg (do not use if devel delay/ <2)

Phenobarb 20mg/kg (use in overdose scenarios or in kids <6mo, can cause resp depression, hypotension, sedation)

After giving 1 second line med, if pt still seizing in 5 minutes, give a second med

50
Q

Features of simple vs complicated febrile seizures

A

Simple = generalized, <15 mins, no reoccurance within 24hrs, no focal neuro deficits on exam

Complex = focal, >15 mins, >2 in 24hrs, post ictal focal deficits

51
Q

RF for reoccurance of febrile seizure?

A
  • family history of febrile seizure
  • family history of epilepsy
  • seized at a lower temp
  • complex
  • age <1yr
52
Q

EEG pattern for Absence Sz

A

3Hz spike and wave

53
Q

Tx for Absence Sz

A

Ethosuximide
Most will outgrow by adolesence

54
Q

6 day old infant, now having 2-3 min tonic clonic seizures occurring 20-30 times per day with associated apneas. Normal pregnancy, uncomplicated delivery, normal neurological exam. Dad had seizures as a baby. Dx and Tx?

A

Benign familial neonatal epilepsy (SeLFNIE)
- Autosomal dominant
- resolves by 6 weeks- 1 year
- will respond to Na channel blockers (carbamazepine, phenobarb)

55
Q

EEG pattern in Infantile spasms

A

Hypsarrythmia (high amplitide waves on background of irregular spikes)

56
Q

Clinical presentation of Infantile spasms

A

Flextion at the waist, extension of the limbs, occurs in CLUSTERS and can be associated with sleep transitions

57
Q

Sandifer syndrome

A

Spasms of neck extension and back arching, spares the limbs, occurs around feeding.

Associated with GERD and can mimic Infantile spasms

58
Q

EEG pattern in SELECTS/Benign Rolandic Epilepsy

A

Centrotemporal spikes

59
Q

Dravet Syndrome

A

Complex, clustering febrile seizures that progress to occurring without fever and cause devel delay

60
Q

Side effects of carbamazepine?

A

SJS
Agranulocytosis
Hepatitis
Rash
SIADH
Ataxia

61
Q

Side effects of Topiramate?

A

Kidney stones
Cognitive slowing
Weight loss
Metabolic acidosis

62
Q

Aura with deja vu, or stomach upset followed by fixed stare, fumbling with fingers, lip smacking

A

Temporal lobe epilepsy

63
Q

Abortive ED management of migraine?

A

IVF bolus
Acetaminophen +NSAID
Metoclopramide
If still in pain at 1hr-> consult neuro for ?VPA

64
Q

Inheritance pattern of DMD?

A

X linked recessive (duplication deletion on chromosome 21)

65
Q

Inheritance patter of myotonic dystrophy?

A

AD

66
Q

What treatment prolongs ambulation time and improves respiratory status in DMD?

A

Steroids

67
Q

Clinical presentation of myotonic dystrophy?

A
  • Bilateral non fatigable ptosis
  • Tented mouth
  • Narrow high arched palate
  • Inverted V lip
  • Distal muscle wasting first and then proximal
  • Reflexes preserved
  • Cognitive impairment
68
Q

Clinical presentation of myasthenia gravis:

A
  • Fatiguable weakness and ptosis
  • Can also have difficulty swallowing and slurred speech
  • DTRs diminished but not lost
69
Q

Clinical presentation of botulinum toxin?

A
  • Constipation followed by bulbar weakness and descending acute flaccid paralysis
  • Fatiguability
70
Q

Infections associated with Guillain Barre?

A

Campylobacter
Mycoplasma pneumoniae

71
Q

Clinical picture Guillain Barre?

A
  • Numbness followed by ascending weakness
  • Absent DTRs or hyporeflexia
  • Can get dysautonomia