Neurology Flashcards

1
Q

Define Macrocephaly

A
  • > 95th percentile or > 2 percentile lines above height/weight
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2
Q

define microcephaly

A
  • < 5th percentile or < 2 percentile lines below height/weight
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3
Q

posterior fontanelle closes when

A
  • 2-3 months
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4
Q

anterior fontanelle closes when

A

1-3 years

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5
Q

How can you test CN III, IV and VI in a pediatric patient

A
  • following objects
  • fixating
  • EOMs
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6
Q

how can you test for CN V in infant

A
  • sucking, swallowing
  • light touch to face
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7
Q

How can you test for CN IX and X in infant

A
  • IX: glossopharyngeal: salivation
  • X: vagus
    • both: gag reflex
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8
Q

List some neonatal primitive reflexes. These should disappear at ?

A
  • Moro: when head is extended, arms and legs both flex
  • Grasp
  • Rooting: tactile stimulus near mouth, mouth pursues stimulus
  • Placing
  • Trunk incurvation: reflex occurs the side of the infant’s spine is stroked or tapped while the infant lies on the stomach. The infant will twitch his or her hips toward the touch
  • disappear at 4-6 months
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9
Q

gait is usually wide based and unstead until what age

A

6 yo

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10
Q

Static evolution of neurologic symptoms

A
  • seen in first few months and do not change over time
    • congenital abnormalities or brain injury
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11
Q

Progressive evolution of neurologic symptoms

A

degenerative disease or neoplasm

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12
Q

Intermittent and brief evolution of neurologic symptoms

A

epileptic or migraine syndromes

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13
Q

Saltatory evolution of neurologic symptoms

A
  • bursts of symptoms followed by partial recovery
    • demyelinating and vascular diseases
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14
Q

What is considered developmental delay

A
  • not reaching a milestone 2 standard deviations below the norm
  • disability: permanent delay
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15
Q

List the 4 developmental milestone categories

A
  1. gross motor
  2. fine motor
  3. cognitive language
  4. social
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16
Q

Intellectual disability can be broken down into what 2 categories

A
  1. adaptive behavior: limitations in conceptual, social, and/or practical skills
  2. intellectual functioning: limitations in general mental capacity including learning, reasoning, problem solving, etc
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17
Q

Which atypical movement describes the inability to make voluntary smooth, accurate, and coordinated movements - cerebellum

A

Ataxia

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18
Q

which atypical movement describes the extreme slowness and stiffness of voluntary movement

A

bradykinesia

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19
Q

which atypical movement describes the syndrome of continous involuntary random movements that usually occur at rest and may appear to be fidgety, dancing, or writhing

A

chorea

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20
Q

which atypical movement describes the syndrome of abnormal muscle contractions that lead to twisting, jerking, spasms, or stiffness at rest of during attempts at movement

A

Dystonia

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21
Q

which atypical movement describes the condition of very rapid and brief shock-like jerks

A

Myoclonus

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22
Q

which atypical movement describes the increase in muscle stiffness that worsens with rapid movement and may be associated with increased reflexes and weakness

A

spasticity

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23
Q

which atypical movement describes the repetitive, stereotyped and sometimes complex involuntary movements or sounds that may appear similar to purposeful actions

A

Tics

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24
Q

What is Cerebral palsy

A
  • motor impairment resulting from brain damage
  • 70% caused during prenatal period
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25
Q

List the 4 different types of Cerebral palsy

A
  1. spastic: 70-80%
  2. athetoid/dyskinetic: writhing
  3. ataxic: imbalance
  4. atonic: flaccid
  • many have normal or above normal intellect
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26
Q

is cerebral palsy hereditary?

A

No

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27
Q

Cerebral palsy treatment

A
  • early recognitition and intervention
  • anti-spasmodics
  • botulinum toxin
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28
Q

what is the most common cause of recurrent HA in children

A

Migraine

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29
Q

in a Migraine, pain is usually bi- or unilateral in children? In teens?

A
  • bilateral in children
  • unilateral in teens
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30
Q

tx of migraine

A

eliminate triggers

31
Q

Tonic convulsion

A

sustained contraction

32
Q

clonic convulsion

A

contraction alternating with relaxation

33
Q

myoclonic convulsion

A

spasm of a muscle or group of muscles

34
Q

atonic convulsion

A

sustained relaxation of muscles

35
Q

if ictus phase (Acute seizure) affect both sides of brain, then patient will be

A

unconscious

36
Q

post ictal phase

A
  • aleration of consciousness following most seizures
  • HA, irritable
37
Q

partial seizures can be broken down into

A
  • simple partial: conscious
  • complex partial: impaired consciousness
38
Q

febrile are extremely common in what age groups

A

3 months to 5 years

39
Q

febrile seizure characteritistics

A
  • 85% are simple seizures
  • associated with sudden spike in temperature
  • usually lasts <15 minutes
  • often + FH
  • < 5% will later have epilepsy
40
Q

what med is given if febrile sz lasts longer than 10-15 min

A

benzodiazepines

41
Q

absence seizure most commonly affects what patient population

A
  • female
  • 4-6 years of age onset
42
Q

what is an absence seizure

A
  • brief loss of environmental awareness accompanied by simple automatisms
    • usually hx “starring into space”
    • not responsive during episode
    • no memory of even
    • no postictal confusion
43
Q

absence seizure can be provoked by

A
  • hyperventilation
  • flashing lights
44
Q

What is status epilepticus

A
  • prolonged seizure lasting longer than 20-30 minutes
45
Q

tx of status epilepticus

A
  • ABCs
  • Benzodiazepine (valium/ativan)
46
Q

West syndrome

A
  • epilepsy syndrome of infancy and childhood
  • characterized by infantile spasms, developmental regression and a specific pattern on EEG testing called hypsarrhythmia (chaotic brain waves)
47
Q

What are infantile spasms? When do they usually start in an infant?

A
  • onset usually 3-7 months old
  • the seizures primarily consist of a sudden bending forward of the body with stiffening of arms and legs; some children arch their backs as they extend their arms and legs
    • happen in clusters
48
Q

when do infantile spasms occur during the day

A
  • just before sleep
  • upon awakening
49
Q

what EEG pattern is consistent with infantile spasms

A

hypsarrthymia pattern

50
Q

tx of infantile spasms

A
  • ACTH
  • vigabatrin
  • Ketogenic diet
    • poor prognosis
51
Q

What is the most frequent permanently disabling birth defect

A
  • spina bifida
52
Q

what is spina bifida

A
  • defective closure of the neural tube at end of week 4
    • anomalies of L/S vertebrae (most common) or spinal cord
53
Q

how is spina bifida prevented

A

folic acid

54
Q

spina bifida is associated with hydrocephalus and Arnold-Chiari malformations which are

A
  • cerebellum herniating through foramen magnum
    • sx: occipital HA that radiates upward and worsens with valsalva
55
Q

patients with spina bifida have a high incidence of allergy to

A

latex

56
Q

What is Dandy walker syndrome

A
  • posterior fossa cyst that causes enlargement of 4th ventricle
  • see hydrocephalus in 90% of patients
57
Q

what is hydrocephalus

A
  • slowly evolving accumulation of CSF over weeks to months
    • due to obstruction vs overproduction of CSF
    • increases ICP and causes ventricular distention
58
Q

treatment of hydrocephalus

A
  • temporary: loop diuretic, acetazolamide
  • surgical: shunt-VP most common
59
Q

Most common hereditary neuromuscular disease

A

Duchenne muscular dystrophy

60
Q

how is Duchenne muscular dystrophy inherited

A
  • X-linked recessive (Xp21.2)
61
Q

50-80% of patients with Duchenne muscular dystrophy have what heart condition

A

cardiomyopathy

62
Q

prognosis of Duchenne muscular dystrophy

A
  • onset age 3 yo
  • wheelchair bound age 10 yo
  • death by 18-20 yo
  • *Proximal progressive weakness and calf hypertrophy
63
Q

Patients with Duchenne muscular dystrophy exhibit Gower’s sign which is

A

using hands to push up from floor to standing

64
Q

how is Duchenne muscular dystrophy diagnosed

A
  • muscle biopsy
  • absence of dystrophin
  • EMG
  • serum CPK levels elevated
65
Q

What is Emery-Dreifuss musclar dystrophy and when does it present

A
  • group of myopathies that primarily affects muscles of hip and shoulder
  • middle to late childhood and early adult
    • wheelchair by age 30
66
Q

how is Limb-girdle musclar dystrophy inherited

A
  • x-linked recessive long arm Xq28
67
Q

Limb-girdle musclar dystrophy is associated with what heart problem

A

severe cardiomyopathy

68
Q

List the 2 types of neurofibromatosis

A
  • type 1: autosomal dominant (chromosome 17)
    • peripheral type
  • type 2: autosomal dominant (chromosome 22)
    • central type
69
Q

clinical presentation

  • cafe-au-lait spots
  • axillary, groin freckling
  • greater incidence of CNS tumors
  • essential HTN
A

Neurofibromatosis

70
Q

hallmark of guillain barre syndrome is

A
  • symmetric ascending loss of motor strength
71
Q

majority of guillain barre syndrome is caused by

A

campylobacter jejuni

72
Q

cause of myasthenia gravis

A
  • rare autoimmune disorder where antibodies form against Ach nicotinic receptors at neuromuscular junction
73
Q

how is Spinal musclar atrophy syndrome inherited? What is it

A
  • autosomal recessive
    • defect on chromosome 5
  • progressive weakness of LMN due to degeneration of anterior horn cells
74
Q

Spinal musclar atrophy syndrome symptoms

A
  • progressive flaccid weakness
    • symmetric
    • lower extremity -> upper extremity
  • remember: normal cognitive, social, and language development