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KG - Pediatrics Exam 2 > Neuro > Flashcards

Neuro Flashcards

1
Q

what characterizes macrocephaly?

A

> 95th percentile or > 2 percentile lines above height/weight

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2
Q

what characterizes microcephaly?

A

< 5th percentile or <2 percentile lines below height/weight

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3
Q

what physical exam characteristics should one pay close attention to when looking to see if there may be a neuro defect?

A
  • hair, skin teeth, nails
  • fontanelles
  • ears, eyes
  • hands, feet
  • midline defects
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4
Q

when do the anterior, sphenoidal, and posterior fontanelles close?

A 
posterior = 2-3 months
sphenoidal = 6 months
anterior = 1-3 yrs
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5
Q

how to evaluate CN I?

A

smell, can assess based on age

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6
Q

how to evaluate CN II?

A

pupillary light reflex, visual acuity

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7
Q

how to evaluate CN III, IV, VI?

A

following objects, fixating, oculocephalic reflex, EOMs

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8
Q

how to evaluate CN V?

A

sucking/swallowing, light touch

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9
Q

how to evaluate CN VII?

A

observe face at rest, crying/blinking

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10
Q

how to evaluate CN VIII?

A

hearing

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11
Q

how to evaluate CN IX, X?

A

gag reflex, sucking, salivation

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12
Q

how to evaluate CN XI?

A

posture, spontaneous movement

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13
Q

how to evaluate CN XII?

A

tongue movement

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14
Q

signs of upper motor neuron lesion

A
  • spastic paralysis
  • increased tone
  • increased DTRs/+babinski in older children
  • minimal muscle atrophy/strength loss
  • fasciculations absent
  • may have sensory disturbances
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15
Q

signs of lower motor neuron lesions

A
  • flaccid paralysis
  • decreased tone
  • absent DTRs
  • profound muscle atrophy
  • fasciculations present
  • may have sensory disturbances
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16
Q

reflex - moro

description, age, origin in CNS

A
  • sudden head extension, leads to extension then flexion of limbs
  • age 0-6 months
  • brainstem
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17
Q

reflex - grasp

description, age, origin in CNS

A
  • finger in palm, flexion of fingers, and elbow and shoulder
  • 0-6 months
  • brainstem
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18
Q

reflex - rooting

description, age, origin in CNS

A
  • tactile stimulus near mouth, mouth pursuing stimulus
  • age 0-6 months
  • brainstem
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19
Q

reflex - trunk incurvation

description, age, origin in CNS

A
  • stroke skin near vertebrae, curvature of spine opposite
  • age 0-6 months
  • spinal cord
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20
Q

reflex - placing

description, age, origin in CNS

A
  • places foot on exam surface when dorsum of foot in contact w/ edge
  • age 0-6 months
  • cerebral cortex
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21
Q

reflex - crossed extension

description, age, origin in CNS

A
  • hold one leg in extension and stimulate dorsum of foot, flex/ext/add/toe fanning opposite leg
  • age 0-6 months
  • spinal cord
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22
Q

reflex - tonic neck

description, age, origin in CNS

A
  • supine, turn head results in ipsilateral extension of arm and leg
  • age 0-6 months
  • brainstem
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23
Q

reflex - parachute

description, age, origin in CNS

A
  • infant sitting, tilt results in extension of ipsilateral arm
  • 6months - life
  • brainstem
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24
Q

reflex - landau

description, age, origin in CNS

A
  • infant held about waist and suspended, extension of neck produces extension of limbs
  • 6 months - 2 years
  • brainstem
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25
Q

in a neuro eval, what should be observed in strength of infants/toddlers?

A
  • infants = symmetry of movements when held supine

- toddlers = reach high, run, walk, hop, climb stairs

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26
Q

in a neuro eval, what should be observed in tone of infants? (both passive and active)

A
  • passive = some resistance to stretch normal

- active = posture adopted when placed in particular position

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27
Q

in a neuro eval, what should be observed in gait of infants/toddlers?

A
  • infants = crawling
  • toddlers = normally wide-based gait and unsteady, gradually closes until age 6
  • ALSO, cerebelar function = finger to nose, RAM, heel to shin, heel to toe walking
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28
Q

in a neuro eval, what should be tested for sensory?

A
  • dull vs. sharp
  • temperature
  • proprioception/vibration
  • graphesthesia
  • stereognosis
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29
Q

in a neuro eval, how should one test mental status?

A
  • developmental milestones

- age, location, gender, colors, numbers, shapes, hobbies, social interaction

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30
Q

define: static (in evolution of symptoms)

A

seen in first few months and do not change over time (ie: congenital abnormalities or brain injury)

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31
Q

define: progressive (in evolution of symptoms)

A

degenerative disease or neoplasm

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32
Q

define: intermittent and brief (in evolution of symptoms)

A

epileptic or migraine syndromes

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33
Q

define: saltatory (in evolution of symptoms)

A

bursts of symptoms followed by partial recovery (ie: demyelinating and vascular disease)

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34
Q

common workup for neurologic impairment in peds? (9 tests)

A

1) serum enzymes (CK)
2) molecular genetic markers - DNA markers
3) nerve conduction velocities
4) electromyography (EMG)
5) muscle imaging (w/ MRI?)
6) muscle biopsy
7) nerve biopsy
8) EKG channelopathies
9) ECHO - muscular dystrophy

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35
Q 
trisomy 13
(type of disorder & characteristics)
A
  • recognized chromosomal disorder

- cleft lip, midline defect

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36
Q 
trisomy 18
(type of disorder & characteristics)
A
  • recognized chromosomal disorder

- weak cry, early death

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37
Q

5P

type of disorder & characteristics

A
  • recognized chromosomal disorder

- cri-du-chat syndrome

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38
Q

3 genetic diseases associated w/ microcephaly

A
  • microcephaly vera (autosomal rec.)
  • microcephaly w/ lissencephaly
  • a genesis of corpus collosum
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39
Q

infections (congenital) TORCH associated w/ microcephaly

A
  • rubella
  • CMV
  • toxoplasmosis (usu from the cat)
  • syphilis
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40
Q 
angelman syndrome
(type of disorder & characteristics)
A
  • MR, ataxia, seizures

- microcephaly w/ syndrome

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41
Q

prader-willi

type of disorder & characteristics

A
  • hypotonia, cryptorchidism, obesity

- microcephaly w/ syndrome

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42
Q

smith-lemli opitz

type of disorder & characteristics

A
  • cyptorch, hypospadias, seizures

- microcephaly w/ syndrome

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43
Q

cornelia de lange

type of disorder & characteristics

A
  • anteverted nostrils, carp mouth, micromelia, synophrys

- microcephaly w/ syndorme

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44
Q

rubinstein-taybi

type of disorder & characteristics

A
  • broad thumbs and toes, narrow nose, maxillary hypoplasia

- microcephaly w/ syndrome

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45
Q

hallermann-streiff

type of disorder & characteristics

A
  • micropthalmia, small nose

- microcephaly w/ syndrome

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46
Q

4 toxic microcephalic conditions?

A
  • fetal alcohol syndrome
  • PKU
  • hypoxic- ischemia
  • intrauterine or neonatal injury
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47
Q

define: ataxia

movement disorder

A
  • inability to make voluntary smooth, accurate, and coordinated movements
  • can look inebriated
  • cerebellum
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48
Q

define: bradykinesia

movement disorder

A

extreme slowness and stiffness of voluntary movement

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49
Q

define: choreo

movement disorder

A
  • syndrome of continuous involuntary random movements that usu occur at rest and may appear to be fidgety, dancing, or writhing
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50
Q

define: dystonia

movement disorder

A
  • syndrome of abnormal muscle contractions that lead to twisting, jerking, spasms, or stiffening at rest or during attempts at movement
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51
Q

define: myoclonus

movement disorder

A

condition of very rapid and brief shock-like jerks

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52
Q

define: spasticity

movement disorder

A
  • increase in muscle stiffness that worsens with rapid movement and may be associated w/ increased reflexes and weakness
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53
Q

define: tics

movement disorder

A

repetitive, stereotyped, and sometimes complex involuntary movements or sounds that may appear similar to purposeful actions

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54
Q

define: tremors

movement disorder

A

rhythmic involuntary back and forth shaking at rest or with movement

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55
Q

what are common causes of headache?

A
  • URI
  • fever
  • sinusitis
  • migraine
  • tension
  • cluster
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56
Q

what are life-threatening causes of headache?

A
  • meningitis, encephalitis, cerebral abscess
  • subarachnoid hemorrhage
  • trauma
  • hydrocephalus, VP shunt malformation
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57
Q

what are “other” causes of headache?

A
  • toxin/substance abuse
  • postictal phase (after seizure)
  • HTN
  • psychogenic
  • sleep deprivation
58
Q

migraine

info & presentation

A
  • most common cause of recurrent HA in kids
  • common sx = visual aura (classic type) vs no aura (common type)
  • throbbing, nausea/vomiting, family history, precipitating factors
  • pain bilateral in kids, unilateral in teens
59
Q

migraine

treatment

A
  • ELIMINATE TRIGGERS
  • prophylaxis if >2/wk or >4/month & triggers have been eliminated
  • symptomatic management - alkaloids, nasal spray, pill at onset
60
Q

when should one WORRY about headaches?

A
  • most severe on awakening, awaken in middle of night
  • severely exacerbated by coughing or bending
  • acute onset without prev history
  • present daily w/ worsening
  • accompanied w/ vomiting
  • focal neuro signs
  • fever, ill appearing
61
Q

when should one IMAGE w/ a headache?

A
  • abnormal neuro exam
  • concern of space occupying lesion
  • if your gut tells you to image!
62
Q

define: convulsion

A

violent involuntary contraction

63
Q

define: tonic (convulsion)

A

sustained contraction

64
Q

define: clonic (convulsion)

A

contraction alternating w/ relaxation

65
Q

define: myoclonic (convulsion)

A

spasm of a muscle or group of muscles

66
Q

define: atonic (convulsion)

A

sustained relaxation of muscles

67
Q

define: seizure

A

sudden onset of certain symptoms

68
Q

define: epilepsy

A

chronic nervous system disorder characterized by repeated convulsions of any type (on a reg basis that person is treated for)

69
Q

define: aura

A

stereotyped forewarning of an attack, may be sensory/motor/behavioral

70
Q

define: ictus

A

seizure phase itself

  • motor activity involuntary
  • if both sides involved, unconscious
  • DTRs suppressed
  • stereotyped motor activity
  • upward deviation of eyes (often)
  • amnesia
  • bladder/bowel incontinence may be present
71
Q

define: post ictal phase

A

alteration of consciousness following most seizures (usu stunned state)

  • headache, irritable
  • sensory and motor impairment temporary
72
Q

name 2 types of partial seizures

A
  • simple partial = conscious

- complex partial = impaired consciousness

73
Q

name types of generalized seizures (6)

A
  • absence
  • tonic-clonic
  • atonic
  • myoclonic
  • tonic
  • clonic
74
Q

what perinatal conditions can cause seizures?

A
  • cerebral malformations
  • intrauterine infection
  • hypoxic-ischemic
  • trauma
  • hemorrhage
75
Q

what infections can cause seizures?

A
  • encephalitis
  • meningitis
  • brain abscess
76
Q

what metabolic conditions can cause seizures?

A
  • hypoglycemia
  • hypocalcemia
  • hypomagnesemia
  • hyponatremia
  • hypernatremia
  • storage diseases
  • porphyria
  • pyridoxine deficiency
77
Q

what poisonings can cause seizures?

A
  • lead
  • cocaine
  • drugs
78
Q

what neurocutaneous syndromes can cause seizures?

A
  • tuberous sclerosis
  • neurofibromatosis
  • sturge-weber syndrome
  • klippel-trenaunay-weber syndrome
79
Q

what systemic disorders can cause seizures?

A
  • vasculitis
  • SLE
  • hypertensive encephalopathy
  • renal failure
  • hepatic encephalopathy
80
Q

what “other” things can cause seizures?

A
  • trauma
  • tumor
  • fevers (febrile seizures)
81
Q

febrile seizures

info & presentation

A
  • 85% simple seizures
  • very common
  • peak ages 3 months - 5 years old
  • usu w/ sudden spike in temp
  • usu lasts abt 15 min
  • usu w/ family history
  • < 5% later acquire epilepsy
82
Q

febrile seizure

management

A
  • airway
  • benzodiazepines if durations > 10-15 min
  • figure out cause of fever
  • exclude meningitis w/ septic work up
  • most children < 12 months need LP (12-18 months grey zone)
83
Q

absence seizures

info & presentation

A
  • females > males
  • onset 4-6 yrs old
  • sx = brief loss of environmental awareness w/ simple automatisms (looks like “staring into space”
  • can be provoked by flashing lights/hyperventilation
  • usu short in duration
  • can have dozens/day
  • not responsive during episode
  • no postictal confusion
  • no memory of event
84
Q

status epilepticus

info & presentation

A
  • seizure lasting > 20-30 min
  • concern for irreversible brain injury
  • 50% no definitive etiology (25% w/ acute brain injury, 20% w/ hx congenital malformation/brain injury)
85
Q

status epilepticus

treatment

A
  • ABCs, suction, O2, intubation
  • benzodiapepine (valium, ativan)
  • if persists, consider phenytoin/fosphenytoin, then phenobarbital, then valproate
  • when stable, EEG, LP, imaging
86
Q

infantile spasms

info & presentation

A
  • typical onset 3-7 months
  • brief, symmetric contractions of extremities/trunk/neck muscles (looks like “twitching”)
  • happens in clusters
  • occur before sleep or upon awakening
87
Q

infantile spasms

diagnostic

A
  • EEG - hypsarrthymia pattern

- MRI to determine etiology

88
Q

infantile spasms

treatment

A
  • ACTH (cortisol stimulator)
  • vigabatrin
  • ketogenic diet
  • very poor prognosis (can signal neuro deterioration)
89
Q

what are the developmental milestone categories?

A

gross motor, fine motor, language, cognitive, social

90
Q

what is considered a developmental delay?

A

not reaching a milestone 2 standard deviations below the norm
- also, delays in one area may be associated w/ delays in other areas

91
Q

when is a developmental delay considered a disability?

A

when the delay is permanent

91
Q

what is the Denver II Development Screening Test? (Describe)

A
  • test that provides a broad variety of standardized items to give a quick overview of the child’s development
  • enables tester to compare a child’s development w/ over 2,000 children who were in standardized pop, like a growth curve
  • contains behavior rating scale
  • ** key = early detection & referral
91
Q

cerebral palsy

etiology

A
  • motor impairment from brain damage
  • 70% caused during prenatal period (majority time cause unknown)
  • abt 4-9% directly related to asphyxia at delivery
  • incidence rate 2/1000 live births (not rare at all)
91
Q

4 classifications of cerebral palsy

A

divided based on movement impairments:

  • spastic (70-80% cases)
  • athetoid/dyskinetic
  • ataxic
  • atonic
91
Q

describe Arnold-Chiari malformations (spina bifida associated w/ this)

A
  • cerebellum herniation thru foramen magnum
  • sx - occipital HA that radiates upward, worsens w/ valsava
  • visual/balance problems, CN compressions, muscle weakness may be present
  • treatment = pain management & surgical decompression
92
Q

cerebral palsy

causes

A
  • hypoxia
  • birth trauma
  • premature birth
  • infections
  • trauma
  • toxins
  • structural brain anomalies
  • NOT HEREDITARY
92
Q

spina bifida

info & presentation

A
  • 1/1000 births in US - most frequent/permanently disabling birth defect
  • defective closure of neural tube at end of wk 4
  • anomalies of L/S vertebrae or spinal cord
  • huge range of severity
  • common to see dimple or tuft of hair over sacral area of back
  • prevented by folic acid administration
  • associated w/ Arnold-Chiari malformations
  • associated w/ hydrocephalus
92
Q

describe spina bifida w/ MENINGOMYELOCELE

A
  • most severe
  • SC or nerve roots exposed on back in sac
  • area covered w/ sores & CSF leaks may be present
  • leg paralysis/incontinence
  • chiari malformations
  • needs immediate surgical closure
93
Q

describe spina bifida w/ MENINGOCELE

A
  • rarest form
  • only meninges exposed in back in cyst like sac
  • underlying SC anatomically and functionally intact
  • no hydrocephalus or neuro deficits
  • surgically repaired
94
Q

dandy walker syndrome

A
  • enlargement of 4th ventricle
  • posterior fossa cyst
  • hydrocephalus in 90%
  • partial/complete absence of cerebral vermis
  • slower motor development
  • progressive enlargement of skill allows less HC symptoms early on
  • 50% normal IQ
  • problems w/ balance, fine motor, stiffness
95
Q

hydrocephalus

info & presentation

A
  • slowly evolving accum of CSF over weeks to months
  • etiology can be obstruction or overproduction of CSF
  • sx related to ventricular distention and increased ICP
96
Q

hydrocephalus

treatment

A
  • temporary = loop diuretic, acetazolamide

- surgical intervention = remove obstructive lesion, shunt - VP most common (VP = ventriculoperitoneal)

97
Q

cerebral palsy

management

A
  • OT/PT/ST
  • anti-spasmodics (dantrolene, benzos, baclofen)
  • botulinum toxin if spasms in one particular area
98
Q

hydrocephalus - intraventricular foramina obstruction

A
  • acute/slow onset
  • any age
  • unilateral
  • etiology = congenital, mass
99
Q

hydrocephalus - aqueduct of sylvius obstruction

A
  • in utero or infancy
  • slowly evolving
  • etiology = NF, toxo, mass, congenital dysplasia, IVH
100
Q

hydrocephalus - impaired flow form 4th ventrical obstruction

A
  • enlarged occipital shelf
  • slow evolving
  • etiology = dandy-walker, atresia of foramina of lushka and magendie
101
Q

hydrocephalus - congenital bone lesion obstruction

A
  • slow evolving
  • lower cranial nerve dysfunction
  • etiology = achondroplasia, rickets, posterior fossa distortion
102
Q

hydrocephalus - extraventricular obstruction (communicating) obstruction

A
  • acute/slowly moving
  • etiology = hypoplasia or damage of arachnoid villi
  • s/p IVH with blood debris
103
Q

complete spinal cord trauma

A

nerve damage obstructs every signal coming form brain to body parts below injury

104
Q

incomplete spinal cord trauma

A

some residual motor and sensory function remains below level of SCI

105
Q

list of neuromuscular diseases w/ proximal muscle weakness

A
  • dystrophy
  • duchenne
  • limb-girdle
  • dermatomyositis, polymyositis
  • kugelberg-welander disease (late onset spinal muscular atrophy)
106
Q

acute transverse myelitis

diagnosis

A
  • MRI (lighter areas show inflammation)

- CSF analysis

107
Q

list of neuromuscular diseases w/ ophthalmoplegia and limb weakness

A
  • myasthenia gravis
  • botulism
  • myotonic dystrophy
  • miller fisher variant of GB syndrome
108
Q

list of neuromuscular diseases w/ facial and bulbar weakness

A
  • myasthenia gravis
  • botulism
  • polio
  • miller fisher variant of GB syndrome
  • myotonic dystrophy
  • facioscapulohumeral dystrophy
109
Q

spinal muscular atrophy syndrome

A
  • autosomal rec.
  • gen. def. chromosome 5
  • progressive weakness of LMN due to degeneration of anterior horn cells
  • majority present in infancy
  • similar to ALS in adults
  • progressive flaccid weakness (symmetric), dec/abs DTR, muscle atrophy
  • difficulty sitting, failure to stand
  • decreased facial exp, drooling
  • death from resp. dysfunction/infection
  • *normal cognitive/language/social dev
110
Q

acute transverse myelitis

info & presentation

A
  • acute inflammation of grey & white matter in one or more spinal cord segments (usu thoracic)
  • onset hours to weeks of bilateral motor, sensory, sphincter deficits below level of lesion
  • back pain, sudden paresthesias, weakness
111
Q

acute transverse myelitis

cause

A

unknown, but related to MS infections, autoimmune or post-infectious inflammation, vasculitis, and certain meds

112
Q

guillain barre syndrome (acute idiopathic polyneuritis

info & presentation

A
  • inflammatory peripheral neuropathy
  • rapid loss of motor strength (ascending)
  • symmetric weakness
  • may have sensory symptoms w/ few findings
  • loss of DTRs early
  • may have autonomic nerve dysfunction
113
Q

guillain barre syndrome (acute idiopathic polyneuritis)

cause

A
  • post-infectious respiratory or GI

- campylobacter jejuni (majority), cytomegalovirus, EBV, and mycoplasma pneumoniae injections, MCV4/infuenza vaccine

114
Q

what is the tension (endrophonium) test?

A

1) administer short acting Cholinesterase inhibitor

2) ptosis and ophthalmoplegia improve w/in seconds

115
Q

guillain barre syndrome (acute idiopathic polyneuritis)

treatment

A
  • IVIG
  • plasmapheresis
  • 1-2% mortality if treated properly, 20% end up on ventilator
116
Q

myasthenia gravis

info

A
  • chronic disease, autoimmune where antibodies form against Ach nicotinic receptors at NM junctions
  • 75% thymus involvement
  • begins teen years
  • rapid fatigue of striated muscle
  • usu immune mediated NM blockade
  • post- synaptic muscle membrane less responsive due to circulating receptor binding antibodies
117
Q

myasthenia gravis

hallmark symptoms

A
  • ptosis, diplopia, ophthalmoplegia, weakness of face/extremities, dysphagia
  • symptoms worsen throughout day or w/ exertion
  • no primary complains of muscle pain, sleepiness
118
Q

myasthenia gravis

diagnosis

A
  • CT scan for thymoma
  • serology for autoantibodies
  • NCS/EMG (repetitive)
  • tensilon challenge test (endrophonium)
119
Q

inheritance pattern for hereditary motor sensory MD (charcot-marie tooth)

A

AD-17p11.2

120
Q

inheritance pattern for duchenne and becker MD?

A

XR - Xp21.2

121
Q

inheritance pattern for emery-dreyfus MD?

A

XR - Xq28

122
Q

inheritance pattern for limb girdle MD (two possibilities)

A

AD - 5q
or
AR - 15q

123
Q

inheritance pattern for congenital MD

A

AR

124
Q

inheritance pattern for hereditary motor sensory MD (charcot-marie tooth)

A

AD-17p11.2

125
Q

duchenne + Becker MD

info & presentation

A
  • most common MD
  • onset 3 yrs, fully expressed by 5-6 yrs, wheelchair bound by 10 yrs (Becker has later onset)
  • cardiomyopathy in 50-80%
    death by 18-20 yrs old (bc resp. problems)
    progressive weakness
  • shows Gower’s sign
  • waddling gait
  • mild intellectual impairment
  • hypertrophy of calves, pelvic weakness
  • proliferation of connective tissue in muscle
126
Q

duchenne + Becker MD

diagnosis

A
  • muscle biopsy - will show degeneration & regeneration w/ connective tissue
  • absence of DYSTROPHIN (Ig protein assoc. w/ muscle fiber plasma membrane)
  • EMG
  • serum CPK levels will be elevated (CPK = creatine phosphokinase)
127
Q

duchenne + Becker MD

treatment

A
  • symptomatic treatment only
  • glucocorticoids
  • new therapy = gene therapy, creatine, aminoglycosides
128
Q

emery-dreifuss MD

A
  • group of myopathies that affect muscles of hip/shoulder
  • middle to late childhood and early adult presentation
  • wheelchair by 30 yrs old
129
Q

limb-girdle MD

A
  • includes other mutations that cause myotubuar myopathy such as adrenoleukocystrophy & block-sulzberger (pigment changes)
  • onset 5-15 yrs old
  • CM severe (must make decision act heart transplant)
130
Q

symptoms of pediatric brain tumors?

A
  • HA
  • nausea, vomiting
  • loss of balance
  • speech problems
  • sleepiness
  • personality changes
  • seizures
  • increased head circumference in infants
131
Q

types of brain tumors?

A
  • gliomas (astrocytoma, brain stem glioma, ependymomas)

- meduloblastomas (15% brain tumors in kids)

132
Q

two types of neurofibromatosis?

A

type 1 = more common, AD (chr. 17), peripheral type

type 2 = AD (chr. 22), incurable, multiple intracranial & spinal tumors, central type

133
Q

neurofibromatosis

presentation

A
  • cafe au lait spots (numerous > .5cm present at birth)
  • neurofibromas form late teens (soft or firm pedunculated tumors along a nerve)
  • axillary/groin freckling
  • greater incidence CNS tumors
  • range cognitive impairment
134
Q

neurofibromatosis

treatment

A
  • surgical excision of tumors

KG - Pediatrics Exam 2 (3 decks)

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