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Peds II > Exam 3 - Neurology > Flashcards

Exam 3 - Neurology Flashcards

1
Q

What is a non-progressive motor impairment secondary to fetal or infantile brain injury?

A

Cerebral palsy

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2
Q

What is the most common subtype of cerebral palsy?

A

Spastic

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3
Q

What are all the subtypes of cerebral palsy?

A
  • Spastic
  • Ataxic
  • Dyskinetic
  • Mixed
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4
Q

The following signs/symptoms are concerning for what disorder?

  • Abnormal tone/posture
  • Retained primitive reflexes
  • Not reaching milestones
  • Excessive irritability
  • Poor feeding, drooling
  • Poor visual attention
A

Cerebral palsy

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5
Q

What is the symptomatic management of cerebral palsy?

A
  • OT, PT, bracing
  • Anti-spasmodics
  • Botulism toxin (for contractures)
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6
Q

The following signs/symptoms are concerning for what disorder?

  • Asymptomatic
  • Bradycardia, HTN, altered respiratory rate
  • Headaches, n/v, behavior changes, papilledema
  • Macrocephaly
  • Hyperreflexia, spasticity
A

Hydrocephalus

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7
Q

How is Hydrocephalus diagnosed?

A

Newborns/infants - US

Older infants/children - MRI or CT

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8
Q

What is the management for hydrocephalus?

A

Refer to neurosurgery for possible shunt placement

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9
Q

When should occipitofrontal circumference (OFC) be measured?

A

Each well child visit b/w birth to 3 y/o

Any child w/ neurologic symptoms or developmental complaints

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10
Q

How is microcephaly defined?

A

Head circumference 2 or more standard deviations below the mean or < 5th percentile

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11
Q

What are the two types of microcephaly?

A

Primary (congenital) - lack of brain development or abnormal development due to timing of insult

Secondary (postnatal) - injury or insult to previously normal brain

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12
Q

If symptomatic microcephaly, what might you expect to see in regards to possible signs/symptoms?

A
  • Delayed milestones
  • Seizures or spasticity
  • Fontanelle may close early and sutures may be prominent
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13
Q

How is macrocephaly defined?

A

Head circumference 2 or more standard deviations above the mean or > 97th percentile

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14
Q

What are the three types of growth patterns seen in macrocephaly and what do they suggest?

A

Rapid growth
- Increased ICP

Catch-up Growth
- Seen in premature infants, neurologically intact (under growth curve and then shoot up, catching up to normal growth)

Normal growth rate
- Familial macrocephaly or megaloencephaly

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15
Q

What is the management for microcephaly and macrocephaly?

A
  • Neurology referral for labs and imaging

- Treat underlying cause

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16
Q

What are the standard rates of growth in head circumference for the following age groups:
1-3 months
4-6 months
6-12 months

What would you do if a child’s measurement was above the average?

A

1-3 months: 2 cm/month
4-6 months: 1 cm/month
6-12 months: 0.5 cm/montn

1) Re-measure to ensure accuracy
2) If measurement is the same, consult neurology due to concern for increased ICP

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17
Q

What is it called when cerebellar tonsils are abnormally shaped and displaced caudally below the foramen magnum?

A

Chiari Malformation - Type I

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18
Q

When do symptoms of Chiari Malformation - Type I typically present?

A

Not until teen or adult

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19
Q

What is a syringomyelia and what can it be associated with?

A

Fluid filled cyst within the spinal cord

Can be associated with Chiari Malformation - Type I

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20
Q

What are signs/symptoms associated with Chiari Malformation - Type I?

A
  • Asymptomatic
  • Neck pain, headaches
  • Increased ICP
  • UMN symptoms (hyperreflexia, weakness)
  • Sleep apnea
  • Worse with Valsalva, coughing, sneezing
  • Syringomyelia symptoms
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21
Q

What are signs/symptoms associated with a syringomyelia?

A
  • Cape-like sensory loss

- Loss of abdominal reflex

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22
Q

What is a Chiari Malformation - Type II?

A

Type I + myelomeningocele

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23
Q

When is Chiari Malformation - Type II typically detected?

A

Prenatally or at birth

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24
Q

What are signs/symptoms associated with Chiari Malformation - Type II?

A
  • Dysphagia
  • Apneic spells and aspiration
  • UE weakness
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25
Q

How are Chiari Malformations diagnosed if not at birth?

A

MRI (tonsils are 5 or more mm below foramen magnum)

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26
Q

What is the management for Chiari Malformations?

A
  • Neurosurgery consultation
  • Asymptomatic Type I - conservative
  • Type II - surgical
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27
Q

What are etiologies/risk factors for spina bifida?

A
  • Low folate
  • Genetics
  • Fever/hyperthermia in 1st trimester
  • Poorly managed diabetes
  • Obesity
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28
Q

What are the type of spinal bifida?

A

Closed = Spina bifida occulta

Open = Meningocele, Myelomeningocele

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29
Q

What is Closed Spinal Dysraphism/Spina Bifida Occulta?

A

Incomplete closure of the spinal cord

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30
Q

What is the presentation of Closed Spinal Dysraphism/Spina Bifida Occulta?

A

Can range from asymptomatic to severe abnormalities.

Hairy patch, dimple, dark spot, swelling on the back at the site of the gap in the spine.

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31
Q

What is Open Spinal Dysraphism/Meningocele?

A

Outpouching of the spinal fluid and meninges through the vertebral cleft.

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32
Q

What is Open Spinal Dysraphism/Myelomeningocele?

A

Meninges and spinal cord protrude from the vertebral cleft.

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33
Q

What symptoms are associated with Open Spinal Dysraphism/Myelomeningocele?

A
  • Sensory loss
  • Paralysis
  • Loss of bladder/bowel control
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34
Q

What is associated with Open Spinal Dysraphism/Myelomeningocele in a majority of cases?

A

Chiari II malformation and hydrocephalus

35
Q

How is Spina Bifida diagnosed?

A

MRI

36
Q

The following clinical manifestations are associated with what disorder?

  • Cafe-au-lait macules
  • Neurofibromas
  • Axillary/inguinal freckling
  • Lisch nodules
  • Optic pathway glioma
A

Neurofibromatosis (NF1)

37
Q

What are neurological abnormalities associated with Neurofibromatosis (NF1)?

A

Macrocephaly, seizures, cognitive deficits

38
Q

What is the management for Neurofibromatosis (NF1)?

A

Multi-disciplinary team of neurology, genetics, and ophthalmology

39
Q

What is the diagnostic criteria for Neurofibromatosis (NF1)?

A

Two or more of the following clinical features must be present:

  • Six or more cafe-au-lait macules
  • Two or more neurofibromas
  • Axillary/inguinal freckling
  • Optic glioma
  • Two or more Lisch nodules
  • Distinctive bony lesion
  • First-degree relative with NF1
40
Q

What are the most common types of primary headaches?

A

Migraine and tension

41
Q

What are the most common types of secondary headaches?

A

Acute febrile illness

42
Q

The following description is associated with which type of headache?

  • Location - focal
  • Duration - 2-72 hours
  • Character - moderate to severe, pulsatile/throbbing
  • Aggravated with activity
  • Associated N/V, photo/phonophobia, +/- aura
A

Migraine (primary)

43
Q

The following description is associated with which type of headache?

  • Location - diffuse
  • Duration - 30 min to 7 days
  • Character - mild to moderate, pressure/non-throbbing
  • Not aggravated with activity
  • May have photo or phonophobia, but not both
A

Tension (primary)

44
Q

How are headaches managed/prevented?

A
  • Track/avoid triggers
  • Exercise, hydration, meal schedule
  • Symptomatic treatment w/ ibuprofen, Tylenol
  • Relaxation therapy, decrease screen time
45
Q

What are some worrisome headache features and when should you considering referring the patient?

A
  • Abnormal neurologic or visual exam
  • Severe headache upon awakening/awaken in middle of night
  • Daily symptoms with progressive worsening
  • Accompanied with persistent vomiting
  • Acute onset without previous history
  • Increased with coughing or bending
46
Q

In what population is pseudotumor cerebri typically seen?

A

Obese teenage girls

47
Q

What is the hallmark symptom of pseudotumor cerebri?

A

Papilledema

48
Q

What are the following signs/symptoms associated with?

  • Headache
  • Papilledema
  • Visual symptoms
  • Pulsatile tinnitus
  • Neck stiffness/back pain
A

Pseudotumor Cerebri

49
Q

How is Pseudotumor Cerebri diagnosed?

A

Diagnosis of exclusion

Neurological evaluation
- MRI
- LP
Ophthalmologic evaluation

50
Q

What is the management for Pseudotumor Cerebri?

A
  • Acetazolamide (reduce rate of CSF production)
  • Topiramate (help control HA)
  • Weight loss
51
Q

What is a sudden, transient disturbance of brain function manifested by involuntary sensory, motor, autonomic symptoms with or without loss of consciousness?

A

Seizure

52
Q

Define epilepsy.

A

2 or more unprovoked seizures occuring more than 24 hours apart

53
Q

What are the phases of a seizure?

A
  • Aura: sense of physical or emotional warning of imminent seizure
  • Ictal: active seizure
  • Post-ictal: recovery period
54
Q

What are focal seizures?

A

Seizures occurring in localized region of the brain

55
Q

Describe the presentation of a simple focal seizure.

A

Conscious: motor, sensory, autonomic symptoms lasting seconds to minutes

56
Q

Describe the presentation of a complex focal seizure.

A

Impaired awareness: interruption of behavior or staring, yelling, arm movements lasting < 3 minutes

57
Q

What are generalized seizures?

A

Abnormal electrical charges in both hemispheres simultaneously

58
Q

Describe the varying types of seizures below:

  • Absence
  • Myoclonic
  • Tonic
  • Clonic
  • Atonic
A

All are with loss of consciousness or awareness and varying post-ictal state

Absence = blank stare, automatisms

Myoclonic = sudden, brief muscle contractions

Tonic = rigid

Clonic = rhythmic jerking

Atonic = sudden loss of muscle control

59
Q

What is the most common type of generalized seizure?

A

Tonic/clonic = Rigid/Rhythmic jerking

60
Q

The following clinical features are associated with what type of seizure?

  • Sudden impairment of consciousness w/o loss of tone
  • Provoked by hyperventilation
  • Presents b/w age 4-10 and often remits by puberty
  • Lasts 9-10 seconds and may occur 10 times/day
A

Absence Seizures

61
Q

How are absence seizures diagnosed?

A

History, exam, EEG

62
Q

What is the 1st line medication for absence seizures?

A

Ethosuximide

63
Q

What are absence seizures provoked by?

A

Hyperventilation

64
Q

What are clinical features associated with febrile seizures?

A
  • Convulsion with temp > 38C (100.4F)
  • Age 6 months to 5 years
  • Often associated with virus
  • (+) genetic predisposition
65
Q

Compare a simple febrile seizure to a complex febrile seizure.

A

Simple - most common, lasting < 15 minutes

Complex - focal (one side of body), lasting > 15 minutes or occurs > 1 in 24 hours

66
Q

What is the management for febrile seizures?

A
  • Generally self-limiting

- IV benzodiazepines if symptoms lasting > 5 minutes

67
Q

What is an acute immune-mediated polyneuropathy that is typically preceded by illness, most commonly Campylobacter jejuni?

A

Guillian-Barre Syndrome

68
Q

What is the most common cause of acute flaccid paralysis in healthy infant/child?

A

Guillian-Barre Syndrome

69
Q

The following signs/symptoms are associated with what disorder?

  • Ascending symmetric weakness
  • Neuropathic pain
  • Gait instability or refusal to walk
  • Absent reflexes
A

Guillian-Barre Syndrome

70
Q

How is Guillian-Barre Syndrome diagnosed?

A
  • Electrodiagnostic studies (EMG/NCV)
  • CSF analysis (increased protein with normal WBC)
  • Spinal MRI with and w/o contrast
71
Q

What is the management of Guillian-Barre Syndrome?

A
  • Hospitalization and close monitoring if rapidly progressing
  • IVIG or plasma exchange in severe cases
72
Q

The following signs/symptoms are associated with what disorder?

  • Descending weakness
  • Constipation, poor feeding
  • Hypotonia, loss of DTR’s
  • Irritable and lethargic
A

Botulism

73
Q

What diagnostic studies are used to evaluate for botulism?

A
  • Stool sample

- EMG/NCV

74
Q

What is the management for Botulism?

A

Hospitalization and close monitoring

- Botulism immune globulin

75
Q

In Duchenne Muscular Dystrophy, what may be elevated before onset of symptoms?

A

Elevated muscle enzymes (CK > 10-20 times normal)

76
Q

What specific sign is associated with Duchenne Muscular Dystrophy?

A

Gower’s sign (use hands to push up from floor)

77
Q

The following signs/symptoms are associated with what disorder?

  • Progressive weakness (proximal before distal, UE before LE)
  • Gower’s sign
  • Pseudohypertrophy of calves
A

Duchenne Muscular Dystrophy

78
Q

What are some conditions associated with Duchenne Muscular Dystrophy?

A
  • Cardiomyopathy
  • Orthopedic complications (scoliosis, fractures)
  • Growth delay
  • Cognitive impairment
79
Q

What diagnostic studies can be used to evaluate for Duchenne Muscular Dystrophy?

A
  • Elevated muscle enzymes (CK > than 10-20 times normal)

- Genetic testing

80
Q

What is the management of Duchenne Muscular Dystrophy?

A

Glucocorticoids

81
Q

What is the typical age of onset in Duchenne Muscular Dystrophy?

A

Age 2-3 (earlier onset then Becker’s)

82
Q

How does Becker Muscular Dystrophy differ from Duchenne Muscular Dystrophy?

A
  • Symptom onset is later
  • Muscle involvement not as severe
  • CK elevated > 5 times normal, not > 10-20
  • Cardiomyopathy may be more predominant
83
Q

Is Guillian-Barre Syndrome associated with descending or ascending weakness?

A

Ascending

84
Q

Is Botulism associated with descending or ascending weakness?

A

Descending

Peds II (11 decks)

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