Demyelinating, Myelitis, Encephalitides Flashcards

1
Q

In what ethnic group are pediatric demyelinating diseases more common?

A

Blacks

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2
Q

What antibodies are often found in about 1/3 of children with an acute demyelinating syndrome?

A

myelin oligodendrocyte glycoprotein (MOG) antibodies

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3
Q

What are the two general criteria for diagnosis of MS?

A

Dissemination in time and space

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4
Q

What qualifies as dissemination in space for diagnosis of MS? (2017 McDonald criteria)

A

involvement of at least two of four areas in the CNS (periventricular, juxtacortical, infratentorial, spinal cord)

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5
Q

What qualifies as dissemination in time for diagnosis of MS? (2017 McDonald criteria)

A

simultaneous presence of enhancing and non-enhancing lesions or new lesions on follow-up MRI

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6
Q

Remote history of what virus is associated with pediatric-onset MS?

A

EBV

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7
Q

What type of MS do children usually have?

A

relapsing-remitting

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8
Q

How does progression of MS in children differ than adults?

A

children have higher relapse rates but quicker recovery

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9
Q

How does MS present in children? Give 4 general symptoms.

A

optic neuritis, gait disturbance, brainstem symptoms, sensory deficits

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10
Q

What are two MRI findings in MS?

A

ovoid T2 lesions and FLAIR hyperintensities in the periventricular white matter

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11
Q

What is elevated in the CSF of MS patients?

A

lymphocytes and protein

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12
Q

What lab finding indicates ADEM over MS?

A

anti-MOG

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13
Q

In MS, how does lesion burden and cognitive impairments in children compare to adults?

A

children have higher rates of cognitive impairment and greater MRI lesion burden

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14
Q

What are two factors predictive of increased long-term disability in pediatric MS?

A

lack of full recovery after an initial progressive course and number of relapses

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15
Q

What are the two first-line medications used in pediatric MS?

A

beta-interferon and glatiramer acetate

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16
Q

What percentage of patients with MS have neurogenic bladder? What types?

A

80%, DSD and detrusor hyperactivity

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17
Q

How is the course of transverse myelitis characterized?

A

acute monophasic

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18
Q

In what ages is pediatric transverse myelitis most common?

A

bimodal distribution: 0-5, 10 -17

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19
Q

In what level of the spinal cord is pediatric TM most common?

A

thoracic

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20
Q

What is seen on MRI in pediatric TM?

A

T2 hyperintensities and T1 isointense signals over multiple segments of the cord

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21
Q

What are 3 disease categories associated with pediatric TM?

A

viruses, systemic inflammatory disorders, multifocal CNS disorders

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22
Q

Is TM generally more severe in children or adults?

A

children

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23
Q

When does pediatric TM symptoms nadir usually occur?

A

within a week of presentation

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24
Q

What are the treatments for pediatric TM?

A

High-dose steroids for one week with consideration for IVIG or PLEX

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25
Q

What is a child with cervical transverse myelitis at risk for?

A

respiratory decompensation

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26
Q

What are three non-intuitive factors associated with a poor prognosis in pediatric TM?

A

female sex, spinal shock, and rapidity to nadir

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27
Q

What presents with optic neuritis and transverse myelitis?

A

Neuromyelitis Optica (NMO)

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28
Q

What antibody is associated with NMO?

A

aquaporin-4 (AQP4-IgG)

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29
Q

What two structures get damaged in NMO?

A

myelin and astrocytes

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30
Q

Who is the typical NMO patient?

A

a non-white female in early adolescence

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31
Q

What clinical manifestation of NMO is more severe in children than adults?

A

vision loss

32
Q

What are 3 areas of the brain that are aquaporin-4 rich?

A

hypothalamus, periventricular gray matter, and subcortical white matter

33
Q

How are the MRI enhancements in NMO characterized?

A

“cloud-like”

34
Q

Why can NMO resemble ADEM?

A

because it can present with encephalopathy

35
Q

How does postrema involvement in NMO present?

A

with intractable emesis

36
Q

How does treatment for pediatric NMO differ from TM?

A

NMO patients are more likely to get long-term immunosuppression

37
Q

What are the two most common functional deficits in NMO?

A

vision loss and paraplegia

38
Q

What medication has been shown to be effective treating spasms in NMO?

A

carbamazepine

39
Q

What are the MRI findings in acute disseminated encephalomyelitis (ADEM)?

A

large diffuse white matter lesions with poor demarcation

40
Q

In what age range do children typically present with ADEM?

A

5 to 8 years old

41
Q

What is the typical clinical presentation of ADEM?

A

rapid onset of encephalopathy with multifocal neurological deficits

42
Q

What are common neurological signs in ADEM?

A

hemiplegia, pyramidal signs, vision changes

43
Q

Why is testing for a viral etiology important in ADEM?

A

because acyclovir can be helpful

44
Q

Which viruses can commonly precede ADEM?

A

HSV, VZV, EBV, WNV

45
Q

What MRI finding in ADEM can be associated with future MS?

A

periventricular lesions

46
Q

What is encephalitis?

A

inflammation of the brain tissue with neurologic dysfunction

47
Q

What is the typical presentation for encephalitis?

A

flu-like illness followed by behavior changes, headaches, nausea, and vomiting

48
Q

What disease can cause temporal lobe seizures?

A

HSV

49
Q

What are the two disease processes that cause encephalitis?

A

autoimmune and viral processes

50
Q

What is the treatment for pediatric encephalitis?

A

empiric antibiotics and acyclovir

51
Q

What are three factors predictive of long-term deficits in pediatric encephalitis?

A

viral etiology, abnormal brain imaging, and longer hospital stay

52
Q

What 4 things are children with a history of encephalitis at risk for?

A

headaches, sleep problems, behavior problems, and learning problems

53
Q

What is the most common cause of viral encephalitis not associated with epidemics?

A

HSV

54
Q

What is the major pathologic process in VZV encephalitis?

A

vasculitis

55
Q

What vessels are commonly involved in VZV related vasculitis?

A

MCA and carotid

56
Q

How does VZV encephalitis typically present?

A

with zoster ophthalmic followed by vasculitic hemiplegia

57
Q

How is Zika thought to affect the CNS?

A

It is thought to cause neuronal cell death and impair neurogenesis

58
Q

What MSK abnormality can be seen with Zika?

A

arthrogryposis

59
Q

What receptor is targeted by antibodies in anti-NMDAR encephalitis?

A

NR1

60
Q

How does anti-NMDAR encephalitis present?

A

psychiatric symptoms, altered LOC, seizures, and dyskinesia

61
Q

What should be suspected in a boy presenting with new-onset tics and obsessive compulsions?

A

PANDAS

62
Q

What are the typical brain MRI findings in anti-NMDAR encephalitis?

A

often normal, may have T2 cortical and sub-cortical intensities

63
Q

Treatment for catatonia in anti-NMDAR encephalitis?

A

benzodiazepines

64
Q

Treatment for movement disorder in anti-NMDAR encephalitis?

A

immune therapy and tetrabenazine

65
Q

How does limbic encephalitis typically present?

A

rapid memory loss, temporal lobe seizures, and psychiatric symptoms

66
Q

What is Ophelia syndrome?

A

limbic encephalitis associated with Hodgkin’s lymphoma

67
Q

What underlying malignancy is associated with opsoclonus myoclonus?

A

neuroblastoma

68
Q

What presents with unilateral brain inflammation, partial seizures, and focal myoclonus?

A

Rasmussens’ encephalitis

69
Q

What is definitive treatment for Rasmussens’ encephalitis?

A

hemispherectomy

70
Q

What are the principal viruses associated with AFM?

A

Enterovirus D68 and A71

71
Q

What is the average age for presentation with AFM?

A

5 years old

72
Q

What remains intact on neurologic exam in AFM?

A

sensation

73
Q

What is the treatment for AFM?

A

supportive care

74
Q

For how long can a child continue to recover from AFM?

A

Over a year

75
Q

What is predictive of worse outcome in AFM?

A

severe weakness at nadir

76
Q

What are two rheumatologic diseases that can present with new-onset movement disorders?

A

SLE and Sjogren syndrome