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PRM Board Exam > Demyelinating, Myelitis, Encephalitides > Flashcards

Demyelinating, Myelitis, Encephalitides Flashcards

(76 cards)

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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
What is a child with cervical transverse myelitis at risk for?
respiratory decompensation
26
What are three non-intuitive factors associated with a poor prognosis in pediatric TM?
female sex, spinal shock, and rapidity to nadir
27
What presents with optic neuritis and transverse myelitis?
Neuromyelitis Optica (NMO)
28
What antibody is associated with NMO?
aquaporin-4 (AQP4-IgG)
29
What two structures get damaged in NMO?
myelin and astrocytes
30
Who is the typical NMO patient?
a non-white female in early adolescence
31
What clinical manifestation of NMO is more severe in children than adults?
vision loss
32
What are 3 areas of the brain that are aquaporin-4 rich?
hypothalamus, periventricular gray matter, and subcortical white matter
33
How are the MRI enhancements in NMO characterized?
"cloud-like"
34
Why can NMO resemble ADEM?
because it can present with encephalopathy
35
How does postrema involvement in NMO present?
with intractable emesis
36
How does treatment for pediatric NMO differ from TM?
NMO patients are more likely to get long-term immunosuppression
37
What are the two most common functional deficits in NMO?
vision loss and paraplegia
38
What medication has been shown to be effective treating spasms in NMO?
carbamazepine
39
What are the MRI findings in acute disseminated encephalomyelitis (ADEM)?
large diffuse white matter lesions with poor demarcation
40
In what age range do children typically present with ADEM?
5 to 8 years old
41
What is the typical clinical presentation of ADEM?
rapid onset of encephalopathy with multifocal neurological deficits
42
What are common neurological signs in ADEM?
hemiplegia, pyramidal signs, vision changes
43
Why is testing for a viral etiology important in ADEM?
because acyclovir can be helpful
44
Which viruses can commonly precede ADEM?
HSV, VZV, EBV, WNV
45
What MRI finding in ADEM can be associated with future MS?
periventricular lesions
46
What is encephalitis?
inflammation of the brain tissue with neurologic dysfunction
47
What is the typical presentation for encephalitis?
flu-like illness followed by behavior changes, headaches, nausea, and vomiting
48
What disease can cause temporal lobe seizures?
HSV
49
What are the two disease processes that cause encephalitis?
autoimmune and viral processes
50
What is the treatment for pediatric encephalitis?
empiric antibiotics and acyclovir
51
What are three factors predictive of long-term deficits in pediatric encephalitis?
viral etiology, abnormal brain imaging, and longer hospital stay
52
What 4 things are children with a history of encephalitis at risk for?
headaches, sleep problems, behavior problems, and learning problems
53
What is the most common cause of viral encephalitis not associated with epidemics?
HSV
54
What is the major pathologic process in VZV encephalitis?
vasculitis
55
What vessels are commonly involved in VZV related vasculitis?
MCA and carotid
56
How does VZV encephalitis typically present?
with zoster ophthalmic followed by vasculitic hemiplegia
57
How is Zika thought to affect the CNS?
It is thought to cause neuronal cell death and impair neurogenesis
58
What MSK abnormality can be seen with Zika?
arthrogryposis
59
What receptor is targeted by antibodies in anti-NMDAR encephalitis?
NR1
60
How does anti-NMDAR encephalitis present?
psychiatric symptoms, altered LOC, seizures, and dyskinesia
61
What should be suspected in a boy presenting with new-onset tics and obsessive compulsions?
PANDAS
62
What are the typical brain MRI findings in anti-NMDAR encephalitis?
often normal, may have T2 cortical and sub-cortical intensities
63
Treatment for catatonia in anti-NMDAR encephalitis?
benzodiazepines
64
Treatment for movement disorder in anti-NMDAR encephalitis?
immune therapy and tetrabenazine
65
How does limbic encephalitis typically present?
rapid memory loss, temporal lobe seizures, and psychiatric symptoms
66
What is Ophelia syndrome?
limbic encephalitis associated with Hodgkin's lymphoma
67
What underlying malignancy is associated with opsoclonus myoclonus?
neuroblastoma
68
What presents with unilateral brain inflammation, partial seizures, and focal myoclonus?
Rasmussens' encephalitis
69
What is definitive treatment for Rasmussens' encephalitis?
hemispherectomy
70
What are the principal viruses associated with AFM?
Enterovirus D68 and A71
71
What is the average age for presentation with AFM?
5 years old
72
What remains intact on neurologic exam in AFM?
sensation
73
What is the treatment for AFM?
supportive care
74
For how long can a child continue to recover from AFM?
Over a year
75
What is predictive of worse outcome in AFM?
severe weakness at nadir
76
What are two rheumatologic diseases that can present with new-onset movement disorders?
SLE and Sjogren syndrome

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