Demyelinating, Myelitis, Encephalitides Flashcards
In what ethnic group are pediatric demyelinating diseases more common?
Blacks
What antibodies are often found in about 1/3 of children with an acute demyelinating syndrome?
myelin oligodendrocyte glycoprotein (MOG) antibodies
What are the two general criteria for diagnosis of MS?
Dissemination in time and space
What qualifies as dissemination in space for diagnosis of MS? (2017 McDonald criteria)
involvement of at least two of four areas in the CNS (periventricular, juxtacortical, infratentorial, spinal cord)
What qualifies as dissemination in time for diagnosis of MS? (2017 McDonald criteria)
simultaneous presence of enhancing and non-enhancing lesions or new lesions on follow-up MRI
Remote history of what virus is associated with pediatric-onset MS?
EBV
What type of MS do children usually have?
relapsing-remitting
How does progression of MS in children differ than adults?
children have higher relapse rates but quicker recovery
How does MS present in children? Give 4 general symptoms.
optic neuritis, gait disturbance, brainstem symptoms, sensory deficits
What are two MRI findings in MS?
ovoid T2 lesions and FLAIR hyperintensities in the periventricular white matter
What is elevated in the CSF of MS patients?
lymphocytes and protein
What lab finding indicates ADEM over MS?
anti-MOG
In MS, how does lesion burden and cognitive impairments in children compare to adults?
children have higher rates of cognitive impairment and greater MRI lesion burden
What are two factors predictive of increased long-term disability in pediatric MS?
lack of full recovery after an initial progressive course and number of relapses
What are the two first-line medications used in pediatric MS?
beta-interferon and glatiramer acetate
What percentage of patients with MS have neurogenic bladder? What types?
80%, DSD and detrusor hyperactivity
How is the course of transverse myelitis characterized?
acute monophasic
In what ages is pediatric transverse myelitis most common?
bimodal distribution: 0-5, 10 -17
In what level of the spinal cord is pediatric TM most common?
thoracic
What is seen on MRI in pediatric TM?
T2 hyperintensities and T1 isointense signals over multiple segments of the cord
What are 3 disease categories associated with pediatric TM?
viruses, systemic inflammatory disorders, multifocal CNS disorders
Is TM generally more severe in children or adults?
children
When does pediatric TM symptoms nadir usually occur?
within a week of presentation
What are the treatments for pediatric TM?
High-dose steroids for one week with consideration for IVIG or PLEX
What is a child with cervical transverse myelitis at risk for?
respiratory decompensation
What are three non-intuitive factors associated with a poor prognosis in pediatric TM?
female sex, spinal shock, and rapidity to nadir
What presents with optic neuritis and transverse myelitis?
Neuromyelitis Optica (NMO)
What antibody is associated with NMO?
aquaporin-4 (AQP4-IgG)
What two structures get damaged in NMO?
myelin and astrocytes
Who is the typical NMO patient?
a non-white female in early adolescence
What clinical manifestation of NMO is more severe in children than adults?
vision loss
What are 3 areas of the brain that are aquaporin-4 rich?
hypothalamus, periventricular gray matter, and subcortical white matter
How are the MRI enhancements in NMO characterized?
“cloud-like”
Why can NMO resemble ADEM?
because it can present with encephalopathy
How does postrema involvement in NMO present?
with intractable emesis
How does treatment for pediatric NMO differ from TM?
NMO patients are more likely to get long-term immunosuppression
What are the two most common functional deficits in NMO?
vision loss and paraplegia
What medication has been shown to be effective treating spasms in NMO?
carbamazepine
What are the MRI findings in acute disseminated encephalomyelitis (ADEM)?
large diffuse white matter lesions with poor demarcation
In what age range do children typically present with ADEM?
5 to 8 years old
What is the typical clinical presentation of ADEM?
rapid onset of encephalopathy with multifocal neurological deficits
What are common neurological signs in ADEM?
hemiplegia, pyramidal signs, vision changes
Why is testing for a viral etiology important in ADEM?
because acyclovir can be helpful
Which viruses can commonly precede ADEM?
HSV, VZV, EBV, WNV
What MRI finding in ADEM can be associated with future MS?
periventricular lesions
What is encephalitis?
inflammation of the brain tissue with neurologic dysfunction
What is the typical presentation for encephalitis?
flu-like illness followed by behavior changes, headaches, nausea, and vomiting
What disease can cause temporal lobe seizures?
HSV
What are the two disease processes that cause encephalitis?
autoimmune and viral processes
What is the treatment for pediatric encephalitis?
empiric antibiotics and acyclovir
What are three factors predictive of long-term deficits in pediatric encephalitis?
viral etiology, abnormal brain imaging, and longer hospital stay
What 4 things are children with a history of encephalitis at risk for?
headaches, sleep problems, behavior problems, and learning problems
What is the most common cause of viral encephalitis not associated with epidemics?
HSV
What is the major pathologic process in VZV encephalitis?
vasculitis
What vessels are commonly involved in VZV related vasculitis?
MCA and carotid
How does VZV encephalitis typically present?
with zoster ophthalmic followed by vasculitic hemiplegia
How is Zika thought to affect the CNS?
It is thought to cause neuronal cell death and impair neurogenesis
What MSK abnormality can be seen with Zika?
arthrogryposis
What receptor is targeted by antibodies in anti-NMDAR encephalitis?
NR1
How does anti-NMDAR encephalitis present?
psychiatric symptoms, altered LOC, seizures, and dyskinesia
What should be suspected in a boy presenting with new-onset tics and obsessive compulsions?
PANDAS
What are the typical brain MRI findings in anti-NMDAR encephalitis?
often normal, may have T2 cortical and sub-cortical intensities
Treatment for catatonia in anti-NMDAR encephalitis?
benzodiazepines
Treatment for movement disorder in anti-NMDAR encephalitis?
immune therapy and tetrabenazine
How does limbic encephalitis typically present?
rapid memory loss, temporal lobe seizures, and psychiatric symptoms
What is Ophelia syndrome?
limbic encephalitis associated with Hodgkin’s lymphoma
What underlying malignancy is associated with opsoclonus myoclonus?
neuroblastoma
What presents with unilateral brain inflammation, partial seizures, and focal myoclonus?
Rasmussens’ encephalitis
What is definitive treatment for Rasmussens’ encephalitis?
hemispherectomy
What are the principal viruses associated with AFM?
Enterovirus D68 and A71
What is the average age for presentation with AFM?
5 years old
What remains intact on neurologic exam in AFM?
sensation
What is the treatment for AFM?
supportive care
For how long can a child continue to recover from AFM?
Over a year
What is predictive of worse outcome in AFM?
severe weakness at nadir
What are two rheumatologic diseases that can present with new-onset movement disorders?
SLE and Sjogren syndrome
