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