Demyelinating Diseases Flashcards
Multiple Sclerosis
chronic inflammatoy disorder characterized by progressive neurological decline that manifests in different clinical phenotypes
MS risk factors
gender (female)
family history
geograpahical latitude
low vitamin D levels
Chronic MS symptoms
fatigue exacerbated by heat (Uhthoff’s phenomenon) that improves with cooler temperatures
sleep disturbances, depression, euphoria, urinary retention, paroxysmal spasms, and cognitive decline
treatment for paroxysmal spasms in MS
carbamazepine
neuropsych testing in MS
deficits in long term memory (most common), verbal fluency, as well as speed and working memory
epilepsy and MS
rare complication, seen in only 2-5%
MS symptoms
broad spectrum of acute focal neurological symptoms, including but not limited to focal sensory or motor deficits, monocular visual loss, diplopia, and gait disturbance
MS diagnostic criteria
2017 McDonald Criteria
2017 McDonald Criteria
diagnosis based on patients that have evidence of demyelination with dissemination in space and time
can be based on clinical changes (relapse), feature son imaging, and CSF findings
Dissemination in Space (DIS)
development of lesions in two distinct anatomical locations indicating a multifocal disease process
Dissemination in Time (DIT)
appearance of new CNS lesions on imaging over time, or lesions of different appearance (bright vs dark) indicating new and old
oligoclonal bands in CSF now considered indication of DIT as well
remitting-relapsing MS (RRMS)
clearly defined attacks with neurologic recovery (complete or incomplete) between them
secondary progressive MS (SPMS)
seen in patients who initially had RRMS but now experiencing a gradual worsening regardless of occasional attacks
early aggressive treatment may delay this progression
primary progressive MS (PPMS)
progressive decline of neurological function without clearly defined attacks over the course of at least 1 year
Clinically Isolated Syndrome (CIS)
defined as a monophasic demyelinating episode in someone who otherwise has no clinical history of demyelinating disease
CIS treatment
initiation of a disease-modifying therapy (DMT) after CIS can delay time to a definitive diagnosis of MS
radiologically isolated syndrome (RIS)
diagnosed in a patient with an MRI that is consistent with MS but they have no clinical history of transient focal neurological deficits
when diagnosed, patients have a 33% chance of having a clinical demyelinating attack over the following two years
risk of MS is higher of oligoclonal bands are present in CSF
typical findings in CSF in MS
elevated protein
elevated CSF IgG index
oligocloncal bands in CSF that do not present in the serum
possibly slight elevation of WBC count (lymphocytic preodminant)
typical finding in MRI in MS
demyelinating lesions related to MS are ovoid, >3mm in diameter, and are typically located in particular regions: periventricular (Dawson’s fingers), juxtacortical, cortical, or spinal cord
acute: contrast enhance due to damage to blood brain barrier
chronic: hyperintense on T2/FLAIR sequences
typical demyelinating attacks
optic neuritis
transverse myelitis
optic neuritis presentation
presents with monocular decreased visual acuity, loss of color vision, and an afferent pupillary defect (APD) due to demyelinating lesion of the optic nerve
often painful with extraocular movements
children more likely to present with headache and bilateral symptoms
can be initial presentation of MS or NMO
optic neuritis funduscopic exam
swollen optic disc
optical coherence tomography (OCT) in optic neuritis
thinning of the retinal nerve fiber layer weeks to months after an acute attack
visual evoked potentials (VEPs) in optic neuritis
prolonged P100 latency, even in patients with distant histories of ON with no residual visual deficits