CC5: Demyelinating Disorders Flashcards
diseases of mylein can be categorized:
myelinoclastic or dysmyelinating
destruction of normal myelin
myelinoclastic
metabolic abnormality,
breakdown abnormal myelin
dysmyelinating
chronic, demyelinating, autoimmune disease of the brain and spinal cord, usually characterized by recurrent attacks and remission of neurologic dysfunction
multiple sclerosis
MS gene pathogenesis:
HLA-DRB1
MS virus pathogenesis:
EBV, HHV, chlamidia, measles
MS metabolic pathogenesis:
vit d, obesity
MS can cause:
axonal injury, focal demylinated lesions, phagocytic microglia, lymphocytes, gliolisis, wallerian degneration
severe eye pain left eye, sees black spot
MS
higher in females and white people
MS
most common MS signs
optic neuritis
brainsten syndrome
cerebellar
transverse myelitis
optic neuritis is common for
MS
esions of the medial longitudinal fasciculus (MLF). Paresis of adduction on lateral gaze & associated nystagmus in the abducting eye
Internuclear ophthalmoplegia
Patients present with unilateral intense paroxysmal pain of the electrical discharge type (“painful flashes”)
Trigeminal Neuralgia
what are cerebellar syndromes?
coordination difficulty: ataxia, gait, tremor, dysarthia
May be the initial presentation of MS:
transverse myelitis
MS diagnosis is based on:
history and physical finding were there is history or evidence of at least two attacks separated in space and time of at least one month that can’t be explained by one single lesion
5 Key principles in the diagnosis of MS
- Identification of a syndrome “typical” of MS-related demyelination
- Objective evidence of CNS involvement
- Demonstration of dissemination in space
- Demonstration of dissemination in time
- “no better explanation” other than MS
what is the McDonald criteria
2010-Diagnostic Criteria for MS
what is Schumacher Criteria 1965
older 5 key principles of MS diagnosis
expalin Mcdonald ceiteria
diagnosis MS-no better explanation
possible MS-not all criteria met
not MS-another better diagnosis
where can you find lesions in MS mri?
T2 lesions around lateral ventirvle, brainstem and cerebellum
Neurodegenerative lesions appear on mri as
black holes
MS lab studies:
CSF
IgG, oligoclonal bands, BMP
Measures, in time, the response of a sensory tract, nucleus and/or cortical area to an external stimuli
Evoked potentials
Visual evoked potentials VEP is sensitive to
optic neuritis P100
Worsening of a sign or symptom with exercise of increased temperature
Uthhoffs phenomenon
Sudden sensation of electric shock down spine and arms when patient flexes neck
Lhermitte’s sign
can MS relapse?
yes. relapsing remmiting MS.
Mediated by lymphocytes & stimulated by exposure to infectious agents.
adaptive immunity
Protection that relays on mechanisms that exist before infection, capable of rapid response, react essentially the same way to repeated infections.
innate immunity
what guide innate immunity
microglia, monocytes, dendritic cells
what guides adaptive immunity
t cells, b cells
Earliest expression of MS
CIS clinically isolated syndrome
Earliest expression of MS
CIS clinically isolated syndrome
the best choice to support the clinical diagnosis of MS
MRI
DIS and DIT
the most important diagnostic CSF study
CSF qualitative assessment for oligoclonal bands (OCBs)
Once known as Devic’s Disease
Neuromyelitis Optica Spectrum Disorder (NMOSD)
Characterized by a combination of bilateral ON and cervical
myelopathy
Neuromyelitis Optica Spectrum Disorder (NMOSD)
neuromyelitis optica antibodies:
astrocytes and oligodendroyctes IgG
neuromylelitis opctica clinical presentation:
relapsing, area postrema vomiting, Optic Nerve and myelopathy occur, MRI lesions
NMOSD mri findings
diencephalic lesions and cerebral syndrome
- AQP-4-IgG diagnostic
2 or more clinical core characteristics
* 1 must be ON, acute myelitis, or area postrema syndrom
NMO vs. MS
NMO is more severe,
MRI has longer bands
bilateral ON
medullary lesion-vomit
CSF
IgG-astrocyte
Uniphasic syndrome occurring is association with an immunization or vaccination or systemic viral infection
Acute disseminated encephalomyelitis (ADEM)
Usually develops after URTI, EBV, CMV, mycoplasma, HIV
ADEM
ADEM is more common in
children
ADEM can present in two forms:
encephalitic and myelitic
ADEM features
preceding events of vaccine
multifocal or diffuse CNS signs
vessel damage
can ADEN relapse
no, rapid recovery
