MS, MG, MD Flashcards
Weakness
Considered disorders of nerve conduction
Autoimmune, Lymphocytes?
Common to what two disorders?
Multiple Sclerosis and Myasthenia Gravis
Autoimmune disease which causes inflammatory demylnation of CNS
Reduces nerurons ability to function
Multiple Sclerosis
Found primarily in young adults, usually under age of 55 at onset (typically mid-20’s to 30)-if older onset, may be more severe
Women>men (may be more severe in men)
Epidemiology of which condition?
Multiple sclerosis
CIS
RRMS
primary progessive
secondary progressive
progressive relapsing
Different forms of which condition?
MS
Likely autoimmine- higher risk with other autoimmune diseases
High risk: Northern Europe, Southern Canada, Northern US
Low risk: Asian, African, American Indian
MS
- Patients of Western European lineage (genetics) who live in temperate zones (environmental)
- If patients move from one “zone” to another before age 15, he/she appears to adopt that zone’s risk
- Less risk closer to equator—is sunlight or Vitamin D protective?
- HLA-DR2 ?, HLA-DRB1 association
- Exposure to viral infections—EBV? Varicella?
- Genetic predisposition? Increased risk if (+) FHX
- Exposure to vaccines?
- Smoking? Alcohol?
- Month of birth? May>November
Risk factors for which condition?
MS
Inflammation –> demyelination –> axonal degeneration
Still an area of research
Pathogenesis of what condition?
MS
Shows response to treatment with immunomodulating rx
Disruption of BBB= rx that blocks T cell movement into CSF improve MS sx
MS
Signs/Sx:
Weakness, fatigue, numbness, tingling, unsteadiness in a limb, spastic paraparesis, retobulbar neuritis/optic neuritis (blurred or dimmed vision, blind spots (central vision), pain w. eye movement, HA, sudden color blindness, imparied night vision, impaired contrast sensitivity, diplopia), disequilibrium/vertigo, pain, sphincter disturbance (urinary urgency or hesitancy)
MS
Relapse/remission
Onset between 15-50
Optic neuritis
Lhermitte’s sign
Internuclear opthalmoplegia
Fatigue
Uhtoff’s phenomenon
These are suggestive or not suggestive of MS?
SUGGESTIVE
Steady progression
Onset before age 10 or after 50
Cortical deficits-aphasia, apraxia, alexia, neglect
Rigidity, sustained dystonia
Convulsions
Early dementia
Deficity developing in minutes
Suggestive/Not suggestive of MS?
NOT suggestive
TIA/CVA
B12 deficiency
HIV
Lyme Neuroborreliosis
Neurosyohilis
Acute disseminated encephalomyelitis
Acute hemorrhagic leukoencephalitis
Acute/subacute transverse myelitis
DDX with which condition?
MS
Certain criteria must be met (McDonald)
Must show two or more different areas in central areas of white matter affected to different times
Only one area may be clinically affected—but a “probable” diagnosis can be made in patients with multi-focal disease on imaging but with only one clinical attack or with a history of at least two clinical attacks but signs of only one lesion
Dx for which condition?
MS
Imaging test of choice to diagnosis clinically suspected MS?
MRI
Lesion is the cerebral or spinal plaque - descrete region of demyelination with initially preserved axon
Found in the periventricular, juxtacortical, infratentorial and/or spinal cord
MRI results of which condition?
MS
Increased Gadolinium enhancement may indicate ______ lesion?
Acute or chronic
Acute
Is an acute lesion symptomatic/asymptomatic in MS?
Asymptomatic!
What other lab should be performed for MS?
Lumbar puncture
Also useful to rule out other dx: B12, Lyme, etc
May have mild lymphocytosis, may have IgG in CSF
Probably normal opening pressure
Albumin in CSF indicates disruption in BBB
Lumbar puncture results in which condition?
MS
Presense of ________ on lumbar puncture is highly suggestive of MS
oligoclonal bands
What is the most common form of MS?
Relapsing-remitting MS
How many forms of MS are recognized?
4!
Initial episode then months or years before new sx emerge or previous sx return
Can lead to incomplete remissions and progressive disability with weakness, spatsticity, ataxia of limbs, impaired vision, and urinary inconinence
PE: optic atrophy, nystagmus, dysarthria, pyramidal/sensory/cerebellar deficits in one or multiple limbs
What form of MS?
RRMS
Acute exacerbation of sx lasting says to weeks, at minimum 24 hours
Defintion of what?
Relapse
Can be previous sx or new sx
Relapse triggers/infection/trauma/stress/pregnancy?
Frequency of relapse is variable –> more common in early dx
Uhthoff’s phenomenon
What form of MS?
RRMS
In some patients, clinical course changes to secondary progressive (SPMS). Some studies indicate that most patients will progress to SPMS
Primary progressive (PPMS)—less common (~10%), Symptoms are steadily progressive from onset.
Progressive relapsing MS (PRMS)—a subset of MS which has some remissions, but steady decline still dominates disease course.
Other forms of what dx?
MS
Fully functional in all neurological systems 15 years after disease onset
What severity of MS?
Benign
Rapidly progressive course leading to significant disability
What severity of MS?
Malignant
No cure
Tx aimed at improving quality of life and limiting disability
Strong support network- family, therapists, social services
Tx for what?
MS
What are acute attacks of MS most commonly treated with?
Glucocorticoids
(methylprednisolone 1g IV x 3-7 days)
Aimed at reducing relapses and slowing progression of MS?
Disease Modifying Therapy
Historically SC or IM (IV for certain patients)
Now 3 oral options
What type of therapy for MS?
Disease Modifying Therapy
- Interferon-b– cytokine that modulates immune responsiveness
- Betaseron / Extavia(Interferon-b1b)
- 250mcg SC administered QOD
- Rebif (Interferon-b1a)
- 44mcg SC administered 3x/week
- Avonex (Interferon-b1a)
- 30mcg IM administered weekly
- Plegridy (Pegylated Interferon-b1a)
- 125mcg SC every other week
Tx for which condition?
MS
Injection site reactions
Flu-like sx
Depression
Elevated LFTs
Leukopenia/anemia
S/E of which type of drugs for MS therapy?
Interferons
What can develop and limit the effectiveness of IFNB treatment in MS?
Neutralizing antibody formation
Mixture of amino acids antigenically similar to myelin protein. Works by competing for T cells - but process not entirely understood
Daily 20mg SC admin
No routine bloodwork neeed
What tx for MS?
Glatiramer (Copaxone | Teva)
Injection site reactions (MC)
Transient flushing/anxiety/chest pains
S/E of which drug for MS?
Glatiramer (Copaxone)
First oral agent approved for MS
Sphingosine 1-phosphate receptor modulator on T-cells
Results in down-regulation of receptors and T-cell sequestration in lymphoid tissue
Generally well tolerated
What drug tx for MS?
Fingolimod (Gilenya | Novartis)
Elevated LFT’s
bradycardia (transient)
macular edema
skin cancers
varicella infections
S/E of which drug for MS?
Fignolimod