Multiple Sclerosis Flashcards
Multiple Sclerosis
the most common immune-mediated inflammatory demyelinating disease of the central nervous system. Pathologically characterized by multifocal areas of demyelination with loss of oligodendrocytes, astroglial scarring and Axonal injury
Test of choice in dx MS
MRI
Characteristic lesions of MS
Cerebral or spinal plaque
Requirement for diagnosis of MS
demonstration of central nervous system lesion in time and space, based on either clinical findings alone or a combination of clinical and MRI findings
Dissemination in space
on MRI one or more T2 lesion in at least two of four MS typical regions (periventricular, juxtacortical, indratentorial, spinal cord) OR a different clinical attack implicating a different central nervous system site
Dissemination in time
on MRI simultaneous presence of asymptomatic gandolinium enhancing and nonenhancing lesions at any time OR a new t2 and or gadolinium enhancing lesion on follow up MRI with reference of a baseline scan OR by the development of a second attack
Patients with two or more attacks who have objective clinical evidence of one lesion need evidence of
dissemination in space
Patients with one attack who have objective clinical evidence of two or more lesions need evidence of
dissemination in time
Lumbar puncture findings with MS
Elevation of CSF immunoglobulin relative to other protein compounds, increased igG index
Features suggestive of MS
Relapse and remissions. Onset between 15 and 50, optic neuritis, lhermitte sign (electric sensation that travels down your neck and back and also feels present in the arms, legs, fingers, and toes. The pain is usually strongest when you bend your neck forward), internuclear ophthalmoplegia- impairment of horizontal eye movements, fatigue, heat sensitivity (uhthoff phenomenon)
Features atypical for MS
steady progression, age before 10, age after 50, cortical deficits such as aphasia, apraxia, neglect. Rigidity, sustained dystonia, convulsions, early dementia, deficit deveoping within minutes
Clinically isolated syndrome
the first attack of a disease compatible with MS that exhibits characteristics of inflammatory demyelination but has yet to fulfill MS diagnostic criteria, if untreated with most likely lead to a diagnosis of MS
Relapsing-Remitting MS
clearly defined relapses with full recovery or with sequelae and residual deficit upon recovery None or minimal disease progression. Most will eventually enter progressive.
Secondary Progressive MS
initial RRMS followed by progession with or without occasional relapses, minor remission.
Primary Progressive MS
Disease progression from the onset with occasional plateuas and temporary minor improvements. 10 percent of adult cases.
MS is ____ mediated
immune
Immune cells are made where
throughout the body except the brain and spinal cord. Made in the tonsils, thymus, bone marrow, spleen, lymphoid tissue
Diagnose MS
clinically
Nerve damage and myelin loss
Axons have a protective myelin coating that is necessary for normal conduction of electrical impulses. In MS the immune system destroys the myelin resulting in slowed (not absent)* conduction and exposure of axons. Exposed axons can then be severed causing them to lose nerve function once degraded causing permanent loss of the axon. Results in permanent loss.
Patho of blood brain barrier breakdown
BBB prevents the entrance of T cells into the nervous system. infections or virsuses can alter this barrier and make it permeable to T cells. Once it regains integrity after the infection has cleared the T cells are still trapped inside the brain
Autoimmunology pathio
Immune system T cells are attacking the nervous system myelin leading to plaques and lesions within the white matter. This destroys oligodendrocytes leading to demyelination, can remyelinate early but not completely and with each attack will lead to further remylination
Inflammation
T cells attacking myelin leads to cytokines and antibodies to come and cause inflammation. BBB leaks lead to swelling and activation of macrophages
Most common initial symptoms of MS
changes in sensation of arms, legs, face, optic neuritis, weakness, internuclear opyhalmoplegia – nystagmus, eye cannot look inward., unsteady when walking, balance problems
Lhermitte sign
Electric sensation run down the back into the limbs by bending the neck forward. Means lesion in the dorsal column of the cervical cord or caudal medulla
Uhthoff phenomenon
worsening of neurological symptoms in MS where the body gets overheated from weather, exercise, fever, saunas, hot tubs
Main symptoms of MS
fatigue, cognitive impairment, mood swings, depression, optic neuritis, nystagmus, diplopia, dysphagia, muscle weakness, muscle spasms, pain sensation, parasethesias, bowel incontinence, diarrhea or constipation, urinary incontinence or frequency
Who gets MS
Diagnosed between 15 and 50, more common in women, caucasians
Name for early MS lesions showing thinly remylinated axons
Shadow plaques
3 tests that help diagnose MS
MRI, LP spinal fluid, evoked potential
Diagnostic criteria for MS
Time and space – damage has occured in at least two separate areas at two different points in time. Can take months to years to properly diagnose
What do you need if… 1 clinical attack and evidence of 1 lesion
DIS – another lesion or another attack proving it is from a different location and a new time (DIT), DIT need another attack
1 attack and 2 lesions need
DIT need another attack
2 attacks, 1 lesion
DIS a lesion that is new or an attack showing a new lesion in a new location
2 attacks, 2 lesions or 1 lesion with a history of a prior attack
None – has 2 attacks (DIT) and 2 lesions/hx of lesion in a different place (DIS)
Dissemination in space
demonstrated with MRI by one or more t2 lesions in at least two of four places: periventricular, juxtacortical, infratorial or spinal cord — OR! – by the development of a further attack implicating a different central nervous system site.
Dissemination in time
MRI by the simulataneous presence of asmpyomatic gadolinium-enhancing and nonenhancing lesions at any time – OR – a new T2 and/or gandolinium enhancing lesion on a follow up MRI (needed to have a baseline scan) – OR – by the development of a second clinical attack
Positive CSF in MS
Oligoclonal IgG bands in the CSF or elevated IgG
Test of choice for MS
MRI*
Evoked potential testing
Electrical events generated in the central nervous system by peripheral stimulation of a sensory organ, tests abnormal nervous system function, detects subclinical lesion in a site remote from a region of clinical diagnosis of multifocal MS and those not easily seen on imagin (optic nerves, dorsal columns)
LP in MS
Oligoclonocal banding MAY be seen and can help with the diagnosis as the proteins separate and forms bands. LP is not a requirement in the dx. Can help to rule out other conditions. Elevation of CSF IFF relative to other protein components. igA and igM may also be increased. + test is OCB or increased igG
RRMS
85 percent have this –> secondary progressive. Unpredictable attacks which may or may not leave permanent deficits followed by periods of remission
Clinically Isolated Syndrome
a first neurological event suggestive of demylination, at high risk for developing definite MS if it is accompanied by multiple, clinically silent asymptomatic lesions on MRI typical of MS
Primary progressive MS
Gradual progression of the disease from he onset with no relapses or remissions
Secondary progressive MS
Initial RRMS that suddenly begins to decline without periods of remission or replaces, just have the symptoms and get worse
Progressive relapsing MS
Steady decline since onset with superimposed attacks
Some examples of disease modifying drugs
Fingolimod, Teriflunomide, Alemtuzamab, Mitoxantrone, Natulizaumab
Relapse management
Relapse is a new symptom or a sudden worsening of old symptom lasting at least 24 hours and usually accompanied by an objective change in neuro findings. Treat w corticosteroids if it interferes w functioning, a 3-5 day course of methylprednisolone w or w out taper and rehab to restore function following a relapse
Spasicity mgmt
Eliminate sources of pain, stretch, ROM, weight bering, cryotherapy, aerobics, EMG feedback, electric stimlation, pool therapy. Meds baclogen intrathecal, Zanaflex, Klonopin, Neurontin
Procedures with spasicity
botox, baclofen pump, cryotherapy cool and stretching, tendonontomy, rhizotomy, myelotomy, motor point blocks
Heat sensitivity
common in MS. Cold can also occur. Sligh temp elevation can cause worsened symptoms – pseudoexaccerbation. Cool strategies beneficial during hot weather, exercise, cooking