Week 5- Multiple Sclerosis Flashcards
PART 1: INTRO AND BACKGROUND
PART 1: INTRO AND BACKGROUND
What is multiple sclerosis (MS)?
-Disease that impacts CNS, in which immune system is triggered to attack it.
What is MS characterized by?
Course of demyelination mediated relapses and remissions with an unpredictable course.
Pathophysiology:
- Abnormal immuno-mediated response attacks ________, ___________, and the axons themselves through the CNS. Creates “holes” which slows AP and saltatory conduction.
- Activation of immune cells that cross BBB, enter CNS, and initiate damaging inflammatory cascade of events.
-myelin, oligodendrocytes, and axons
- Is MS a gradual continuous attack?
- Remyelination occurs but is often __________.
- No, the nervous system is able to regain composure (REMISSION) until next attack happens.
- Incomplete
- With time, anti-inflammatory responses/remyelination cannot keep up and we see demyelinated areas undergo ________, what is this?
- ______ matter > ______ matter
- Gliosis, which is a proliferation of neurological tissues resulting in scarring.
- white matter > gray matter (as desease progresses)
- MS is the most common cause of disability in what populations?
- What is the average age of onset?
- young, middle-aged adults
- 15-50
What is the cause of MS?
-Largely unknown (idiopathic)
What is the hypothesis for MS?
-Perfect storm between genetic predisposition and environmental antigens resulting in auto-immune response.
What are some predisposing factors to MS?
- Women > Men (3:1)
- Caucasian/Nordic
- Higher income contries
- Temp Zones (Western EU and NA)
- Low vitamin D exposure during childhood/teenage years.
What are the (3) things that help with diagnosis of MS?
- Clinical Presentation
- MRI
- Additional laboratory tests
With clinical presentation, patient must show S/Sx consistent with ____________.
-demyelination
- On a MRI we are looking for the signs of ________ (________ build up) which often take time to show.
- We are looking for dissemination in ________ and ______. What does this mean?
- With MS, we need to see lesions in 2 of what 4 MS typical areas to get a diagnosis.
- gliosis, plaque build up
- space and time, different areas of CNS and changes of plaque over time.
- Periventricular, juxtacortical, infratentorial, spinal cord
What are some additional lab tests that can be used to help with diagnosis of MS?
- Visual evoked potentials
- Lumbar puncture
SIGNIFICANT decline in number of attacks, lesion sites, and disability in patients that participate in ________ ______ _________ protocols.
-early drug treatment protocols
PART 2: S/Sx OF MS
PART 2: S/Sx OF MS
- What is a Clinically Isolated Syndrome (CIS)?
- Is it monofocal or multifocal?
- First episode of neurologic symptoms that lasts at least 24 hrs and is later on determined to be caused by demyelination.
- Can be either monofocal or multifocal.
- Clinically Isolated Syndrome most commonly affects what 3 areas?
- How are they initially treated?
- Optic nerves, brainstem, or spinal cord
- High-dose glucocorticoids for acute symptoms.
Do all CIS progress to MS?
-No, there are some cases of one big attack then lies dormant.
Risk factors for progression from CIS to MS:
- ____________ presentation
- > /=__ T2 MRI lesions
- Oligoclonal bands present in ______, not in serum.
- polysymptomatic presentation
- > /=2 T2 MRI lesions
- present in CSF, not in serum
- ____ + _____ findings indicative of earlier events = confirmed MS Diagnosis
- Why is this common?
- CIS + MRI
- Because a lot of times, the first couple of events the patient won’t have anything or an MRI or think anything is going on.
- CIS + MRI findings = __-__% chance of MS developing.
- CIS without MRI findings = __% chance of MS developing.
- 60-80%
- 20%
What are the (3) most common early involved structures with CIS?
- Optic Nerve
- Brainstem
- Spinal Cord
Optic Neuritis:
- Unilateral reduced visual _______.
- Orbital pain particularly with _____ _________.
- Reduced ________ vision.
- Afferent ________ defect.
- Retrobulbar or mild _____ swelling.
- Unilateral reduced visual acuity.
- Orbital pain particularly with eye movement.
- Reduced color vision.
- Afferent pupillary defect.
- Retrobulbar or mild disc swelling.
Brainstem:
- Bilateral internuclear __________.
- Ataxia and gaze evoked __________.
- ____ nerve palsy.
- _____-_____ symptoms.
- Facial ________ loss.
- ________
- ________
- ________
- Bilateral internuclear ophthalmoplegia.
- Ataxia and gaze evoked nystagmus.
- 6th nerve palsy.
- Multi-focal symptoms.
- Facial sensory loss.
- Vertigo
- Ataxia
- Dysarthria
Spinal Cord:
- Incomplete transverse myelitis.
- Positive _________ sign.
- _________ symptoms.
- Asymmetric limb ________.
- Symptom progression between __ hours and ___ days.
- Incomplete transverse myelitis.
- Positive Lhermitte’s sign.
- Sphincter symptoms.
- Asymmetric limb weakness.
- Symptom progression between 4 hours and 21 days.
What is a Lhermitte’s sign?
-When bending neck and tucking chin to chest, complain of jolt sensation passing neck and into back.
List of MS Clinical S/Sx:
- Motor function
- Sensory function
- Visual deficits
- Cognitive function
- Poor tolerance to temp increases***
- Fatigue***
- Pain
- Sleep Disorders
- Speech and swallow impairments
- Dizziness
- Bowel and Bladder dysfunction
- Motor impairments of MS tend to be _________.
- Include things such as _______, ________, and __________.
- asymmetrical
- weakness, spasticity, coordination
Loss of sensation tends to be ______, usually we will see ____________.
- rare
- paresthesias
What visual deficit is most commonly seen in MS?
-Optic neuritis
What is the hallmark sign of cognitive dysfunction?
-Slowed information processing speed.
Pain following MS:
- _____________ most common.
- ___________ _______ presents as brief shooting pain in extremities.
- ____
- _________ ________ pain can result from ascending sensory tracts getting damaged and see demyelination.
- Trigeminal Neuralgia
- Paroxysmal limb pain
- HA
- Chronic Neuropathic pain
MS and Heat Insensitivity:
- ___% of MS patients are sensitive to increases in core body temp.
- What is the neuroblockade hypothesis?
- What is Uhthoff symptom?
- 80%
- De-myelinated neurons ability to conduct APs decreases as temp increases.
- Increase in presence of neurological symptoms in response to heating condition. (known as pseudo-exacerbation)
MS and Fatigue:
- Up to ___% of MS patients experience some type of acute chronic fatigue.
- Does it worsen as the day progresses?
- What exacerbates it?
- What do we see with both primary and secondary fatigue?
- What can fatigue ultimately lead to?
- 80%
- Yes
- Heat and exercises
- central, peripheral, psychological factors
- Can often lead to fear of fatigue → decreased physical activity → disuse → worsening disability
- MS Exacerbation: new and recurrent MS symptoms lasting >___ hours.
- _____________ are transient worsening of symptoms that occurs due to stress, infection, overheating, or overexertion and are
- 24 hours
- pseudo-exacerbations, <24 hours
PART 3: MS SUBTYPES AND MEDICAL MANAGEMENT
PART 3: MS SUBTYPES AND MEDICAL MANAGEMENT
What are the (3) major types of MS?
- Relapsing-Remitting MS (RRMS)
- Secondary Progressive MS (SPMS)
- Primary Progressive MS (PPMS)
Relapsing Remitting MS:
- Approximately ___% are initially diagnosed with RRMS.
- Clearly defined episodes of acute worsening of neurologic function (_______), followed by partial or complete recovery (_________) with periods of time between that patients are clinical free of disease progression.
- Relapses with either full recovery or some remaining neurological signs/symptoms and residual deficit on recovery.
- 85%
- relapses, remissions
Secondary Progressive MS (SPMS):
- Initially presents as ___ period followed by a steady worsening of neurological function, with or without relapses.
- Remissions are _____, or can have ________.
- RRMS
- Remissions are minor, or can have plateau.
What is the average time it will take someone with RRMS to progress to rediagnosis of SPMS?
-20 years
Primary Progressive MS (PPMS):
- Continuous worsening from initial onset without distinct ________ or ________.
- May have occasional plateaus and temporary ________ improvements.
- relapses or remissions
- minor improvements
What is the 4th declassified type of MS?
Progressive Relapsing MS (PRMS)
Progressive Relapsing MS (PRMS):
- Steady worsening with ________ and _________.
- Why has this gone away?
- relapses and remissions
- These patients could have been classified into the other subtypes.
What are the (3) goals taken for medical management of MS?
- Acute Exacerbation Treatment
- Disease Modifying Medications
- Symptom Management
What is the main way we deal with acute exacerbations of MS?
Immunosuppression drugs to treat acute flare ups and shorten duration of episode.
- Adrenocorticotropic hormone (ACTH)
- Methylprednisolone
- Prednisone
Disease modifying drugs are used for what purpose?
-Reduce frequency and severity of clinical attacks, educe development of lesion sites, slow down the progression of disability.
- _________ drugs are used to slow progression of disease and decrease symptoms. What is the downside?
- _________ are daily subcutaneous injections aimed to mimic the effects of myelin protein.
- _________ and _________ are the last line of defense.
- Interferon (make you feel like crap)
- Copaxone
- Tysabri and Novantrone
What are the (4) main things symptom management is aimed at with MS?
- Fatigue
- Spasticity
- Pain
- Urinary dysfunction
PART 4: OUTCOME MEASURES AND PROGNOSIS
PART 4: OUTCOME MEASURES AND PROGNOSIS
What is the EDSS?
Expanded Disability Status Scale
-Best and most widely known scale for quantifying disability in MS.
What are the (8) functional systems of the EDSS? (Looking at “do these patients have impairments in these functional systems”)
- Pyramidal (motor impairments)
- Cerebellar (trunk/limb ataxia)
- Brainstem (CN, extraoccular)
- Sensory (rarely full loss)
- Bowel and Bladder (Spinal Cord)
- Visual (common)
- Cerebral (cognitive, behavioral alterations)
- Other (any other neurological)
EDSS Levels 0-10:
- 0 = _____ neurological exam
- 1 = No disability, minimal signs in ____ FS
- 1.5 = No disability, minimal signs in _____ FS
- 2 = Minimal disability in ____ FS
- 2.5 = Minimal disability in _____ FS
- 3 = Fully ambulatory, moderate disability in ____ FS, or mild disability in __-__ FS
3. 5 = Fully ambulatory but with moderate disability in _____ FS and minimal or more disability in several other FS - 4 = Fully ambulatory without aid, self-sufficient, up and about some 12 hours a day, able to walk without aid or rest at least 500m without aid/rest.
- 4.5 = Fully ambulatory without aid, up and about much of the day, able to work a full day, may otherwise have some limitation of full activity or require minimal assistance, able to walk without aid or rest at least 300 meters.
- 5 = Ambulatory without aid/rest for about 200 meters; disability significant enough to impair full daily activities (e.g., to work a full day without special provisions)
- 5.5 = Ambulatory without aid for about 100 meters; disability significant enough to preclude full daily activities
- 6 = Intermittent or unilateral constant assistance (cane, crutch, brace) required to walk about 100 meters with or without resting
- 6.5 = Constant bilateral assistance (canes, crutches, braces) required to walk about 20 meters without resting
- 7 = Unable to walk beyond approximately 5 meters even with aid, essentially restricted to wheelchair; wheels self in standard wheelchair and transfers alone; up and about in wheelchair some 12 hours a day
- 7.5 = Unable to take more than a few steps; restricted to wheelchair; may need aid in transfer; wheels self but cannot carry on in standard wheelchair a full day; May require motorized wheelchair
- 8 = Essentially restricted to bed or chair or perambulated in wheelchair, but may be out of bed itself much of the day; retains many self-care functions; generally has effective use of arms
1
Disability Categorization by EDSS Score:
- __-__ = normal to mild disability
- __-__ = mild to moderate disability
- __-__ = moderate to severe disability
- __-__ = severe disability with restriction to bed or WC
- 0-3.5 = normal to mild disability
- 4-5.5 = mild to moderate disability
- 6-7.5 = moderate to severe disability
- 8-9.5 = severe disability with restriction to bed or WC
What is the main criteria to the EDSS?
- High criteria on ambulation, not looking at a lot other.
- Decrease in interrater reliability with lower scores.
- Not perfectly linear.
- COGNITION is never mentioned.
- What is another scale for quantifying disability in MS that is used less often?
- It is a simple measure that is entirely based off of ________ ability.
- Disease Step Scale
- ambulation
Disease Steps Scale:
- 1 = Functionally ______ with no limitations on activity or lifestyle.
- 2 = _____ disability, mild symptoms or signs.
- 3 = Early cane, use of a cane or other form of unilateral support for greater distances, but can walk at least ___ feet without it.
- 4 = Late cane, cane dependent, unable to walk ___ feet without a cone or other form of unilateral support.
- 5 = Bilateral support, requires _______ support to walk 25 feet.
- 6 = Confined to _______.
- U = Unclassifiable, used for patients who do not fit above classification.
- normal
- mild
- 25 feet
- 25 feet
- bilateral
- WC
What are some MS Subjective Outcome Measures. (5)
- 12-Item MS Walking Scale
- Fatigue Scale for Motor and Cognitive Function
- MSQOL-54
- Modified Fatigue Impact Scale
- Multiple Sclerosis Impact Scale (MSIS-29)
What is the cutoff score for call risk with the 12-Item MS Walking Scale?
- > /=75
Why is the Fatigue Scale for Motor and Cognitive Function good?
-Able to differentiate between motor and cognitive aspects of fatigue.
Modified Fatigue Impact scale looks at fatigues impact on what 3 aspects (wasn’t initially used/intended for MS)?
- Physical
- Cognitive
- Psychosocial
MSIS-29 looks at physical (20) and psychosocial (9) difficulties related in MS in past ____ weeks.
-2 weeks
1
1
Disease Progression:
- Varied and inconsistent response ________ patients.
- Patterns typically similar _______ patients.
- What is the mean time before use of unilateral AD?
- Life expectancy ~___ years less than age-matched peers.
- between
- within
- 15-20 years
- 10 years
What EDSS levels do PTs tend to be the most helpful with MS?
-Levels 3-6
Disease Prognosis:
- White matter lesions on MRI at initial clinical presentation demonstrated an ___% chance of developing MS in 7-10 years.
- Appearance of a new lesion on T2 weighted MRI < ___ months after initial clinical episode = poor prognosis.
- MRI Findings: Favorable prognostic factors including low total lesion burden, low active lesion formation, and negligible myelin or axon loss.
- 88%
- 3 months
Disease Prognosis:
- _____ of MS
- Level of recovery after ________ insult
- Time between ________
- Location of _____ lesion site
- Initiation of medical management
- Type of _________ at initial insult
- Gender
- Age of onset
- NEUROLOGICAL FINDINGS AFTER ___ YEARS
- Type
- initial insult
- relapses
- 2nd lesion
- symptoms
- 5 years
- If a patient has ________ impairments at diagnosis, this is indicative of a quicker overall progression.
- Males or females have faster progression?
- Neurological findings at ___ years is one of the most important prognostic factors for MS.
- cognitive
- males
- 5 years
