Podcast
Questions and Answers
In relapsing-remitting multiple sclerosis (RRMS), what is the typical pattern of disease progression?
In relapsing-remitting multiple sclerosis (RRMS), what is the typical pattern of disease progression?
- A progressive course from the onset with occasional plateaus or temporary minor improvements.
- Unpredictable attacks (relapses) followed by periods of partial or complete recovery (remissions). (correct)
- Steady progression of disability from the onset, with acute relapses but no remissions.
- Gradual worsening of symptoms from the onset without distinct relapses or remissions.
Which type of multiple sclerosis is characterized by a gradual neurological deterioration from the onset of the disease, without distinct relapses or remissions?
Which type of multiple sclerosis is characterized by a gradual neurological deterioration from the onset of the disease, without distinct relapses or remissions?
- Progressive-relapsing MS (PRMS)
- Relapsing-remitting MS (RRMS)
- Secondary-progressive MS (SPMS)
- Primary-progressive MS (PPMS) (correct)
A patient initially diagnosed with relapsing-remitting MS (RRMS) begins to experience a steady worsening of their symptoms, independent of relapses. This progression indicates a transition to which form of MS?
A patient initially diagnosed with relapsing-remitting MS (RRMS) begins to experience a steady worsening of their symptoms, independent of relapses. This progression indicates a transition to which form of MS?
- Secondary-progressive MS (SPMS) (correct)
- Primary-progressive MS (PPMS)
- Progressive-relapsing MS (PRMS)
- Benign MS
Which of the following best describes progressive-relapsing multiple sclerosis (PRMS)?
Which of the following best describes progressive-relapsing multiple sclerosis (PRMS)?
How does age of onset typically differ between relapsing-remitting MS (RRMS) and primary-progressive MS (PPMS)?
How does age of onset typically differ between relapsing-remitting MS (RRMS) and primary-progressive MS (PPMS)?
Which of the following statements accurately compares the relapse patterns in relapsing-remitting MS (RRMS) and secondary-progressive MS (SPMS)?
Which of the following statements accurately compares the relapse patterns in relapsing-remitting MS (RRMS) and secondary-progressive MS (SPMS)?
To differentiate between RRMS and PPMS, what key aspect of the patient's initial disease presentation would be most informative?
To differentiate between RRMS and PPMS, what key aspect of the patient's initial disease presentation would be most informative?
A patient with MS experiences an acute worsening of symptoms (relapse). After several weeks, the symptoms improve, but some residual deficits persist, representing an incomplete recovery. Which type of MS is MOST likely?
A patient with MS experiences an acute worsening of symptoms (relapse). After several weeks, the symptoms improve, but some residual deficits persist, representing an incomplete recovery. Which type of MS is MOST likely?
In a patient with a long-standing diagnosis of RRMS, what clinical finding would MOST strongly suggest a transition to secondary progressive MS?
In a patient with a long-standing diagnosis of RRMS, what clinical finding would MOST strongly suggest a transition to secondary progressive MS?
A researcher is studying different MS subtypes. They want to identify patients whose disease is characterized by progression from onset, with occasional plateaus or temporary minor improvements, but without distinct relapses or remissions. Which subtype is the researcher most likely studying?
A researcher is studying different MS subtypes. They want to identify patients whose disease is characterized by progression from onset, with occasional plateaus or temporary minor improvements, but without distinct relapses or remissions. Which subtype is the researcher most likely studying?
A patient newly diagnosed with MS is being educated about their disease course. Which statement accurately describes the prognosis of Primary Progressive MS (PPMS) compared to Relapsing-Remitting MS (RRMS)?
A patient newly diagnosed with MS is being educated about their disease course. Which statement accurately describes the prognosis of Primary Progressive MS (PPMS) compared to Relapsing-Remitting MS (RRMS)?
A patient with MS experiences a series of acute exacerbations with partial recovery after each. However, from the onset, there has been a clear and steady worsening of their overall function independent of the relapses. Which type of MS does this MOST likely represent?
A patient with MS experiences a series of acute exacerbations with partial recovery after each. However, from the onset, there has been a clear and steady worsening of their overall function independent of the relapses. Which type of MS does this MOST likely represent?
Which of the MS subtypes benefit MOST from therapies designed to reduce the frequency and severity of acute relapses?
Which of the MS subtypes benefit MOST from therapies designed to reduce the frequency and severity of acute relapses?
A patient with MS is described as having "inflammatory" disease activity. Which MS subtypes are MOST likely to have this characteristic?
A patient with MS is described as having "inflammatory" disease activity. Which MS subtypes are MOST likely to have this characteristic?
A 55-year-old patient presents with steadily worsening leg weakness and gait unsteadiness over the past two years. They deny any distinct relapses or remissions. MRI shows spinal cord lesions but few brain lesions. What type of MS is most likely?
A 55-year-old patient presents with steadily worsening leg weakness and gait unsteadiness over the past two years. They deny any distinct relapses or remissions. MRI shows spinal cord lesions but few brain lesions. What type of MS is most likely?
An accurate prognosis for a patient with MS requires differentiating between disease courses. Progression independent of relapse is a defining feature of which MS subtypes?
An accurate prognosis for a patient with MS requires differentiating between disease courses. Progression independent of relapse is a defining feature of which MS subtypes?
Disease modifying therapies (DMTs) aim to change the natural course of MS by preventing or delaying the progression of the disease. A patient with which MS subtype would be LEAST likely to benefit from a DMT?
Disease modifying therapies (DMTs) aim to change the natural course of MS by preventing or delaying the progression of the disease. A patient with which MS subtype would be LEAST likely to benefit from a DMT?
A 40-year-old patient has been diagnosed with RRMS for the past 10 years. Over the last year, they've noticed a gradual increase in their baseline fatigue and leg weakness, even between relapses. What is the MOST likely explanation?
A 40-year-old patient has been diagnosed with RRMS for the past 10 years. Over the last year, they've noticed a gradual increase in their baseline fatigue and leg weakness, even between relapses. What is the MOST likely explanation?
After an MS relapse that affected the brainstem, a patient has residual diplopia and imbalance. Six months later, what indicates whether they are still in the relapsing-remitting phase or have transitioned to secondary progressive?
After an MS relapse that affected the brainstem, a patient has residual diplopia and imbalance. Six months later, what indicates whether they are still in the relapsing-remitting phase or have transitioned to secondary progressive?
Compared to RRMS, which statement is MOST accurate regarding diagnostic findings or clinical course of primary progressive.
Compared to RRMS, which statement is MOST accurate regarding diagnostic findings or clinical course of primary progressive.
A clinical trial is enrolling patients with progressive forms of MS to test a new neuroprotective agent. Which patient would MOST likely qualify for enrollment?
A clinical trial is enrolling patients with progressive forms of MS to test a new neuroprotective agent. Which patient would MOST likely qualify for enrollment?
After many years a majority of patients with RRMS eventually transition to which subtype of MS?
After many years a majority of patients with RRMS eventually transition to which subtype of MS?
Which disease course will most closely resemble primary progressive MS?
Which disease course will most closely resemble primary progressive MS?
Which disease course is marked by clear relapses with full recovery and little disease progression for more than 10 years?
Which disease course is marked by clear relapses with full recovery and little disease progression for more than 10 years?
Which characteristic dictates the change from RRMS to secondary progressive?
Which characteristic dictates the change from RRMS to secondary progressive?
Which disease progress has to have the presence of relapses?
Which disease progress has to have the presence of relapses?
A patient is diagnosed with Primary Progressive MS (PPMS). Which of the following signs or symptoms, if present, would be MOST unexpected and warrant reconsideration of the diagnosis?
A patient is diagnosed with Primary Progressive MS (PPMS). Which of the following signs or symptoms, if present, would be MOST unexpected and warrant reconsideration of the diagnosis?
Compared to patients with RRMS, individuals with PPMS are LESS likely to exhibit which of the following?
Compared to patients with RRMS, individuals with PPMS are LESS likely to exhibit which of the following?
After 15 years of well-controlled RRMS, a patient begins to experience steadily increasing fatigue, cognitive difficulties, and balance problems, without acute relapses. This clinical change suggests:
After 15 years of well-controlled RRMS, a patient begins to experience steadily increasing fatigue, cognitive difficulties, and balance problems, without acute relapses. This clinical change suggests:
Which of the following is the MOST important factor for prognostication when initially counseling a patient newly diagnosed with MS?
Which of the following is the MOST important factor for prognostication when initially counseling a patient newly diagnosed with MS?
While all MS subtypes involve demyelination and axonal damage, what is the PRIMARY distinguishing factor that separates Primary Progressive MS (PPMS) from the other forms of the disease?
While all MS subtypes involve demyelination and axonal damage, what is the PRIMARY distinguishing factor that separates Primary Progressive MS (PPMS) from the other forms of the disease?
A neurologist is evaluating a patient with suspected MS. Which historical factor would MOST strongly suggest the diagnosis of Relapsing-Remitting MS (RRMS)?
A neurologist is evaluating a patient with suspected MS. Which historical factor would MOST strongly suggest the diagnosis of Relapsing-Remitting MS (RRMS)?
Distinguishing between MS subtypes influences management decisions. For which subtype are medications targeting acute relapses considered LEAST effective?
Distinguishing between MS subtypes influences management decisions. For which subtype are medications targeting acute relapses considered LEAST effective?
In progressive relapsing MS which is most correct?
In progressive relapsing MS which is most correct?
Which criteria is not part of the diagnosis for MS?
Which criteria is not part of the diagnosis for MS?
Which disease course does not include any relapses?
Which disease course does not include any relapses?
A MRI is done on a patient, what part of the MRI will show an amount of lesions and show high signal intensity?
A MRI is done on a patient, what part of the MRI will show an amount of lesions and show high signal intensity?
Which sign or symptom is from plaques in sensory pathways?
Which sign or symptom is from plaques in sensory pathways?
What is the hallmark of Huntington's Disease?
What is the hallmark of Huntington's Disease?
What structure of the brain is affected in Huntington's disease?
What structure of the brain is affected in Huntington's disease?
What electrolyte should you check for in Restless Leg Syndrome?
What electrolyte should you check for in Restless Leg Syndrome?
In MS, which disease course involves initial relapses with recovery, but then transitions to a phase of steady neurological decline without distinct relapses?
In MS, which disease course involves initial relapses with recovery, but then transitions to a phase of steady neurological decline without distinct relapses?
A patient is diagnosed with MS and presents with a continuous decline in neurological function from the onset, with no distinct relapses. What is the most likely subtype?
A patient is diagnosed with MS and presents with a continuous decline in neurological function from the onset, with no distinct relapses. What is the most likely subtype?
Which MS subtype is characterized by a progressive course from onset, but also includes distinct relapses along the way?
Which MS subtype is characterized by a progressive course from onset, but also includes distinct relapses along the way?
A patient with MS experiences new neurological symptoms that resolve completely within a few weeks. However, they have occurred multiple times in the past year. What is the MOST likely form of the disease at this stage?
A patient with MS experiences new neurological symptoms that resolve completely within a few weeks. However, they have occurred multiple times in the past year. What is the MOST likely form of the disease at this stage?
Which of the following findings would be MOST suggestive of primary progressive MS (PPMS) rather than relapsing-remitting MS (RRMS)?
Which of the following findings would be MOST suggestive of primary progressive MS (PPMS) rather than relapsing-remitting MS (RRMS)?
Which of the following is the MOST critical factor in differentiating secondary progressive MS (SPMS) from relapsing-remitting MS (RRMS)?
Which of the following is the MOST critical factor in differentiating secondary progressive MS (SPMS) from relapsing-remitting MS (RRMS)?
An individual previously diagnosed with relapsing-remitting MS (RRMS) is now experiencing a consistent increase in disability, even between relapses. What is the MOST likely explanation?
An individual previously diagnosed with relapsing-remitting MS (RRMS) is now experiencing a consistent increase in disability, even between relapses. What is the MOST likely explanation?
In a patient with RRMS, what clinical sign or symptom most suggests the disease has transitioned to SPMS?
In a patient with RRMS, what clinical sign or symptom most suggests the disease has transitioned to SPMS?
A researcher is investigating new treatments for progressive MS. Which patient group would be MOST suitable for a clinical trial focusing on neuroprotective strategies?
A researcher is investigating new treatments for progressive MS. Which patient group would be MOST suitable for a clinical trial focusing on neuroprotective strategies?
A patient with MS has a disease course characterized by distinct relapses with partial recovery. Over the last few years their baseline function has worsen independent of relapse, what form of MS does this MOST likely represent?
A patient with MS has a disease course characterized by distinct relapses with partial recovery. Over the last few years their baseline function has worsen independent of relapse, what form of MS does this MOST likely represent?
Essential tremors are not present when?
Essential tremors are not present when?
What is a common medication treatment for Essential Tremor?
What is a common medication treatment for Essential Tremor?
Which of the following are key symptoms of Parkinson's disease? (Select all that apply)
Which of the following are key symptoms of Parkinson's disease? (Select all that apply)
Which of the following describes a key aspect of Parkinson's pathology?
Which of the following describes a key aspect of Parkinson's pathology?
What is the first-line treatment for Parkinson's disease?
What is the first-line treatment for Parkinson's disease?
What are COMT inhibitors?
What are COMT inhibitors?
What are the key hallmarks of Huntington's Disease? (Select all that apply)
What are the key hallmarks of Huntington's Disease? (Select all that apply)
Huntington's Disease is what type of dominant inheritance?
Huntington's Disease is what type of dominant inheritance?
What is the primary pathology associated with Huntington Disease?
What is the primary pathology associated with Huntington Disease?
Huntington's disease results in the expansion of which of the following?
Huntington's disease results in the expansion of which of the following?
Which of the following statements about the pathology of Huntington Disease is correct?
Which of the following statements about the pathology of Huntington Disease is correct?
Which of the following is a common treatment for Huntington Disease?
Which of the following is a common treatment for Huntington Disease?
What is a key difference between Huntington's disease and Parkinson's disease?
What is a key difference between Huntington's disease and Parkinson's disease?
What is the pathology of restless leg syndrome?
What is the pathology of restless leg syndrome?
Which of the following treatments are commonly used for Restless Leg Syndrome? (Select all that apply)
Which of the following treatments are commonly used for Restless Leg Syndrome? (Select all that apply)
What cells are destroyed in Multiple Sclerosis?
What cells are destroyed in Multiple Sclerosis?
A patient that presents with what optic issue should be considered for MS?
A patient that presents with what optic issue should be considered for MS?
What is Lhermitte's sign?
What is Lhermitte's sign?
What distinguishes relapsing-remitting multiple sclerosis from secondary progressive multiple sclerosis?
What distinguishes relapsing-remitting multiple sclerosis from secondary progressive multiple sclerosis?
Diagnosis of Multiple Sclerosis (MS) requires which of the following?
Diagnosis of Multiple Sclerosis (MS) requires which of the following?
What is the primary treatment for primary progressive multiple sclerosis (MS)?
What is the primary treatment for primary progressive multiple sclerosis (MS)?
Flashcards
Essential Tremor: Etiology
Essential Tremor: Etiology
Usually unexplained; tremor at any age, enhanced by stress, involves hands/head, bilateral.
Essential Tremor: Clinical Features
Essential Tremor: Clinical Features
Involuntary rhythmic movement; various amplitudes; not present at rest but exam otherwise normal.
Essential Tremor: Diagnosis
Essential Tremor: Diagnosis
Clinical exam; tremor present; ask patient: does caffeine make it worse/ alcohol better?
Essential Tremor: Treatment
Essential Tremor: Treatment
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Parkinson's Disease: Etiology
Parkinson's Disease: Etiology
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Parkinson's Disease: Clinical Features
Parkinson's Disease: Clinical Features
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Parkinson's Disease: Diagnosis
Parkinson's Disease: Diagnosis
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Parkinson's Disease: Treatment
Parkinson's Disease: Treatment
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Huntington's Disease: Etiology
Huntington's Disease: Etiology
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Huntington's Disease: Clinical Features
Huntington's Disease: Clinical Features
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Huntington's Disease: Diagnosis
Huntington's Disease: Diagnosis
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Huntington's Disease: Treatment
Huntington's Disease: Treatment
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Restless Leg Syndrome: Etiology
Restless Leg Syndrome: Etiology
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Restless Leg Syndrome: Clinical Features
Restless Leg Syndrome: Clinical Features
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Restless Leg Syndrome: Diagnosis
Restless Leg Syndrome: Diagnosis
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Restless Leg Syndrome: Treatment
Restless Leg Syndrome: Treatment
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Study Notes
Essential Tremor
- Involves involuntary rhythmic movement of a body part with varying amplitudes.
- Etiology is mostly unexplained.
- Can occur at any age.
- Stress enhances tremors.
- Typically affects hands, head, or both.
- It is usually bilateral.
- Tremors are not present at rest.
- The exam is otherwise normal.
- Over time it will become more obvious.
- Impacts manual skills.
- Small amounts of alcohol can alleviate it.
- Treatment depends on severity.
- Propranolol 60-240 mg daily.
- Primidone should be titrated.
- Alprazolam, topiramate, gabapentin, and botulinum toxin A are options for treatment.
- Deep Brain Stimulation is an interventional therapy.
- It might be a risk factor for Parkinson disease.
Parkinson Disease
- A neurodegenerative disorder characterized by generalized bradykinesia, resting tremor, or rigidity.
- Movement disorder.
- Most commonly idiopathic.
- Parkinson disease onset is usually between ages 45-65.
- Dopamine depletion is the key pathology.
- An imbalance of dopamine and acetylcholine.
- Involves death of dopaminergic neurons.
- The Substantia Nigra gradually disappears which controls movement and connects to the motor cortex.
- Lewy bodies are eosinophilic with alpha-synuclein found in other diseases, significance unknown.
- Cardinal features include tremor, rigidity, bradykinesia, and postural instability.
- Non-motor symptoms include affective disorders like depression, anxiety, and apathy, psychosis, cognitive changes, fatigue, sleep disorders, anosmia, autonomic disturbances, sensory complaints, pain, and seborrheic dermatitis.
- Dementia or mild cognitive impairment may develop.
- Resting tremor, present at rest, diminishes with movement
- Cogwheel rigidity is a symptom.
- Stooped posture and almost expressionless faces are symptoms.
- Bradykinesia presents as slow or lessened movement or an absence of movement.
- Difficulty initiating movement, legs freeze up and presents as a shuffling gait.
- Postural instability causes problems with balance causing falls.
- Resting tremor occurs at 4-6 cycles per second.
- Tremor enhances with stress and less severe during activity.
- One limb is usually affected and then progresses into generalizing more limbs.
- Rigidity is a resistance to passive movement.
- Flexed posture.
- Bradykinesia is a slowness of voluntary movements.
- Decreased automatic movements.
- Immobility of the face, widened palpebral fissures, decreased blinking, and decreased facial expression can occur.
- Seborrhea of the scalp and face, Myerson sign, drooling, and soft voice can be seen.
- ROM impaired and no muscle weakness.
- Difficult rising from a chair or shuffling steps, unsteady, and has difficulty turning.
- Diagnosis involves a good physical exam and history to exclude medication side effects and other neurodegenerative diseases.
- Absence of specific labs or imaging.
- Treatment involves blocking acetylcholine.
- Levodopa is a precursor to dopamine.
- Carbidopa inhibits the enzyme that breaks down levodopa to dopamine
- Sinemet – combo drug is also a treatment.
- Amantadine is used for dyskinesias or mild motor symptoms with an unclear mode of action.
- Pramipexole, and ropinirole act on the dopamine receptors and is used for early and late stages
- MOA inhibitors have an unclear MOI.
- Rasagiline and selegiline can inhibit the breakdown of dopamine and slow progression.
- COMT inhibitors = catecholamine-O-methyltransferase inhibitors that reduce metabolism of levodopa, create a more sustained plasma levels and decrease the breakdown of dopamine.
- Istradefylline in a treatment
- Anticholinergics alleviate tremors and rigidity more, that cause side effects.
- Antipsychotics can cause confusion and psychotic symptoms and occurs as a medication side.effect or illness
- Tai chi, antidepressants, anti-anxiety medications, and assist devices for ADL's.
- Brain stimulation and brain stimulation – implantable device to BG
- All patients should be referred.
Huntington Disease
- Hallmarks include chorea and dementia.
- Clinical onset of 30-50 y.o causing fatal outcomes within 15-20 years.
- Rare cases require genetic testing.
- A neurodegenerative disease with autosomal dominant genetics.
- Due to repeats on the CAG expression on the HTT protein.
- Degeneration of neurons in the putamen, caudate, and cerebra cortex.
- Caudate Putamen is apart of the Dorsal Striatum.
- Causes movement(inhibition) problems.
- Part of Basal Ganglia that CONTROLS MOVEMENT, and CONNECTS TO MOTOR CORTEX.
- Atrophy, especially in the caudate and putamen.
- Tissue loss and expansion of lateral ventricles.
- Chorea~ purposeless, dance-like.
- Athetosis~slow "snake-like".
- Can't surpress because sleep.
- Abnormal eye movements and poor coordination.
- Signs and symptoms include motor, and cognitive and psychiatric disturbances.
- Initially abnormal movements or intellectual changes occur.
- Mental changes can cause behavioral changes, irritability, moodiness, antisocial behavior, or psychiatric changes.
- Dyskinesia may be minor and cause restlessness and fidgeting which turns into chorea and dystonia.
- Diagnosis requires genetic testing which is the gold standard.
- Use MRI/CT to diagnose cerebral atrophy.
- No cure or way to stop progression and treatments are for symptoms.
- Lots of additional support – language, and speech therapy.
- Selectively depletes dopamine.
- Deutetrabenazine depletes monoamines.
- Deep brain stimulation is a treatment option.
Restless Leg Syndrome
- Relatively common
- Can be primary or idiopathic or secondary
- May have a genetic component.
- Involves restlessness and sensory disturbances, which leads to urge to move legs, especially occurring during evening and at night.
- Associated with periodic limb movements during sleep or relaxed wakefulness.
- Important to check ferritin levels.
- Treat with iron if needed.
- Can use Gabapentin, Pregabalin, Pramipexole, and Levodopa.
Multiple Sclerosis (MS)
- The nervous system is attacked and destroys the myelin.
- Is a demyelination.
- BBB increases.
- T cells enter and activated by myelin
- The cells will the express more cells to get in.
- Myelin specific T cells-Dilates cells, make ABYs to proteins, and myelin Oligodendrocytes destroyed and lose myelin protection, PLAQUES.
- Remyelination will eventually stop.
- Has genetic and environmental factors, such as female sex, certain genes, environmental deficiencies such as vitamin d levels, and infections.
- The immune system attacks the white matter of the brain and or spinal cord.
- It is chronic and proggressive, which degrades myelin sheath from lymphocytes and macrophages.
- Plaques are a variety of symptoms.
- Initial complaints are from the age 20-50.
- Common symptoms are weakness, numbness/tingling in sensory, plaques for some areas.
- Unsteadiness of a limb, spastic paraparesis(PLAQUES in MOTOR).
- Optic neuritis, diplopia, disequilibrium.
- Sphincter Disturbance, ANS.
- types are irreversibly damaged.
- Relapsing Remitting which is most common. Initial presentation occurs for months, then progress to years
- new or original symptoms may occur.
- The relapse is incomplete with remissions which cause disability.
- Secondary Progressive, eventual immune attack is constant.
- Primary Progressive, constant attacks from onset. Steadily progressing from being able and disability has developed early.
- Progressive elapsing.
- There is no explanation was causes the exacerbations.
- The three categories of symptoms include dysarthria, optic nerve neuritis and nystamus. -Diagnostic is an MRI of the brain and cervical cord which looks like a white matter plaque. You will NEED imaging for areas. -T1 Weight with hypointense areas shows areas damage to the axons. -Gadolinium will highlight areas of inflammation. -T2 shows shows high signal intensity of lesions -CAN"T diagnose w/o 2 regains affected at different times.
- Must abide by McDonalds criteria.
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