Clin Med Unit 3 Essential Tremor and Parkinson Disease

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Questions and Answers

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?

  • 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?

  • 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)?

<p>Progressive worsening of the disease from the start, with clear acute relapses, with or without full recovery (C)</p> Signup and view all the answers

How does age of onset typically differ between relapsing-remitting MS (RRMS) and primary-progressive MS (PPMS)?

<p>RRMS generally starts in the 20s and 30s, whereas PPMS tends to begin later, around the 40s and 50s. (D)</p> Signup and view all the answers

Which of the following statements accurately compares the relapse patterns in relapsing-remitting MS (RRMS) and secondary-progressive MS (SPMS)?

<p>RRMS has distinct relapses with full recovery, whereas SPMS has fewer relapses with incomplete recovery. (C)</p> Signup and view all the answers

To differentiate between RRMS and PPMS, what key aspect of the patient's initial disease presentation would be most informative?

<p>The presence of distinct clinical relapses followed by remissions. (A)</p> Signup and view all the answers

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?

<p>Relapsing Remitting (A)</p> Signup and view all the answers

In a patient with a long-standing diagnosis of RRMS, what clinical finding would MOST strongly suggest a transition to secondary progressive MS?

<p>Gradual worsening of neurological function independent of acute relapses. (D)</p> Signup and view all the answers

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?

<p>Primary Progressive MS (PPMS) (A)</p> Signup and view all the answers

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)?

<p>PPMS generally has a worse prognosis than RRMS, often leading to earlier disability accumulation. (C)</p> Signup and view all the answers

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?

<p>Progressive-Relapsing (PRMS). (C)</p> Signup and view all the answers

Which of the MS subtypes benefit MOST from therapies designed to reduce the frequency and severity of acute relapses?

<p>Relapsing-Remitting (RRMS). (C)</p> Signup and view all the answers

A patient with MS is described as having "inflammatory" disease activity. Which MS subtypes are MOST likely to have this characteristic?

<p>Relapsing Remitting (RRMS) and Progressive Relapsing (PRMS). (A)</p> Signup and view all the answers

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?

<p>Primary-Progressive MS. (B)</p> Signup and view all the answers

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?

<p>Primary Progressive and Secondary Progressive MS. (B)</p> Signup and view all the answers

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?

<p>Primary Progressive MS. (D)</p> Signup and view all the answers

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?

<p>Their RRMS is converting to Secondary-Progressive MS. (B)</p> Signup and view all the answers

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?

<p>Continued worsening of the imbalance that affects ambulation. (A)</p> Signup and view all the answers

Compared to RRMS, which statement is MOST accurate regarding diagnostic findings or clinical course of primary progressive.

<p>PPMS is more commonly associated with spinal cord involvement than brain lesions. (C)</p> Signup and view all the answers

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?

<p>Both B and C (A)</p> Signup and view all the answers

After many years a majority of patients with RRMS eventually transition to which subtype of MS?

<p>Secondary progressive (A)</p> Signup and view all the answers

Which disease course will most closely resemble primary progressive MS?

<p>Progressive relapsing (A)</p> Signup and view all the answers

Which disease course is marked by clear relapses with full recovery and little disease progression for more than 10 years?

<p>Benign (C)</p> Signup and view all the answers

Which characteristic dictates the change from RRMS to secondary progressive?

<p>Gradual worsening of symptoms independent of relapses (A)</p> Signup and view all the answers

Which disease progress has to have the presence of relapses?

<p>Both progressive relapsing and relapsing remitting (D)</p> Signup and view all the answers

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?

<p>An acute episode of optic neuritis with near-complete recovery. (A)</p> Signup and view all the answers

Compared to patients with RRMS, individuals with PPMS are LESS likely to exhibit which of the following?

<p>Antibody-Mediated Inflammation in the Central Nervous System (C)</p> Signup and view all the answers

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:

<p>Transition to secondary progressive MS (B)</p> Signup and view all the answers

Which of the following is the MOST important factor for prognostication when initially counseling a patient newly diagnosed with MS?

<p>Identifying the subtype of MS (RRMS, SPMS, PPMS, PRMS). (D)</p> Signup and view all the answers

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?

<p>Absence of distinct relapses and remissions. (B)</p> Signup and view all the answers

A neurologist is evaluating a patient with suspected MS. Which historical factor would MOST strongly suggest the diagnosis of Relapsing-Remitting MS (RRMS)?

<p>Previous episodes of sensory disturbance and vision loss that resolved spontaneously. (A)</p> Signup and view all the answers

Distinguishing between MS subtypes influences management decisions. For which subtype are medications targeting acute relapses considered LEAST effective?

<p>Primary Progressive MS (PPMS). (D)</p> Signup and view all the answers

In progressive relapsing MS which is most correct?

<p>There is a slow disease progression with relapses (D)</p> Signup and view all the answers

Which criteria is not part of the diagnosis for MS?

<p>Slow disease progression over time (D)</p> Signup and view all the answers

Which disease course does not include any relapses?

<p>Primary Progressive (A)</p> Signup and view all the answers

A MRI is done on a patient, what part of the MRI will show an amount of lesions and show high signal intensity?

<p>T2 weighted (C)</p> Signup and view all the answers

Which sign or symptom is from plaques in sensory pathways?

<p>Numbness/tingling (C)</p> Signup and view all the answers

What is the hallmark of Huntington's Disease?

<p>Chorea (A)</p> Signup and view all the answers

What structure of the brain is affected in Huntington's disease?

<p>Putamen (D)</p> Signup and view all the answers

What electrolyte should you check for in Restless Leg Syndrome?

<p>Ferritin (D)</p> Signup and view all the answers

In MS, which disease course involves initial relapses with recovery, but then transitions to a phase of steady neurological decline without distinct relapses?

<p>Secondary-Progressive MS (D)</p> Signup and view all the answers

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?

<p>Primary-Progressive MS (D)</p> Signup and view all the answers

Which MS subtype is characterized by a progressive course from onset, but also includes distinct relapses along the way?

<p>Progressive-Relapsing MS (A)</p> Signup and view all the answers

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?

<p>Relapsing-Remitting MS (B)</p> Signup and view all the answers

Which of the following findings would be MOST suggestive of primary progressive MS (PPMS) rather than relapsing-remitting MS (RRMS)?

<p>Gradual, steady worsening of symptoms from disease onset (A)</p> Signup and view all the answers

Which of the following is the MOST critical factor in differentiating secondary progressive MS (SPMS) from relapsing-remitting MS (RRMS)?

<p>Progression of disability independent of acute relapses. (C)</p> Signup and view all the answers

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?

<p>The patient has transitioned to secondary progressive MS. (C)</p> Signup and view all the answers

In a patient with RRMS, what clinical sign or symptom most suggests the disease has transitioned to SPMS?

<p>Gradual worsening of motor function between relapses. (C)</p> Signup and view all the answers

A researcher is investigating new treatments for progressive MS. Which patient group would be MOST suitable for a clinical trial focusing on neuroprotective strategies?

<p>Patients with PPMS experiencing steady neurological decline. (D)</p> Signup and view all the answers

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?

<p>Secondary-Progressive MS (SPMS) (A)</p> Signup and view all the answers

Essential tremors are not present when?

<p>At rest (A)</p> Signup and view all the answers

What is a common medication treatment for Essential Tremor?

<p>Propranolol (A), Primidone (@)</p> Signup and view all the answers

Which of the following are key symptoms of Parkinson's disease? (Select all that apply)

<p>Bradykinesia, Resting Tremor, Rigidity (A)</p> Signup and view all the answers

Which of the following describes a key aspect of Parkinson's pathology?

<p>Dopamine Depletion (A), Accumulation of Alpha-Synuclein (C)</p> Signup and view all the answers

What is the first-line treatment for Parkinson's disease?

<p>Levodopa (A)</p> Signup and view all the answers

What are COMT inhibitors?

<p>Catecholamine O-Methyltransferase inhibitors (A)</p> Signup and view all the answers

What are the key hallmarks of Huntington's Disease? (Select all that apply)

<p>Chorea (A), Cognitive decline (C)</p> Signup and view all the answers

Huntington's Disease is what type of dominant inheritance?

<p>Autosomal dominant (A)</p> Signup and view all the answers

What is the primary pathology associated with Huntington Disease?

<p>Repeats of CAG expression of HIT (B)</p> Signup and view all the answers

Huntington's disease results in the expansion of which of the following?

<p>Lateral Ventricles (A)</p> Signup and view all the answers

Which of the following statements about the pathology of Huntington Disease is correct?

<p>Increased Dopamine (A)</p> Signup and view all the answers

Which of the following is a common treatment for Huntington Disease?

<p>Tetrabenazine (A)</p> Signup and view all the answers

What is a key difference between Huntington's disease and Parkinson's disease?

<p>Huntington's is characterized by chorea, while Parkinson's is characterized by bradykinesia. (A)</p> Signup and view all the answers

What is the pathology of restless leg syndrome?

<p>Dopamine deficiency in the brain (A)</p> Signup and view all the answers

Which of the following treatments are commonly used for Restless Leg Syndrome? (Select all that apply)

<p>Gabapentin (B), Iron supplements (A), Dopamine agonists (C)</p> Signup and view all the answers

What cells are destroyed in Multiple Sclerosis?

<p>Oligodendrocytes (A)</p> Signup and view all the answers

A patient that presents with what optic issue should be considered for MS?

<p>Optic Neuritis (A)</p> Signup and view all the answers

What is Lhermitte's sign?

<p>Electric shock in the neck when flexing the head forward (A)</p> Signup and view all the answers

What distinguishes relapsing-remitting multiple sclerosis from secondary progressive multiple sclerosis?

<p>Relapsing-remitting involves periods of relapse and remission, while secondary progressive gradually worsens over time. (A)</p> Signup and view all the answers

Diagnosis of Multiple Sclerosis (MS) requires which of the following?

<p>Symptoms occurring over time and space (A)</p> Signup and view all the answers

Signup and view all the answers

What is the primary treatment for primary progressive multiple sclerosis (MS)?

<p>Ocrelizumab (A)</p> Signup and view all the answers

Flashcards

Essential Tremor: Etiology

Usually unexplained; tremor at any age, enhanced by stress, involves hands/head, bilateral.

Essential Tremor: Clinical Features

Involuntary rhythmic movement; various amplitudes; not present at rest but exam otherwise normal.

Essential Tremor: Diagnosis

Clinical exam; tremor present; ask patient: does caffeine make it worse/ alcohol better?

Essential Tremor: Treatment

Depends on severity; Propranolol, Primidone, Alprazolam, Topiramate, Gabapentin, Botulinum toxic A, DBS.

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Parkinson's Disease: Etiology

Idiopathic dopamine depletion; onset 45-65 y.o.; rarely familial. Imbalance of dopamine and acetylcholine.

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Parkinson's Disease: Clinical Features

Generalized bradykinesia, resting tremor, rigidity. Non-motor: depression, anxiety, psychosis, fatigue, anosmia.

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Parkinson's Disease: Diagnosis

Good PE and history; exclude medication side effects; exclude other neurodegenerative diseases or, responds to treatment.

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Parkinson's Disease: Treatment

Levodopa, Carbidopa, Amantadine, Dopamine agonists, MAO inhibitors, COMT inhibitors, Anticholinergics, Antipsychotics, PT/OT/Speech therapy, Tai chi.

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Huntington's Disease: Etiology

Neurodegenerative; autosomal dominant; repeats on the CAG expression on HTT protein; degeneration of neurons in putamen, caudate.

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Huntington's Disease: Clinical Features

Motor, cognitive, psychiatric disturbances; abnormal movements or intellectual changes; dyskinesia --> chorea, cerebral atrophy

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Huntington's Disease: Diagnosis

Genetic testing (gold standard); MRI/CT shows cerebral atrophy. Family history is indicative.

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Huntington's Disease: Treatment

No cure; treat symptoms; Tetrabenazine/Deutetrabenazine; Deep brain stimulation; gene therapy.

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Restless Leg Syndrome: Etiology

Primary/idiopathic or secondary; genetic component; sensory disturbances; urge to move legs, periodic limb movements during sleep.

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Restless Leg Syndrome: Clinical Features

Restlessness and sensory disturbances urge to move legs; evening and at night; periodic limb movements during sleep or relaxed wakefulness.

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Restless Leg Syndrome: Diagnosis

Check ferritin levels; RLS is primarily a clinical diagnosis. Rule out secondary causes.

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Restless Leg Syndrome: Treatment

Iron if needed; Gabapentin, Pregabalin, Pramipexole, Levodopa. Manage secondary causes.

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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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