MG/MS (Exam #3) Flashcards
What is the most common disorder of NM transmission?
Myasthenia Gravis (MG)
What is the bimodal distribution of Myasthenia Gravis (MG) (gender and age)?
- Females 20-30 years
- Males 60-80 years
What condition involves autoimmune attack on ACh receptors at NM junction → decreased ACh transmission?
Myasthenia Gravis (MG)
What is Myasthenia Gravis (MG) often triggered by?
STRESS (illness, surgery, emotional stress, pregnancy/childbirth, meds)
What three meds/classes of meds should be avoided with Myasthenia Gravis (MG)?
- Fluoroquinolones
- BBs
- Hydroxychloroquine
What two sxs are seen with Myasthenia Gravis (MG)?
- Muscle fatigability
- Fluctuating weakness
Both worse at end of day/after exercise
What condition involves muscle fatigability and fluctuating weakness worse at end of day/after exercise?
Myasthenia Gravis (MG)
What ocular finding is often seen with Myasthenia Gravis (MG)? What other sxs may be seen?
Ptosis
- Also, diplopia
What sxs of Myasthenia Gravis (MG) may be seen involving the face?
“Myasthenic sneer”
What sxs of Myasthenia Gravis (MG) may be seen involving the neck? Limbs?
Weakness in neck = “dropped head syndrome”
Weakness in limbs (arms > legs)
What condition involves “dropped head syndrome”?
Myasthenia Gravis (MG)
What sxs of Myasthenia Gravis (MG) may be seen involving respiratory?
“Myasthenic crisis”
What two tests will be + with Myasthenia Gravis (MG)?
- +AChR Ab
- +MuSK Ab
What diagnostic test can be used to evaluate for Myasthenia Gravis (MG), and what will be seen?
EMG/NCS shows decreased response/fatigability
What condition involves the Ice Pack Test, and what does a positive test look like?
Myasthenia Gravis (MG) - Difficulty maintaining upward gaze with ptosis
What condition involves the “Tensilon” Test, and what does a positive test look like?
Myasthenia Gravis (MG) - Administer AChE-I with immediate sxs improvement
What is the first line tx for Myasthenia Gravis (MG)?
Pyridostigmine (Mestinon)
- AChE-Is
What organ may be affected with Myasthenia Gravis (MG), and what is seen on CT/MRI?
Thymus
- Thymic hyperplasia or thymoma
What are the two recommended rapid/short-acting immunotherapy txs for Myasthenia Gravis (MG)? What are the two chronic immunotherapy tx?
Acute/Short-Acting:
- Plasma exchange/plasmapheresis
- IVIG
Chronic:
- Steroids
- Azathioprine
What is the leading cause of permanent disability in young adults?
Multiple Sclerosis (MS)
What age group is Multiple Sclerosis (MS) often seen?
15-45 years
- F > M
What condition involves immune-mediated attack on axons/myelin sheaths → demyelination?
Multiple Sclerosis (MS)
What condition involves plaques seen on imaging?
Multiple Sclerosis (MS)
What are the four types of Multiple Sclerosis (MS)?
Which is most common? Which is most aggressive?
- Relapsing-Remitting Disease (RRMS) = MOST common
- Secondary Progressive (SPMS)
- Primary Progressive (PPMS) = MOST aggressive
- Clinically Isolated Syndrome (CIS)
What type of Multiple Sclerosis (MS) involves clearly defined relapses with full recovery or only mild defect?
Relapsing-Remitting Disease (RRMS)
What type of Multiple Sclerosis (MS) involves initially RRMS with gradual worsening?
Secondary Progressive (SPMS)
What type of Multiple Sclerosis (MS) involves progressive sxs increase from disease onset with NO remission?
Primary Progressive (PPMS)
What condition involves separation of TIME and SPACE, and what does this mean?
Multiple Sclerosis (MS) - Episodes occur months/years apart + affect different anatomic locations
What is the characteristic timeline seen with Multiple Sclerosis (MS)?
Separation of TIME and SPACE
- Episodes occur months/years apart + affect different anatomic locations
What is often the first sxs seen with Relapsing-Remitting Disease (RRMS)?
Paresthesias
What three sxs are often see with Relapsing-Remitting Disease (RRMS)?
- Paresthesias
- Optic neuritis (eye pain leading to vision loss)
- Weakness
What are two pathognomonic signs of Multiple Sclerosis (MS)? Describe each.
- Lhermitte sign: neck flexion causes electrical shock down back/limbs
- Marcus Gunn Pupil = BOTH pupils dilate with bright light on affected pupil
What condition involves Marcus Gunn Pupil, and what is this?
Multiple Sclerosis (MS) - BOTH pupils dilate with bright light on affected pupil
What condition involves Lhermitte sign, and what is this?
Multiple Sclerosis (MS) - Neck flexion causes electrical shock down back/limbs
What are the two ways by which Multiple Sclerosis (MS) can be diagnosed?
- Clinical alone
- Clinical + MRI
What is the recommended dx test for Multiple Sclerosis (MS), and what is shown?
MRI with AND without contrast
- Shows plaques disseminated in TIME and SPACE
What is McDonald Criteria (2), and what condition is it associated with?
Multiple Sclerosis (MS)
- Damage in more than one place in CNS
AND
- Damage occurred more than once
What is seen with Multiple Sclerosis (MS) on CSF analysis?
Oligoclonal bands
What condition involves oligoclonal bands with a normal total WBC count?
Multiple Sclerosis (MS)
What is the recommended tx for ALL Multiple Sclerosis (MS) patients?
Followed by Neurology
What is the general first line tx for Relapsing-Remitting Disease (RRMS), and what are the two main goals of this therapy?
Disease-modifying therapy
- Decrease relapse rate
- Lower long-term risk of disease progression
What is the first line tx for ACUTE Relapsing-Remitting Disease (RRMS)?
STEROIDS
Besides steroids for tx of ACUTE Relapsing-Remitting Disease (RRMS), what two other tx options can be considered?
- ACTH
- Plasma exchange/plasmapheresis
What three drugs/classes of drugs are used to manage other sxs associated with Multiple Sclerosis (MS)?
- SSRIs for depression
- Muscle relaxants for muscle spasticity
- Oxybutynin for bladder dysfunction/neurogenic bladder