MS, neuromuscular junction

Question 1

is RRMS or PPMS more common in women than men

Answer 1

RRMS is- the progressive primary is about the same

Question 2

what does the absolute diagnostic criteria for demonstration of dissemination of disease related events requires?

Answer 2

seperation of both time and place

Question 3

clinically isolated demyelinating syndromes —what predicts whether it will become MS?

Answer 3

MRI- less risk if MRI is normal at start of CIDS

Question 4

T-F–MS is almost always a LMN syndrome?

Answer 4

false, UMN

Question 5

what has greater than sensitivity than MRI for MS?

Answer 5

CSF analysis

Question 6

what is the CSF findings in MS?

Answer 6

elevated IgG index, IgG synthetic rate, oligoclonal bands (not found in blood)

Question 7

what tech can help show lesiion demonstrated in space?

Answer 7

unilateral conduction delay of the P100 evoked potential

Question 8

what standard screening tests should be conducted in someone with MS?

Answer 8

CBC, CMP, ESR, ANA, B12, infection or hypercoaguability measures
(ALL SHOULD BE NEGATIE IN STRAIGHT FORWARD MS)

Question 9

separate MS attacks must have how long between them?

Answer 9

30day

Question 10

What might cause a pseudo MS attack or worsening of symptoms?

Answer 10

high fever, vigorous exercise, hot water baths or showers or environmental heat

Question 11

what major categories of illness make up the core differntials for MS?

Answer 11

Inflammatory disease
infectious diseas
disease of myelin
vascular disease
misc.(spinocerebellar, arnold-chiari, B12, hashimoto, Leber's-mitoch)

Question 12

T-F all MS gets worse over time?

Answer 12

trure

Question 13

T-F men with later onset usually do worse than women in MS ?

Answer 13

true with more evident myelopathic symtpoms

Question 14

T-F- MS cerebellar symptoms tend to do worse over time?

Answer 14

true

Question 15

T-F—younger onset means worse long term with MS?

Answer 15

false

Question 16

T-F- optic neuritis at onset for MS tends to have worse long term?

Answer 16

False

Question 17

t-F intervals of MS attacks >1year fear well

Answer 17

True

Question 18

do persistent ambulation difficulties arise sooner in progressive-from-onset or relapsing from onset?

Answer 18

progressive from onset

Question 19

preg and breastfeeding makes MS worse?

Answer 19

false- usually a relative protected time. without breast feeding might be worse

Question 20

how many patients with RRMS become SPMS after 10 years?

Answer 20

50%

Question 21

How many RRMS patients wil need a walking aide 15-23 years later?

Answer 21

50%

Question 22

review the list of disease modifying MS drugs

Answer 22

interferon beta, glatiramer acetate, mitoxantrone, natalizumab, fingolimod, teriflunomide, dimethyl fumarate

Question 23

T-F–MS has a correlation between EBNA-1 IgG and gadolinium enhancing MRI lesions?

Answer 23

true

Question 24

MS patients need what tests for diagnosis?

Answer 24

MRI and CSF for oligoclonal bands and IgG inde

Question 25

review a list of common MS symptoms

Answer 25

neurogenic bladder, fatigue, spasticity, sexual dysfunction, depression

Question 26

does ALS start limb or face symptoms first?

Answer 26

limb onset usually

Question 27

what are common ALS management things?

Answer 27

biPAP, feeding tube, communication devices, medications for drooling, ALS clinic rehab, clinical trials

Question 28

what is the mean time of after diabetes onset to development of neuropathy?

Answer 28

8 years Type 1 longer Type 2 may actually be before

Question 29

is burning pain or proprioception large myelinated fibers?

Answer 29

proprioception- pain is small unmyelinated

Question 30

does regulated blood glucose reverse polyneuropathy?

Answer 30

no but may lessen pain

Question 31

what medications do we use to treat neuropathy pain?

Answer 31

gabapentin, amitriptyline and pregabalin

Question 32

what are the common infections of guillane barre?

Answer 32

CMV, EBV, campy, Hib

Question 33

what are the antibodies against?

Answer 33

schwan cells/myelin | on peripheral nerves

Question 34

does guillane barre effect sensation or motor?

Answer 34

both and then may effect breathing, heart rate, urinary retention, ileus

Question 35

what does nerve conduction studies show for guillane barre?

Answer 35

demyelinating pattern

Question 36

what does CSF show in guillane barre?

Answer 36

elevated total protein and no or few WBCs

Question 37

what is Rx for guillane barre?

Answer 37

admit- monitor respiratory- may need IVIG or plasmapheresis

Question 38

although many improve with guillane barre with time, what may persist in 50%-

Answer 38

neuropathic signs- paresthesias and numbness

Question 39

what are antibodies of mysenthia gravis agains?

Answer 39

AcH receptors > reduction in action potentials of the muscle membrane and therefore muscle weakness

Question 40

what is the most common presenting symptom of myesthenia gravis?

Answer 40

ptosis and diplopia

Question 41

how do we diagnose myasthenia gravis?

Answer 41

blood test acetylcholine receptor antibodies cholinesterase inhibitors like tensilon or prostigmine and weakness should improve slow rate repetitive nerve stimulation > decrement

Question 42

what is symptomatic medication for myasthenia gravis?

Answer 42

pyridostigmine

Question 43

what is immunosuppresive treatment for myasthenia gravis?

Answer 43

prednisone, azathiprine, mycophenolate mofetil

Question 44

what is used to treat myasthenia in acute severe crisis?

Answer 44

IVIG plasmapharesis

Question 45

what organ may be removed to treat myasthenia gravis?

Answer 45

thmymectomy

Question 46

is lambert eaton presynaptic or post synaptic?

Answer 46

pre

Question 47

what are clinical features of lambert eaton?

Answer 47

fatiguing weakness, dry mouth, ED

Question 48

what is lambert eaton often associated with?

Answer 48

malignancy- small cell lung carcinoma

Question 49

how do we diagnose lambert eaton?

Answer 49

blood test P/Q ca channel antibodies, high rate repetitive nerve stimulation increment

Question 50

is botulism a pre or post synaptic disorder?

Answer 50

pre synaptic

Question 51

where do botulism symptoms start and then where do they fo?

Answer 51

bulbar then limbs and then respiratory

Question 52

what is management of botulism?

Answer 52

supportive- ICU vent feeding. antitoxin

Question 53

how do we diagnose polymyositis?

Answer 53

Creatine Kinase level elevation 50x | -muscle biopsy invasion of fibers by Tcells

Question 54

How do we treat molymyositis?

Answer 54

prednisone

Question 55

what is unique about dermatomyositis?

Answer 55

rashes- heliotrope rash-

Question 56

what is the difference in diagnostic biposy of dermatomyositis?

Answer 56

perivascular inflammatory infiltrates, perifascicular atrophy is pathognomonic

Question 57

what is the clinical hallmark of inclusion body myositis?

Answer 57

early weakness and atrophy of quads and forearms (wrist and finger flexors) and foot drop

Question 58

what does muscle biopsy of inclusion body myositis show?

Answer 58

endomysial inflammation with basophilic granular indclusions around the edges of vacuoles

Question 59

does inclusion body myositis respond to immunosupressors?

Answer 59

no

Question 60

what is the most common inherited neuromuscular disease in adults?

Answer 60

myotonic dystrophty- CTG

Question 61

why does myotonia occur in MD?

Answer 61

chloride channel abnormality

Question 62

where do we see muscle wasting in myotonic dystrophy?

Answer 62

temporal wasting

Question 63

what clinical features not related to muscle are often found with myotonic dystrphy,

Answer 63

cataracts, arrhythmias, hypogonadism, hypersomnia, frontal balding, retardation apathy