MS, neuromuscular junction Flashcards
is RRMS or PPMS more common in women than men
RRMS is- the progressive primary is about the same
what does the absolute diagnostic criteria for demonstration of dissemination of disease related events requires?
seperation of both time and place
clinically isolated demyelinating syndromes —what predicts whether it will become MS?
MRI- less risk if MRI is normal at start of CIDS
T-F–MS is almost always a LMN syndrome?
false, UMN
what has greater than sensitivity than MRI for MS?
CSF analysis
what is the CSF findings in MS?
elevated IgG index, IgG synthetic rate, oligoclonal bands (not found in blood)
what tech can help show lesiion demonstrated in space?
unilateral conduction delay of the P100 evoked potential
what standard screening tests should be conducted in someone with MS?
CBC, CMP, ESR, ANA, B12, infection or hypercoaguability measures
(ALL SHOULD BE NEGATIE IN STRAIGHT FORWARD MS)
separate MS attacks must have how long between them?
30day
What might cause a pseudo MS attack or worsening of symptoms?
high fever, vigorous exercise, hot water baths or showers or environmental heat
what major categories of illness make up the core differntials for MS?
Inflammatory disease infectious diseas disease of myelin vascular disease misc.(spinocerebellar, arnold-chiari, B12, hashimoto, Leber's-mitoch)
T-F all MS gets worse over time?
trure
T-F men with later onset usually do worse than women in MS ?
true with more evident myelopathic symtpoms
T-F- MS cerebellar symptoms tend to do worse over time?
true
T-F—younger onset means worse long term with MS?
false
T-F- optic neuritis at onset for MS tends to have worse long term?
False
t-F intervals of MS attacks >1year fear well
True
do persistent ambulation difficulties arise sooner in progressive-from-onset or relapsing from onset?
progressive from onset
preg and breastfeeding makes MS worse?
false- usually a relative protected time. without breast feeding might be worse
how many patients with RRMS become SPMS after 10 years?
50%
How many RRMS patients wil need a walking aide 15-23 years later?
50%
review the list of disease modifying MS drugs
interferon beta, glatiramer acetate, mitoxantrone, natalizumab, fingolimod, teriflunomide, dimethyl fumarate
T-F–MS has a correlation between EBNA-1 IgG and gadolinium enhancing MRI lesions?
true
MS patients need what tests for diagnosis?
MRI and CSF for oligoclonal bands and IgG inde
review a list of common MS symptoms
neurogenic bladder, fatigue, spasticity, sexual dysfunction, depression
does ALS start limb or face symptoms first?
limb onset usually
what are common ALS management things?
biPAP, feeding tube, communication devices, medications for drooling, ALS clinic rehab, clinical trials
what is the mean time of after diabetes onset to development of neuropathy?
8 years
Type 1 longer
Type 2 may actually be before
is burning pain or proprioception large myelinated fibers?
proprioception- pain is small unmyelinated
does regulated blood glucose reverse polyneuropathy?
no but may lessen pain
what medications do we use to treat neuropathy pain?
gabapentin, amitriptyline and pregabalin
what are the common infections of guillane barre?
CMV, EBV, campy, Hib
what are the antibodies against?
schwan cells/myelin
on peripheral nerves
does guillane barre effect sensation or motor?
both and then may effect breathing, heart rate, urinary retention, ileus
what does nerve conduction studies show for guillane barre?
demyelinating pattern
what does CSF show in guillane barre?
elevated total protein and no or few WBCs
what is Rx for guillane barre?
admit- monitor respiratory- may need IVIG or plasmapheresis
although many improve with guillane barre with time, what may persist in 50%-
neuropathic signs- paresthesias and numbness
what are antibodies of mysenthia gravis agains?
AcH receptors > reduction in action potentials of the muscle membrane and therefore muscle weakness
what is the most common presenting symptom of myesthenia gravis?
ptosis and diplopia
how do we diagnose myasthenia gravis?
blood test acetylcholine receptor antibodies
cholinesterase inhibitors like tensilon or prostigmine and weakness should improve
slow rate repetitive nerve stimulation > decrement
what is symptomatic medication for myasthenia gravis?
pyridostigmine
what is immunosuppresive treatment for myasthenia gravis?
prednisone, azathiprine, mycophenolate mofetil
what is used to treat myasthenia in acute severe crisis?
IVIG plasmapharesis
what organ may be removed to treat myasthenia gravis?
thmymectomy
is lambert eaton presynaptic or post synaptic?
pre
what are clinical features of lambert eaton?
fatiguing weakness, dry mouth, ED
what is lambert eaton often associated with?
malignancy- small cell lung carcinoma
how do we diagnose lambert eaton?
blood test P/Q ca channel antibodies, high rate repetitive nerve stimulation increment
is botulism a pre or post synaptic disorder?
pre synaptic
where do botulism symptoms start and then where do they fo?
bulbar then limbs and then respiratory
what is management of botulism?
supportive- ICU vent feeding. antitoxin
how do we diagnose polymyositis?
Creatine Kinase level elevation 50x
-muscle biopsy invasion of fibers by Tcells
How do we treat molymyositis?
prednisone
what is unique about dermatomyositis?
rashes- heliotrope rash-
what is the difference in diagnostic biposy of dermatomyositis?
perivascular inflammatory infiltrates, perifascicular atrophy is pathognomonic
what is the clinical hallmark of inclusion body myositis?
early weakness and atrophy of quads and forearms (wrist and finger flexors) and foot drop
what does muscle biopsy of inclusion body myositis show?
endomysial inflammation with basophilic granular indclusions around the edges of vacuoles
does inclusion body myositis respond to immunosupressors?
no
what is the most common inherited neuromuscular disease in adults?
myotonic dystrophty- CTG
why does myotonia occur in MD?
chloride channel abnormality
where do we see muscle wasting in myotonic dystrophy?
temporal wasting
what clinical features not related to muscle are often found with myotonic dystrphy,
cataracts, arrhythmias, hypogonadism, hypersomnia, frontal balding, retardation apathy
