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RCA Test 2 > neuromuscular diseases > Flashcards

neuromuscular diseases Flashcards

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0
Q

Multiple sclerosis causes

A

Unsure (genetic?)
Mineral deficiency ?
Vitamin D3
Virus? Epistein bar, herpies

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1
Q

Multiple sclerosis physiology

A 
Upper motor neuron 
Inflammatory response 
Glial cells destroyed 
Demyelination of white matter
Impulses are slowed or blocked 
Increase in sodium channels 
Myelin sheath and cells destroyed
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2
Q

Multiple sclerosis prevalence

A

Northern climates

20-30 years of age

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3
Q

Multiple sclerosis

A

Optic neuritis - 25% partial or total loss of vision
Any part of CNS
Brain and spinal cord

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4
Q

Multiple sclerosis signs and symptoms (early)

A 
1st symptom in 50% numbness in one or more limbs 
Weakness 
Spastic 
Babinski sign 
Emotional instability 
Bladder disfunction
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5
Q

Multiple sclerosis symptoms (late)

A 
Severe muscle spasm 
Urinary incontinence 
Difficulty swallowing 
Contractures 
Spastic ataxic 
URTI
Surgery 
Stress
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6
Q

Multiple sclerosis (course)

A 
Recovery over weeks to months 
Incomplete 
Exacerbations and remission
Slurred speech, bed ridden, Incontinence
12 to 15 years
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7
Q

Multiple sclerosis (diagnosis)

A

CSF - increase # of gamma globulin and immunoglobulin

MRI - scarred lesions in brain

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8
Q

Multiple sclerosis treatment

A 
Steroids 
Decadron
Interferon (proteins formed when cells are exposed to a virus) 
ACTH
Decrease inflammation 
Physical therapy, supportive
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9
Q

Myasthenia gravis (patho)

A

Nerve impulse fails to pass muscles at myoneural junction

Motor end plate

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10
Q

Myasthenia gravis

A 
Rare disease occurring in young adults 
Auto immune 
Women effected more then men (2 to1) 
aspiration is common
URTI occur due to ineffective cough 
May require artifical airway
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11
Q

Eaton lambert syndrome

A

Special form of myasthenia gravis
Found in people with oat cell carcinoma
Visual symptoms less frequent
Trunk , pelvic and shoulder girdle involved
Weakness with exertion, but temp in muscle power at first

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12
Q

Myasthenia gravis (patho)

A

Inability of motor end plate to secrete acetylcholine
Excessive cholinestrase at nerve endings
Non response of muscle fiber to acetylcholine
Antibodies to acetylcholinestetase receptors at end plate

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13
Q

Myasthenia gravis (clinical)

A

Droopy eyelids (ptosis)
Usually muscles of eyes, face, jaws, neck first then limbs
Proximal greater than distal (vision)
Muscle weakness with gravis prognosis
Repeated use of muscle leads to progressive weakness restored by rest

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14
Q

Myasthenia gravis (diagnosis)

A

EMG -electromyography
IV edrophonium chloride (Tensilon) test
-short acting anticholinestrase
-increase in muscle strength test is positive

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16
Q

Myasthenia gravis (treatment)

A 
*Anticholinesterase 
Neostigmine
Pyridostigmine 
*Steroids 
Plasmapheresis 
Atropine - blocks acetylcholine 
Tensilon (underdosage and overdosage decrease muscle strength)
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17
Q

Myasthenia Gravis (general)

A 
in advance stages: effects all muscles 
NO cardiac or sensory involvement 
Rapid or slow with exacerbation and remission 
Thymic turmors 25% 
thymic hyperplasia 80%
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18
Q

Myasthenia Gravis (drugs to avoid)

A

Muscle relaxants - Morephine
barbiturates
tranquilizers
neomycin

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19
Q

ALS (general)

A 
Lou Gehrig Disease 
Upper or lower motor neurons 
Effects men more than women 
Maybe Genetic - SOD type 1
Slow viral infection ?
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20
Q

ALS (patho)

A 
myelin sheath destroyed - scar tissue 
Anterior horn cells are affected
Loss of motor NOT sensory
spinal cord lateral tracts / medulla ventral tracts 
nerve impulses blocked
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21
Q

ALS (Clinical)

A

fatigue, awkwardness of fine finger movements
Dysphagia (difficulty swallowing)
Spasticity of flexor muscles
progressive muscle weakness, atrophy, fasciculations (muscle twitching)
Jaw Clonus

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22
Q

ALS (theories)

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A

excess naturally glutamate in synaptic cleft,- cell death
oxidative injury (malfunction of SOD1
Protein aggregates
axonal strangulation

23
Q

ALS (outcomes)

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A

One side of body more involved with progression
person remains alert no sensory loss
death within 5 to 10 years (resp or bulbar) paralysis

24
Q

ALS (medical management)

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A

Heparin prophylactically to decrease P.E.
Nasogastric tube
home care ventilation for respiratory failure

25
Poliomyelitis (general)
Acute febrile diaseae Cause by three types of virus Paralysis common with type 1 Salk vaccine, safe "live virus"
26
Poliomyelitis (patho)
Incubation 7 to 21 days virus attacks anterior horn cells of spinal cord Motor pathways damaged, NOT sensory Motor units become enlarged with recovery
27
Bulbar Poliomyelitis
attacks medulla and basal structures of brain and cranial nerves
28
Poliomyelitis (clinically)
No symptoms 90 to 95% of time mild systemic symptoms aseptic meningitis 3 to 4 days after muscle weakness in 48 hours to weeks
29
Poliomyelitis (clinically2)
``` spinal cord (limb weakness) brain stem, crainal nerves, cerebral lower motor neurons decrease reflexes atrophy, no sensory loss, pain ```
30
Poliomyelitis (outcomes)
Some recovery after 3 to 4 months Resp failure -due to involvement of resp control center, muscles of diaphragm, intercostals lose neurons at a faster rate than normal
31
Poliomyelitis (treatments)
Supportive: Physiotherapy | ventilation/suctioning
32
Post polio syndrome
progressive weakness with aging
33
Musclar Dystrophy (general)
``` gradual wasting of skeletal muscle progressive with increased weakness diminution of muscle mass confined to wheel chair NO REMISSION ```
34
Muscular Dystrophy (general)
Four clinical forms of the disease | Microscopic abnormalities of skeletal muscles
35
Muscular Dystrophy (Form of)
``` Duchenne Symptoms noted before age of 5 spinal deformity waddling gait, muscle wasting involves HEART muscles! (leads to death) X-linked recessive (ALL BOYS) ```
36
Muscular Dystrophy (Becker)
milder form of Duchenne musclar dystrophy
37
Facio-Scapulo-Humeral MD
type of muscle dystrophy M/F equally effected weakness/wasting of shoulder girdle/upperarms onset puberty weakness of facial muscles first years of life
38
Limb-Girdle MD
type of muscular dystrophy M/F equally, early or late in life, slow course most of the muscles of the body affected
39
Myotonic MD
type of muscular dystrophy M/F equally effected occurs earlier and more severe later gens delayed relaxation of muscles cataracts in both eyes may occur, reproductive functions distrubed
40
Muscular Dystrophy (treatment)
origins in genetic mutation | supportive treatment: physical and occupational therapy
41
Miscellaneous causes of resp muscle weakness
Malnutrition, electrolytes (hypokalemia, hypomagnesemia, hypophosphatemia, hypochloremia) Iatrogenic causes due to treatment with corticosteroids Botulism, tetanus (infectious causes)
42
Guillain-Barre syndrome (general)
an inflammatory disease that effects PNS autoimmune response rapid symmetrical limb weakness ascending pattern
43
Guillain-Barre syndrome (general)
#1 cause of acute NM weakness Acute inflammatory Neuropathy all ages commonly in 30-50 yr, Males
44
Guillain-Barre syndrome (etiology)
occurs 2 wks after recent URTI or GI infection Campylobacter jejuni (major cause) occurs with: HIV, mycoplasma, Hep, epstine occurs after immunizations associated with hodgkins lymphoma
45
Guillain-Barre syndrome (patho)
Injury to PNS ONLY! immunological cell mediated/humoral (antibody) reaction lymphocytes sensitized and attach to myelin sheaths on peripheral nerves. Demyelination by macrophages remove damaged myelin
46
Guillain-Barre syndrome (patho)
all peripheral nerves affected become inflamed impediment of nerve impulses -myelin damage schwann cells proliferate+remyelination
47
Guillain-Barre syndrome (clinical)
3 main stages of disease acute onset 1-3 weeks Plateau symptoms remain but dont get worse Recovery few weeks to 2 yrs, full recovery Glove and stocking distribution entrance complaint- difficulty walking, trouble up and down stairs, trouble rising from chair
48
Guillain-Barre syndrome (symptoms)
progressive ascending skeletal muscle paralysis tingling sensation + numbness (distal paresthesia) Loss of deep tendon reflexes SENSORY nerve impairment under and over reaction of SNS or PNS CARDIAC arrhythmias malignant hypertension
49
Guillain-Barre syndrome (symptoms)
peripheral facial weakness decrease gag reflex; cranial nerve 9,10 decrease ability to swallow muscle paralysis involves ab, diaphragm, arms postural hypoten, thermal reg, reten urine mechanical vent
50
Guillain-Barre syndrome (clinical assessment)
ABG - acute ven failure, resp acidosis hypoemia PFT's - reduce lung vol & capac diminish B/S w/crackles + wheezes CXR - normal
51
Guillain-Barre syndrome (clinical assessment)
past hx - preceding infection, weakness lumbar puncture - CSF - protein reduced nerve conduction (blocked/slow) electromyography- single muscle fiber activate progressive motor weakness >1 limb+areflexia
52
Guillain-Barre syndrome (treatment)
plasmapheresis or TPE (shorten course) IV immunoglobulin all pts need hospitalized high risk vent fail
53
plasmapheresis
removes portion of patients circulating blood, centrifuged, separate elements, mixed with 50% ALBUMIN 50% SALINE, reinfused, old plasma discarded 5 treatments in a row lasting 90 mins not for mild symptoms contraindicated: acute MI, angina, sepsis, autonomic dysfunction .
54
Guillain-Barre syndrome (Prognosis)
80% of pts make total recovery in 1 year 25% req mech vent 10% relapse

RCA Test 2 (4 decks)

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