neuromuscular diseases Flashcards
Multiple sclerosis causes
Unsure (genetic?)
Mineral deficiency ?
Vitamin D3
Virus? Epistein bar, herpies
Multiple sclerosis physiology
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
Multiple sclerosis prevalence
Northern climates
20-30 years of age
Multiple sclerosis
Optic neuritis - 25% partial or total loss of vision
Any part of CNS
Brain and spinal cord
Multiple sclerosis signs and symptoms (early)
1st symptom in 50% numbness in one or more limbs Weakness Spastic Babinski sign Emotional instability Bladder disfunction
Multiple sclerosis symptoms (late)
Severe muscle spasm Urinary incontinence Difficulty swallowing Contractures Spastic ataxic URTI Surgery Stress
Multiple sclerosis (course)
Recovery over weeks to months Incomplete Exacerbations and remission Slurred speech, bed ridden, Incontinence 12 to 15 years
Multiple sclerosis (diagnosis)
CSF - increase # of gamma globulin and immunoglobulin
MRI - scarred lesions in brain
Multiple sclerosis treatment
Steroids Decadron Interferon (proteins formed when cells are exposed to a virus) ACTH Decrease inflammation Physical therapy, supportive
Myasthenia gravis (patho)
Nerve impulse fails to pass muscles at myoneural junction
Motor end plate
Myasthenia gravis
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
Eaton lambert syndrome
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
Myasthenia gravis (patho)
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
Myasthenia gravis (clinical)
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
Myasthenia gravis (diagnosis)
EMG -electromyography
IV edrophonium chloride (Tensilon) test
-short acting anticholinestrase
-increase in muscle strength test is positive
Myasthenia gravis (treatment)
*Anticholinesterase Neostigmine Pyridostigmine *Steroids Plasmapheresis Atropine - blocks acetylcholine Tensilon (underdosage and overdosage decrease muscle strength)
Myasthenia Gravis (general)
in advance stages: effects all muscles NO cardiac or sensory involvement Rapid or slow with exacerbation and remission Thymic turmors 25% thymic hyperplasia 80%
Myasthenia Gravis (drugs to avoid)
Muscle relaxants - Morephine
barbiturates
tranquilizers
neomycin
ALS (general)
Lou Gehrig Disease Upper or lower motor neurons Effects men more than women Maybe Genetic - SOD type 1 Slow viral infection ?
ALS (patho)
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
ALS (Clinical)
fatigue, awkwardness of fine finger movements
Dysphagia (difficulty swallowing)
Spasticity of flexor muscles
progressive muscle weakness, atrophy, fasciculations (muscle twitching)
Jaw Clonus
ALS (theories)
excess naturally glutamate in synaptic cleft,- cell death
oxidative injury (malfunction of SOD1
Protein aggregates
axonal strangulation
ALS (outcomes)
One side of body more involved with progression
person remains alert no sensory loss
death within 5 to 10 years (resp or bulbar) paralysis
ALS (medical management)
Heparin prophylactically to decrease P.E.
Nasogastric tube
home care ventilation for respiratory failure
Poliomyelitis (general)
Acute febrile diaseae
Cause by three types of virus
Paralysis common with type 1
Salk vaccine, safe “live virus”
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
Bulbar Poliomyelitis
attacks medulla and basal structures of brain and cranial nerves
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
Poliomyelitis (clinically2)
spinal cord (limb weakness) brain stem, crainal nerves, cerebral lower motor neurons decrease reflexes atrophy, no sensory loss, pain
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
Poliomyelitis (treatments)
Supportive: Physiotherapy
ventilation/suctioning
Post polio syndrome
progressive weakness with aging
Musclar Dystrophy (general)
gradual wasting of skeletal muscle progressive with increased weakness diminution of muscle mass confined to wheel chair NO REMISSION
Muscular Dystrophy (general)
Four clinical forms of the disease
Microscopic abnormalities of skeletal muscles
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)
Muscular Dystrophy (Becker)
milder form of Duchenne musclar dystrophy
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
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
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
Muscular Dystrophy (treatment)
origins in genetic mutation
supportive treatment: physical and occupational therapy
Miscellaneous causes of resp muscle weakness
Malnutrition,
electrolytes (hypokalemia, hypomagnesemia, hypophosphatemia, hypochloremia)
Iatrogenic causes due to treatment with corticosteroids
Botulism, tetanus (infectious causes)
Guillain-Barre syndrome (general)
an inflammatory disease that effects PNS
autoimmune response
rapid symmetrical limb weakness ascending pattern
Guillain-Barre syndrome (general)
1 cause of acute NM weakness
Acute inflammatory Neuropathy
all ages commonly in 30-50 yr, Males
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
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
Guillain-Barre syndrome (patho)
all peripheral nerves affected become inflamed
impediment of nerve impulses -myelin damage
schwann cells proliferate+remyelination
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
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
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
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
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
Guillain-Barre syndrome (treatment)
plasmapheresis or TPE (shorten course)
IV immunoglobulin
all pts need hospitalized high risk vent fail
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 .
Guillain-Barre syndrome (Prognosis)
80% of pts make total recovery in 1 year
25% req mech vent
10% relapse
