N
Signs and Symptoms of ALS: alpha motor neuron involvement
- muscle weakness + evidence of denervation (fasciculations and atrophy)
what is myesthenia gravis? how common is this?
- NMJ disorder
- immunological disorder in which antibodies are directed against the POSTsynaptic nicotine acetylcholine receptors
- MOST common neuromuscular transmission disorder
What is LEMS associated with?
small cell lung cancer
steroid myopathies tend to be...
proximal without involvement of ocular, bulbar, or facial muscles
onset of DMD/BMD?
onset is usually in childhood around age 3
Signs and Symptoms of ALS: lateral corticospinal tract
- weakness, spasticity, hyperreflexia, babinski sign
myesthenia gravis is bimodal, describe the 1st peak and 2nd peak
1st peak in 2nd-3rd decade; Women > Men
2nd peak in 7th-8th decade; Men > Women
What is spinal muscular atrophy? What is it characterized by?
group of alpha motor neuron diseases without spasticity deficits
characterized by degeneration of cranial motor nuclei and anterior horn cells
inheritance, onset, progression and life expectancy of muscular dystrophy
-x-linked
- childhood age 3
- proximal muscle weakness, including neck flexors predominates; pseudohypertrophy of calf muscles->fatty infiltration
- progressive muscle changes
DMD is progressive and children would most likely need the use of a wheelchair by age ______
10-12 y.o
How do initial ALS symptoms begin and how do they spread?
Initial symptoms begin with weakness in localized area → gradual spread to muscles in same region → contralateral UE → ipsilateral LE → contralateral LE → Bulbar muscles
defining characteristics of myesthenia gravis and muscle weakness
-ocular
- bulbar
- limb
worse at what time of day?
- ocular (most common): ptosis and diplopia
- bulbar: dysarthria and dysphagia
- limb: usually proximal and symmetric
worse towards end of the day
What does myopathie mean? what is the most common symptom?
nonspecific term for disorders of skeletal muscle
weakness
what gene is involved in MD? What is Gowers sign?
- dystrophin
- climb up legs to erect, move hands up thighs
DMD death is usually around_____ due to
20
respiratory insufficiency and aspiration
describe the progression, typical time to fatality and cure?
-fatal within 3-5 years of dx
- lost motor while maintaining cognitive fx
- no cure but riluzole can slow down progression
what is myasthenic crisis?
presentation with respiratory weakness- medical emergency
what are positive and negative symptoms of myopathies?
Symptoms may be positive (pain, stiffness, cramps) or negative (weakness, fatigue)
Describe limb-girdle MD in regards to what muscles are affected, speed of progression and effects from one side to the other
- symmetrical, slowly progressive and proximal muscles most affected
True of false: BMD reduces life expectancy but not as severely as DMD?
true
what symptoms are NOT present in ALS
NO sensory sign deficits and eye movement unaffected
What is LEMS?
pre-synaptic NMJ condition
Caused by antibodies directed against the presynaptic terminal
Reduction of pre-synaptic Ca = reduction in release of ACh = weakness
what are inflammatory myopathies treated with?
corticosteroids
what is the typical age of onset for limb-girdle MD? what gender affected?
10-30 years and both genders affected equally
free box
:)