ALS Flashcards
describe epidemiology of ALS
onset of mid to late 50s.
5-10% have family hx
what percentage of ALS cases are limb-onset and what percent are bulbar-onset?
70-80% limb
20-30% bulbar
describe the etiology of ALS
most cases have unknown etiology but small percent are hereditary.
what is the hallmark sign of ALS?
UMN and LMN pathology
what cranial nerves are involved with ALS?
5,7,9,10,12
ALS involves a degeneration of motor neurons in 4 areas…what are they?
primary motor cortex, corticospinal tract, brainstem, spinal cord
can’t neighboring neurons help out the neurons that are denervated?
yes but only for a certain amount of time. Once more than 50% of motor units are lost they can’t help anymore
talk about the progression of ALS throughout the body
it spreads throughout a whole region before moving onto a different region. It also begins distal then moves proximal.
what are the most frequent clinical manifestations of ALS?
focal asymmetrical muscle weakness that begins in either UE or LE. Weakness of the bulbar muscles.
what are examples of LMN sxs?
muscle weakness, hyporeflexia, hypotonicity, atrophy, muscle cramps, fasciculations
what are examples of UMN sxs?
spasticity, babinski, hoffman, hyperreflexia, muscle weakness
what are the impairments associated with Bublar pathology?
facial and neck weakness, dysphagia, dysarthria, sialorrhea, pseudobulbar affect
what is the definition of cachexia?
extreme weight loss and wasting
how often are cognitive impairments in ALS and what type of cognitive impairment is associated with ALS?
35% of people with ALS. Frontotemporal dementia
what is pseudobulbar affect?
exaggerated crying and then laughing, untriggered
why is fatigue common in ALS?
because the surviving motor units are overburdened.
what ocular pathology is related to ALS?
none really. if the pt lives longer then they may have opthalmoplegia
how are the sphincter muscles affected in ALS?
generally spared
what must a pt have in order for an ALS diagnosis?
LMN signs by clinical exam, EMG, or neuro examination
UMN signs clinical exam
Progression of the disease within a region or other regions by clinical exam
according to the el escorial criteria, what must a pt have in order to have definite ALS?
LMN and UMN signs in 3 regions
according to the el escorial criteria, what must a pt have in order to have definite Familial ALS?
LMN and UMN sxs in 1 region and identified DNA gene
according to El Escorial criteria, if a pt has LMN sxs in 2 regions but no UMN sxs, what type of ALS diagnosis would they have?
suspected ALS
what is the average duration for onset to death?
27-43 months
what typically causes death in ALS patients?
respiratory failure
