ALS Flashcards
most common adult-onset motor neuron disease:
ALS
commonly known as _______ disease
Lou Gehrig’s
ALS means:
amyotrophic: no muscle
lateral: spinal cord where motor neurons are located
sclerosis: hardening of the affected muscles
two classifications of ALS:
familial ALS: family hx –> mostly autosomal dominant
sporadic ALS: no family hx
______% develop limb onset ALS
______% develop bulbar onset ALS
regardless of initial involvement, both ______ and ____ are implicated in the disease
70-80%
20-30%
UMN;LMN
ALS is always _______ and with disease progression individual will become ______ in all aspects of life
fatal; dependent
Epidemiology: higher incidence in men or women?
average age of onset?
men; 50s
Etiology is largely _______
Possible causes:
excess ______ causing ecotoxicity
____ mutation
inflammation/autoimmune response
growth factor abnormalities
viral infections
apoptosis (programmed cell death)
unknown
glutamate
SOD - 1
known risk facks of ALS:
age
gender
family hx
clusters (ex. western pacific ALS/PDC)
disease-causing mutations
unknown risk factors for ALS:
diet
vigorous physical activity
trauma
neurotoxicant exposure
lifestyle factors
ALS involves the loss of ______ neurons in the _______ cortex, _____ and ______
-typically ALS progresses ________ a body region before moving to a neighboring region of the body
-_____ onset ALS may spread to the cervical region
motor; motor, spinal cord, brainstem
within/single
bulbar
CN commonly affected includes:
CN V, VII, IX, X, XII
ALS involves degeneration and eventual loss of motor neurons, but early on reinnervation from healthy axons to denervated muscles. With disease progression:
—______occurs
reinnervation cannot keep up with the rate of denervation
muscle atrophy
ALS does not just affect motor areas but nonmotor such as
ANS
basal ganglia
cerebellum
frontotemporal
oculomotor
sensory systems
Clinical manifestations in ALS:
extent of motor neuron loss
combination of UMN and LMN loss
non-motor area affected
pattern of onset
body regions affected
sec. complication
stage of disease
LMN Signs:
muscle weakness
hyporeflexia
hypotonicity
muscle cramps
atrophy
fasciculations
UMN signs
spasticity
hyperreflexia
pathologic reflexes
muscle weakness
bulbar pathology:
spastic bulbar palsy (____)
flaccid bulbar palsy (____)
is mixed palsy common?
Clinical presentation: (5)
UMN
LMN
yes
bulbar muscle weakness
dysphagia
dysarthria
sialorrhea
pseudobulbar affect
What is the leading cause of death in individuals with ALS?
pt. complaints: (5)
respiratory failure
fatigue
decrease activity level
inability to tolerate supine
headache due to hypoxia
changed in speech
cognitive deficits may include:
decreased language comprehension
impaired memory
difficulty w/abstrct reasoning
frontotemporal dementiaia
bulbar onset is more predictive of ____ impairment than limb onset
cognitive
clinical manifestations: rare (3)
sensory
bowel and bladder
ocular palsy
there is no single test to diagnose someone with ALS
a diagnosis of ALS is made by ______ of disease progression and testing to rule out other neurologic diseases
tests to rule out: (6)
observation
EMG, lab studies, neuroimaging, nerve conduction testing, and/or muscle and nerve biopsies
symptom onset to diagnosis can range average from _____-____ months
diagnostic criteria to aid physicians in the diagnosis of ALS (2)
8-15 months
el escorial criteria
Awaji-shima criteria
