ALS Flashcards
what is the most common and fatal motor neuron disease
ALS
mortality rate
2-5 years
variable
what is ALS characterized by
progressive degeneration and loss of motor neurons in the spinal cord, brainstem, motor cortex
what is specifically degenerated
anterior horn cells
corticobulbar tracts
corticospinal tracts
what is onset
> 50 years
men or women are more affected
men, slightly
what causes the UMN symptoms
degeneration of UMN cell bodies in motor cortex
what causes the LMN symptoms
alpha motor neurons LMN of spinal cord
what is one cause of ALS
glutamate excitotoxicity
what does glutamate excitotoxicity increase
increased CSF, plasma, post-mortem tissue
risk factors for ALS
male > female
caucasian, non-hispanic
geographical clusters
family hx
55-75 y/o
priot trauma / TBI
diagnosis of ALS
difficult
dx by exclusion*
clinical exam
EMG/NCV
MRI
CSF, blood and urine tests – high creatine phosphokinase levels
what does a clinical dx of ALS require
pattern of observed and reported symptoms of both UMN/LMN
persistent decline in physical fxn that can’t be attributed to other disorders
what is the El escorial criteria for dx of ALS
exclusion of all other dx + progressive functional decline + progressive UMN + LMN deficits in more than or at least 1 region of the body
OR
LMN deficits in more than or at least 1 limb/region as defined by clinical examination and/or EMG in 2 clinical regions
what regions are involved with El escorial criteria
defined as bulbar, cervical, thoracic, lumbosacral
what is included in the gold coast criteria for dx of ALS
investigations excluding other disease processes AND
progressive motor impairment documented by hx or repeated clinical assessment, preceded by normal fxn AND UMN/LMN signs in at least 1 region of the body OR LMN dysfunction in at least 2 regions
what regions are involved with gold coast criteria for dx of ALS
bulbar, UE/LE
what does El escorial criteria dx lead to
suspected, possible, probable and definite ALS
what does gold coast criteria for dx lead to
+ ALS or - ALS
cardinal sign of ALS
muscle weakness - focal, asymmetrical muscle weakness beginning in LE or UE or bulbar weakness
do you see more UMN weakness or LMN
LMN weakness
what happens to eye, bowel and bladder
normal spared until terminal stages
what do respiratory impairments look like for this population
decreased strength
reduction of VC (25-35%)
early S&S of muscle weakness
what do cognitive impairments look like for this population
variable
verbal fluency, language comprehension, memory, abstract reasoning
who are cognitive impairments more common in
bulbar onset
additional impairments
pain
fatigue
ANS symptoms
what is spared in this disease
sensory pathways
ocular motor fxn
sphincter (bowel/bladder)
