ALS Flashcards
what is ALS
a neurodegenerative disease that effects the motor neuros of the BS, brain and the spinal cord
what is the most common neuro disease in the adult population
ALS
what age does ALS occur at
at any age but normally after 50s
when does death occur for these indivduals
3-5 from onset
what type of ALS is more prominent limb or bulbar
limb onset
70% - 80%
how prevent is the bulbar onset of ALS
20 - 30%
Limb-onset ALS presentation
Initial involvement in extremities
Bulbar-onset presentation
initial involving the bulbar muscle - diffucty speaking, chewing, and swallowing
what pop is the bulbar more prevalent in
middle aged women
how many people with ALS have a family presentation with it
5 -10%
Autosomal dominant (in most cases)
hat are known risk factors for ALS
disease causing mutations
clusters - western pacific ALS
family history
age
gender (male > females)
possible risk factors
neurotoxiant disorders
certian occupations
diet
lifestyle factors
vigours physical axtivity
what is the pathophysiology of ALS
progressive degeneration and loos of motor neurons in the spinal cord, motor cortex, and motor cortex
early versus late in the disease progression neurosn
early - healthy intaxct neurons can replace the degenerated neurons
later - there are not more healthy neuron to replace the degenerated ones
what areas impact with ALS
UMN
corticospinal tract
brainstem nuclei for CN 5, 7, 9, 10, 12
anterior horn of the spinal cord
what is normally spared with ALS
brainstem nuclei that are controlling the extral ocular movemnts
motor nuclei that are controlling the pelvic floor striated muscles - anal and external sphincter
sensory system
what is the most frequently presenting impairment
focal assymetrical weakness
muscle weakness is isolated to. a specific muscle
what is the caridnal sign of ALS
muscle weakness
LMN path
muscle weakness
hyporeflexia
hypotonicity
atrophy
muscle cramp
faciulations
what is a fasciculation
a muscle twitch
UMN patho
hyperreflexia
hypertonicty - spacity
muscle weakness
patho relfexes
bulbar patho
dysphalgia
dysarrthia
sialorrhea
what is sialorrhea
excessive saliva flow
what is what is pseudobulbar affect
Inappropriate involuntary laughing and crying due to a nervous system disorder.
Respiratory impairments
seen with ALs
Inspiratory/expiratory muscle weakness,
dyspnea/DOE,
nocturnal respiratory difficulty, orthopnea,
hypoventilation,
secretion retention, ineffective cough
what is orthopneas
Discomfort when breathing while lying down flat;
neck issues and ALS
neck stiffness
heavy head sensation
hard time stablizing the head
what are 2nd impairment that you might see with muscle weakness
contracture
sublexation
tendon shortening
decreased ROM
adhesive capcilitus
amb difficulties
foot drop
what symptoms do we look for the diagnosis of ALS
LMN degradation - neuroexamination
UMN degration
progressive spread if signs within the specfic region
what must be absent for the issue to be ALS
Electrophysiologic evidence of other disease processes that might explain the signs of LMN or UMN degeneration
Neuroimaging evidence of other disease processes that might explain the observed clinical and electrophysiologic signs