ALS - background

subtypes of motor neuron disorders

amyotrophic lateral sclerosis, primary lateral sclerosis, progressive bulbar palsy, progressive muscular atrophy

amyotrophic lateral sclerosis: degeneration of ____ tracts, neurons in the ____ cortex and _____, and ____ horn cells

corticospinal, motor, brainstem, anterior

primary lateral sclerosis: degeneration of _____

upper motor neurons

progressive bulbar palsy: degeneration of ____ neurons of CN ____ and ____

motor, IX, XII

progressive muscular atrophy: loss of ____ neurons in the ____ and _____

motor, spinal cord, brainstem

UMN lesion is

• ____ the ____ horn

• ____ the ____ nerves

above, anterior, above, motor nuclei of cranial

LMN lesion is

• _____ the ____ horn

• _____ the ____ nerves

below, anterior, below, motor nuclei of cranial

ALS results in variety of ____ and ____ symptoms 

UMN, LMN 

ALS = degeneration of motor neurons in the ___, ___ and ____

spinal cord, brainstem, motor cortex

average onset of ALS

mid to late 50s

ALS affects ____ slightly more

men

cause of ALS is mostly ____

unknown 

known risk factors for ALS

• ____ mutations

• ____ gender

• _____

• _____ Hx

• _____

disease causing, male, clusters, family, age

possible risk factors for ALS

• ____ exposures

• ____ physical activity

• ____

• ____

• ____ characteristics

• ____ factors

neurotoxicant, vigorous, trauma, diet, occupational, lifestyle

ALS has involvement from ____ areas like:

• ____ nervous system

• _____

• _____

• _____ lobe

• _____

• _____

non-motor, autonomic, basal ganglia, cerebellum, frontotemporal, oculomotor, sensory 

4 main phenotypes of ALS

LMN, UMN, bulbar, respiratory

LMN pathology ALS

• _____ weakness, _____reflexia, ______, _____, muscle ____, ____

muscle, hypo, hyotonicity, atrophy, cramps, fasciculations

UMN pathology ALS

• ____, ____ reflexes, _____reflexia, ____ weakness

spasticity, pathologic, hyper, muscle

bulbar ALS 

• ____ mm weakness, ____, ____, sialorrhea, ____ affect 

bulbar, dysarthria, dysphagia, pseudobulbar 

pseudobulbar affect: ____ changes in mood at is outside of ____

uncontrolled, norm

respiratory ALS

• ____ mm weakness, _____, noctural ____ difficulty, ____, hypoventilation, ___ retention, ineffective _____

respiratory, dyspnea, respiratory, secretion, cough

frontotemporal demential related impairments ALS

• ____ and ____ impairments

cognitive, behavioral

other potential rare impairments with ALS 

• _____ impairment 

• ____ dysfunction 

• ____ palsy 

sensory, bowel, bladder, ocular 

secondary ALS impairments

• ____, weight ____. cachexia, decreased ____, tendon ____, joint ____, joint _____ etc

sensory, loss, ROM, shortening, contracture, subluxaiton

no definitive ____ tests or _____ for ALS

diagnostic, biomarker

time interval from symptom onset to Dx = ____-____mo

8, 15

used to support Dx and exclude others 

• ____ presentation 

• ____ studies 

• EMG 

• ____ conduction velocity

• ____ and ____ biopsies 

• ____ studies 

clinical, labratory, nerve, muscle, nerve, neuroimaging 

help to Dx with ALS - Presence of

• _____ signs by clinical, electrophysiologic or neuropathological exam

• ____ signs by clinical exam

• progression of disease within a ____ or to ____ by clinical exam or via med history

LMN, UMN, region, new region

ALS is ____ and ____

• progression from ____ limitations to ____ restrictions is inevitable

progression, deteriorating, activity, participation

in most pts with ALS, death occurs within ____ to ____ yrs after ____ onset and usually results from ___ failure

3, 5, symptom, respiratory

factor that has strongest correlation to prognosis = ____

age 

patients les than ___-___ yo had better 5 yr survival rates

35, 40

_____ onset ALS better prognosis than ____ onset

limb, bulbar

factors contributing to poor prognosis

• ____ between symptom onset and Dx

• presence of ___ or FTD

• ____ distress

delay, dementia, psychological

Riluzole (Rilutek) is a _____

glutamate inhibitor 

Riluzole (Rilutek)

• extends survival ____-____ mo

• standard dose is ____mg ____x a day

2, 3, 50, 2

side effects of Riluzole (Rilutek): ___ toxicity, asthenia, ___, vomitting, and ____

liver, nausea, dizziness

Radicava (Edaravone) is a _____

free radical scavenger

Radicava (Edaravone)

• more effective in ____ stages 

• continuous cycle of TX: delivered IV daily for ___-___ days, followed by ____ for next ____-____ days 

earlier, 10, 14, no drug, 10, 14

side effects of Radicava (Edaravone): contusion, ____ disturbance, _____, dermatits, eczema

gait, headache

ALS requires ____ care

palliative

no ___ for ALS but stil considered ___ disease

• ____ management

  • _____

  • ____ care

  • address ____ as they arise

cure, treatable, symptomatic, individualized, supportive, impairments

most common reported symtpoms of ALS 

fatigue 

multidisciplinary ALS goals

• informing ____ and ____ about the diagnosis and prognosis

• ____ of communication problems, insomnia, anxiety, depression, spasticity, fatigue, cramps

• ____ management

• management of ____ insufficiency and ____ decisions

• diagnosis and management of ____ and ____ impairments

• ____ therapies

• ____ care

patient, family, management, nutrition, respiratory, ventilation, cognitive, behavior, drug, palliative