ALS

special test and measures for muscle performance?

- MMT - isokinetic muscle strength testing - handheld dynamometry

what are the other 3 things that are spared with ALS?

- Onuf's nucleus - sensory system - spinocerebellar tracts

management of dysphagia

- adjust eating position (tuck chin during swallowing) - modify food and fluid consistency

what is FTD characterized by?

- cognitive decline - executive functioning impairments - difficulties with planning/organization/concept abstraction - personality/behavioral changes

LMN impairments may lead to secondary impairments. What are the 4 secondary impairments?

- decreased ROM - joint subluxation - tendon shortening (achilles) - joint contractures (claw hand deformity)

middle stage ALS impairments

- develops an increase in number of impairments and severity of impairments - patient experiences increasing signs and symptoms

ALS without FTD

- difficulty with verbal fluency and language comprehension - changes in memory - difficulty with abstract reasoning - generalized impairments in intellectual function

with UMN impairments, you may have difficulty with swing phase of gait due to?

- distal spasticity - decreased balance

s/s of respiratory insufficiency

- dyspnea with exertion - daytime sleepiness - orthopnea (pillows) - weak cough - inability to blow nose - tachypnea - increased use of accessory respiratory muscles - paradoxical breathing - morning headaches

management of dysarthria

- exaggerate articulation - decrease rate of speech - as disease progresses, focus on decreasing the patients dependence on speech as the primary method of communication

special tests and measures for motor function

- examine hand function - examine initiation - modification and control of movement patterns should be examined

what are the early signs of respiratory impairments?

- fatigue - dyspnea on exertion - difficulty sleeping supine - frequent awakening at night - recurrent sighing - excessive daytime sleepiness - morning headaches due to hypoxia

special test and measures for joint integrity, ROM and muscle length

- functional ROM (AROM, AAROM, PROM) - muscle length - soft tissue flexibility and extensibility

disease specific measures

- functional status of patient - uses scale from 4 (normal) to 0 (unable to attempt) - correlates with measures of UE and LE strength - respiratory items

other LMN symptoms

- hyporeflexia - flaccidity - muscle cramping

where does the focal, asymmetrical muscle weakness usually begin?

- in LE or UE - bulbar

what are the initial symptoms of dysarthria?

- inability to project voice - enunciation problems

flaccid dysphagia

- liquids may regurgitate into the nose - cough reflex may be weak increasing the risk of aspiration

what are respiratory impairments related to?

- loss of respiratory muscle strength - decrease in vital capacity

what 2 things is sialorrhea due to?

- lower facial muscle weakness - absence of automatic spontaneous swallowing

with dysphagia, patients are at risk for suboptimal food intake due to

- muscle atrophy - jaw weakness - fatigue - drooling - choking on fluid and food

muscle cramp management

- muscle stretching - adequate hydration and nutrition - anticonvulsant meds if not relieved

special tests and measures for CN integrity?

- oral motor function - phonation - speech production

management of respiratory impairments

- pneumococcal and yearly influenza vaccinations - prevention of aspiration - effective oral and pulmonary secretion management

respiratory tests and measures

- respiratory symptoms and muscle function - breathing pattern - chest expansion - respiratory sounds - cough effectiveness - FVC (handheld spirometer)

three types of PT interventions

- restorative/remediation - compensatory - preventive

test and measures frequently applicable to patients with ALS include

- sensory function - muscle performance - motor function - coordination/balance - gait - functional status - environment - respiratory function - cognitive function

Fatigue is another LMN impairment. What 4 things may fatigue be related to with ALS?

- sleep disturbances - respiratory impairments - hypoxia - depression

UMN signs

- spasticity - hyperreflexia - clonus - babinski - hoffmann

ALS is a progressive degeneration and loss of MOTOR NEURONS in what 3 places?

- spinal cord - BS - brain (motor cortex)

how can you test posture?

- static - dynamic - postural alignment and body mechanics during self-care functional mobility skills, functional activity and work conditions

what are the signs of respiratory impairments as ALS progresses?

- truncated speech - orthopnea - dyspnea at rest - paradoxical breathing - accessory muscle use - weak cough

when do you test for sensation?

- when patient complains of sensory symptoms - if sensory involvement is suspected

average duration of ALS

27-43 months

what CNs are spared?

3, 4 and 6

which CNs are most affected with ALS?

5, 7, 9, 10 and 12

what does ALS stand for?

Amyotrophic Lateral Sclerosis

special tests and measures for tone and reflexes

DTR and pathological reflexes should be tested to distinguish between UMN and LMN involvement

what is ALS also known as?

Lou Gehrig's disease

management of pseudo bulbar affect

SSRIs or tricyclic anti-depressants

eventually, a patient that has dysarthria with ALS becomes _________

anorthic (doesn't talk)

management of sialorrhea

anticholinergic medications to decrease saliva

limb onset ALS have better or worse prognosis than those with bulbar onset

better

which types of ALS are more likely to have cognitive impairments?

bulbar onset

patients with brisk, widespread fasciculations are instructed to avoid what?

caffeine and nicotine

progression is spread in a __________ manner before developing rostral or caudal symptoms

contiguous (s/s spread locally - within spinal cord segments - before moving to other regions)

what is the first sign that they have sialorrhea?

drooling at night

what kind of palsy can Dysarthria occur with?

either type (spastic or flaccid)

what kind of palsy can Dysphagia occur with?

either types (spastic or flaccid)

sialorrhea

excessive saliva and drooling

what is a LMN symptom that is common in individuals with ALS?

fascicultions

what is the most frequent LMN impairment?

focal, asymmetrical muscle weakness

with LMN impairments, you can get cervical extensor weakness. Which way does the head typically fall?

forward and becomes completely flexed as the disease progresses

what kind of dementia is considered part of the ALS spectrum?

frontotemporal dementia (FTD)

with the late stage of ALS, the restorative intervention is directed toward remediation of?

impairments from other systems (edema and pressure sores)

where does muscle weakness usually occur initially?

isolated muscles more distally

early stage ALS activity limitations

may or may not cause minor activity limitations

if cognitive impairments are expected, what test or measure can you do?

mini mental - referral for a neuropsychological eval may be indicated

what kind of palsy is common with ALS?

mixed palsy (spastic or flaccid bulbar palsy)

compensatory intervention

modifying activities, tasks or the environment to minimize activity limitations and participation restrictions

swelling of distal limb may develop owing to lack of what?

muscle pumping action in weakened extremity

what is a cardinal sign of ALS (due to UMN or LMN)?

muscle weakness

pharyngeal weakness results in _____ tone

nasal

is pain a direct impairment of ALS?

no

are there specific balance test and measures specific for ALS?

no, but can do berg TUG or functional reach

are there specific gait tests and measures for ALS?

no, but documentation within a particular time period over a certain distance is good

is skin integrity usually a problem?

no, not even in late stage

are urinary symptoms a normal impairment of ALS?

no, they are abnormal

with the early and middle stages of ALS do restorative interventions last?

no, they are temporary - work to increase muscle strength and aerobic capacity

is there a cure for ALS?

no; drug trials are ongoing

early stage ALS participation restrictions

none

middle stage ALS activity limitations

noted

late stage ALS participations restrictions

numerous - patient becomes increasingly more functionally limited owing to lack of voluntary motor control - dependent in all aspects of mobility and self-care - may require mechanical ventilation

late stage ALS impairments

numerous and increasingly more severe impairments

patients who have been on the ventilator for long periods of time may develop the inability to voluntarily close their eyes or _______________

ophthalmoplegia

medical management for individuals with ALS may be considered _________

palliative - an approach that improves QOL of patients and their families

what is pseudobulbar affect?

poor emotional control

what does the diagnosis of ALS require?

presence of - LMN signs - UMN signs - progression of disease within a region or to other regions

what is necessary to determine the extent and rate of progression of the disease?

re-examination at regular intervals

restorative intervention

remediating or improving impairments and activity limitations

death from ALS usually results from?

respiratory failure

early stage ALS impairments

resultant impairments

what kind of palsy is pseudo bulbar affect usually caused by?

spastic bulbar palsy

quality of life measures

specific QOL represents five distinct areas of health - mobility - ADLs - eating and drinking - communication - emotional functioning

middle stage ALS participation restrictions

start to develop

age at time of onset has the strongest or weakest relationship to prognosis?

strongest

spastic dysphagia

uncoordinated closure of epiglottis - allows liquids or solids to pass to the larynx

does ALS typically have UMN or LMN s/s

variety of both

spastic dysarthria

voice sounds forced; more effort is needed to move air thru upper airway

flaccid dysarthria

voice sounds hoarse or breathy

what may Dysarthria be caused by?

weakness of: - tongue - lip - jaw - larynx - pharynx

what is key in the PT exam?

what is important, relevant, and valued by the individual

can UMN signs decrease as the disease progresses?

yes

is fatigue common in patients with ALS?

yes

is sensation spared with ALS?

yes

is sialorrhea experienced early in those with bulbar onset?

yes

as motor neurons degenerate, can intact axons sprout and reinnervate partially denervated muscles..at least for awhile?

yes - can compensate until motor unit loss is about 50%

are external ocular muscles usually spared?

yes - if late degeneration occurs, it does so late in the course

are paresthesia or pain in limbs possible?

yes, especially when muscle weakness and spasticity lead to immobility, adhesive capsulitis or contractures

are depression and anxiety common in patients with ALS?

yes, therefore, screening is important and referral to a psychologist may be needed