ALS and Huntingtons

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Presentation of ALS: Early Symptoms
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Presentation of ALS: Early Symptoms
Common Early Symptoms:
•Muscle twitches
•Muscle cramps
•Tight/stiff muscles (spasticity)
•Weakness

•Slurred or nasal speech
•Difficultly swallowing or chewing
-Tripping, clumsy, dropping things
-Emotional lability
ALS Bulbar Onset
Dysarthria
Dysphagia

Dyspnea
Functional limitations
Higher cognitive skills (executive functioning) and dementia (small percentage)
Pain (neuropathic)
ALS: Limb Onset
Muscle weakness (initial onset: commonly unilateral, distal UE or LE)

Muscle tightness

Spasticity

Fasciculations/ muscle twitches

Dyspnea
Functional limitations
Higher cognitive skills (executive functioning) and dementia (small percentage)
Pain (neuropathic)
ALS - Long Term Effects
Long term - these patients will end up on a vent
Maintain their higher cognitive skills à they are fully aware of their body's decline, cognitive symptoms such as anxiety and depression are prevalence
A small percentage of patients may develop dementia over time
Pain à if they do have it, it can be very debilitating and impact the PT session
however, neuropathic pain is not very common
ALS outcome measures
•Outcome Measures:
•ALS-specific Quality of Life-Revised (ALSSQOL-R)
•Numeric Pain Rating Scale (NPRS)

-TUG
-Endurance testing - 6 or 2 MWT (good because we only want gentle aerobic exercise, do not want to exert)
-Tinetti
-mFRT
-w/c push test
ALS W/C management
-As how they complete transfers at home and assess what they are comfortable doing
-Assess wheelchair/ powerchair equipment and setup as well as management
Is ALS LMN or UPM either or, or can it be both?
Flaccid and spastic
Will progress to both, for LMN fasiculations weakness more spastic
ALS PT Role
•Gentle, low-impact aerobic exercises
•ROM and stretching
•Adaptive equipment and assistive devices
•Caregiver training
•Education

-Stationary bike, swimming
-We do have the capacity to strengthen the unaffected muscles
-AFOs, assistive devices, W/C, power chair
-AFOs are good for efficiency
-Strength goals are not appropriate as we are not expecting them to recover from strength deficits
-We work to adapt and modify their mobility
Huntington's disease overview
•Familial, females > males
•Typical onset: age 30-50 years old
•Juvenile HD: 7-10% are diagnosed before third decade of life
•Average prognosis is 17 years from diagnosis
•
•The striatum (caudate nucleus and putamen) is the most affected part of the brain
•Movement
•Mood
•Behavior regulation
Huntington's Disease -"What will goals look like if they are maintenance goals?
"à goals cannot just be maintenance they need to be skilled
-Goal is to maintain what they have and relate it to function
-Write goal about the function they are trying to accomplish, not the strength
-EX: patient will be independent with W/C propulsion
-Use an outcome measures to direct a goal
-Prevention goals: pressure relief, contracture
-Education goals for the caregiver - mobility, assist, pressure relief, etc.
Huntington's disease Key Symptoms-Early on: cognitive impairments, muscle twitches, irritability, feeling fidgety -Chorea - involuntary movement -Impaired coordination -Staging is very loose, not a definitive thing, more of a continuum -Bradykinesia sets in during late stages -Pneumonia and heart failure are common causes of deathHuntington's disease Motor ProgressionPRE: Gait changes EARLY: Mild chorea Dysdiadochokinesia Increased reflexes Abnormal extraocular movements MIDDLE: Chorea, dystonia Rigidity and spasticity Voluntary movement abnormalities Decreased coordination Motor impersistence Balance deficits Dysphagia/dysarthria LATE: Bradykinesia Rigidity and spasticity Severe voluntary movement abnormalities Dysarthria Dysphagia IncontinenceHuntington's Disease CognitivePRE: Difficulty with complex tasks Early: Mild problems with planning, sequencing, organizing, and prioritizing tasks Middle Intellectual decline Memory loss Perceptual problems Lack of insight or self-awareness Difficulty with dual tasking LATE Global dementiaHuntington's disease PsychiatricDepression, aggression, irritability Sadness, depression, irritability Apathy Perseveration Impulsivity Antisocial and suicidal behavior Paranoia Delusions or hallucinations DeliriumHuntington's disease Motor/Physical Symptomsbradykinesia: slowness of movement -akinesia: absence of voluntary movement -Impaired postural control and balance: secondary to impaired motor control -Chorea: starts with finger and hands - progresses to involve all 4 limbs and trunk - progress to face and truncal involvement -Dystonia - abnormal sustained position of a part of the body - twisting of arm with clenched fist, excessive bending of knees -Video: probable stage à early -Increased risk of weight loss secondary to energy expenditure with the constant movementHuntington's disease PT ExaminationPT exam: -Strength - deficits are secondary to immobility and deconditioning -Later in the disease they have strength inpersistance, therefore may break when doing MMTs -ROM -Coordination -Balance -Mobility -GaitHuntington's disease Outcome measures•Berg Balance Scale •Tinetti (harder than berg has gait in it) •TUG •10MWT •Unified Huntington's Disease Rating Scale (UHDRS) •Motor, cognitive, behavioral, and function •Includes standardized ratings of chorea and dystoniaHuntington's disease Cognitive ChangesCognitive Impaired memory Decreased processing Impaired reasoning and judgement PerseverationHuntington's disease Psychological/Emotional ChangesPsychological/Emotional Depression (idiopathic and reactive) Suicidal Psychosis (delusions and hallucinations) Obsessive/compulsiveHuntington's disease PT in the Early Stage•Health and wellness •Aerobic exercise (moderate intensity, 55-90% HR max) •Stretching •Resistance training (3x/week) •Including respiratory muscles •Balance •Core stability •Fall prevention •Encourage fun activities outside of PT -Exercise for more often but for shorter duration in order to prevent excessive fatigue -Add resistance training into exercise program -Exercise outside of PT to help with depressionHuntington's disease PT in the Middle Stage•Greater concern for falls •Balance exercises •Video games (Wii, Dance Dance Revolution) •Gait training •Use of metronome •Treadmill training (safety concerns) •Functional strengthening •Focus attention on maintaining balance before a task (stairs) •Avoid dual tasking •Self-awareness of fatigue factor -Greatest focus is on balance -Closed chain > open chain -More functional strengthening -Better to decrease impact of motor in-persistenceHuntington's disease PT in the Late Stage•Support function and positioning surrounding ADLs •Optimize posture, positioning and seating (comfort) •Environmental modifications •Equipment prescription •Caregiver support •Respite careHuntington's disease Goals•Promote fitness and wellness •Strength •Maintain respiratory function •Assess and prescribe assistive devices and equipment •Gait and balance training •Educate and support caregivers •Prescribe and modify HEPs