Disorders of the PNS & CNS Flashcards
Amyotrophic Lateral Sclerosis (ALS)
description, prognosis, symptoms
- progressive, degenerative disease in which the motor neurons in the brain, spinal cord, and peripheral system are destroyed and replaced by scar tissues (corticospinal tracts and anterior horn cells/ bulbar efferent neurons
- Onset: age 57; death usually occurs in 2-5 yrs
Symptoms:
1. muscle weakness/atrophy (begins distally(hand) and asymetrically)
2. cramps
3. spasticity
4. hyperactive deep tendon reflexes
5. Dsyarthria/dysphagia
6. sensory/eye movement urinary sphincters spared
fatal progressive, AKA Lou Gehrig’s disease
Evaluation for ALS
- goals/piorities should focus on minimizing effects of symptoms on occupational performance
- as disease progress, interventions should expand on physical and social environment
- Assessment/screening tools
- COPM
- ASL Functional Rating Scale
- Purdue Pegboard Test
- Multidemensional Fatigue Inventory
- Dysphagia screening and testing
Intervention for ALS
- use compensatory approach (focus on adapting to disability & preventing secondary complications
- Home eval_safety (positioning, transfers, and skin integrity
- AT, AD, AE, environmental modifications
- Assessment and management of dysphagia
- Social participation
- Exercise/breathing programs/montior fatigue/overexertion/muscle spasm
- fall prevention
AD/AT for ASL
- neck collar/universal cuff (UE stability)
- foot-drop splint, cane, walker (mobility aids/exertion); ankle-foot orthosis (from PT); short opponens splint to improve key pinch; compensatory strategies for sit to stand; button hook
- W/C (high back/recline/lightweight, turns in small space, and head/trunk/extremty support); power W/C with adaptive control (tilt/recline)
- voice op/hands-free tech
- adapti food consistency; swallowing tech; nutrition
compensatory approaches
Intervention:
Initial Stage of
ALS
- Focus on environmental support (DME, communication)
- Modify home, AE
- Exercise, including PROM & light resistive (improves function and reduces spasticity)
- Be cautious with strengthening of muscles with MMT grades above F (+3). Monitor for overwork fatigue
- Consider orthotic supports (AFOs, wrist/thumb splints-short opponens splint)
Family should be updated regularly d/t progression of disease
Intervention:
Mid-late stage of
ASL
- Daily ROM to prevent contractures and reduce spasticity
- Educate on disease progression & choices
- Caregiver training
- Adaptation with ADLs, work, leisure w/ assistance
- compensation
- provide supportive role for psychosocial (memory book)
- Swallow screening
Intervention:
Late stage of
ALS
- P W/C, vent, feeding tube
- MOBILITY: Social w/d linked to anxiety and depression, not because of level of disability (P W/C, public transportation can prevent that)
- Hospice
Erb’s palsy
decription, symptoms
a paralysis of upper brachial plexus (CN 5 & CN6, maybe CN7)
symptoms:
1. paralyzed (supraspinatus/infra, deltoids, biceps, brachialis, subscauplaris)
2. arms cannot be raised; elbow flexion/retraction/protraction are weak
waiter’s tip position
Klumpke’s palsy
decription, symptoms
a paralysis of the lower brachial plexus (CN 7 & CN8); rare
symptoms:
1. paralysis of the hand and wrist, often with ipsilateral Horner’s syndrome (miosis, ptosis, and facial anhidrosis)
2. Limp hands; fingers do not move
Guillain-Barre Syndrome
- inflammatory disease that causes demyellination of axons in peripheral n.
- Maybe from previous viral infections, enteritis, repiratory tract infections, and HIV/AIDS
- 1/3 of ppl dev. symptoms do so days/wks after diarrhea or a respiratory illness (Campylobacter jejuni bacteria-most common)
no cure, ACUTE, 80% walk w/i 6 mo.
The 3 Phases of
Guillain-Barre Syndrome
- RAPID ONSET acute inflammatory phase: weakness occurs in atleast 2 extremeties advances and reacheres its maximum in 2-4 wks; 20% to 30% of ppl need vent.
- Plateau phase : symptoms at most disabiling with little/no change over few days/wks.
- Progressive recoveryinflammatory phase: remyelination and axonal regeneration occur over a period from 6 months to 2 yrs.
-Recovery starts at head/neck and travel distally. most recover sign/all function; residual: fatigue
-Rapid progression: typically symmetrical ascending pattern of flaccid paralysis that begins in the feet; paralysis may occur in respiratory m.
- Etiology unk. may occur after infectious disorder, surg, immunization
- Onset recovery: 2-4 wks after first symptom
Segmental demyleination (with axonal degen in severe cases)
S&S for
Guillain-Barre Syndrome
- Pain (mostly in LE)
- Fatigue
- Absence of deep tendon reflexes
- mild sensory loss in the hands and legs (glove-and-stocking distribution); hypersensitivity reported as sensation recovery
- CN dysfunction/facial palsy
- hypertension, arrhythmias, facial flushing, diarrhea, impotence, urinary retention, and inc. sweating
- B&B
- cog dysfunction
respriatory, speech dys.
Evaluation for
Guillain-Barre Syndrome
- OT referral occurs when GBS is mod-severe, typically 40%
- Plateau phase: screen/eval in ICU; assessment includ communcation, control of physcial envir. comfort/positioning, and anxiety management
- Recovery phase: eval focus on mobility, self care, ADL, communcation, leisure, and workplae and community reintegration (inpatient/outpatient/home/work)
Intervention for
Guillain-Barre Syndrome
For each stages, splint
- Plateau: modify activities for temporary (signs, pic. board, environmental modifications (call button/remote/phone), hands-free adaption, positioning/stability of UE, caregiver edu, anxiety reduction; arm support; head/mouth switch)
- Recovery: dynamic splints (maintain ROM/esp wrist/fingers/ankle): hinged drop-foot orthosis; AD, enviro. adapt/mod; EC, fatigue, FM program, home assessment/mod.
AVOID FATIGUE and PROTECT JOINTS
Progressive resistive exercises should be used CONSERVATIVELY
Educate on diagnosis/phases
Huntington’s disease
- a hereditary neurological disorder that leads to severe physical and mental disabilities. progression: loss of brain cells, affecting movement, cog, emotion, and behavior.
- progressive of loss tissue in frontal cortex, globes pallidus, and thalamus; degeneration of the corpus striatum result in decrease GABA. symptoms progress over a 15-20 yr period
- late stage: unable to talk, walk, or perform ADLs w/o sig A.
FATAL PROGRESSIVE, CHROM 4
Involves both voluntary and involuntary movements
S&S for
Huntington’s disease
inc. stages
- involuntary motor (*Chorea), akathisia/motor restlessness, dystonia)-contributes to weight loss
- voluntary motor (bradykinesia, akinesia (delayed movement/basal gang, incoordination of movements)
-
Cog/behavior (forgetful, concentration, calc, sequen, memory)
* initial stage: dif. maintaining work perform.
* middle: diff. memory and decision
* later: compromised pronunciation because of dysarthria - slowing of saccadic eye/ocular pursuits
- dysphagia
RHYTMIC MOVEMENTS/dancing big movements
Significant deterioration of cognitive and behavioral abilities
Evaluation for
Huntington’s Disease
-
Unified Huntington’s Disease Rating Scale(assesses changes in areas of motor/cog. function, and functional capacity and behavioral abnormalities
2.Eval. Functional ADLs, cog abilities (prob-solv), motor performance, strength, personal interests, and values
Intervention for
Huntington’s Disease
- Early stage: cogn, FM(build-up handles, unbreakable dishes, grabbars), occulomotor control (no driving), depression, work/home eval., community mobility, daily checklist, sequencing, min. effect of chorea/FM incoordin. HEP, reminders to turn off appliances (kitchen timer);** improve safety in kitchen, BR/BA, living room**
- Middle stage: engagement of purposeful activity, eval/recommend AD (walker, W/C, dressing aid), activity tolerance/manage fatigue, positioning (during feeding), roles-finance/drive/job/loss strat, simple written cues, depression/suicide support, adapting in hobbies d/t gait and bal. deficits, maintaining body weight d/t excessive movement
- Late stage: positioning, splinting, communication devices (dysarthira), cont. routines/schedules, facilitate smooth transition to tube feedings, environ. controls
avoid open ended questions
Myasthenia Gravis (MG)
- autoimmune attack on acetylcholine receptors needed for voluntary movementsat the nerve-muscle junction
- progressively disabling
- death occur from respiratory complications
- rare/difficult to diagnosis
- characterized by episodic m. weakness innervated by CN
GOOD PROGNOSIS
Symptoms of
Myasthenia Gravis
- Common symptoms: ptosis (eye droop), diplopia, muscle fatigue after exercise, dysarthria, dysphagia, and proximal limb weakness
- Sensation and deep tendon reflexes are intact
3.Symptoms fluctuate over the course of the day - Relapse period: quadriparesis may develop
- Life treatening respiratory muscle involvement may occur
6.Good prognosis; most improve wks to months after plasma exchange;
M. affected
Oculomotor m
Oropharngeal m
(Weakness of limbs NOT initial symptom)
MAX WEAKNESS: THE 1ST YR AFTER DIAGNOSIS
“Grave muscle weakness”
Treatment for
Myasthenia Gravis (MG)
- AE/EC, avoid muscle exertion/fatigue
- Assessment for eating and swallowing
- Vision disc-test periphreal
- Cargiver goals
- Ability to tolerate act., strength,pacing, rest/sleep
- home safety d/t vision maybe impaired
- Support group; stress reduction
STRONGER IN THE MORNING; AVOID FATIGUE
Multiple Sclerosis (MS)
- Demeylination of CNS (brain and spinal cord)
- Occurs mostly between age 20-50; diagnoised at 30.
- Diagnosis criteria: multi CNS lesions and at least 2 episodes of neurological disturbance in an individual between 10 and 59 yrs.
slowly progressive, prognosis variable with unpredicatble course
not fatal, symptoms unpredictable
4 category
of MS
- clinically isolated syndrome (CIS)- a 1st episode of neurological symptoms (optic, vertigo, numbface, weakness, bladder issues); Not everyone with CIS develops MS.
- relapsing-remitting (RRMS)- clearly defined episode of neuro. sym. followed by remission; disability not always inc. after each episode
- Secondary progressive(SPMS)- ppl w/RRMS, neuro function declines overtime, disability increases
- Primary progressive(PPMS)-** disability begins to inc. w/ 1st epi. of neuro symptoms**
- Progressive-relapse (PRMS)-steadily worsens from the onset, but symptom flare-ups – with or without remissions – are also present
basis of disease progression
Symptoms of MS
- multiple and varied neurologic symptoms and signs, usually with remissions and exacerbations
- onset of symptoms is usually insidious
- paresthesias in 1+ extremities, on trunk, or in the face
- weakness or clumbsiness in the leg or hand is common
- visual disturbances (diplopia, partial blindness, nystagmus, eye pain, etc.)
- Emotional disturbances (lability, euphoria, and reactive depression)
- Balance loss and/or vertigo
- Bladder dysfunction/(catherization)
- Cog. (apthy, memory loss, lack of judgment, inattention)
- sensorimotor, spasticity, inc. reflexes, ataxia, weakness, gait instability
General Intervention for
Neurological System Disorders
- positioning/pressure relief, w/c mobility, bed
- postural control
- motor learning/control retraining/relearning for functional integration of affected limbs
- ADL retraining, adaption
- AD/AT/EC-fatigue
- splinting (tendodesis splint)
- edu/sex/community/coping
- vision/cog-percep compensation
- skin care/b&b
COMBINE REMEDIATION/REHAB: NONPROGRESSIVE
COMPENSATORY: FOR PROGRESSIVE
An OTR® is performing a home evaluation for a client with Stage III amyotrophic lateral sclerosis (ALS). On what will the OTR’s recommendations MOST LIKELY focus?
a] Modifications to keep the client’s lifestyle as close as possible to occupations preferred before diagnosis
b] Environmental adaptations, such as moving frequently used items to easy-to-reach and nearby areas
c] Creation of a first-floor setup and increased accessibility to a wheelchair or durable medical equipment
d] Technology changes, such as a motorized stair lift
Solution : The correct answer is C.
Someone with Stage III ALS is likely to have severe weakness in the lower extremities, and ambulation will be increasingly difficult once a client reaches this stage. Working with a client and his or her family to create a safe, accessible first-floor setup is the best option. The client and family will need recommendations for creating spaces that a wheelchair or power chair can navigate and the most appropriate durable medical equipment, such as a hospital bed and a specialized mattress to prevent pressure sores.
A: ALS is progressive, and the client will need to change his or her lifestyle to adapt to increasing disability.
B: Environmental adaptations may be appropriate for the earlier stages of ALS, when a client is more mobile.
D: A stair lift would not be the best option for a client at this stage, because a family member would be required to carry the wheelchair to the opposite end of the stairs.
An OTR® conducting an analysis of occupational performance of a client with multiple sclerosis needs to choose an assessment that allows consideration of the client’s self-perception of abilities. Which assessment would BEST be able to capture this information?
Mini-Mental State Examination
Assessment of Motor and Process Skills
Modified Fatigue Impact Scale
Canadian Occupational Performance Measure
Solution: The correct answer is D.
The Canadian Occupational Performance Measure is a client-centered measure that allows clients to report their own perceptions of their self-care, leisure, and work or play abilities.
A: The Mini-Mental State Examination provides a summative measure of cognitive skills, but it does not assess a client’s self-perception of ADL abilities.
B: The Assessment of Motor and Process Skills allows for observation and measurement of a client’s motor and process skills related to occupational performance of selected ADL or IADL areas, but it does not assess a client’s self-perception of ADL abilities.
C: The Modified Fatigue Impact Scale measures the impact of fatigue on daily activities, but it does not assess a client’s self-perception of ADL abilities.
