Disorders of the PNS & CNS

Question 1

Amyotrophic Lateral Sclerosis (ALS)

description, prognosis, symptoms

Answer 1

1. 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
2. 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

Question 2

Evaluation for ALS

Answer 2

1. goals/piorities should focus on minimizing effects of symptoms on occupational performance
2. as disease progress, interventions should expand on physical and social environment
3. Assessment/screening tools
   - COPM
   - ASL Functional Rating Scale
   - Purdue Pegboard Test
   - Multidemensional Fatigue Inventory
   - Dysphagia screening and testing

Question 3

Intervention for ALS

Answer 3

1. use compensatory approach (focus on adapting to disability & preventing secondary complications
2. Home eval_safety (positioning, transfers, and skin integrity
3. AT, AD, AE, environmental modifications
4. Assessment and management of dysphagia
5. Social participation
6. Exercise/breathing programs/montior fatigue/overexertion/muscle spasm
7. fall prevention

Question 4

AD/AT for ASL

Answer 4

1. neck collar/universal cuff (UE stability)
2. 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
3. W/C (high back/recline/lightweight, turns in small space, and head/trunk/extremty support); power W/C with adaptive control (tilt/recline)
4. voice op/hands-free tech
5. adapti food consistency; swallowing tech; nutrition

compensatory approaches

Question 5

Intervention:
Initial Stage of
ALS

Answer 5

1. Focus on environmental support (DME, communication)
2. Modify home, AE
3. Exercise, including PROM & light resistive (improves function and reduces spasticity)
4. Be cautious with strengthening of muscles with MMT grades above F (+3). Monitor for overwork fatigue
5. Consider orthotic supports (AFOs, wrist/thumb splints-short opponens splint)

Family should be updated regularly d/t progression of disease

Question 6

Intervention:
Mid-late stage of
ASL

Answer 6

1. Daily ROM to prevent contractures and reduce spasticity
2. Educate on disease progression & choices
3. Caregiver training
4. Adaptation with ADLs, work, leisure w/ assistance
5. compensation
6. provide supportive role for psychosocial (memory book)
7. Swallow screening

Question 7

Intervention:
Late stage of
ALS

Answer 7

1. P W/C, vent, feeding tube
2. MOBILITY: Social w/d linked to anxiety and depression, not because of level of disability (P W/C, public transportation can prevent that)
3. Hospice

Question 8

Erb’s palsy

decription, symptoms

Answer 8

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

Question 9

Klumpke’s palsy

decription, symptoms

Answer 9

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

Question 10

Guillain-Barre Syndrome

Answer 10

1. inflammatory disease that causes demyellination of axons in peripheral n.
2. Maybe from previous viral infections, enteritis, repiratory tract infections, and HIV/AIDS
3. 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.

Question 11

The 3 Phases of
Guillain-Barre Syndrome

Answer 11

1. 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.
2. Plateau phase : symptoms at most disabiling with little/no change over few days/wks.
3. 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)

Question 12

S&S for
Guillain-Barre Syndrome

Answer 12

1. Pain (mostly in LE)
2. Fatigue
3. Absence of deep tendon reflexes
4. mild sensory loss in the hands and legs (glove-and-stocking distribution); hypersensitivity reported as sensation recovery
5. CN dysfunction/facial palsy
6. hypertension, arrhythmias, facial flushing, diarrhea, impotence, urinary retention, and inc. sweating
7. B&B
8. cog dysfunction

respriatory, speech dys.

Question 13

Evaluation for
Guillain-Barre Syndrome

Answer 13

1. OT referral occurs when GBS is mod-severe, typically 40%
2. Plateau phase: screen/eval in ICU; assessment includ communcation, control of physcial envir. comfort/positioning, and anxiety management
3. Recovery phase: eval focus on mobility, self care, ADL, communcation, leisure, and workplae and community reintegration (inpatient/outpatient/home/work)

Question 14

Intervention for
Guillain-Barre Syndrome

For each stages, splint

Answer 14

1. 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)
2. 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

Question 15

Huntington’s disease

Answer 15

1. a hereditary neurological disorder that leads to severe physical and mental disabilities. progression: loss of brain cells, affecting movement, cog, emotion, and behavior.
2. 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
3. late stage: unable to talk, walk, or perform ADLs w/o sig A.

FATAL PROGRESSIVE, CHROM 4

Involves both voluntary and involuntary movements

Question 16

S&S for
Huntington’s disease

inc. stages

Answer 16

1. involuntary motor (*Chorea), akathisia/motor restlessness, dystonia)-contributes to weight loss
2. voluntary motor (bradykinesia, akinesia (delayed movement/basal gang, incoordination of movements)
3. Cog/behavior (forgetful, concentration, calc, sequen, memory)
   * initial stage: dif. maintaining work perform.
   * middle: diff. memory and decision
   * later: compromised pronunciation because of dysarthria
4. slowing of saccadic eye/ocular pursuits
5. dysphagia

RHYTMIC MOVEMENTS/dancing big movements

Significant deterioration of cognitive and behavioral abilities

Question 17

Evaluation for
Huntington’s Disease

Answer 17

1. 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

Question 18

Intervention for
Huntington’s Disease

Answer 18

1. 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**
2. 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
3. Late stage: positioning, splinting, communication devices (dysarthira), cont. routines/schedules, facilitate smooth transition to tube feedings, environ. controls

avoid open ended questions

Question 19

Myasthenia Gravis (MG)

Answer 19

1. autoimmune attack on acetylcholine receptors needed for voluntary movementsat the nerve-muscle junction
2. progressively disabling
3. death occur from respiratory complications
4. rare/difficult to diagnosis
5. characterized by episodic m. weakness innervated by CN

GOOD PROGNOSIS

Question 20

Symptoms of
Myasthenia Gravis

Answer 20

1. Common symptoms: ptosis (eye droop), diplopia, muscle fatigue after exercise, dysarthria, dysphagia, and proximal limb weakness
2. Sensation and deep tendon reflexes are intact
   3.Symptoms fluctuate over the course of the day
3. Relapse period: quadriparesis may develop
4. 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”

Question 21

Treatment for
Myasthenia Gravis (MG)

Answer 21

1. AE/EC, avoid muscle exertion/fatigue
2. Assessment for eating and swallowing
3. Vision disc-test periphreal
4. Cargiver goals
5. Ability to tolerate act., strength,pacing, rest/sleep
6. home safety d/t vision maybe impaired
7. Support group; stress reduction

STRONGER IN THE MORNING; AVOID FATIGUE

Question 22

Multiple Sclerosis (MS)

Answer 22

1. Demeylination of CNS (brain and spinal cord)
2. Occurs mostly between age 20-50; diagnoised at 30.
3. 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

Question 23

4 category
of MS

Answer 23

1. clinically isolated syndrome (CIS)- a 1st episode of neurological symptoms (optic, vertigo, numbface, weakness, bladder issues); Not everyone with CIS develops MS.
2. relapsing-remitting (RRMS)- clearly defined episode of neuro. sym. followed by remission; disability not always inc. after each episode
3. Secondary progressive(SPMS)- ppl w/RRMS, neuro function declines overtime, disability increases
4. Primary progressive(PPMS)-** disability begins to inc. w/ 1st epi. of neuro symptoms**
5. Progressive-relapse (PRMS)-steadily worsens from the onset, but symptom flare-ups – with or without remissions – are also present

basis of disease progression

Question 24

Symptoms of MS

Answer 24

1. multiple and varied neurologic symptoms and signs, usually with remissions and exacerbations
2. onset of symptoms is usually insidious
3. paresthesias in 1+ extremities, on trunk, or in the face
4. weakness or clumbsiness in the leg or hand is common
5. visual disturbances (diplopia, partial blindness, nystagmus, eye pain, etc.)
6. Emotional disturbances (lability, euphoria, and reactive depression)
7. Balance loss and/or vertigo
8. Bladder dysfunction/(catherization)
9. Cog. (apthy, memory loss, lack of judgment, inattention)
10. sensorimotor, spasticity, inc. reflexes, ataxia, weakness, gait instability

Question 25

General Intervention for
Neurological System Disorders

Answer 25

1. positioning/pressure relief, w/c mobility, bed
2. postural control
3. motor learning/control retraining/relearning for functional integration of affected limbs
4. ADL retraining, adaption
5. AD/AT/EC-fatigue
6. splinting (tendodesis splint)
7. edu/sex/community/coping
8. vision/cog-percep compensation
9. skin care/b&b

COMBINE REMEDIATION/REHAB: NONPROGRESSIVE

COMPENSATORY: FOR PROGRESSIVE

Question 26

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

Answer 26

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.

Question 27

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

Answer 27

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.