ALS/HD/MS Flashcards
Area of destruction: Corticobulbar tracts and branstem motor Symptoms: Dysarthria, dysphagia, facial and tongue weakness, nuclei involved and wasting A Primary lateral sclerosis (PLS) B Progressive SPinal Muscular Atrophy
C
Progressive blubar palsy
C
Progressive blubar palsy
Areas of Destruction: Lower motor neurons in the spinal cord Symptoms: Marked muscle wasting of the limbs, trunk, (PMA or PSMA) (LMN form) and sometimes the brainstem and/or the bulbar muscles
A
Primary lateral sclerosis (PLS)
B
Progressive Spinal Muscular Atrophy
C
Progressive blubar palsy
B
Progressive Spinal Muscular Atrophy
Areas of destruction: Destruction of the cortical motor neurons; progressive spastic paraparesis may involve both corticospinal and corticobulbar regions. symptoms: Progressive spastic paraparesis A Primary lateral sclerosis (PLS) B Progressive Spinal Muscular Atrophy
B
Progressive Spinal Muscular Atrophy
.
Destruction of motor neurons within the spinal cord, brainstem, and motor cortex
A ALS B HD C MS
A
ALS
________ ALS makes up about 90 to 95 % of ALS cases and is one of the two primary forms of ALS. Occurs at a random point
A sporadic B familial C genetic
A
sporadic
2nd primary form of ALS, 5 to 10% of individuals get this due to genetic history
A
sporadic
B
familial
C
genetic
B
familial
focal weakness in arms, legs, or bulbar mm's trip or drop things slurred speech abnormal fatigue uncontrolable laughing or crying A late symptoms B progressive symptoms
C
early symptoms
C
early symptoms
Marked muscle atrophy Weight loss Spasticity Muscle cramping Fasciculation Difficulty walking, dressing, fine motor, swallowing, breathing
A
late symptoms
B
progressive symptoms
C
early symptoms
B
progressive symptoms
weakness or muscle atrophy of extremities, cervical extensor weakness, fasciculation, muscle cramps, and loss of reflexes
A
UMN damage
B
LMN damage
C
damage to bulbar nerve
B
LMN damage
speech, swallowing, breathing may be affected A UMN damage B LMN damage
C
damage to bulbar nerve
C
damage to bulbar nerve
\_\_\_\_\_\_\_\_ invovlement usually indicates a poorer prognosis A UMN B LMN
C
bulbar
C
bulbar
younger age at onset onset involving LMN’s located in the spinal cord deficits in either UMN or LMON not a combination of both areas absent or slow changes in respiratory function fewer fasciculations longer time from onset of symptoms to diagnosis
A
postitive/faster progression
B postiive/slower progression C negative/faster progression D negative/slower progression
B
postiive/slower progression
what is typical death after onset of ALS A 1-3 years B 1-2 years`
C
2-5 years
D
6-8 years
C
2-5 years
stage 1 of ALS mild weakness clumsiness ambulatory independent with ADL’s what intervention would you use with focus on client factors?
A
stretch to avoid contractures
B
begin ROM program and add strengthening program of gentle resistance
C
keep patient physically independent as long as possible through pleasurable activities and walking
D
provide compensatory equipment for w/c such as trough or lap tray
B
begin ROM program and add strengthening program of gentle resistance
symptoms: Moderate, selective weakness Slightly decreased independence in ADLs; for example, difficulty climbing stairs, difficulty raising arms, difficulty buttoning clothing Interventions With Focus on Performance Areas of Occupation: Assess self-care, work, and leisure skills impaired by loss of function; if patient continues to work, focus on how to adapt tasks with current deficits; assist with balance between work, home, and leisure activities; include significant others in treatment. Use adaptive equipment to facilitate ADLs (e.g., button hook, reacher, built-up utensils, shower seat, grab bar). Integrate hand orthotic use into daily activities. Perform baseline dysphagia evaluation; reevaluate throughout each stage of the disease. Interventions With Focus on Client Factors: Continue stretching to avoid contractures. Continue cautious strengthening of muscles with MMT grades above F+ (3+). Monitor for overwork fatigue. Consider orthotic support (e.g., AFOs, wrist or thumb splints—short opponens splint). what stage is this of ALS A Stage III B Stage IV
C
Stage II
D
Stage V
C
Stage II
symptoms: Severe, selective weakness in ankles, wrists, and hands Moderately decreased independence in ADLs Tendency to become easily fatigued with long-distance ambulation Slightly increased respiratory effort Interventions With Focus on Performance Areas of Occupation: Prescribe manual or power wheelchair with modifications to eventually allow recline or tilt posture with headrest, elevating leg rests, adequate trunk and arm support. Help patient prioritize activities and provide work simplification. Reassess for adaptive equipment needs (universal cuff to eat). Assess and adapt use of communication devices (e.g., regular phone to cordless or speaker phone; pen and paper to computer with adapted typing aid). Provide support if there is loss of employment or other activities; explore alternative activities. Begin discussing need for home modification, such as installing ramps or moving the bedroom to the lowest floor. Provide education regarding the types of bathroom equipment available for energy conservation and safety. Interventions With Focus on Client Factors: Keep patient physically independent as long as possible through pleasurable activities and walking. Encourage deep breathing exercises, chest stretching, and postural draining if needed.
A Stage III B Stage IV C Stage II D Stage V
A
Stage III
Symptoms: Severe lower extremity weakness Moderate to severe upper extremity weakness Wheelchair dependent Increasingly dependent in ADLs At risk for skin breakdown caused by poor mobility Interventions With Focus on Performance Areas of Occupation: Instruct family in methods to assist patient with self-care, especially bathing, dressing, and toileting; aim to minimize caregiver’s burden and stress. Family training to learn proper transfer, positioning principles, and turning techniques. Instruct in use of mechanical lift if needed for transfers out of bed (patients in slings require head support). Adapt and select essential control devices for telephone, stereo, television, electric hospital bed controls for independent use. Adapt wheelchair for respiratory unit if needed to allow for continued community access. Interventions With Focus on Client Factors: Instruct family and patient in skin inspection techniques. Instruct in use of electric hospital bed and antipressure device. Adapt wheelchair for respiratory unit if needed; reassess adequacy of wheelchair cushion for pressure relief. A Stage III B Stage IV C Stage II
D
Stage V
D
Stage V
symptoms: Hanging-arm syndrome with shoulder pain and sometimes edema in the hand Wheelchair dependent Severe lower extremity weakness (with or without spasticity) Able to perform some ADLs, but fatigues easily Interventions With Focus on Performance Areas of Occupation: Evaluate need for arm slings, overhead slings, mobile arm supports for eating, typing, page turning. Prescribe power wheelchair if the patient wants to be independent with mobility; controls must be adaptable from hand to other mode of control. Evaluate need for assistive technology such as environmental control systems, voice-activated computer; augmentative communication device. Help the patient prioritize activities, and consider negotiating roles with significant others. Reinforce the need for home modifications. Reinforce the need for shower seat or transfer tub bench and shower hose. Assist with patient’s ability to participate in closure activities, such as writing letters or making tapes for children, completing a life history, and writing a log on household management for family. Interventions With Focus on Client Factors: If arm supports are not used, provide arm troughs or wheelchair lap tray for wheelchair positioning; wrist cock-up splints for full resting; hand splints may be needed for positioning. Provide pain and spasm management through the following: Heat, massage as indicated to control spasm and pain Anti-edema measures Active assisted or passive range-of-motion exercises to the weak joints; caution to support and rotate shoulder during abduction and joint accessory motions Isometric contractions of all musculature to tolerance
A
Stage III
B Stage IV C Stage V D Stage VI
B
Stage IV
