Unit 8: ALS Flashcards
What is ALS?
-aka Lou Gehrigs disease
-Late-onset FATAL neurodegenerative disease of UMN and LMN
-Found more often in men than women
-Average age of onset is 58 (has been diagnosed as early at 20 yrs old)
-Voluntary muscle control is affected, and early manifestations indicating UMN or LMN disease vary with site of initial disease process
UMN involvement results in:
-Weakness
-Spasticity
-Hyperreflexia
LMN involvement results in:
-Weakness or muscle atrophy of extremities
-Cervical extensor weakness
-Fasciculations
-Muscle Cramps
-Loss of reflexes
*Typically, eye function. bowel and bladder control, and sensation are not affected
How common is ALS?
-6,000 people in US are diagnosed annually (15/d)
-Approximately 20,000 Americans have ALS at any given time
-60% are male
-93% are Caucasian
-Military veterans (particularly those deployed during gold war) are 2x as likely to develop ALS
How common is ALS?
-6,000 people in US are diagnosed annually (15/d)
-Approximately 20,000 Americans have ALS at any given time
-60% are male
-93% are Caucasian
-Military veterans (particularly those deployed during gold war) are 2x as likely to develop ALS
How is ALS Diagnosed?
-No special test is available to establish the diagnosis, and the cause is unknown.
-Initial symptoms vary widely, and diagnosis is a careful, multistep system of exclusion.
A comprehensive diagnostic workup includes most, if not all, of the following procedures:
-Electrodiagnostic tests including electomyography (EMG) and nerve conduction velocity (NCV)
-Blood and urine studies including high resolution serum protein electrophoresis, thyroid and parathyroid hormone levels and 24-hour urine collection for heavy metals
-Spinal tap
- X-rays, including magnetic resonance imaging (MRI)
-Myelogram of cervical spine
-Muscle and/or nerve biopsy
-A thorough neurological examination
Types of ALS
-Sporadic: Most common in US (90 – 95% of all cases)
-Familia: Small number of cases in US (5–10% of all cases)
-Guamaniam: High incidence was observed in the 1950’s
Progression of ALS:
-The mean survival time with ALS is 3 – 5 years.
-Many live to 5, 10 or more years
Stages of ALS
-Stage I: Ambulatory, no problems with ADL, mild weakness
-Stage II: Ambulatory, moderate weakness in certain
muscles
-Stage III: Ambulatory, severe weakness in certain muscles
-Stage IV: Wheelchair confined, almost independent, severe weakness in legs
-Stage V: Wheelchair confined, dependent; pronounced weakness in legs, severe weakness in arms
-Stage VI: Bedridden, no ADL, maximal assistance required
Evaluations with ALS
- Evaluation should be geared toward clearly defined levels of functional stage of ALS progression.
-Measures specifically useful for ALS: ALS Functional Rating Scale; Purdue Pegboard or other timed upper extremity function test and standard ROM/MMT; Multidimensional Fatigue Inventory
-Re-evaluations should be done at repeated visits due to the progressive nature of the disease.
Goal setting during early stages (Intervention with ALS)
-Optimize strength and ROM using home exercise programs.
-Maintain function in ADL and IADL through use of assistive or adaptive devices.
-Decrease pain and fatigue through use of splints and orthotics.
-Employ joint protection, pain management, energy conservation, and work simplification techniques.
Goal setting during late stages (Intervention with ALS)
-Focus on enabling the caregiver to assist the client safely and effectively.
-Optimize safety, assess positioning, perform safe transfers, and maintain skin integrity.
-Employ AAC equipment.
-Assess and manage dysphagia.
-Optimize social participation.
-Identify and obtain equipment to allow continued mobility.
-Environmental modification
Treatment Options for ALS
-Therapists must be aware of client’s level of tolerance for gadgets, financial resources, and social and cultural context.
- Special treatment considerations: Exercise, Equipment, Assistive technology, Dysphagia management
Rilizole
Antiglutamate agent
-Only medication FDA approved to alter the course of the disease
To assist in evaluation which assessment should be used?
ALS Functional Rating Scale
Because of ALS progression how often is reevaluation
At each visit
