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
