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ALS is also known as ...
Lou Gehrig's disease
What is an initial sign of ALS?
Atrophy of thenar eminence
A diagnosis of ALS is most common at which age?
50's
- Age less than 35
- Psychological health
- Limb onset
- Rating of functional scale
- SNIP - respiratory test
Favorable prognosis for ALS
- LMN signs by clinical, electrophysiological, or neuropathological examination
- UMN signs by clinical observation ( + Babinski found in 30-50% of patients)
- Progression of the disease within a body region or to another body region
- Absence of electrophysiological and pathological evidence of other diseases affecting UMN and LMN
- Disease causing mutations (EX: SOD1, alsin)
- Gender: male > female
- Age
- Family history
- Clusters (EX: Western Pacific ALS/PDC
Known risk factors for ALS
Name pathology/system affected:
Spasticity, pathologic reflexes, hyperreflexia, muscle weakness
UMN pathology
- No specific test
- Patient history, physical exam, neuro exam
Diagnosis
- From onset to death: between 27 and 43 months
- In most patients, death occurs within 3-5 years after symptoms onset - usually results from respiratory failure
- About 10% of patients have a much slower disease progression, living 10 years or longer
Disease course
Name pathology/system affected:
Muscle weakness, hyporeflexia, hypotonicity, atrophy, muscle cramps, fasciculations
LMN pathology
Name pathology/system affected:
Rare impairment sensory impairments, bowel and bladder dysfunction, ocular palsy
Indirect and composite impairments: fatigue, weight loss, cachexia, decreased ROM, tendon shortening, joint contracture, joint subluxation, adhesive capsulitis, pain, balance and postural control impairments, gait disturbances, deconditioning, depression, anxiety
Other
- Affects motor tracts (UMN) and/or motor neurons (LMN) - brain, brainstem, spinal cord, cranial nerves are affected
- Onset limb or bulbar -- rapidly progressive
- Degeneration of: corticospinal tracts, neurons in motor cortex and brainstem, anterior horn cells in spinal cord
Amyotrophic lateral sclerosis (ALS)
Sensation, cognition, eye movement, autonomic, bowel, bladder and sexual function
Usually spared in ALS
- Neurotoxicant exposures (EX: lead, mercury, pesticide exposure, solvent exposure)
- Vigorous physical activity (EX: heavy manual labor, athleticism)
- Trauma (EX: skeletal trauma, fractures, severe electrical shock with unconsciousness)
- Diet (EX: high fat intake, high glutamate intake, low fiber intake, low antioxidant intake)
- Certain occupation characteristics (EX: electrical workers, farmers, industrial occupations)
- Lifestyle factors (EX: cigarette smoking, alcohol intake, anthropometric measures)
Possible risk factors for ALS
Name pathology/system affected:
Frontotemporal dementia-related impairments (EX: loss of insight, emotional blunting), cognitive impairments (EX: attention deficits, deficits in cognitive flexibility), behavioral impairments (EX: irritability, social disinhibition)
ALS - FTD, ALSci, ALSbi
Name pathology/system affected:
Bulbar muscle weakness, dysphagia, dysarthria, sialorrhea, pseudobulbar affect
Bulbar
- No cure for ALS
- Riluzole (glutamate inhibitor) FDA approved for treatment of ALS + vit E
- Extends survival for 2-3 months
- Nuedexta was FDA approved in 2010 to treat pseudobulbar affects
Medications for ALS
- Fatigue
- Respiratory status
- Swallowing status
- Cervical spine muscle weakness - collar
- Communication needs
PT considerations for PT exam in ALS
Name pathology/system affected:
Respiratory muscle weakness (inspiratory and expiratory), dyspnea, exertional dyspnea, nocturnal respiratory difficulty, orthopnea, hypoventilation, secretion retention, ineffective cough
Respiratory
- Maintain patients QOL and their independence in functional mobility and ADLs
Goal of ALS
Asymmetrical, distal motor weakness
Common initial presentation for limb ALS
- Adaptive equipment
- Orthotics
- Airway clearance techniques
- Breathing techniques
- Re-evaluate types of interventions every few months
- Energy conservation
- Psych management for depression
- Nutritional support
PT interventions - considerations for ALS
ALS functional rating scale (ALSFRS) and the revised version ALSFRS-R
- Self reported / clinician interview scale rating function
- 4 (normal function) to 0 (unable to attempt the task)
- Revised version adds respiratory status
Functional outcome measure for ALS
- Dependent on the pattern and severity of the disease
- Multidisciplinary
- Multisystem by every discipline
- AVOID OVERUSE FATIGUE (specifics depend on the person, but in general a weak/denervated muscle is more susceptible to damage)
PT interventions
ALS assessment questionnaire (ALSAQ-40)
- Self reported/clinician interview scale measuring QOL
- Original scale: 40 items that represent 5 distinct areas of health: mobility, ADL, eating and drinking, communication, and emotional functioning
- Now shortened to 11 items
QOL outcome measure for ALS