Neuro - 7 ALS

1

100

ALS is also known as ... 

Lou Gehrig's disease 

200

- Disease causing mutations (EX: SOD1, alsin)
- Gender: male > female
- Age
- Family history
- Clusters (EX: Western Pacific ALS/PDC 

Known risk factors for ALS 

300

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 

400

Name pathology/system affected: 

Bulbar muscle weakness, dysphagia, dysarthria, sialorrhea, pseudobulbar affect 

Bulbar 

500

Asymmetrical, distal motor weakness

Common initial presentation for limb ALS 

2

100

What is an initial sign of ALS? 

Atrophy of thenar eminence 

200

Name pathology/system affected: 

Spasticity, pathologic reflexes, hyperreflexia, muscle weakness 

UMN pathology 

300

- 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) 

400

- 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 

500

- 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 

3

100

A diagnosis of ALS is most common at which age? 

50's

200

- No specific test
- Patient history, physical exam, neuro exam

Diagnosis 

300

Sensation, cognition, eye movement, autonomic, bowel, bladder and sexual function 

Usually spared in ALS 

400

- Fatigue
- Respiratory status
- Swallowing status
- Cervical spine muscle weakness - collar
- Communication needs 

PT considerations for PT exam in ALS 

500

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 

4

100

- Age less than 35
- Psychological health
- Limb onset
- Rating of functional scale
- SNIP - respiratory test

Favorable prognosis for ALS 

200

- 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 

300

- 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 

400

Name pathology/system affected: 

Respiratory muscle weakness (inspiratory and expiratory), dyspnea, exertional dyspnea, nocturnal respiratory difficulty, orthopnea, hypoventilation, secretion retention, ineffective cough 

Respiratory 

500

- 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 

5

100

- 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 

Requirements to be diagnosed with ALS 

200

Name pathology/system affected:

Muscle weakness, hyporeflexia, hypotonicity, atrophy, muscle cramps, fasciculations 

LMN pathology 

300

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

400

- Maintain patients QOL and their independence in functional mobility and ADLs 

Goal of ALS 

500

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