ALS

ALS pathophysiology

characterized by a progressive degeneration and loss of motor neurons in the spinal cord, brain stem and motor cortex resulting in variety of UMN and LMN signs and symptoms

what UMN are affected

corticospinal tract

what CN are affected

trigeminal (CN V)

facial (CN VII)

glossopharyngeal (CN IX)

vagus (CN X)

hypoglossal (CN XII)

anterior horn cells in spinal cord

what CN are spared

occulomotor (CN III)

trochlear (CN IV)

abducents (CN VI)

what tracts and system is spared

sensory

spinocerebellar tract

signs and symptoms at onset are

asymmetrical and focal

LMN pathology

muscle weakness

hyporeflexia

hypotonicity

atrophy

muscle cramps

fasiculations

UMN pathology

muscle weakness

hyperreflexia

spasticity

pathological reflexes

bulbar symptoms

bulbar muscle weakness

dysphagia

dysarthia

sialorrhea

pseudobulbar effects

respiratory symptoms

respiratory muscle weakness (inspiratory and expiratory)

dyspnea

exertional dyspnea

nocturnal respiratory difficulty

orthopnea

hypoventilation

secretion retention

ineffective cough

ALS early stage: common impairments and activity limitations

mild to moderate weakness in specific muscle groups

difficulty with ADL and mobility toward end of this stage

ALS early stage interventions: restorative/preventative

strengthening exercises

endurance exercises

active ROM, active assisted ROM

stretching exercise

ALS early stage interventions: compensatory

determine potential need for adaptive or assistive devices

determine potential need for ergonomic modifications of home/workplace

energy conservation

educate the patient about the disease process, energy conservation and support groups

middle stage ALS: common impairments and activity limitations

progressive decrease in mobility throughout stage

wheelchair needed for long distances; increase wheelchair use towards end of stage

severe muscle weakness in some groups, mild to moderate weakness in other groups

ALS middle stage interventions: compensatory

support weak muscles (assistive and supportive devices, adaptive equipment, slings, orthoses)

modifications to workplace/home (install ramp, move bedroom to first floor)

wheelchair prescription

education of caregivers regarding functional training

ALS middle stage interventions: preventative

active, active assistive and passive ROM, stretching exercises (early middle)

endurance exercises

determine need for pressure relieving devices (pressure distributing mattress)

ALS late stage: common impairments and activity limitations

wheelchair dependent or restricted to bed

complete dependence with ADL

severe weakness of UE, LE, neck and trunk muscles

dysarthria, dysphagia

respiratory compromise

pain

ALS late stage interventions: preventative

passive ROM

pulmonary care

hospital bed and pressure relieving devices

skin care, hygiene

ALS early stage interventions: compensatory

caregiver education regarding transfers, positioning, turning, skin care

mechanical lift