Neurology

Amyotrophic Lateral Sclerosis

근위축측삭경화증

Progressive degeneration of upper and lower motor neurons (Lou Gehrig disease)

How common
Commonly encountered in neurology
Typical age
Variable age

What is it?

Core pathophysiology of Amyotrophic Lateral Sclerosis.

Commonly affected: Affected neural region (peripheral nerve, brain, spinal cord, or muscle)

How it develops

  1. Trigger/predispositionTriggering or predisposing factors for Amyotrophic Lateral Sclerosis
  2. Neural injurySpecific nerve/muscle injury occurs
  3. ManifestationCharacteristic neurologic features appear
  4. CourseRecovery or progression depends on treatment

Symptoms

  • Characteristic neurologic symptomClassic Amyotrophic Lateral Sclerosis feature
  • Motor/sensory changeWeakness, sensory loss, paresthesia
  • Functional impairmentDifficulty with ADLs

How it is examined

  • Neurologic examStrength, sensation, reflex, cranial nerve, cerebellar
  • Specific testsAppropriate workup for Amyotrophic Lateral Sclerosis (NCS/EMG/MRI/serology)

Imaging

Plain X-ray generally not used for diagnosis.

  • N/A

Brain/spine MRI or NCS/EMG as appropriate.

  • Region-specific neurologic findings

Non-surgical care

  • PharmacotherapyDiagnosis-specific neurologic medication
  • Rehab/PTFunctional recovery and maintenance
  • Trigger managementModify identified triggers

Conservative options are generally tried first. Medications listed here can have side effects — discuss them with your prescriber.

When surgery is considered

Refractory or structural abnormality

Procedures that may be discussed

  • Nerve decompression or targeted procedure
  • Immunotherapy/plasmapheresis (if autoimmune)

Outlook

Variable based on timing of diagnosis/treatment; most improve with active care.

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