Amyotrophic Lateral Sclerosis

A degenerative disorder affecting upper MOTOR NEURONS in the brain and lower motor neurons in the brain stem and SPINAL CORD. Disease onset is usually after the age of 50 and the process is usually fatal within 3 to 6 years. Clinical manifestations include progressive weakness, atrophy, FASCICULATION, hyperreflexia, DYSARTHRIA, dysphagia, and eventual paralysis of respiratory function. Pathologic features include the replacement of motor neurons with fibrous ASTROCYTES and atrophy of anterior SPINAL NERVE ROOTS and corticospinal tracts. (From Adams et al., Principles of Neurology, 6th ed, pp1089-94)

Also Known As:

Lou Gehrig's Disease; Motor Neuron Disease, Amyotrophic Lateral Sclerosis; Lou Gehrig Disease; Amyotrophic Lateral Sclerosis With Dementia; Amyotrophic Lateral Sclerosis, Guam Form; Amyotrophic Lateral Sclerosis, Parkinsonism-Dementia Complex of Guam; Amyotrophic Lateral Sclerosis-Parkinsonism-Dementia Complex 1; Charcot Disease; Dementia With Amyotrophic Lateral Sclerosis; Gehrig's Disease; Guam Disease; Guam Form of Amyotrophic Lateral Sclerosis; Lou-Gehrigs Disease; ALS Amyotrophic Lateral Sclerosis; Amyotrophic Lateral Sclerosis Parkinsonism Dementia Complex 1; Amyotrophic Lateral Sclerosis, Parkinsonism Dementia Complex of Guam; Disease, Guam; Disease, Lou-Gehrigs; Gehrig Disease; Gehrigs Disease; Sclerosis, Amyotrophic Lateral; ALS - Amyotrophic Lateral Sclerosis

Nervous System Diseases: 14737
- Neurodegenerative Diseases: 44095
  - Motor Neuron Disease: 1851
    - Amyotrophic Lateral Sclerosis: 12080
  - TDP-43 Proteinopathies: 223
    - Amyotrophic Lateral Sclerosis: 12080
- Central Nervous System Diseases: 4371
  - Spinal Cord Diseases: 4420
    - Amyotrophic Lateral Sclerosis: 12080
- Neuromuscular Diseases: 2048
  - Motor Neuron Disease: 1851
    - Amyotrophic Lateral Sclerosis: 12080
Nutritional and Metabolic Diseases: 15
- Metabolic Diseases: 10634
  - Proteostasis Deficiencies: 1476
    - TDP-43 Proteinopathies: 223
      - Amyotrophic Lateral Sclerosis: 12080

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Drug/Important Bio-Agent (IBA)