Amyotrophic lateral sclerosis
The majority of cases occur sporadically without family history. Approximately 5%-10% of cases are genetically inherited. Approximately 15%-20% of familial cases are associated with mutations in the superoxide dismutase-1 (SOD1) gene.
ALS is more likely to occur in men, with a peak incidence in the fifth to seventh decade of life. Veterans are more likely to develop the disease. There are some familial forms of ALS that are associated with concomitant frontotemporal dementia.
Patients typically survive 2-5 years following diagnosis.
G12.21 – Amyotrophic lateral sclerosis
86044005 – Amyotrophic lateral sclerosis
Differential Diagnosis & Pitfalls
- Spinal muscular atrophy
- Primary lateral sclerosis
- Cervical stenosis
- Myasthenia gravis
- Spasmodic dysphonia
- Lambert-Eaton myasthenic syndrome
- Multifocal motor neuropathy
- Chronic inflammatory demyelinating polyneuropathy
- Postpolio syndrome
- Lead toxicity
- Dapsone toxicity
- POEMS syndrome
- Anti-MAG neuropathy
- Kennedy disease