Amyotrophic lateral sclerosis: Difference between revisions
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==Clinical Features== | ==Clinical Features== | ||
*Acute respiratory failure | *Acute [[respiratory failure]] | ||
**Predicted by forced VC <25 mL/kg or 50% decrease from normal | **Predicted by forced VC <25 mL/kg or 50% decrease from normal | ||
*Aspiration [[Pneumonia (Main)|pneumonia]] | *Aspiration [[Pneumonia (Main)|pneumonia]] | ||
*Trauma related to extremity weakness | *Trauma related to extremity [[weakness]] | ||
*Asymmetric weakness without sensory findings with mixed upper and lower motor nerve findings | *Asymmetric [[weakness]] without sensory findings with mixed upper and lower motor nerve findings | ||
==Differential Diagnosis== | ==Differential Diagnosis== | ||
| Line 17: | Line 17: | ||
==Management== | ==Management== | ||
#Nebulized medications | #Nebulized medications | ||
#Steroids | #[[Steroids]] | ||
#Antibiotics | #[[Antibiotics]] for infectious complications | ||
#Assisted ventilation / intubation | #Assisted ventilation / intubation if in [[respiratory failure]] and aligned with [[goals of care]] | ||
==See Also== | ==See Also== | ||
Revision as of 16:05, 18 August 2019
Background
- Progressive muscle atrophy/weakness due to degeneration of upper and lower motor neurons
- Patients will rarely present to the ED undiagnosed
- Likely related to mutated superoxide dismutase (SOD1) gene
- Involvement of the anterior horn cells
Clinical Features
- Acute respiratory failure
- Predicted by forced VC <25 mL/kg or 50% decrease from normal
- Aspiration pneumonia
- Trauma related to extremity weakness
- Asymmetric weakness without sensory findings with mixed upper and lower motor nerve findings
Differential Diagnosis
Weakness
- Neuromuscular weakness
- Upper motor neuron:
- CVA
- Hemorrhagic stroke
- Multiple sclerosis
- Amyotrophic Lateral Sclerosis (ALS) (upper and lower motor neuron)
- Lower motor neuron:
- Spinal and bulbar muscular atrophy (Kennedy's syndrome)
- Spinal cord disease:
- Infection (Epidural abscess)
- Infarction/ischemia
- Trauma (Spinal Cord Syndromes)
- Inflammation (Transverse Myelitis)
- Degenerative (Spinal muscular atrophy)
- Tumor
- Peripheral nerve disease:
- Neuromuscular junction disease:
- Muscle disease:
- Rhabdomyolysis
- Dermatomyositis
- Polymyositis
- Alcoholic myopathy
- Upper motor neuron:
- Non-neuromuscular weakness
- Can't miss diagnoses:
- ACS
- Arrhythmia/Syncope
- Severe infection/Sepsis
- Hypoglycemia
- Periodic paralysis (electrolyte disturbance, K, Mg, Ca)
- Respiratory failure
- Emergent Diagnoses:
- Symptomatic Anemia
- Severe dehydration
- Hypothyroidism
- Polypharmacy
- Malignancy
- Aortic disease - occlusion, stenosis, dissection
- Other causes of weakness and paralysis
- Acute intermittent porphyria (ascending weakness)
- Can't miss diagnoses:
Management
- Nebulized medications
- Steroids
- Antibiotics for infectious complications
- Assisted ventilation / intubation if in respiratory failure and aligned with goals of care
See Also
References
Tintinalli's Emergency Medicine: A Comprehensive Study Guide, 7e (2010), Chapter 167. Chronic Neurologic Disorders