Episode Details

Episode 152: ALS Fundamentals

Future Dr. Rodriguez explains the symptoms of ALS, including UMN and LMN symptoms. Dr. Arreaza discusses the principles of symptomatic treatment by primary care. This is a brief introduction to ALS.  

Written by Adraina Rodriguez, MSIV, Ross University School of Medicine.  

You are listening to Rio Bravo qWeek Podcast, your weekly dose of knowledge brought to you by the Rio Bravo Family Medicine Residency Program from Bakersfield, California, a UCLA-affiliated program sponsored by Clinica Sierra Vista, Let Us Be Your Healthcare Home. This podcast was created for educational purposes only. Visit your primary care provider for additional medical advice.

Arreaza: It is rare but you may encounter it and you should be able to identify the most common symptoms. ALS Challenge in 2014: Ice bucket challenge. 

Adriana: Patrick Quinn was an ALS patient and activist who created the ICE Bicket Challenge and helped raise US$220 million for medical research.

Arreaza: What is ALS?

Adriana: ALS stands for Amyotrophic Lateral Sclerosis, formerly known as Lou Gehrig’s Disease. It is the most common form of acquired motor neuron disease. ALS is a progressive, incurable neurodegenerative motor neuron disorder with Upper motor neuron (UMN) and/or Lower motor neuron symptoms that cause muscle weakness, disability, and eventually death. There is no single diagnostic test that can confirm or entirely exclude the diagnosis of motor neuron disease. 

Arreaza: When should you suspect ALS in a patient?

Adriana: The classic patient presentation is insidious, slowly progressive, and unremitting UMN and/or LMN symptoms present in one of four body segments - cranial/bulbar, cervical, thoracic, and lumbosacral - followed by spread to other segments over a period of months to years. 

Arreaza: What would you see on the physical exam when the Patient is in the clinic? There is a system to send signals from your brain to your muscles. It involves basically two neurons: Upper and lower motor neurons. The UMN goes from your cerebral cortex to your spinal cord and there it connects to a lower motor neuron through synapsis. The LMN then sends the signal to your muscles, causing contraction or relaxation. Tell us about the UMN and LMN symptoms.

Adriana:

• LMN Symptoms: Weakness, Fasciculations, Muscular atrophy, Decreased muscle tone (flaccidity) and reduced or absent reflexes. 
• UMN Symptoms: Increased tone and increased extremity deep-tendon reflexes, presence of any reflexes in muscles that are profoundly weak and wasted, pathological reflexes (crossed adductors, jaw jerk, Hoffman sign, Babinski sign 50%), syndrome of pseudobulbar affect (inappropriate laughing, crying, forced yawning).

Arreaza: What are important factors to help narrow your differential to ALS?
Multifocal motor neuropathy, cervical radiculomyelopathy, benign fasciculations, inflammatory myopathies, post-polio syndrome, monomelic amyotrophy, hereditary spastic paraplegia, spinobulbar muscular atrophy, myasthenia gravis, hyperthyroidism, and many others.

There are pertinent negatives to look out for: 

• Usually negative neuropathic or radiculopathic pain, sensory loss, sphincter dysfunction, ptosis, or extraocular muscle dysfunction (20-30% positive sensory symptoms or “pins and needles” and “electricity” in the affected limbs).
• Note: Cognitive dysfunction does not exclude ALS

Arreaza: What are the diagnostic criteria for ALS

Adriana: Gold Coast Criteria 2019 proposed over El Escorial criteria:

• Progressive upper and lower motor neuron symptoms and signs in one limb or body segment, OR
• Progressive lower motor neuron symptoms and signs