User Tools

Site Tools


n_mnd

Differences

This shows you the differences between two versions of the page.

Link to this comparison view

Both sides previous revision Previous revision
Next revision
Previous revision
n_mnd [2020/06/14 00:02]
gary1 [Amyotrophic lateral sclerosis (ALS)]
n_mnd [2021/02/24 21:25]
gary1 [Progressive Bulbar Palsy (PBP)]
Line 21: Line 21:
 =====Amyotrophic lateral sclerosis (ALS)===== =====Amyotrophic lateral sclerosis (ALS)=====
   *aka Lou Gehrig's disease   *aka Lou Gehrig's disease
-  * progressive degeneration of motor nerve cells in the brain (upper motor neurons) and spinal cord (lower motor neurons)+  * progressive degeneration of motor nerve cells in the brain (upper motor neurons) and spinal cord (lower motor neurons) associated with impaired folding of neuronal intracellular proteins resulting in mSOD1 toxicity and TDP‐43 pathology 
 +  * in 2021 it was reported than a new compound NU-9 was able to reduce this protein abnormal folding, improve mitochondrial and endoplasmic reticulum function and reverse the neuronal degeneration over 90 days in mice(([[https://doi.org/10.1002/ctm2.336|Improving mitochondria and ER stability helps eliminate upper motor neuron degeneration that occurs due to mSOD1 toxicity and TDP‐43 pathology]]))
   * most develop it between 40-70yrs, especially 60-70yrs but can occur younger   * most develop it between 40-70yrs, especially 60-70yrs but can occur younger
   * similar incidence as [[MS]] and accounts for 5 of every 100,000 adult deaths   * similar incidence as [[MS]] and accounts for 5 of every 100,000 adult deaths
Line 39: Line 40:
 ====Progressive Bulbar Palsy (PBP)==== ====Progressive Bulbar Palsy (PBP)====
   *25% of cases of ALS; mainly causes a LMN [[bulbar_palsy|bulbar palsy]]   *25% of cases of ALS; mainly causes a LMN [[bulbar_palsy|bulbar palsy]]
 +  *early cases may have:
 +    * difficulty with pronunciations, particularly lateral consonants (linguals) and velars
 +    * drooling of saliva
 +    * gagging, choking, aspiration of food and saliva
 +    * 25% develop widespread symptoms common to ALS
 +  * later, loss of gag reflex, tongue fasciculations, and inability to protrude the tongue occur
 +  * some may develop pseudobulbar palsy and emotional changes
 +  * death, usually from aspiration pneumonia, usually occurs within 1-3 yrs of onset of disorder
  
 ====Progressive Muscular Atrophy (PMA)==== ====Progressive Muscular Atrophy (PMA)====
n_mnd.txt · Last modified: 2021/02/24 21:25 by gary1