In case you missed it, the big news this week is the FDA
fast-tracked the approval of a treatment for ALS. You can read about it
here: CNN Health
and here: FDAPress Release.
This
is fantastic news for those living with ALS.
I have received a few inquiries asking if this drug
could be helpful for treating Kennedy’s Disease.
First off, I am not a doctor and not as knowledgeable
as some others with KD concerning this topic. What I do know is that ALS is a
condition that attacks the upper motor neurons. Kennedy’s Disease attacks the
lower motor neurons. For this reason, I do not believe the new drug will be
beneficial for those of us living with KD.
So what is the difference between the two upper and
lower motor neurons? I found the following explanation to be helpful.
“Upper motor neurons
originate in the motor region of the brain stem. They are not
responsible for the stimulation of the muscle which is targeted as they do
not carry information down to the final common pathway. They
work through a neurotransmitter called glutamate which transmits the nerve
impulses from upper to lower motor neurons where it is detected by
glutamatergic receptors. On the other hand, lower motor neurons receive
impulses from the upper motor neurons and connect the spinal cord and brain
stem to the muscle fibers. They are the cranial and spinal nerves. They
work by making use of the glutamate which is released from the upper motor
neurons, and this triggers depolarization in the lower motor neurons. A series
of actions occur which end up signalling the muscle to contract. The cell bodies
of the lower motor neurons are located in the neuraxis, and their axons leave
and synapse with the muscles in the body. On the other hand, the upper motor
neurons synapse with the lower motor neurons as they are unable to leave the
central nervous system.”
Source: DifferenceBetween Net
Nonetheless, even if this new treatment is not the answer, it
is progress and a help for those living with ALS.
I'm no doc either but as far as I understand for it to be called ALS *BOTH* motor neurons would have to be affected - so this drug could be interesting for KD patients.
ReplyDeleteThe MND type that affects the upper motor neurons is called "primary lateral sclerosis", the type that affects the lower motor neurons is called "progressive muscular atrophy".
So, I don't know, maybe this is good news - but it seems to be really, really expensive.