There are four main "muscle things" that occur
with ALS: Spasticity, cramping, fasciculations, and fibrillations. The first three are
uncomfortable and spasticity and cramping can be very painful.
Spasticity is an upper motor neuron problem and is present to some degree in
ALS. For some patients it is minimal, for others extreme. Spasticity
can actually be helpful in maintaining function as the rigidity
helps replace normal muscle strength, but it causes jerky, hard to
control movements. Spasticity causes a tightening of muscles that
results in a stiffening of that part of the body in an exaggerated
reflex. It is actually triggering both the muscles to flex and the
muscles to extend that part of the body at the same time. It can
occur in any muscles - the arms, legs, back, abdomen, or neck. A
simple touch can trigger it and it may last only a moment or persist
indefinitely. Spasticity isn't always painful but it can be,
especially if it triggers muscle cramps that add to the pain.
Muscle cramps are very common in ALS, probably due to muscle fatigue or
irritability from screwed up nerve impulses. They can be terribly
painful and can occur in any muscle; feet, legs, arms, abdomen,
chest, back, and (my all time favorite for sheer pain!) the neck and
jaw. They can be in small muscles or affect big ones -- good
old-fashioned charlie horses. As the muscles are affected by ALS,
cramping is noted in that area. The cramping becomes less severe
with time because the weakening muscles simply can't work up a good
cramp anymore. They tend to be repetitive. Once a muscle starts
cramping up, it does so over and over for a miserable hour or so.
They tend to occur more if you have overdone exercise, if the muscle
is cold, or if circulation is decreased. Holding a book up to read
in bed on a cool night will just about guarantee some whopping good
hand cramps and result in interesting finger gestures!
Fasciculations (muscle twitchings) are probably due to nerve irritability. They
occur in smaller muscle bundles inside large muscle bundles and can
be observed as well as felt. Fasciculations are not so much painful
as irritating. It can feel as if someone is popping corn under your skin!
They can be incredibly persistent and keep you from sleeping.
Fibrillations occur in single muscle fibers within a muscle. They cannot be felt but can be seen on EMG (Electromyelogram). Fibrillations occur in conditions besides ALS.
Is it Spasticity or Cramping?
The first step in relieving these recurring pains is determining whether they are due to spasticity or cramping. Medications that work for spasticity don't necessarily work for muscle cramps and vice-versa.
Spasticity affects larger areas of the body - arm, leg, trunk,
neck. All the muscles in the area tighten up and the entire area may be
so tight it hurts. Muscle cramps are generally limited to a single
muscle and the pain, although intense even in small muscles, is limited
Spasticity often begins with an odd sensation, sometimes
described as a quiver, rushing and spreading through the area and
becoming painful as they tighten the muscles and more painful as the muscles
tire. Muscle cramps start out painful and just get worse as they tighten
Spasticity in the legs generally causes them legs to stiffen out. In
the trunk, back or neck it causes your body to arch a bit. In the arms
it may flex or extend the arm. Spasticity is often most striking when
you first wake up or start to move after sitting still for a while and
can often be connected to a sensory trigger. Of course that trigger can
be as mild as a gentle touch so the connection may not be made.
Muscle cramps often affect the hands and feet making fingers and toes curl
incredibly tightly. Spasticity is more widespread, less likely to affect
just those muscles, and more likely to stiffen them out than to curl
Spasticity can usually be
helped by medication but can be a very stubborn problem. One
consideration in treating spasticity is to find a balance between
relieving excessive and painful spasticity and maintaining a certain
level of spasticity which can be helpful by replacing muscle strength.
The meds for spasticity are primarilyBaclofen, Dantrium and Zanaflex.
Although Baclofen is often listed
under meds for cramping and prescribed for it, the indications say
"Clinically, baclofen is used to treat spasticity." In my experience,
Baclofen did not reduce cramping at all. However, if spasticity is
triggering the cramping, Baclofen may reduce cramping by reducing the
spasticity. Some patients experience weakness or tiredness while taking
it but these problems can often be reduced if the dosage is decreased.
In 1996 the FDA approved the use of Baclofen, delivered by an implanted
pump, for the treatment of spasticity due to spinal cord injury and this
is now being used for ALS patients.
less likely to cause weakness but may cause sleepiness. This can often
be minimized by starting with a low dose and gradually increasing it
until spasticity is relieved.
Dantrium has been used for spasticity, but because of its
potential for causing liver problems, is less frequently used since
other meds are now available. It does seem to still be used for bladder
control problems caused by spasticity however.
Quinine (Qualaquin, Quinine Sulfate) is a drug
long used for muscle cramping but in 1995 the FDA made it available by prescription only because of its very rare but potentially severe risks (heart arrythmias, birth defects, blood disorders, hearing loss). Even so I would certainly recommend asking your doctor about trying quinine. Additionally, the FDA removed muscle cramping from its list of uses because leg cramps are not life threatening and therefore not worth the potential risk of quinine. Making matters worse for ALS patients who experience severe muscle cramps daily, the FDA limited quinine to a single formulation available only as Qualaquin -- which costs around $5 a tablet! All of this was done and continues in effect even though research shows quinine to be effective against cramping, and that no other drug is significantly effective. #*%&! So we are left to suffer or
find a doctor willing to order Qualaquin outside of the FDA listed use, and pray that insurance will cover it.
If you find a doctor willing to write a prescription for generic quinine sulfate, save money by having the prescription filled by an Internet Canadian pharmacy. Or drink Tonic Water. The amount of quinine is probably too low to have any effect, however.
I do not recall what dose I took, but do know that too
large a dose will cause weakness. I am told the smallest tablet made is
260mg. I would recommend beginning with half a tablet and increasing to
a whole one if it is ineffective. It is also available in 200 mg
capsules. It is much less expensive than the other drugs prescribed,
and, in my experience, the most effective.
Like any drug, quinine does have
some potential side effects (and cannot be taken by pregnant women) but
it does not cause physical and psychological dependence as Valium does
in long term use. Aside from the weakness when I took the larger dose, I
noticed no other immediate side effects. After more than 5 years of
daily use, I did notice sweet foods started to loose any sweet taste,
especially chocolate. Quinine is very bitter so I assumed it was the
culprit since I wasn't on any other meds at the time. It was a minor
problem and when I did stop taking quinine, I found the ability to fully
enjoy an Oreo was gradually restored!
Valium and other muscle relaxants may be tried for spasticity but are probably better
used for muscle cramps. However, their side effects and cost make them a
second choice to quinine in my opinion.
I have not heard of anyone having any real success in reducing the fasciculations (muscle
twitching) with any medication.