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 in area.
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 up.
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 them.
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.
Zanaflex is 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,
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