Another question on Fasiculations

  : : I know this is an overworked subject, but I have read in a couple of articles that generalized fasiculations can be a presenting feature of ALS. I have read here that fasiculations occur ONLY after weakness and in focal groups. Are there two types of the disease?? Also a previous poster made the remark that someone here had said speech and swallowing problems were most often seen together and one would not present without the other. Is this also the case??  Thank you.
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  : Dear George:
  : Isolated fasciculation with a normal neurological examination by an experienced neurologist, specially if followed up by a similar normal examination in a few months, is almost NEVER ALS. An EMG examination is frequently requested, however, to confirm the diagnosis of benign fasciculation syndrome, and to calm the worries of the patient.
  : AND
  : Speech and swallowing difficulties in ALS tend to go together, although occasional dissociations occur. The difiiculties are from a combination of pseudobulbar (upper motor neuron) and bulbar (lower motor neuron) involvement.
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  : The following is a set of qustions answered in an ealier posting:
  :
  : : 1. How frequently are fasciculations the first symptom found in an ALS patient?
  : : Widespread profuse fasciculations are the hallmark of ALS. Most ALS experts will agree that the diagnosis of ALS is in doubt if no fasciculation is found, clinically and on EMG.
  : : Fasciculations are an early symptom of ALS, and may indeed precede any complaints of weakness by the patient. ALMOST ALL patients with fasciculations who have ALS, however, will have an abnormal neurological examination (i.e. show signs of weakness, atrophy, corticospinal involvement, or bulbar/pseudobulbar involvement) when examined by an experienced neurologist.
  : : 2. If the fasiculations are not accompanied by atrophy or weakness, what is the probability
  : : that they are ALS related? Again, I have read that they are only ALS indicative if they are
  : : the RESULT of weakness/atrophy. Thus, I'd think that fasciculations in the absence of the
  : : other symptoms would be a pretty darn good sign.
  : : A normal neurological exam (except for fasciculations) by an experienced and good neurologist makes ALS a very unlikely diagnosis. "Symptoms" are what the patient reports, and "signs" are what the physician observes. Your last sentence is correct if it reads "in the absence of the
  : : other signs".
  : : 3. How many people suffer from benign fasciculations, and how often do they last on average?
  : : Sporadic fasciculation is incredibly common, occurs in the majority of normal individuals off and on. Some individuals are more perceptive to these twitches and tend to report them, sometimes from fear of having a serious neurological problem. Most experts agree that a subset of individuals have more prominent, widespread, and persistent fasciculation than should be seen normally. These people often diagnosed to have the benign fasciculations syndrome. This syndrome is obviously quite common, but a prevalence figure is not available. One hundred and thirty seven individuals with benign fasciculations were identified and studied at the Mayo Clinic over a period of 28 years. Of these, 121 were available for a follow-up interview after a mean of about seven years; none had developed ALS, and about half had had a significant improvement. Interestingly, about 16% of the cases have a viral infection preceding onset of symptoms, and about 20% have emotional stresses.
  : : 4. Other than ALS and "nothing", what else could explain fasciculations?
  : : A long list of causes - root disease, nerve entrapments (such as carpal tunnel syndrome), specific forms of neuropathy (specially demyelinating), radiation injury to nerves, other forms of motor neuron disease (SMA, Kennedy's disease), the cramp-fasciculation syndrome, syringomyelia, thyroid disease, caffeine, some other drugs, etc.
  : : I guess you are regarding the benign fasciculations under "nothing".
  : : 5. How do you define weakness? How do I think about this vis-a-vis soreness, stiffness, aching,
  : : or the feeling of fatigue in muscles?
  : : Weakness is loss of strength. Specifically, on history, an inability to execute specific actions (such as lifting heavy things, opening jars, turning keys, climbing steps, rising from a chair, getting feet into the car, walking on heels/toes, getting out of bed, etc.) due to a lack of strength. Numerous individual muscles are tested for strength during a neurological exam.
  : : 6. If someone is experiencing twitching, along with muscle soreness and fatigue, but can still go out
  : : and run 3+ miles, is that pretty indicative of a non-ALS situation?
  : : Not necessarily. Needs a good neurological exam. One hand may be a little weak, for instance.
  : : 7. What percent of ALS patients experience symptoms in one limb first, versus multiple limbs? My
  : : understanding is that directionally it tends to occur more often in one limb first.
  : : ALS usually starts in one limb (or else, in the bulbar- face/swallowing/tongue muscles), and then spreads to other areas.
  : : 8. If the twitches are primarily in the calves and eyelids, with some roving twitches elsewhere, is
  : : this a positive sign? It seems stress related and/or benign twitches occur most often in calves and
  : : eyes.
  : : Yes, in most cases this pattern suggests a benign problem.
  : : I hope this is useful to you and to others.
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  Thank you for your response. Could you briefly describe some of the symptoms associated with pseudobulbar and symptoms of bulbar involvement in the speech and swallowing areas??
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The symptoms may be difficult to distinguish:
Pseudobulbar- a specific type of slurring of speech (slowed, "hot potatoes"), choking while swallowing, excessive tendency to gag, tendency to laugh or cry easily
Bulbar- a somewhat different change of speech (nasal, hoarse), drooling, twitching around the lower face, wasting and weakness of the tongue, sagging of the jaw with weakness of chewing, weakness of swallowing.
Examination is relatively simpler.