Published on 03/03/2015 by admin
Filed under Neurology
Last modified 03/03/2015
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70 Other Peripheral Motor Hyperactivity Syndromes
H. Royden Jones, Jr.
This is another hyperkinetic presumed peripheral nerve hyperexcitability syndrome that occurs even more rarely than the stiff person syndrome. As noted above, there are a number of synonyms. It is a very unusual neurologic disorder also characterized by the continuous firing of peripheral nerves, and thus muscle fibers. It is much more subtle in its presentation. Typically the age of onset is in the teenage years, but it may be seen in adulthood. This appears to be autoimmune in origin (anti–potassium channel antibodies are positive in some patients.) This is a nonspecific finding per se as these antibodies may be found in a variety of neurologic syndromes.
Here the patient first notes what appears to be an almost continuous firing of groups of muscle fibers usually appearing to mimic fasciculations. These occur concomitantly in both agonists and antagonists. The patient often notes a sense of fatigue. Usually there are no significant associated muscle spasms, cramping, or pain; however, in some instances such may occur. The patient may note a degree of weakness because of the inconsistent firing of opposing muscle groups, leading to ineffective mechanical function. Some patients have excessive sweating.
Netters Neurology
