Neuromyotonia
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2008-06-14T14:51:40Z
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{{Merge|Isaacs syndrome|date=April 2008}}
{{Infobox_Disease |
Name = {{PAGENAME}} |
Image = |
Caption = |
DiseasesDB = 31818 |
ICD10 = {{ICD10|G|71|1|g|70}} |
ICD9 = {{ICD9|333.90}} |
ICDO = |
OMIM = |
MedlinePlus = |
eMedicineSubj = |
eMedicineTopic = |
MeshID = D020386 |
}}
'''Neuromyotonia''', also known as ''Isaacs' Syndrome'', is spontaneous [[muscle|muscular]] activity resulting from repetitive motor unit action potentials of peripheral origin.
==Causes==
It develops as a result of both acquired or [[hereditary disease]]s. Acquired form is more frequent and is usually caused by [[antibody|antibodies]] against neuromuscular junction.
Autoreactive antibodies can be detected in a variety of peripheral (e.g. [[myasthenia gravis]], [[Lambert-Eaton myasthenic syndrome]]) and central nervous system (e.g. [[paraneoplastic cerebellar degeneration]], [[paraneoplastic limbic encephalitis]]) disorders. Their causative role has been established in some of these diseases but not all. Neuromyotonia is considered to be one of these with accumulating evidence for [[autoimmune disorder|autoimmune origin]] over the last few years. Some neuromyotonia cases do not only improve after [[blood plasma|plasma]] exchange but they may also have antibodies in their [[blood plasma|serum]] samples against [[ion channel|voltage-gated potassium channels]].<ref>{{cite journal |author=Maddison P |title=Neuromyotonia |journal=Clinical neurophysiology : official journal of the International Federation of Clinical Neurophysiology |volume=117 |issue=10 |pages=2118–27 |year=2006 |pmid=16843723 |doi=10.1016/j.clinph.2006.03.008}}</ref> Moreover, these antibodies have been demonstrated to reduce potassium channel function in neuronal cell lines.
==Presentation==
As a result of muscular hyperactivity patients may present with muscle cramps, [[myotonia]]-like symptoms, excessive sweating, [[myokymia]] and [[fasciculation]]s. A very small proportion of cases with neuromyotonia may develop central nervous system findings in their clinical course, causing a disorder called [[Morvan's syndrome]] and they may also have antibodies against potassium channels in their serum samples. [[Sleep disorder]] is only one of a variety of clinical conditions observed in Morvan's syndrome cases ranging from confusion and memory loss to [[hallucination]]s and [[delusion]]s.
==Treatment==
There is no known cure for neuromyotonia. The long-term prognosis for individuals with the disorder is uncertain. [[Anticonvulsants]], including [[phenytoin]] and [[carbamazepine]], usually provide significant relief from the stiffness, muscle spasms, and pain associated with neuromyotonia. Plasma exchange may provide short-term relief for patients with some forms of the acquired disorder.
==References==
<references/>
{{PNS diseases of the nervous system}}
[[Category:Neurological disorders]]