neuromyotonia การใช้

• Antibodies directed against VGKC were first reported in neuromyotonia.
• Other patients present with isolated neuromyotonia or limbic encephalitis.
• Neuromyotonia is considered to be one of these with accumulating evidence for autoimmune origin over the last few years.
• Autoimmune neuromyotonia is typically caused by antibodies that bind to potassium channels on the motor nerve resulting in continuous / hyper-excitability.
• The principal autoimmune diseases studied are myasthenia gravis, the Lambert-Eaton myasthenic syndrome, limbic encephalitis, other types of autoimmune encephalitis and acquired neuromyotonia.
• Other ion channels can also be disrupted, such as the potassium channels causing inefficient repolarization at the presynaptic membrane as in neuromyotonia . ( reference 5)
• The classic presentation is with Morvan s syndrome, a disease with the features of neuromyotonia ( i . e . peripheral hyperexcetability ) and limbic encephalitis.
• A variety of conditions have similar symptoms to SPS, including myelopathies, dystonias, spinocerebellar degenerations, primary lateral sclerosis, neuromyotonia, and some psychogenic disorders.
• In some rare cases, acquired neuromyotonia has been misdiagnosed as amyotrophic lateral sclerosis ( ALS ) particularly if fasciculations may be evident in the absence of other clinical features of ALS . However, fasciculations are rarely the first sign of ALS as the hallmark sign is weakness.
• The neurologist or specialist may run a series of more specialized tests, including needle electromyography EMG / and nerve conduction studies ( NCS ) ( these are the most important tests ), chest CT ( to rule out paraneoplastic ) and specific blood work looking for voltage-gated potassium channel antibodies, acetylcholine receptor antibody, and serum immunofixation, TSH, ANA ESR, EEG etc . Neuromyotonia is characterized electromyographically by doublet, triplet or multiplet single unit discharges that have a high, irregular intraburst frequency.