Post-irradiation neuromyotonia of the masseter muscle

INTRODUCTION: Neuromyotonia is a late and rare complication of radiation therapy, consisting of involuntary sustained muscle contractions with a delay in relaxation. OBSERVATION: We report the case of a 68-year-old man who developed neuromyotonia of the masseter muscle 6 years after irradiation for tonsil carcinoma. CONCLUSION: This observation underlines the importance of a correct diagnosis that can lead to an efficient treatment by carbamazepine.     

Hereditary paroxysmal ataxia with neuromyotonia.

The clinical manifestations of a patient with hereditary paroxysmal ataxia and neuromyotonia are described. Generalized tremor, triggered by sudden movements, and spasms of hand and foot muscles were the main clinical findings. Electromyogram (EMG) and nerve blocking studies led to the diagnosis of neuromyotonia. Treatment with acetozolamide was of no therapeutic value, confirming previous observations about the difference in response of paroxysmal ataxia with and without neuromyotonia.     

Ocular neuromyotonia: a case report

Ocular neuromyotonia is a rare clinical entity, which is characterized by spontaneous spasm of ocular muscles resulting in paroxysmal diplopia and strabismus. Inappropriate discharges from ocular motor neurons or axons with unstable cell membranes may cause the sustained contraction of their respective ocular muscles. In most cases, ocular neuromyotonia resulted from radiation therapy to the parasellar and sellar regions or compressive lesions on ocular motor nerves. Among 30 patients reported in the literatür only six patients were accepted as idiopathic. Here, we reported a patient with third nerve myotonia representing with spells of diplopia. No lesion was found and she was accepted as idiopathic ocular neuromyotonia.     

Paroxysmal neuromyotonia: a new sporadic channelopathy

Neuromyotonia is a heterogeneous group of genetic and autoimmune channelopathies resulting in hyperexcitability of peripheral nerves. We report an unusual case of neuromyotonia, which to our knowledge has not been previously described. The patient developed intermittent attacks of severe painful muscle stiffness accompanied by sweating, myokymia and raised serum creatine kinase. Genetic analysis of KCNA1, KCNQ2 and SCN4A genes did not identify pathogenic mutation. Serum voltage-gated potassium channel antibody was also negative. He was successfully treated with acetazolamide and carbamazepine. This appears to be a new neuromuscular disease, "paroxysmal neuromyotonia", the etiology of which is still unknown.     

The generator site in acquired autoimmune neuromyotonia

Objective

To investigate the origin of ectopic activity in neuromyotonia (NMT).

Ocular neuromyotonia with spastic lid closure.

Ocular neuromyotonia is characterized by tonic spasms of extraocular muscles evoked by eccentric gaze that induces transient strabismus and diplopia. We report the case of a 70-year-old woman who initially presented with unilateral deficits in fifth and sixth cranial nerve functions attributed to a fifth cranial nerve schwannoma. After radiation treatment, she developed neuromyotonia and synkinesis of the ipsilateral third cranial nerve. During the attacks of neuromyotonia, the left eyelids were often spastically closed, a phenomenon not previously reported. The ocular neuromyotonia regressed spontaneously within 3 years, but components of the synkinesis persisted. Ephaptic transmission in a damaged third cranial nerve may be responsible for the neuromyotonia and synkinesis. Synkinesis is a more enduring manifestation.