1/20. Reversible F-wave hyperexcitability associated with antibodies to potassium channels in Isaacs' syndrome. We report a case of Isaacs' syndrome showing F-wave hyperexcitability which was reversible after either epidural block or plasmapheresis. A 14-year-old girl with progressive muscle cramp and myokymia in the legs showed high amplitude, long duration, polyphasic F-waves after either tibial nerve or peroneal nerve stimulation. Potassium current of PC-12 cell membrane was suppressed by her serum, IgM of which strongly reacted with proteins of approximately 50 kDa of the lysates of a PC-12 cell line. After an epidural nerve block with 1 per cent lidocaine 10 ml, the myokymia temporally improved, during which time the hyperexcitable F-waves became normalized. We treated her with double filtration plasmapheresis, which resulted in remarkable improvement of myokymia; simultaneously, the abnormal F-waves showed a decrease in amplitude, duration, and in the number of phases. We concluded that there was hyperexcitability of the proximal site of motor fibers in our patient, and possible immune mechanisms gave rise to the myokymia and unusual F-wave features. ( info) |
2/20. Multiple paraneoplastic diseases associated with thymoma. Here we report a patient with a lymphoepithelial thymoma who developed in chronological sequence limbic encephalitis, neuromyotonia and myasthenia gravis. The patient presented with limbic encephalitis associated with an invasive thymoma and improved after surgery and cytotoxic therapy. Two months after thymectomy, neuromyotonia associated with hyperhidrosis and mild motor neuropathy occurred and the patient was given plasma-exchange and prednisone therapy. Five months later he developed mild generalised myasthenia gravis. Anti-acetylcholine receptor antibodies, previously repeatedly negative, were found positive at the onset of clinical signs of myasthenia gravis. ( info) |
3/20. Isolated finger flexion: a novel form of focal neuromyotonia. Two almost identical elderly women are described who presented with gradually progressive painless involuntary flexion of the ring and middle fingers over 12 months, leading eventually to contractures. The flexion deformity persisted during sleep and was the sole neurological abnormality. Both patients had advanced chronic obstructive pulmonary disease and were on long term salbutamol and oxygen. Neurophysiological studies indicated that this was due to neuromyotonia mainly involving flexor digitorum superficialis muscles without evidence of underlying peripheral neuropathy, proximal conduction block, or generalised neuromyotonia. Voltage gated potassium channel antibodies were negative. The clinical and neurophysiological picture remained static over a 2 year follow up period. It is suggested that this is a novel form of acquired focal neuromyotonia and speculate both on its cause and distribution. ( info) |
4/20. Successful immunoglobulin treatment in a patient with neuromyotonia. Neuromyotonia is characterized by spontaneous and continuous muscle fibre activity leading to muscle cramps, pseudomyotonia, myokymia and weakness. Electromyographic recordings show typical findings. An auto-immune mechanism has been suggested in at least a subset of patients. Various therapies have been tried with different outcome. A patient with neuromyotonia responding well to high-dose immunoglobulin treatment is presented. ( info) |
5/20. Idiopathic ocular neuromyotonia: a neurovascular compression syndrome? Ocular neuromyotonia in the muscles innervated by the right oculomotor nerve was diagnosed in a patient without a history of radiation therapy. electromyography of the levator palpebrae showed continuous motor unit activity. brain MRI disclosed a close contact between the right third cranial nerve and a basilar artery dolichoectasia. The patient partly benefited from carbamazepine therapy. This unique finding suggests that neurovascular compression syndrome could be an hitherto unrecognised cause of ocular neuromyotonia. ( info) |
6/20. Isaacs' syndrome successfully treated by immunoadsorption plasmapheresis. We report a 70-year-old woman with Isaacs' syndrome (acquired neuromyotonia) who showed a marked improvement after immunoadsorption plasmapheresis (IAP). She developed hyperhidrosis in her teens, and slowly progressive symptoms of neuromyotonia for over 50 years. An in vitro investigation of her serum with patch-clamp technique suggested the presence of antibodies against potassium channels. She was treated with IAP, which brought disappearance of her symptoms. Though the symptoms started to recur in 3 weeks, moderate improvement has been maintained by immunosuppressive drug treatment. ( info) |
7/20. Neuromyotonia: autoimmune pathogenesis and response to immune modulating therapy. BACKGROUND: Neuromyotonia (NMT) has been postulated to be an autoimmune channelopathy, probably by affecting voltage gated potassium channels (VGKC) leading to excitation and abnormal discharges [Sinha et al., Lancet 338 (1991) 75]. OBJECTIVE: To report three patients with NMT who had other associated immune-mediated conditions, i.e., myasthenia gravis, thymoma and various types of peripheral neuropathies. One patient had peripheral neuropathy and involvement of pre- and post-synaptic neuromuscular junction. RESULTS: All three patients had evidence of polyneuropathy and neuromyotonic discharges on electrodiagnostic studies. Elevated acetylcholine receptor antibodies were noted in all patients and malignant thymoma was found in two patients with metastasis. All three patients showed moderate to marked response to plasma exchange. CONCLUSIONS: These findings strongly suggest a humoral autoimmune pathogenesis of NMT, probably by K( ) channel involvement, affecting acetylcholine quantal release and postsynaptic membrane. Clinicians should be aware of this association of immune-mediated conditions in NMT patients and marked improvement with plasma exchange. ( info) |
| Morvan's 'fibrillary chorea' or Morvan's syndrome is characterized by neuromyotonia (NMT), pain, hyperhydrosis, weight loss, severe insomnia and hallucinations. We describe a man aged 76 years with NMT, dysautonomia, cardiac arrhythmia, lack of slow-wave sleep and abnormal rapid eye movement sleep. He had raised serum antibodies to voltage-gated K( ) channels (VGKC), oligoclonal bands in his CSF, markedly increased serum norepinephrine, increased serum cortisol and reduced levels and absent circadian rhythms of prolactin and melatonin. The neurohormonal findings and many of the clinical features were very similar to those in fatal familial insomnia, a hereditary prion disease that is associated with thalamic degenerative changes. Strikingly, however, all symptoms in our MFC patient improved with plasma exchange. The patient died unexpectedly 11 months later. At autopsy, there was a pulmonary adenocarcinoma, but brain pathology showed only a microinfarct in the hippocampus and no thalamic changes. The NMT and some of the autonomic features are likely to be directly related to the VGKC antibodies acting in the periphery. The central symptoms might also be due to the direct effects of VGKC antibodies, or perhaps of other autoantibodies still to be defined, on the limbic system with secondary effects on neurohormone levels. Alternatively, changes in secretion of neurohormones in the periphery might contribute to the central disturbance. The relationship between VGKC antibodies, neurohormonal levels, autonomic, limbic and sleep disorders requires further study. ( info) |
9/20. Ocular neuromyotonia: a case report. Ocular neuromyotonia is a rare motility disorder occurring after tumor irradiation near the skull base or as a consequence of vascular abnormalities. Ocular myasthenia, convergence spasm and a cyclic third nerve palsy must be considered as differential diagnoses. The case of a 32-year-old woman suffering from intermittent diplopia six months after radiation therapy of a recurrent pituary gland adenoma is presented. ( info) |
10/20. Chronic postoperative epidural abscess with ascending neuromyotonia. IMPLICATIONS: Clinicians are reminded that epidural abscesses may not follow epidural space violation, may present a confusing constellation of back pain and paraparesis, and may have serious consequences if not diagnosed accurately and managed aggressively with spinal decompression. ( info) |