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Biomedical subjects

N J Brautaset

Publications and source records attributed to N J Brautaset.

12 recordsLinked to original sources

A rare form of painful nondystrophic myotonia.

OBJECTIVE: In this paper we report a painful nondystrophic myotonia which has not been previously described. Pain is a rare symptom in myotonia. We report a myotonic disorder in a 34-year-old woman and her 14-year-old daughter. Painful cramps occur during and after exercise in the mother, and both patients can demonstrate unusual contractions in the tongue. In the present study we try to evaluate the mechanisms behind the unique finding of trains of high amplitude of positive waves, not seen in the earlier known myotonic conditions. METHODS: Clinical investigations and electromyography with single and dual channel recordings and muscle morphometry were performed. RESULTS: The electromyographic recordings reveal positive waves, fibrillation potentials and myotonic discharges. In addition, extraordinary findings were made of trains of high frequency positive potentials with very high amplitudes and with conduction block along the muscle fibres. CONCLUSIONS: In this new form of myotonia with likely dominant heredity, the specific finding of trains of high amplitude positive waves indicates ephaptic transmission within bundles of neighbouring muscle fibres.

Adolescent↗

A double-blind placebo-controlled study assessing the efficacy and tolerability of 50 mg sumatriptan tablets in the acute treatment of migraine. Sumatriptan Tablets S2CM07 Study Group.

BACKGROUND: Oral sumatriptan 50 mg has been found to have good efficacy and tolerability in the acute treatment of migraine but has been less well studied than the 100 mg dose. METHODS: This was a double-blind, parallel-group study (Glaxo Wellcome protocol number S2CM07) comparing the efficacy and safety of sumatriptan 50 mg tablets with placebo in the acute treatment of migraine. Patients treated three migraine attacks with study medication; a second, optional dose was available for treating recurrent headache. Of the 560 patients randomized, 485 treated at least one attack, 411 at least two attacks, and 362 three attacks. The primary efficacy measure was the proportion of patients who had obtained complete or almost complete headache relief at 4 h after dosing. RESULTS: For all attacks, a significantly greater proportion of patients experienced headache relief at 4 h with sumatriptan 50 mg tablets than with placebo (59% to 62% versus 32% to 42%; P = 0.005). The same was true at 3 h across all attacks, and at 2 h for attacks 1 and 2 (49% versus 23% and 45% versus 29%, respectively). Although sumatriptan and placebo were associated with similar incidences of recurrence, sumatriptan was associated with a longer time to recurrence. The incidence of adverse events with sumatriptan was similar to that with placebo, and there was no increase in adverse events associated with use of a second dose to treat recurrence. CONCLUSIONS: Sumatriptan 50 mg tablets are well tolerated and efficacious in relieving migraine headache.

Acute Disease↗

Generator sites for spontaneous activity in neuromyotonia. An EMG study.

A 16-year-old female patient with symptoms and signs compatible with neuromyotonia was studied with various neurophysiological tests and with muscle biopsy. Nerve conduction studies revealed signs of axonal motor neuropathy. EMG showed denervation in distal muscles, and moderate neurogenic changes in other muscles. Abundant spontaneous motor unit activity was recorded in all muscles. This activity did not disappear upon proximal nerve blockade with local anaesthetics. Based on the shape of spontaneous discharges and their behaviour on nerve stimulation and during voluntary effort, the site of generation was suggested. This varied for different discharges, from proximally in the nerve, to various sites along the intramuscular nerve tree. In some axons there were signs of conduction block proximal to the generation site for the spontaneous discharges. Different axons showed various degrees of abnormality; local hyperexcitability triggering new impulses only after the passage of a preceding impulse, increased hyperexcitability generating spontaneous activity, total impulse blocking, and finally axonal degeneration. Treatment with dihydantoin reduced the spontaneous activity with concomitant clinical improvement.

Adolescent↗

Oculopharyngeal muscular dystrophy in Norway. Survey of a large Norwegian family.

OBJECTIVES: Oculopharyngeal muscular dystrophy was first described by Taylor in 1915. A substantial number of cases have been reported from North America, the disorder being most frequent in Quebec, Canada. MATERIAL: The onset of disease is in middle life, most often presenting with ptosis and a slight degree of ophthalmoplegia, followed some years later by dysphagia and often by proximal limb weakness. The course is slowly progressive, but the dysphagia may become severe and has caused death by starvation in several cases. The disorder is transmitted autosomally dominant in families. RESULTS: The present report describes a large Norwegian family with 13 affected members of whom we have examined eight. Transmission is autosomally dominant, mode of presentation may be either by ptosis or by dysphagia. In rare cases gait difficulties may be the presenting symptom. CONCLUSION: Electromyographic and muscle biopsy examinations demonstrate a primary myogenic disorder.

Aged↗

[Lumbar disk prolapse treated with chymopapain].

We report results from 108 consecutive patients followed up for one year after chymopapain injection. The patients were selected on strict clinical and radiological criteria. In nine patients (8%) the result was poor, and they were surgically treated within one year after the injection. 99 (92%) patients improved and after one year 87 of these (81% of the total material) had returned full time to their previous occupation. The only serious complication was one case of septicemia and possible discitis. The patient made a full recovery after antibiotic treatment. On the basis of these results, we shall continue to use chymopapain injection to treat selected patients with sciatica caused by herniated lumbar discs.

Adolescent↗

Provokable bilateral vertebral artery compression diagnosed with transcranial Doppler.

BACKGROUND: Head and neck movements may cause vascular compression that produces a syndrome of vertebrobasilar insufficiency. Vertebrobasilar circulation was examined noninvasively in two patients who were able to provoke these symptoms repeatedly on demand. CASE DESCRIPTIONS: Blood flow velocities in the basilar artery (case 1) and both posterior cerebral arteries (both cases) were measured continuously by transcranial Doppler sonography while the patients voluntarily performed the offending maneuvers and reproduced their symptoms. The provocative maneuvers evoked an immediate and precipitous drop in blood flow velocity, producing symptoms within seconds. Upon relief, the blood flow velocities showed a transient overshoot before returning to baseline values. CONCLUSIONS: These observations indicate that the symptoms of vertebrobasilar insufficiency were due to real reductions in blood flow and demonstrate the usefulness of transcranial Doppler sonography to diagnose bilateral extracranial vertebral artery compression.

Adult↗

Multimodal evoked responses and cerebrospinal fluid oligoclonal immunoglobulins in patients with multiple sclerosis.

One hundred patients with possible, probable and definite multiple sclerosis (MS) were examined with somatosensory (SER), visual (VER) and brain stem auditory (BAER) evoked responses. Paired samples of cerebrospinal fluid (CSF) and serum were examined with agarose gel electrophoresis to detect intrathecally synthesized oligoclonal immunoglobin bands. Comparison of the number of abnormal CSF and evoked response tests showed that the CSF examination was slightly more sensitive in all diagnostic groups when compared to the results of multimodal evoked responses but that the two sets of test were in part supplementary. Oligoclonal immunoglobulin bands in the CSF were present in all patients with a duration of the disease of less than six months. VER seemed to be the most sensitive of the evoked tests in this particular group of patients.

Brain Stem↗