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V Stojanovic

Publications and source records attributed to V Stojanovic.

7 recordsLinked to original sources

The features of myasthenia gravis with autoantibodies to MuSK.

OBJECTIVES: To determine if myasthenia gravis (MG) with antibodies to MuSK is a distinct subgroup of seronegative MG. METHODS: We assayed antibodies to muscle specific tyrosine kinase (MuSK) in 55 MG patients who had no antibodies to acetylcholine receptors and looked for the specific phenotype, comparing clinical features of anti-MuSK positive and anti-MuSK negative MG patients. RESULTS: MG with anti-MuSK antibodies was characterised by a striking prevalence of female patients (15 women, two men). Age at onset ranged from 22 to 52 years, with 70.6% of patients presenting at < 40 years of age. The majority of patients (82.4%) had prevalent involvement of facial and bulbar muscles. One third of them did not respond well to anticholinesterase drugs. Steroid immunosuppression was effective in eight patients (44.4%). Nine patients underwent thymectomy; six of these had no thymus pathology, while three had a hyperplastic thymus. At the end of the observation period, six (35.3%) patients were in remission, five (29.4%) improved, four (23.6%) did not change, and two (11.7%) had died. CONCLUSIONS: MG patients with antibodies to MuSK have characteristic clinical features that are different from features of the remaining seronegative MG patients. This emphasises the predictive value of anti-MuSK antibody analysis in seronegative MG patients.

Adult↗

Hereditary persistent distal cramps.

A disease consisting of persistent muscle cramps involving distal muscle groups that occurred in 12 members of the same family is described. The cramps appeared on exertion and in full relaxation or during sleep. In the third generation they appeared in the second decade; in the fourth and fifth generations in childhood with higher frequency and intensity of cramps. The disease is not sex linked and seems to be dominantly inherited. Electromyography showed no myotonic response on insertion. Motor unit potentials were normal. Continual waxing and waning electrical discharges corresponding to clinically visible contractions of parts of the muscles were present. Repetitive nerve stimulation caused no change in the amplitude of evoked muscle potentials. On spinal anaesthesia or nerve block the muscle contractions continued but became painless. The movements were only stopped with local infiltration of anaesthetic into the muscle. There were no cramps on ischaemic work. Drug studies revealed no benefit on carbamazepine, slight relief with meprobamate, and complete disappearance with potassium chloride. The remission outlasted the treatment for three months and then cramps of milder degree reappeared. Repeated potassium chloride treatment was not effective. The cramps increased on hydrochlorothiazide, and 12 hours after spinal anaesthesia. In the authors' opinion the disease should be considered as not belonging to any known nosological entity.

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