Journal of neurology
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Journal of neurology · Jan 1985
Antimyotonic therapy with tocainide under ECG control in the myotonic dystrophy of Curschmann-Steinert.
Ten patients suffering from advanced myotonic dystrophy with severe myotonic symptoms were treated with 800-1200 mg/day of the anti-arrhythmic drug tocainide (Xylotocan). All patients reported a marked subjective improvement of myotonia, which was confirmed by objective tests. Except for a slight QT-prolongation in one patient, the ECG was not significantly altered by the treatment. ⋯ In these patients suffering from myotonic dystrophy with typical cardiomyopathy no deleterious effects of the drug were observed, especially no cardiac arrhythmias which would have necessitated interruption of treatment. Therefore, the authors recommend symptomatic therapy with tocainide for myotonia and paramyotonia congenita, as well as in myotonic dystrophy patients suffering from marked myotonic stiffness. ECG and 24-h ECG should be carefully recorded as necessary in any treatment with anti-arrhythmic drugs.
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Journal of neurology · Jan 1985
Case ReportsMyasthenia gravis associated with adrenocortical insufficiency. Report of two cases.
Two cases of Addison's disease associated with myasthenia gravis are reported. This association has been described only rarely in the literature. ⋯ It is most likely that the origin of the adrenocortical insufficiency in the second case is tuberculous. The pathogenetic mechanism of these associations is briefly discussed.
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Single drug therapy with either phenytoin or primidone resulted in complete seizure control in 11 of 35 patients (31%) referred to an epilepsy clinic for treatment of uncontrolled chronic epilepsy with complex-partial seizures. Complete seizure control was associated with an increase in the mean plasma concentrations from 14 micrograms/ml to 23 micrograms/ml phenytoin and from 34 micrograms/ml to 40 micrograms/ml phenobarbitone with no change in the antiepileptic drug. ⋯ Non-compliance was admitted by eight patients (23%). Optimum single drug therapy is of considerable clinical value in intractable epilepsy with complex-partial seizures.
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In a series of 109 carotid arterial reconstructions cranial nerve injury was observed in 14 of 102 patients. Ipsilateral peripheral hypoglossal nerve injury occurred in nine patients with carotid occlusive disease. The marginal mandibular nerve was injured in three patients and recurrent laryngeal nerve dysfunction was noted in four patients. ⋯ The nerves are injured by retraction to clear the operative field or by postoperative haematoma. Risk factors include crossing of the hypoglossal nerve close to the carotid bifurcation or procedures requiring long arteriotomy or skeletonization of the internal carotid artery. Unilateral cranial nerve injury is usually mild but will require delay of controlateral carotid reconstruction to avoid severe bilateral cranial nerve palsy.
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Journal of neurology · Jan 1981
Case ReportsCerebral disseminated coagulation. An encephalitis-like syndrome in a boy with dissecting aortic aneurysm.
A boy was suspected of having meningoencephalitis probably of viral origin, although all microbiological, including virological, tests were negative. Postmortem examination revealed a dissecting aortic aneurysm and disseminated intravascular coagulation involving the central nervous system exclusively. The clinical evolution, the age of the patient, and the association of DAA with this cerebral disseminated coagulation is very unusual.