Arrhythmias are common in patients with MD with infrahisian conduction abnormalities. The prophylactic implantation of a pacing system when the HV interval is > or =70 ms seems appropriate. The PM protects the patient against the clinical consequences of paroxysmal profound bradycardia and facilitates the diagnosis and management of frequent paroxysmal tachyarrhythmias.

Prolongation of the HV interval is the most common conduction abnormality in MD and can be reliably recognized only by invasive EP testing. It raises the issue of prophylactic pacing to limit the incidence of sudden death in MD. Atrial and ventricular arrhythmias are often inducible, although their predictive value remains to be determined. Young age emerged as the most powerful predictor of inducible ventricular tachyarrhythmias. Conversely, we found no relationship between ECG or EP abnormalities recorded during invasive testing and the DNA mutation size or severity of peripheral muscle involvement.

Cardiac tachyarrhythmias have rarely been studied in young patients with myotonic dystrophy type 1 (DM1). The authors observed major cardiac rhythm disturbances in 11 patients aged 10 to 18 years. Tachyarrhythmic events were more frequent than impulse conduction disorders. Wide variations in CTG expansion were observed among the population. Since physical exercise was a prominent arrhythmogenic factor, systematic exercise tests with EKG monitoring may be indicated in young patients with DM1.

The relationship between MR morphologic and functional RV abnormalities and EP testing suggests potential for the use of MR imaging as a noninvasive method to estimate the individual risk of arrhythmia in patients with myotonic dystrophy.

A case-referent study was undertaken to look for occupational risk factors among patients with glioma treated in a neurological hospital in Paris between 1975 and1984. In the study group were 125 men with gliomas (aged < 65) and 238 patients (also < 65) admitted for nonneoplastic, non-malformative vascular diseases in the same department during the same period constituting the reference group. All diagnoses were confirmed by tomodensitometry. Information on occupational history was obtained from a postal questionnaire and from medical records. Comparison ofcases and referents showed a significant excess risk among teachers (OR = 4-1) and a raised risk among wood workers (OR = 1 6). Four ofnine cases ofglioma who had been employed as wood workers reported that a colleague had suffered from glioma (those reports were confirmed by hospital records). None were reported among 11 referent wood workers. Using a complementary questionnaire on wood work, exposure assessment to wood preservatives and solvents showed that frequent exposure to organochlorine wood preservatives and to organic solvents occurred more often among cases than referent wood workers (p < 0-1O).

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RÉSUMÉLe décret travailleur traite des dispositions réglementaires relatives à la prévention des risques liés aux travaux sous rayonnements ionisants. À ce titre, il complète le système réglementaire existant qui traite de la prévention des risques en général.