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Philippe Corcia, MD, PhD; Helene-Farnase Jafari-Schluep, MD; Dominique Lardillier, MD; Hassan Mazyad, MD; Pieric Giraud, MD; Pierre Clavelou, MD, PhD; Jean Pouget, MD; William Camu, MD, PhD

Arch Neurol. 2003;60:553-557.

Background  The origin of amyotrophic lateral sclerosis (ALS) remains largely unknown but seems to be multifactorial. We believe that ALS clusters may help clinicians understand or analyze the role of environmental factors in ALS pathogenesis.

Objective  To describe a cluster of conjugal ALS in southeastern France.

Patients  We describe 9 couples in which both spouses were affected by ALS. Eight of the 9 had lived in southeastern France. In all cases, the spouses were married for more than 10 years. Three couples lived in the same département and 2 of them in the same city.

Results  To our knowledge, such a large cluster of conjugal ALS cases has not been previously reported. No precise environmental factors could be identified at the origin of these conjugal cases.

Conclusions  We suggest that genetic and environmental factors, or both, may explain the occurrence of this cluster of ALS.

From the Departments of Neurology, University Hospital Bretonneau, Tours (Dr Corcia), Hôpital Gui de Chauliac, Montpellier (Drs Jafari-Schluep, Mazyad, and Camu), CHU La Timone, Marseille (Drs Lardillier and Pouget), Hôpitaux de Lyon, Lyon (Dr Giraud), and Hôpital Nord Cebazat, Clermont-Ferrand (Dr Clavelou), France.

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