channelopathy ͉ FEB3 locus ͉ convulsions ͉ epilepsy ͉ neuronal excitability I t has long been known that there is a major genetic component in the etiology of febrile seizures (FS), and an autosomaldominant (AD) inheritance with incomplete penetrance has been proposed in large pedigrees or groups of families with FS (1). Six loci for familial FS have been reported, but no genes were identified; they have been mapped at chromosomes 8q13-21

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DJ-1 gene mutations have been found to cause early-onset Parkinson's disease. We report a family from southern Italy with three brothers affected by early-onset parkinsonism, dementia, and amyotrophic lateral sclerosis. Molecular analysis of the DJ-1 gene in two living patients showed a novel homozygous mutation in exon 7 (E163K) and a new homozygous mutation (g.168_185dup) in the promoter region of the gene. Both mutations cosegregated with the disease and were detected in a heterozygous state in the patients' mother and their healthy siblings. Our findings expand the spectrum of clinical presentations associated with mutations in DJ-1 gene.