Familial amyotrophic lateral sclerosis with dementia: a second canadian family.

A family with adult-onset amyotrophic lateral sclerosis (ALS) and dementia is described. The father presented with lower motor neurone disease affecting the legs. A son presented with bulbar paralysis. Neither developed dementia. A daughter presented with spastic right lower limb weakness. Two years later, she developed a rapidly progressive mental disorder. Her cortical pathology had a distinctive fronto-temporal distribution. The rarity of published reports on ALS with dementia in families outside the Western Pacific and the factor of parental consanguinity conspired to make genetic counseling for this family difficult.