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Familial amyotrophic lateral sclerosis with dementia: A second Canadian family
Author(s) -
Pinsky Leonard,
Finlayson M. H.,
Libman I.,
Scott B. H.
Publication year - 1975
Publication title -
clinical genetics
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.543
H-Index - 102
eISSN - 1399-0004
pISSN - 0009-9163
DOI - 10.1111/j.1399-0004.1975.tb00317.x
Subject(s) - amyotrophic lateral sclerosis , dementia , daughter , consanguinity , family history , medicine , spastic , disease , psychology , pediatrics , psychiatry , pathology , cerebral palsy , biology , evolutionary biology
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 consangcinity conspired to make genetic counselling for this family difficult.