RT Journal Article
SR Electronic
T1 The syndrome of cognitive impairment in amyotrophic lateral sclerosis: a population-based study
JF Journal of Neurology, Neurosurgery & Psychiatry
JO J Neurol Neurosurg Psychiatry
FD BMJ Publishing Group Ltd
SP 102
OP 108
DO 10.1136/jnnp-2011-300188
VO 83
IS 1
A1 Julie Phukan
A1 Marwa Elamin
A1 Peter Bede
A1 Norah Jordan
A1 Laura Gallagher
A1 Susan Byrne
A1 Catherine Lynch
A1 Niall Pender
A1 Orla Hardiman
YR 2012
UL http://jnnp.bmj.com/content/83/1/102.abstract
AB Background Despite considerable interest, the population-based frequency, clinical characteristics and natural history of cognitive impairment in amyotrophic lateral sclerosis (ALS) are not known.Methodology The authors undertook a prospective population-based study of cognitive function in 160 incident Irish ALS patients and 110 matched controls. Home-based visits were conducted to collect demographic and neuropsychological data. Patients were classified using the recently published consensus criteria and by a domain-based classification of both executive and non-executive cognitive processes.Results 13.8% of patients fulfilled the Neary criteria for frontotemporal dementia. In addition, 34.1% of ALS patients without evidence of dementia fulfilled the recently published consensus criteria for cognitive impairment. Non-demented ALS patients had a significantly higher frequency of impairment in language and memory domains compared to healthy controls. These deficits occurred primarily in patients with executive dysfunction. 14% of ALS patients had evidence of cognitive impairment without executive dysfunction, and no cognitive abnormality was detected in almost half the cohort (46.9%).Conclusion Co-morbid dementia occurs in approximately 14% of patients with a new diagnosis of ALS. Cognitive impairment, predominantly but not exclusively in the form executive dysfunction, is present in more than 40% of ALS patients who have no evidence of dementia. Cognitive impairment in ALS is not a universal feature, and its manifestations may be more heterogeneous than previously recognised.