BACKGROUND: Patients with ALS are often told that the disease spares cognition; however, recent evidence suggests deficits in frontal executive skills occur in a sizable minority of ALS patients. In many instances, the frontal executive deficits represent the co-occurrence of frontotemporal lobar dementia (FTLD) and ALS. METHODS: Word generation, a simple frontal task that takes <2 minutes, was tested in 100 consecutive patients with ALS seen in the authors' multidisciplinary clinic. Any patient with a prior dementia diagnosis was excluded from the study. A subset of 44 patients agreed to undergo further neuropsychological testing and clinical interview to confirm or deny a diagnosis of dementia. RESULTS: Diminished word generation was found in one-third. Of the patients with abnormal word generation who agreed to further evaluation, nearly all were shown to meet research criteria for FTLD. In addition, one-quarter of the patients with normal word generation who agreed to further evaluation met research criteria for FTLD; these patients had new-onset personality changes. CONCLUSIONS: This study suggests that frontal executive deficits are present in half of ALS patients, many of whom meet strict research criteria for FTLD. Word generation tests are a useful screening tool in this cohort.
BACKGROUND:Patients with ALS are often told that the disease spares cognition; however, recent evidence suggests deficits in frontal executive skills occur in a sizable minority of ALSpatients. In many instances, the frontal executive deficits represent the co-occurrence of frontotemporal lobar dementia (FTLD) and ALS. METHODS:Word generation, a simple frontal task that takes <2 minutes, was tested in 100 consecutive patients with ALS seen in the authors' multidisciplinary clinic. Any patient with a prior dementia diagnosis was excluded from the study. A subset of 44 patients agreed to undergo further neuropsychological testing and clinical interview to confirm or deny a diagnosis of dementia. RESULTS: Diminished word generation was found in one-third. Of the patients with abnormal word generation who agreed to further evaluation, nearly all were shown to meet research criteria for FTLD. In addition, one-quarter of the patients with normal word generation who agreed to further evaluation met research criteria for FTLD; these patients had new-onset personality changes. CONCLUSIONS: This study suggests that frontal executive deficits are present in half of ALSpatients, many of whom meet strict research criteria for FTLD. Word generation tests are a useful screening tool in this cohort.
Authors: P N Leigh; S Abrahams; A Al-Chalabi; M-A Ampong; L H Goldstein; J Johnson; R Lyall; J Moxham; N Mustfa; A Rio; C Shaw; E Willey Journal: J Neurol Neurosurg Psychiatry Date: 2003-12 Impact factor: 10.154
Authors: Nicholas T Olney; Madeleine S Goodkind; Catherine Lomen-Hoerth; Patrick K Whalen; Craig A Williamson; Deborah E Holley; Alice Verstaen; Laurel M Brown; Bruce L Miller; John Kornak; Robert W Levenson; Howard J Rosen Journal: Brain Date: 2011-12-06 Impact factor: 13.501
Authors: Mariely DeJesus-Hernandez; Ian R Mackenzie; Bradley F Boeve; Adam L Boxer; Matt Baker; Nicola J Rutherford; Alexandra M Nicholson; NiCole A Finch; Heather Flynn; Jennifer Adamson; Naomi Kouri; Aleksandra Wojtas; Pheth Sengdy; Ging-Yuek R Hsiung; Anna Karydas; William W Seeley; Keith A Josephs; Giovanni Coppola; Daniel H Geschwind; Zbigniew K Wszolek; Howard Feldman; David S Knopman; Ronald C Petersen; Bruce L Miller; Dennis W Dickson; Kevin B Boylan; Neill R Graff-Radford; Rosa Rademakers Journal: Neuron Date: 2011-09-21 Impact factor: 17.173
Authors: Bob Olsson; Erik Portelius; Nicholas C Cullen; Åsa Sandelius; Henrik Zetterberg; Ulf Andreasson; Kina Höglund; David J Irwin; Murray Grossman; Daniel Weintraub; Alice Chen-Plotkin; David Wolk; Leo McCluskey; Lauren Elman; Leslie M Shaw; Jon B Toledo; Jennifer McBride; Pilar Hernandez-Con; Virginia M-Y Lee; John Q Trojanowski; Kaj Blennow Journal: JAMA Neurol Date: 2019-03-01 Impact factor: 18.302