- •We established a screening tool for behavioral symptoms for Japanese ALS/FTD cases.
- •The tool was efficacious for Japanese patients same as the original English version.
- •Combining other measures, the tool revealed some characteristics of Japanese ALS/FTD.
Amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD) share common clinical, genetic and neuropathological features. Some ALS patients have behavioral/personality changes, which could result in significant obstacles in the care provided by family members and caregivers. An easy screening tool would contribute greatly to the evaluation of these symptoms.
We translated the ALS-FTD-Questionnaire, developed in the Netherlands, into Japanese (ALS-FTD-Q-J) and examined the clinimetric properties (internal consistency, construct and clinical validity). Patients with ALS and/or behavioral variant FTD (bvFTD) were evaluated alongside healthy controls in this multicenter study. All ALS patients, regardless of bvFTD status, were further evaluated by the frontal behavioral inventory (FBI) and for frontal/executive function, cognition, anxiety/depression, and motor functions.
Data from 146 subjects were analyzed: ALS (92), ALS-bvFTD (6), bvFTD (16), and healthy controls (32). The internal consistency of the ALS-FTD-Q-J was good (Cronbach α = 0.92). The ALS-FTD-Q-J showed construct validity as it exhibited a high correlation with the FBI (r = 0.79). However, correlations were moderate with anxiety/depression and low with cognitive scales, in contrast to the original report, i.e. a moderate correlation with cognition and a low correlation with anxiety/depression. The ALS-FTD-Q-J discriminated ALS patients from (ALS-)bvFTD patients and controls. Thus, the ALS-FTD-Q-J is useful for evaluating Japanese ALS/FTD patients.
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- Are amyotrophic lateral sclerosis patients cognitively normal?.Neurology. 2003; 60: 1094-1097
- Prevalence and patterns of cognitive impairment in sporadic ALS.Neurology. 2005; 65: 586-590
- The cognitive profile of ALS: a systematic review and meta-analysis update.J. Neurol. Neurosurg. Psychiatry. 2015;
- How common are behavioural changes in amyotrophic lateral sclerosis?.Amyotroph. Lateral Scler. 2011; 12: 45-51
- The syndrome of cognitive impairment in amyotrophic lateral sclerosis: a population-based study.J. Neurol. Neurosurg. Psychiatry. 2012; 83: 102-108
- The ALS-FTD-Q: a new screening tool for behavioral disturbances in ALS.Neurology. 2012; 79: 1377-1383
- The prevalence of frontotemporal dementia.Neurology. 2002; 58: 1615-1621
- Familial aggregation in frontotemporal dementia.Neurology. 1998; 50: 1541-1545
- C9orf72 in Japanese amyotrophic lateral sclerosis (ALS).Rinsho Shinkeigaku. 2013; 53: 1074-1076
- Japanese amyotrophic lateral sclerosis patients with GGGGCC hexanucleotide repeat expansion in C9ORF72.J. Neurol. Neurosurg. Psychiatry. 2013; 84: 398-401
- Analysis of C9orf72 repeat expansion in 563 Japanese patients with amyotrophic lateral sclerosis.Neurobiol. Aging. 2012; 33 (e11–6): 2527
- Analyses of the MAPT, PGRN, and C9orf72 mutations in Japanese patients with FTLD, PSP, and CBS.Parkinsonism Relat. Disord. 2013; 19: 15-20
- Frequency of the C9orf72 hexanucleotide repeat expansion in patients with amyotrophic lateral sclerosis and frontotemporal dementia: a cross-sectional study.Lancet Neurol. 2012; 11: 323-330
- Expanded GGGGCC hexanucleotide repeat in noncoding region of C9ORF72 causes chromosome 9p-linked FTD and ALS.Neuron. 2011; 72: 245-256
- El Escorial revisited: revised criteria for the diagnosis of amyotrophic lateral sclerosis.Amyotroph. Lateral Scler. Other Motor Neuron Disord. 2000; 1: 293-299
- Frontotemporal lobar degeneration: a consensus on clinical diagnostic criteria.Neurology. 1998; 51: 1546-1554
- Sensitivity of revised diagnostic criteria for the behavioural variant of frontotemporal dementia.Brain. 2011; 134: 2456-2477
- Frontal behavioral inventory: diagnostic criteria for frontal lobe dementia.Can. J. Neurol. Sci. 1997; 24: 29-36
- The Montreal cognitive assessment, MoCA: a brief screening tool for mild cognitive impairment.J. Am. Geriatr. Soc. 2005; 53: 695-699
- The FAB: a frontal assessment battery at bedside.Neurology. 2000; 55: 1621-1626
- Phonological fluency is uniquely impaired in Japanese-speaking schizophrenia patients: confirmation study.Psychiatry Clin. Neurosci. 2011; 65: 672-675
- The hospital anxiety and depression scale.Acta Psychiatr. Scand. 1983; 67: 361-370
- Performance of the amyotrophic lateral sclerosis functional rating scale (ALSFRS) in multicenter clinical trials.J. Neurol. Sci. 1997; 152: S1-S9
- Clinicopathologic study on an ALS family with a heterozygous E478G optineurin mutation.Acta Neuropathol. 2011; 122: 223-229
- Hanja (ideogram) alexia and agraphia in patients with semantic dementia.Neurocase. 2010; 16: 146-156
Published online: May 18, 2016
Accepted: May 17, 2016
Received in revised form: April 18, 2016
Received: January 19, 2016
© 2016 Elsevier B.V. All rights reserved.