Consensus criteria for the diagnosis of frontotemporal cognitive and behavioural syndromes in amyotrophic lateral sclerosis

References

• Strong MJ. The syndromes of frontotemporal dysfunction in amyotrophic lateral sclerosis. Amyotroph Lateral Scler. 2008; 9: 323–38
   PubMed Web of Science ®Google Scholar
• Hodges JR, Miller B. The classification, genetics and neuropathology of frontotemporal dementia. Introduction to the special topic papers: part 1. Neurocase. 2001; 7: 31–5
   PubMed Web of Science ®Google Scholar
• Rascovsky K, Hodges JR, Kipps CM, Johnson JK, Seeley WW, Mendez MF, et al. Diagnostic criteria for the behavioural variant of frontotemporal dementia (bvFTD): current limitations and future directions. Alzheimer Dis Assoc Disord. 2007; 21: S14–8
   PubMed Web of Science ®Google Scholar
• Lomen-Hoerth C, Strong MJ. Cognition in amyotrophic lateral sclerosis. Amyotrophic Lateral Sclerosis, H Mitsumoto, S Przedborksi, P Gordon, M de Bene. Marcel Dekker, London 2006; 115–38
   Google Scholar
• Roberson ED, Hesse JH, Rose KD, Slama H, Johnson JK, Yaffe K, et al. Frontotemporal dementia progresses to death faster than Alzheimer's disease. Neurology. 2005; 65: 719–25
   PubMed Web of Science ®Google Scholar
• Rascovsky K, Salmon DP, Lipton AM, Leverenz JB, de Carli C, Jagust WJ, et al. Rate of progression differs in frontotemporal dementia and Alzheimer's disease. Neurology. 2005; 65: 397–403
   PubMed Web of Science ®Google Scholar
• Olney RK, Murphy J, Forshew D, Garwood E, Miller BL, Langmore S, et al. The effects of executive and behavioural dysfunction on the course of ALS. Neurology. 2005; 65: 1774–7
   PubMed Web of Science ®Google Scholar
• Poloni M, Capitani E, Mazzini L, Ceroni M. Neuropsychological measures in amyotrophic lateral sclerosis and their relationship with CT scan-assessed cerebral atrophy. Acta Neurol Scand. 1986; 74: 257–60
   PubMed Web of Science ®Google Scholar
• Frank B, Haas J, Heinze HJ, Stark E, Munte TF. Relation of neuropsychological and magnetic resonance findings in amyotrophic lateral sclerosis: evidence for subgroups. Clin Neurol Neurosurg. 1997; 99: 79–86
   PubMed Web of Science ®Google Scholar
• Ringholz GM, Appel SH, Bradshaw M, Cooke NA, Mosnik DM, Schulz PE. Prevalence and patterns of cognitive impairment in sporadic ALS. Neurology. 2005; 65: 586–90
   PubMed Web of Science ®Google Scholar
• Lomen-Hoerth C, Murphy J, Langmore S, Kramer JH, Olney RK, Miller B. Are amyotrophic lateral sclerosis patients cognitively normal?. Neurology. 2003; 60: 1094–7
   PubMed Web of Science ®Google Scholar
• Neumann M, Sampathu DM, Kwong LK, Truax AC, Micsenyi MC, Chou TT, et al. Ubiquitinated TDP-43 in frontotemporal lobar degeneration and amyotrophic lateral sclerosis. Science. 2006; 314: 130–3
   PubMed Web of Science ®Google Scholar
• Arai T, Hasegawa M, Akiyama H, Ikeda K, Nonaka T, Mori H, et al. TDP-43 is a component of ubiquitin-positive tau-negative inclusions in frontotemporal lobar degeneration and amyotrophic lateral sclerosis. Biochem Biophys Res Comm. 2006; 351: 602–11
   PubMed Web of Science ®Google Scholar
• Kwong LK, Neumann M, Sampathu DM, Lee VMY, Trojanowski JQ. TDP-43 proteinopathy: the neuropathology underlying major forms of sporadic and familial frontotemporal lobar degeneration and motor neuron disease. Acta Neuropathol. 2007; 114: 63–70
   PubMed Web of Science ®Google Scholar
• Neumann M, Mackenzie IR, Cairns NJ, Boyer PJ, Markesbery WR, Smith CD, et al. TDP-43 in the ubiquitin pathology of frontotemporal dementia with VCP gene mutations. J Neuropathol Exp Neurol. 2007; 66: 152–7
   PubMed Web of Science ®Google Scholar
• Wilson CM, Grace GM, Munoz DG, He BP, Strong MJ. Cognitive impairment in sporadic ALS. A pathological continuum underlying a multisystem disorder. Neurology. 2001; 57: 651–7
   PubMed Web of Science ®Google Scholar
• Yang W, Sopper MM, Leystra-Lantz C, Strong MJ. Microtubule-associated tau protein positive neuronal and glial inclusions in amyotrophic lateral sclerosis. Neurology. 2003; 61: 1766–73
   PubMed Web of Science ®Google Scholar
• Yoshida M. Amyotrophic lateral sclerosis with dementia: the clinicopathological spectrum. Neuropathology. 2004; 24: 87–102
   PubMed Web of Science ®Google Scholar
• Jackson M, Lennox G, Lowe J. Motor neuron disease-inclusion dementia. Neurodegeneration. 1996; 5: 339–50
   PubMedGoogle Scholar
• Woulfe J, Kertesz A, Munoz DG. Frontotemporal dementia with ubiquitinated cytoplasmic and intranuclear inclusions. Acta Neuropathol. 2001; 102: 94–100
   PubMed Web of Science ®Google Scholar
• Murphy JM, Henry RG, Langmore S, Kramer JH, Miller BL, Lomen-Hoerth C. Continuum of frontal lobe impairment in amyotrophic lateral sclerosis. Arch Neurol. 2007; 64: 530–4
   PubMedGoogle Scholar
• Guedj E, Le Ber I, Lacomblez I, Dubois B, Verpillat P, Didic M, et al. Brain spect perfusion of frontotemporal dementia associated with motor neuron disease. Neurology. 2007; 69: 488–90
   PubMed Web of Science ®Google Scholar
• Lomen-Hoerth C, Anderson T, Miller B. The overlap of amyotrophic lateral sclerosis and frontotemporal dementia. Neurology. 2002; 59: 1077–9
   PubMed Web of Science ®Google Scholar
• World Federation of Neurology Research Group on Neuromuscular Disease. El Escorial World Federation of Neurology criteria for the diagnosis of amyotrophic lateral sclerosis. J Neurol Sci. 1994;124(Suppl):96–107.
   PubMed Web of Science ®Google Scholar
• Brooks, BR, Miller, RG, Swash, M, Munsat, TL, for the World Federation of Neurology Research Group on Motor Neuron Diseases. El Escorial revisited: revised criteria for the diagnosis of amyotrophic lateral sclerosis. Amyotroph Lateral Scler Other Motor Neuron Disord. 2000;1:293–9.
   PubMed Web of Science ®Google Scholar
• Chaudhari KR, Crump S, Al-Sarraj S, Anderson V, Cavanagh J, Leigh PN. The validation of El Escorial criteria for the diagnosis of amyotrophic lateral sclerosis: a clinicopathological study. J Neurol Sci. 1995; 129: 11–2
   PubMed Web of Science ®Google Scholar
• Ross MA, Miller RG, Berchert L, Parry GJ, Barohn RJ, Armon C, et al. Toward earlier diagnosis of amyotrophic lateral sclerosis. Neurology. 1998; 50: 768–72
   PubMed Web of Science ®Google Scholar
• Phukan J, Pender NP, Hardiman O. Cognitive impairment in amyotrophic lateral sclerosis. Lancet Neurology. 2007; 6: 994–1003
   PubMed Web of Science ®Google Scholar
• Wheaton MW, Salamone AR, Mosnik DM, McDonald RO, Appel SH, Schmolck HI, et al. Cognitive impairment in familial ALS. Neurology. 2007; 69: 1411–7
   PubMed Web of Science ®Google Scholar
• Ince PG, Tomkins J, Slade JY, Thatcher NM, Shaw PJ. Amyotrophic lateral sclerosis associated with genetic abnormalities in the gene encoding Cu/Zn superoxide dismutase: molecular pathology of five new cases, and comparison with previous reports and 73 sporadic cases of ALS. J Neuropathol Exp Neurol. 1998; 57: 895–904
   PubMed Web of Science ®Google Scholar
• Rosen DR, Siddique T, Patterson D, Figlewicz DA, Sapp P, Hentati A, et al. Mutations in Cu/Zn superoxide dismutase gene are associated with familial amyotrophic lateral sclerosis. Nature. 1993; 362: 59–62
   PubMed Web of Science ®Google Scholar
• Masè G, Ros S, Gemma A, Bonfigli L, Carraro N, Cazzato G, et al. ALS with variable phenotypes in a six-generation family caused by leu144phe mutation in the SOD1 gene. J Neurol Sci. 2001; 191: 11–8
   PubMed Web of Science ®Google Scholar
• Battistini S, Giannini F, Greco G, Bibbò G, Ferrera L, Marini V, et al. SOD1 mutations in amyotrophic lateral sclerosis. Results from a multicentre Italian study. J Neurol. 2005; 252: 782–8
   PubMed Web of Science ®Google Scholar
• Wicks, P, Abrahams, S, Papps, B, Al-Chalabi, A, Shaw, C, Leigh, PN, , et al. SOD1 and cognitive dysfunction in familial amyotrophic lateral sclerosis. J Neurol. 2008; in press.
   Google Scholar
• Brown J, Ashworth A, Gydesen S, Sorensen A, Rossor M, Hardy J, et al. Familial non-specific dementia maps to chromosome 3. Hum Mol Genet. 1995; 4: 1625–8
   PubMed Web of Science ®Google Scholar
• Parkinson N, Ince PG, Smith MO, Highley R, Skibinski G, Andersen PM, et al. ALS phenotypes with mutations in CHMP2B (charges multivesicular body protein 2B). Neurology. 2006; 67: 1074–7
   PubMed Web of Science ®Google Scholar
• Cannon A, Baker M, Boeve B, Josephs K, Knopman D, Petersen R, et al. CHMPB2 mutations are not a common cause of frontotemporal lobar degeneration. Neurosci Lett. 2006; 398: 83–4
   PubMed Web of Science ®Google Scholar
• Blair, IP, Vance, C, Durnall, JC, Williams, KL, Thoeng, A, Shaw, CE, , et al. CHMPB2 mutations are not a common cause of familial or sporadic ALS. J Neurol Neurosurg Psychiat. 2007; doi: 10.1136/jnnp/2007.140541.
   Web of Science ®Google Scholar
• Gellera C, Colobrita C, Ticozzi N, Castellotti B, Bragato C, Ratti A, et al. Identification of new ANG gene mutations in a large cohort of Italian patients with amyotrophic lateral sclerosis. Neurogenetics. 2008; 9: 33–40
   PubMed Web of Science ®Google Scholar
• Hutton M, Lendon CL, Rizzu P, Baker M, Froelich S, Houlden H, et al. Association of missense and 5′′-splice-site mutations in tau with the inherited dementia FTDP-17. Nature. 1998; 393: 702–5
   PubMed Web of Science ®Google Scholar
• Lynch T, Sano M, Marder KS, Bell KL, Foster NL, Defendini RF, et al. Clinical characteristics of a family with chromosome 17-linked disinhibition-dementia-Parkinsonism-amyotrophy complex. Neurology. 1994; 44: 1878–84
   PubMed Web of Science ®Google Scholar
• Wilhelmsen KC, Forman MS, Rosen HJ, Alving LI, Goldman J, Feiger J, et al. 17q-linked frontotemporal dementia-amyotrophic lateral sclerosis without tau mutations with tau and (-synuclein inclusions. Arch Neurol. 2004; 61: 398–406
   PubMed Web of Science ®Google Scholar
• Zarranz JJ, Ferrer I, Lezcano E, Forcadas MI, Eizaguirre B, Atarés B, et al. A novel mutation (K317M) in the MAPT gene causes FTDP and motor neuron disease. Neurology. 2005; 64: 1578–85
   PubMed Web of Science ®Google Scholar
• Kumar-Singh S, van Broeckhoven C. Frontotemporal lobar degeneration: current concepts in the light of recent advances. Brain Pathol. 2007; 17: 104–13
   PubMed Web of Science ®Google Scholar
• Le Ber I, Camuzat A, Hannequin D, Pasquier F, Guedj E, Rovelet-Lecrux A, et al. Phenotype variability in progranulin mutation carriers: a clinical, neuropsychological, imaging and genetic study. Brain. 2008; 131: 746
   Web of Science ®Google Scholar
• Pickering-Brown SM, Rollinson S, Du Plessis D, Morrison KE, Varma A, Richardson AMT, et al. Frequency and clinical characteristics of progranulin mutation carriers in the Manchester frontotemporal lobar degeneration cohort: comparison with patients with MAPT and no known mutations. Brain. 2008; 131: 721–31
   PubMed Web of Science ®Google Scholar
• López de Munain A, Alzualde A, Gorostidi A, Otaegui D, Ruiz-Martinez J, Indakoetxea B, et al. Mutations in progranulin gene: clinical, pathological, and ribonucleic acid expression findings. Biol Psychiatry. 2008; 63: 946–52
   PubMed Web of Science ®Google Scholar
• Schymick JC, Yang Y, Andersen PM, Vonsattel JP, Greenway M, Momeni P, et al. Progranulin mutations and amyotrophic lateral sclerosis or amyotrophic lateral sclerosis-frontotemporal dementia phenotypes. J Neurol Neurosurg Psychiat. 2007; 78: 754–6
   PubMed Web of Science ®Google Scholar
• Spina S, Murrell JR, Huey ED, Wassermann EM, Pietrini P, Baraibar MA, et al. Clinicopathological features of frontotemporal dementia with progranulin sequence variation. Neurology. 2007; 68: 820–7
   PubMed Web of Science ®Google Scholar
• Hamilton RL, Bowser R. Alzheimer's disease pathology in amyotrophic lateral sclerosis. Acta Neuropathol. 2004; 107: 515–22
   PubMed Web of Science ®Google Scholar
• Neary D, Snowden JS, Gustafson L, Passant U, Stuss D, Black S, et al. Frontotemporal lobar degeneration. A consensus on clinical diagnostic criteria. Neurology. 1998; 51: 1546–54
   PubMed Web of Science ®Google Scholar
• McKhann GM, Albert MS, Grossman M, Miller B, Dickson D, Trojanowski JQ. Clinical and pathological diagnosis of frontotemporal dementia. Arch Neurol. 2001; 58: 1803–9
   PubMed Web of Science ®Google Scholar
• Gibbons ZC, Richardson A, Neary D, Snowden JS. Behaviour in amyotrophic lateral sclerosis. Amyotroph Lateral Scler. 2008; 9: 67–74
   PubMed Web of Science ®Google Scholar
• Gregory CA, Serra-Mestres J, Hodges JR. Early dementia of the frontal variant of frontotemporal dementia: how sensitive are standard neuroimaging and neuropsychological tests?. Neuropsychiatry Neuropsychol Behav Neurol. 1999; 12: 128–35
   PubMedGoogle Scholar
• Hachinski V, Iadecola C, Petersen RC, Breteler MM, Nyenhuis DL, Black SE, et al. National Institute for Neurological Disorders and Stroke – Canadian Stroke Network vascular cognitive impairment harmonization standards. Stroke. 2006; 37: 2220–41
   PubMed Web of Science ®Google Scholar
• Woolley-Levine S, Moore D, Katz J. What is the best verbal bedside tool to screen for cognitive impairment in ALS?. Amyotroph Lateral Scler. 2007; 8: 101
   PubMedGoogle Scholar
• Abrahams S, Leigh PN, Harvey A, Vythelingum GN, Grise D, Goldstein LH. Verbal fluency and executive dysfunction in amyotrophic lateral sclerosis (ALS). Neuropsychologia. 2000; 38: 734–47
   PubMed Web of Science ®Google Scholar
• Yeudall LT, Fromm D, Reddon JR, Stefanyk WO. Normative data stratified by age and sex for 12 neuropsychological tests. J Clin Psychol. 1986; 42: 918–46
   Web of Science ®Google Scholar
• Crawford JR, Moore JW, Cameron IM. A NART-based equation for the estimation of premorbid perfomance. Br J Clin Psychol. 1992; 31: 327–9
   PubMed Web of Science ®Google Scholar
• Flaherty-Craig C, Eslinger P, Stephens B, Simmons Z. A rapid screening battery to identify frontal dysfunction in patients with ALS. Neurology. 2006; 67: 2070–2
   PubMed Web of Science ®Google Scholar
• Woolley-Levine S, York M, Haring K, Lee C, Goodson W, Struut A, et al. Development of a cognitive behavioural screen for use with ALS patients: preliminary data. Amyotroph Lateral Scler. 2007; 8: 102
   Google Scholar
• Mioshi E, Dawson K, Mitchell J, Arnold R, Hodges JR. The Addenbrooke's Cognitive Examination Revised (ACE-R): a brief cognitive test battery for dementia screening. Int J Geriatr Psychiatry. 2006; 21: 1078–85
   PubMed Web of Science ®Google Scholar
• Nasreddine ZS, Phillips NA, Bédirian V, Charbonneau S, Whitehead V, Collin I, et al. The Montreal Cognitive Assessment, MoCA: a brief screening tool for mild cognitive impairment. J Am Geriatr Soc. 2005; 53: 695–9
   PubMed Web of Science ®Google Scholar
• Kertesz A, Davidson W, Fox H. Frontal behavioural inventory: diagnostic criteria for frontal lobe dementia. Can J Neurol Sci. 1997; 24: 29–36
   PubMed Web of Science ®Google Scholar
• Blair M, Kertesz A, Davis-Faroque N, Hsiung GY, Black SE, Bouchard RW, et al. Behavioural measures in frontotemporal lobar dementia and other dementias: the utility of the frontal behavioural inventory and the neuropsychiatric inventory in a national cohort study. Dement Geriatr Cogn Disord. 2008; 23: 406–15
   Web of Science ®Google Scholar
• Cummings JL, Mega M, Gray K, Rosenberg-Thompson S, Carusi SA, Gornbein J. The neuropsychiatric inventory: comprehensive assessment of psychopathology in dementia. Neurology. 1994; 44: 2308–14
   PubMed Web of Science ®Google Scholar
• Paulsen JS, Stout JC, de la Pena J, Romero R, Twafik-Reddy Z, Swenson MR, et al. Frontal-behavioural syndromes in cortical and subcortical dementia. Assessment. 1996; 3: 327–37
   Google Scholar
• Grossman AB, Woolley-Levine S, Bradley WG, Miller RG. Detecting neurobehavioural changes in amyotrophic lateral sclerosis. Amyotroph Lateral Scler. 2007; 8: 56–61
   PubMed Web of Science ®Google Scholar
• Benton AL, Hamsher KD, Sivan AB. Multilingual aphasia examination: manual of instructions3rd edn. AJA Associates, Iowa City 1994
   Google Scholar
• Delis DC, Kaplan E, Kramer JH. Delis-Kaplan executive function system technical manual. The Psychological Corporation, San Antonio, TX 2001
   Google Scholar
• Morris JC, Heyman A, Mohs RC, Hughes JP, van Belle G, Fillenbaum G, et al. The Consortium to Establish a Registry for Alzheimer's Disease (CERAD). Part I. Clinical and neuropsychological assessment of Alzheimer's disease. Neurology. 1989; 39: 1159–65
   PubMed Web of Science ®Google Scholar
• Heaton R, Chelune GJ, Talley J, Kay GG, Curtiss G. Wisconsin Card Sorting Test (WCST) manual: revised and expanded. Psychological Assessment Resources, Odessa, Fl 1993
   Google Scholar
• Delis DC, Squire LR, Bihrle A, Massman P. Componential analysis of problem-solving ability: performance of patients with frontal lobe damage and amnesic patients on a new sorting test. Neuropsychologia. 1992; 30: 697
   Web of Science ®Google Scholar
• Jensen AR, Rohwer WD, Jr. The Stroop colour-word test: a review. Acta Psychologica. 1966; 25: 36–93
   PubMed Web of Science ®Google Scholar
• Corrigan JD, Hinkeldey MS. Relationships between parts A and B of the Trail Making Test. J Clinic Psychol. 1987; 43: 402–9
   PubMed Web of Science ®Google Scholar
• Rey A. Psycological examination of traumatic encephalopathy. Archieves de Psychologic. 1941; 28: 286–340
   Google Scholar
• Delis DC, Kramer JH, Kaplan E, Ober BA. California Verbal Learning Test manual-adult version (second edition). The Psychological Corporation, New York 2000
   Google Scholar
• Warrington EK. Recognition Memory Test. NFER-Nelson, WindsorUK 1984
   Google Scholar
• Sweet JJ, Demakis GJ, Ricker JH, Millis SR. Diagnostic efficiency and material specifictiy of the Warrington Recognition Memory Test. A collaborative multisite investigation. Arch Clin Neuropsychol. 2000; 15: 301–9
   PubMed Web of Science ®Google Scholar
• Wechsler D. Wechsler Memory Scales-Third Edition (WMS-III). Administration and Scoring Manual. The Psychological Corporation, San Antonia, TX 1997
   Google Scholar
• Kendrick DC, Gibson AJ, Moyes IC. The revised Kendrick battery: clinical studies. Br J Soc Clin Psychol. 1979; 18: 329–40
   PubMed Web of Science ®Google Scholar
• Lezak MD. Neuropsychological Assessment3rd edn. New Oxford Press, New York 1995
   Google Scholar
• Peterson LR, Peterson MJ. Short-term retention of individual verbal items. Journal of Experimental Psychology. 1959; 58: 193–8
   PubMedGoogle Scholar
• Smith A. Symbol digit modalities test. Western Psychological Services, Los Angeles, CA 1991
   Google Scholar
• Gronwall DMA. Paced auditory serial addition task: a measure of recovery from concussion. Percept Mot Skills. 1997; 44: 367–73
   Web of Science ®Google Scholar
• Wechsler D. Wechsler Adult Intelligence Scale3rd edn. The Psychological Corporation, San Antonio, TX 1997
   Google Scholar
• Goodglass H, Kaplan E. Boston Diagnostic Aphasia Examination – 2nd edn. Lea & Febiger, Malvern, PA 1983
   Google Scholar
• McKenna P, Warrington EK. The Graded Naming Test. NFER-Nelson, Windsor 1983
   Google Scholar
• Howard D, Patterson K. Pyramid and Palm Trees. Thames Valley Test Company, Bury St Edmunds, Suffolk 1992
   Google Scholar
• Dunn LM, Dunn ES. Peabody Picture Vocabulary Test-III. MN, Circle Pinns 1997
   Google Scholar
• Dunn LM, Whetton C, Burley J. The British Picture Vocabulary Test. GL Assessment, LondonUK 1997
   Google Scholar
• Bishop D. Test for reception of grammar: version 2 (TROG-2). Pearson Assessment, LondonUK 2003
   Google Scholar
• Woodward JL, Benedict RHB, Roberts VJ, Goldstein FC, Kinner KM, Capruso DX, et al. Short-form alternatives to the Judgement of Lines Orientation test. J Clin Exp Psychol. 1996; 18: 898–904
   PubMed Web of Science ®Google Scholar
• Benton AL, van Allen MW. Contribution to neuropsychological assessment. Oxford University Press, New York 1983
   Google Scholar
• Colarusso RP, Hammill DD. Motor-Free Visual Perception Test – Revised. Academic Therapy Publicaitons, Novato, CA 1996
   Google Scholar
• Warrington EK, James M. Visual Object and Space Perception Battery (VOSP). Pearson Assessment, LondonUK 1991
   Google Scholar
• Bright P, Jaldow E, Kopelman MD. The National Adult Reading Test as a measure of premorbid intelligence: a comparison with estimates derived from demographic variables. JINS. 2002; 8: 847–54
   PubMed Web of Science ®Google Scholar
• Uttl B. North American Adult Reading Test: age norms, reliability, and validity. J Clin Exp Neuropsych. 2002; 24: 1123–37
   PubMed Web of Science ®Google Scholar
• Wechsler D. Wechsler Test of Adult Reading. The Psychological Corporation, San Antonio, TX 2001
   Google Scholar
• Baddeley A, Emslie H, Nimmo-Smith I. Speed and Capacity of Language Processing. Pearson, LondonUK 1992
   Google Scholar
• Saxton JA, Ratcliff G, Dodge H, Pandav R, Baddeley A, Ganguli M. Speed and capacity of language processing test: normative data from an older American community-dwelling sample. Appl Neuropsychol. 2001; 8: 193–203
   PubMedGoogle Scholar
• Beardsall L, Huppert F. Short NART, CCRT and Spot-the-Word: comparisons in older and demented persons. Br J Clin Psychol. 1997; 36: 619–22
   PubMed Web of Science ®Google Scholar
• Wechsler D. Wechsler Abbreviated Scale of Intelligence (WASI). The Psychological Corporation, San Antonio, TX 1999
   Google Scholar
• Axelrod BN. Validity of the Wechsler Abbreviated Scale of Intelligence and other very short forms of estimating intellectual function. Assessment. 2002; 9: 17–23
   PubMed Web of Science ®Google Scholar
• Brink TL, Yesavage JA, Lum O, Heersema P, Adey MB, Rose TL. Screening tests for geriatric depression. Clinical Gerontologist. 1982; 1: 37–44
   Google Scholar
• Hamilton M. A rating scale for depression. J Neurol Neurosurg Psychiatry. 1960; 23: 56–62
   PubMed Web of Science ®Google Scholar
• Beck AT, Ward CH, Mendelson M, Mock J, Erbaugh J. An inventory for measuring depression. Arch Gen Psychiatry. 1961; 4: 561–71
   PubMed Web of Science ®Google Scholar
• Beck AT, Steer RA, Brown GK. Manual for the Beck Depression Inventory-II. Psychological Corporation, San Antonio, Texas 1996
   Google Scholar
• Zigmond AS, Snaith RP. The hospital anxiety and depression scale. Acta Psychiat Scand. 1983; 67: 361–70
   PubMed Web of Science ®Google Scholar
• Nyenhuis DL, Luchetta T, Ymamoto C, Terrien A, Bernardin L, Rao SM, et al. The development, standardization, and initial validation of the Chicago Multiscale Depression Inventory. J Pers Assess. 1998; 70: 386–401
   PubMed Web of Science ®Google Scholar
• Wicks P, Abrahams S, Masi F, Hejda-Forde S, Leigh PN, Goldstein LH. Prevalence of depression in a 12-month consecutive sample of patients with ALS. Eur J Neurol. 2007; 14: 993–1001
   PubMed Web of Science ®Google Scholar
• Strong MJ, Gordon PH. Primary lateral sclerosis, hereditary spastic paraplegia and amyotrophic lateral sclerosis – discrete entities or spectrum?. Amyotroph Lateral Scler Other Motor Neuron Disord. 2005; 6: 8–16
   PubMed Web of Science ®Google Scholar
• Strong MJ. The basic aspects of therapeutics in amyotrophic lateral sclerosis. Pharmacol Ther. 2003; 98: 379–414
   PubMed Web of Science ®Google Scholar
• Strong MJ, Hudson AJ, Alvord WG. Familial amyotrophic lateral sclerosis, 1850–1989: a statistical analysis of the world literature. Can J Neurol Sci. 1991; 18: 45–58
   PubMed Web of Science ®Google Scholar
• Eisen A, Schulzer M, MacNeil M, Pant B, Mak E. Duration of amyotrophic lateral sclerosis is age dependent. Muscle Nerve. 1993; 16: 27–32
   PubMed Web of Science ®Google Scholar
• Jablecki CK, Berry C, Leach J. Survival prediction in amyotrophic lateral sclerosis. Muscle Nerve. 1989; 12: 833–41
   PubMed Web of Science ®Google Scholar
• Worms PM. The epidemiology of motor neuron diseases: a review of recent studies. J Neurol Sci. 2001; 191: 3–9
   PubMed Web of Science ®Google Scholar
• Cashman, NR, Cudkowicz, ME, Davidson, MC, Pioro, EP, Rosenfeld, J and the ALS Patient Care Database . Gender effects on duration and onset age of amyotrophic lateral sclerosis. Amyotroph Lateral Scler Other Motor Neuron Disord. 2001;2:41–2.
   Google Scholar
• Nelson LM, Matkin C, Longstreth WT, Jr, McGuire V. Population-based case control study of amyotrophic lateral sclerosis in western Washington State. II. Diet Am J Epidemiol. 2000; 151: 164–73
   Google Scholar
• Nelson LM, McGuire V, Longstreth WT, Jr, Matkin C. Population-based case-control study of amyotrophic lateral sclerosis in western Washington state. I. Cigarette smoking and alcohol consumption. Am J Epidemiol. 2000; 151: 156–63
   PubMed Web of Science ®Google Scholar
• Mott RT, Dickson DW, Trojanowski JQ, Zhukareva V, Lee VM, Forman M, et al. Neuropathological, biochemical, and molecular characterization of the frontotemporal dementias. J Neuropathol Exp Neurol. 2005; 64: 420–8
   PubMed Web of Science ®Google Scholar
• Josephs KA, Holton JL, Rossort MN, Godbolt AK, Ozawa T, Strand K, et al. Frontotemporal lobar degeneration and ubiquitin immunohistochemistry. Neuropathol Appl Neurobiol. 2004; 30: 369–73
   PubMed Web of Science ®Google Scholar
• Migheli A, Pezzulo T, Attanasio A, Schiffer D. Peripherin immunoreactive structures in amyotrophic lateral sclerosis. Lab Invest. 1993; 68: 185–91
   PubMed Web of Science ®Google Scholar
• Wong N, He BP, Strong MJ. Characterization of neuronal intermediate filament protein expression in cervical spinal motor neurons in sporadic amyotrophic lateral sclerosis (ALS). J Neuropathol Exp Neurol. 2000; 59: 972–82
   PubMed Web of Science ®Google Scholar
• Strong MJ. Neurofilament metabolism in sporadic amyotrophic lateral sclerosis. J Neurol Sci. 1999; 169: 170–7
   PubMed Web of Science ®Google Scholar
• Leigh PN, Dodson A, Swash M, Brion J-P, Anderton BH. Cytoskeletal abnormalities in motor neuron disease. An immunohistochemical study. Brain. 1989; 112: 521–35
   PubMed Web of Science ®Google Scholar
• van Welsem ME, Hogenhuis JA, Meininger V, Metsaars WP, Hauw JJ, Seilhean D. The relationship between Bunina bodies, skein-like inclusions and neuronal loss in amyotrophic lateral sclerosis. Acta Neuropathol. 2002; 103: 583–9
   PubMed Web of Science ®Google Scholar
• Wharton S, Ince PG. Pathology of motor neuron disorders. Motor Neuron Disorders, PJ Shaw, MJ Strong. Butterworth Heinemann, Philadelphia 2003; 17–50, 1st edn.
   Google Scholar
• Noda K, Katayama S, Watanabe C, Yamamura Y, Nakamura S. Gallyas- and tau-positive glial structures in motor neuron disease with dementia. Clin Neuropath. 1999; 18: 218–25
   PubMed Web of Science ®Google Scholar
• Morris HR, Khan MN, Janssen JC, Brown JM, Perez-Tur J, Baker M, et al. The genetic and pathological classification of familial frontotemporal dementia. Arch Neurol. 2001; 58: 1813–6
   PubMedGoogle Scholar
• Caselli RJ, Windebank AJ, Petersen RC, Komori T, Parisi JE, Okazaki E, et al. Rapidly progressing aphasic dementia and motor neuron disease. Ann Neurol. 1993; 33: 200–7
   PubMed Web of Science ®Google Scholar
• Ferrer I, Roig C, Espino A, Peiro G, Matias Guiu X. Dementia of frontal lobe type and motor neuron disease. A Golgi study of the frontal cortex. J Neurol Neurosurg Psychiat. 1991; 54: 932–4
   PubMed Web of Science ®Google Scholar
• Kato S, Oda M, Hayashi H, Kawata A, Shimizu T. Participation of the limbic system and its associated areas in the dementia of amyotrophic lateral sclerosis. J Neurol Sci. 1994; 126: 62–9
   PubMed Web of Science ®Google Scholar
• Kawashima T, Kikuchi H, Takita M, Doh-ura K, Ogomori K, Oda M, et al. Skein-like inclusions in the neostriatum from a case of amyotrophic lateral sclerosis with dementia. Acta Neuropathol. 1998; 96: 541–5
   PubMed Web of Science ®Google Scholar
• Mitsuyama Y. Presenile dementia with motor neuron disease in Japan: clinico-pathological review of 26 cases. J Neurol Neurosurg Psychiat. 1984; 47: 953–9
   PubMed Web of Science ®Google Scholar
• Munoz DG. The pathology of Pick complex. Pick's disease and Pick complex, A Kertesz, DG Munoz. John Wiley and Sons, New York 1998; 211–39
   Google Scholar
• Horoupian DS, Katzman R, Terry RD, Davies P, Hirano A, de Tersa R, et al. Dementia and motor neuron disease: morphometric, biochemical and Golgi studies. Ann Neurol. 1984; 16: 305–13
   PubMed Web of Science ®Google Scholar
• Brun A, Passant U. Frontal lobe degeneration of non-Alzheimer type. Acta Neurol Scand. 1996; 168: 28–30
   Google Scholar
• Giannakopoulos P, Hof PR, Bouras C. Dementia lacking distinctive histopathology: clinicopathological evaluation of 32 cases. Acta Neuropathol (Berl) 1995; 89: 346–55
   PubMed Web of Science ®Google Scholar
• Jackson M, Lowe J. The new neuropathology of degenerative frontotemporal dementias. Acta Neuropathol. 1996; 91: 127–34
   PubMed Web of Science ®Google Scholar
• Soni W, Luthert PJ, Leigh PN, Mann DMA. A morphometric study of the neuropathological substrate of dementia in patients with motor neuron disease. Neuropathol Appl Neurobiol. 1993; 19: 203
   Google Scholar
• Nishikawa Y, Tan C-F, Onodera O, Toyoshima Y, Yamada M, Morita T, et al. Sporadic amyotrophic lateral sclerosis: two pathological patterns shown by analysis of distribution of TDP-43-immunoreactive neuronal and glial cytoplasmic inclusions. Acta Neuropathol. 2008; 116: 169–82
   PubMed Web of Science ®Google Scholar
• Dickson DW, Josephs KA, Amador-Ortiz C. TDP-43 in differential diagnosis of motor neuron disorders. Acta Neuropathol. 2008; 114: 71–9
   Web of Science ®Google Scholar
• Mori F, Tanji K, Zhang H-X, Nishihira Y, Tan C-F, Takahashi H, et al. Maturation process of TDP-43-positive neuronal cytoplasmic inclusions in amyotrophic lateral sclerosis with and without dementia. Acta Neuropathol. 2008; 116: 193–203
   PubMed Web of Science ®Google Scholar
• Mackenzie IRA. The neuropathology of FTD associated with ALS. Alzheimer Dis Assoc Disord. 2008; 21: S44–9
   Web of Science ®Google Scholar