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Chapter 8 - Overviewof clinical assessment of frontotemporal dementia syndromes

from Section 3 - Approach to the diagnosis of FTD

Published online by Cambridge University Press:  05 May 2016

By
Bradford C. Dickerson ,
Simon Ducharme  and
Chiadi U. Onyike
Edited by
Bradford C. Dickerson
Bradford C. Dickerson
Affiliation:
Department of Neurology, Massachusetts General Hospital
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Hodges' Frontotemporal Dementia , pp. 91 - 105
Publisher: Cambridge University Press
Print publication year: 2016

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References

Adams, RD (1966) Further observations on normal pressure hydrocephalus. Proc R Soc Med 59:1135–1140.Google Scholar
American Psychiatric Association (2013a) Neurocognitive disorders. In: Diagnostic and Statistical Manual of Mental Disorders, 5th edn. Arlington VA: American Psychiatric Association.Google Scholar
American Psychiatric Association (2013b)Diagnostic and Statistical Manual of Mental Disorders, 5th edn. Arlington, VA: American Psychiatric Association.Google Scholar
Ames, D, Cummings, J, Wirshing, W, Quinn, B, Mahler, M (1994) Repetitive and compulsive behavior in frontal lobe degenerations. J Neuropsychiatry Clin Neurosci 6:100–113.Google Scholar
Amodio, DM, Frith, CD (2006) Meeting of minds: the medial frontal cortex and social cognition. Nat Rev Neurosci 7:268–277.CrossRefGoogle ScholarPubMed
Baborie, A, Griffiths, TD, Jaros, E et al. (2012) Frontotemporal dementia in elderly individuals. Arch Neurol 69:1052–1060.Google Scholar
Berrios, GE, Girling, DM (1994) Introduction: Pick's disease and the ‘frontal lobe’ dementias. Hist Psychiatry 5:539–547.Google Scholar
Bickart, KC, Brickhouse, M, Negreira, A et al. (2014) Atrophy in distinct corticolimbic networks in frontotemporal dementia relates to social impairments measured using the Social Impairment Rating Scale. J Neurol Neurosurg Psychiatry 85:438–448.Google Scholar
Borroni, B, Alberici, A, Grassi, M et al. (2010) Is frontotemporal lobar degeneration a rare disorder? Evidence from a preliminary study in Brescia county, Italy. J Alzheimers Dis 19:111–116.Google Scholar
Bozeat, S, Gregory, CA, Ralph, MA, Hodges, JR (2000) Which neuropsychiatric and behavioural features distinguish frontal and temporal variants of frontotemporal dementia from Alzheimer's disease? J Neurol Neurosurg Psychiatry 69:178–186.CrossRefGoogle ScholarPubMed
Brodtmann, A, Cowie, T, McLean, C, Darby, D (2013) Phenocopy or variant: a longitudinal study of very slowly progressive frontotemporal dementia. BMJ Case Rep 2013.Google Scholar
Brun, A, Englund, E, Gustafson, L et al. (1994) Clinical and neuropathological criteria for frontotemporal dementia. The Lund and Manchester Groups. J Neurol Neurosurg Psychiatry 57:416–418.Google Scholar
Cerami, C, Marcone, A, Galimberti, D et al. (2011) From genotype to phenotype: two cases of genetic frontotemporal lobar degeneration with premorbid bipolar disorder. J Alzheimers Dis 27:791–797.Google Scholar
Chien, DT, Bahri, S, Szardenings, AK et al. (2013) Early clinical PET imaging results with the novel PHF-tau radioligand [F-18]-T807. J Alzheimers Dis 34:457–468.Google Scholar
Constantinidis, J, Richard, J, Tissot, R (1974) Pick's disease. Histological and clinical correlations. Eur Neurol 11(4):208–217.CrossRefGoogle ScholarPubMed
Cummings, JL (1997) The Neuropsychiatric Inventory: assessing psychopathology in dementia patients. Neurology 48:S10–16.Google Scholar
Davies, RR, Kipps, CM, Mitchell, J et al. (2006) Progression in frontotemporal dementia: identifying a benign behavioral variant by magnetic resonance imaging. Arch Neurol 63:1627–1631.Google Scholar
Dobson-Stone, C, Hallupp, M, Bartley, L et al. (2012) C9ORF72 repeat expansion in clinical and neuropathologic frontotemporal dementia cohorts. Neurology 79:995–1001.CrossRefGoogle ScholarPubMed
Dubois, B, Slachevsky, A, Litvan, I, Pillon, B (2000) The FAB: a Frontal Assessment Battery at bedside. Neurology 55:1621–1626.Google Scholar
Floris, G, Borghero, G, Cannas, A et al. (2013) Bipolar affective disorder preceding frontotemporal dementia in a patient with C9ORF72 mutation: is there a genetic link between these two disorders? J Neurol 260:1155–1157.Google Scholar
Fodero-Tavoletti, MT, Okamura, N, Furumoto, S et al. (2011) 18F-THK523: a novel in vivo tau imaging ligand for Alzheimer's disease. Brain 134:1089–1100.Google Scholar
Funkiewiez, A, Bertoux, M, de Souza, LC, Levy, R, Dubois, B (2012) The SEA (Social cognition and Emotional Assessment): a clinical neuropsychological tool for early diagnosis of frontal variant of frontotemporal lobar degeneration. Neuropsychology 26:81–90.Google Scholar
Garcin, B, Lillo, P, Hornberger, M et al. (2009) Determinants of survival in behavioral variant frontotemporal dementia. Neurology 73:1656–1661.Google Scholar
Gislason, TB, Sjogren, M, Larsson, L, Skoog, I (2003) The prevalence of frontal variant frontotemporal dementia and the frontal lobe syndrome in a population based sample of 85 year olds. J Neurol Neurosurg Psychiatry 74:867–871.Google Scholar
Gorno-Tempini, ML, Hillis, AE, Weintraub, S et al. (2011) Classification of primary progressive aphasia and its variants. Neurology 76:1006–1014.Google Scholar
Grasbeck, A, Englund, E, Horstmann, V, Passant, U, Gustafson, L (2003) Predictors of mortality in frontotemporal dementia: a retrospective study of the prognostic influence of pre-diagnostic features. Int J Geriatr Psychiatry 18:594–601.Google Scholar
Gregory, CA, Serra-Mestres, J, Hodges, JR (1999) Early diagnosis of the frontal variant of frontotemporal dementia: how sensitive are standard neuroimaging and neuropsychologic tests? Neuropsychiatry Neuropsychol Behav Neurol 12:128–135.Google Scholar
Gustafson, L (1987) Frontal lobe degeneration of non-Alzheimer type. II. Clinical picture and differential diagnosis. Arch Gerontol Geriatr 6:209–223.Google Scholar
Hodges, JR, Mitchell, J, Dawson, K et al. (2010) Semantic dementia: demography, familial factors and survival in a consecutive series of 100 cases. Brain 133:300–306.CrossRefGoogle Scholar
Hornberger, M, Piguet, O, Kipps, C, Hodges, JR (2008) Executive function in progressive and nonprogressive behavioral variant frontotemporal dementia. Neurology 71:1481–1488.Google Scholar
Hornberger, M, Shelley, BP, Kipps, CM, Piguet, O, Hodges, JR (2009) Can progressive and non-progressive behavioural variant frontotemporal dementia be distinguished at presentation? J Neurol Neurosurg Psychiatry 80:591–593.Google Scholar
Hornberger, M, Piguet, O, Graham, AJ, Nestor, PJ, Hodges, JR (2010) How preserved is episodic memory in behavioral variant frontotemporal dementia? Neurology 74:472–479.Google Scholar
Hornberger, M, Wong, S, Tan, R et al. (2012) In vivo and post-mortem memory circuit integrity in frontotemporal dementia and Alzheimer's disease. Brain 135:3015–3025.Google Scholar
Hu, WT, Seelaar, H, Josephs, KA et al. (2009) Survival profiles of patients with frontotemporal dementia and motor neuron disease. Arch Neurol 66:1359–1364.Google Scholar
Hu, WT, Chen-Plotkin, A, Grossman, M et al. (2011) Novel CSF biomarkers for frontotemporal lobar degenerations. Neurology 75:2079–2086.Google Scholar
Ibach, B, Koch, H, Koller, M, Wolfersdorf, M; Workgroup for Geriatric Psychiatry of the Psychiatric State Hospitals of Germany, Workgroup for Clinical Research of the Psychiatric State Hospitals of Germany (2003) Hospital admission circumstances and prevalence of frontotemporal lobar degeneration: a multicenter psychiatric state hospital study in Germany. Dement Geriatr Cogn Disord 16:253–264.Google Scholar
Kertesz, A, Davidson, W, Fox, H (1997) Frontal behavioral inventory: diagnostic criteria for frontal lobe dementia. Can J Neurol Sci 24:29–36.Google Scholar
Kertesz, A, McMonagle, P, Blair, M, Davidson, W, Munoz, DG (2005) The evolution and pathology of frontotemporal dementia. Brain 128(Pt 9):1996–2005.CrossRefGoogle ScholarPubMed
Kertesz, A, Blair, M, McMonagle, P, Munoz, DG (2007) The diagnosis and course of frontotemporal dementia. Alzheimer Dis Assoc Disord 21(2):155–163.CrossRefGoogle ScholarPubMed
Kertesz, A, Ang, LC, Jesso, S et al. (2013) Psychosis and hallucinations in frontotemporal dementia with the C9ORF72 mutation: a detailed clinical cohort. Cogn Behav Neurol 26:146–154.Google Scholar
Khan, BK, Yokoyama, JS, Takada, LT et al. (2012) Atypical, slowly progressive behavioural variant frontotemporal dementia associated with C9ORF72 hexanucleotide expansion. J Neurol Neurosurg Psychiatry 83:358–364.Google Scholar
Kipps, CM, Davies, RR, Mitchell, J et al. (2007) Clinical significance of lobar atrophy in frontotemporal dementia: application of an MRI visual rating scale. Dement Geriatr Cogn Disord 23:334–342.Google Scholar
Klunk, WE, Engler, H, Nordberg, A et al. (2004) Imaging brain amyloid in Alzheimer's disease with Pittsburgh Compound-B. Ann Neurol 55:306–319.Google Scholar
Knopman, DS, Petersen, RC, Edland, SD, Cha, RH, Rocca, WA (2004) The incidence of frontotemporal lobar degeneration in Rochester, Minnesota, 1990 through 1994. Neurology 62:506–508.CrossRefGoogle ScholarPubMed
Knopman, DS, Kramer, JH, Boeve, BF et al. (2008) Development of methodology for conducting clinical trials in frontotemporal lobar degeneration. Brain 131:2957–2968.Google Scholar
Le Ber, I, Camuzat, A, Hannequin, D et al. (2008) Phenotype variability in progranulin mutation carriers: a clinical, neuropsychological, imaging and genetic study. Brain 131:732–746.Google Scholar
Lewandowski, K, Cohen, B, Öngur, D (2011) Evolution of neuropsychological dysfunction during the course of schizophrenia and bipolar disorder. Psychol Med 41:225.Google Scholar
Mann-Wrobel, MC, Carreno, JT, Dickinson, D (2011) Meta-analysis of neuropsychological functioning in euthymic bipolar disorder: an update and investigation of moderator variables. Bipolar Disord 13:334–342.CrossRefGoogle ScholarPubMed
Maruyama, M, Shimada, H, Suhara, T et al. (2013) Imaging of tau pathology in a tauopathy mouse model and in Alzheimer patients compared to normal controls. Neuron 79:1094–1108.Google Scholar
Masellis, M, Momeni, P, Meschino, W et al. (2006) Novel splicing mutation in the progranulin gene causing familial corticobasal syndrome. Brain 129:3115–3123.Google Scholar
Mathuranath, PS, Nestor, PJ, Berrios, GE, Rakowicz, W, Hodges, JR (2000) A brief cognitive test battery to differentiate Alzheimer's disease and frontotemporal dementia. Neurology 55:1613–1620.Google Scholar
McGirr, A, Mohammed, S, Kurlan, R, Cusimano, MD (2013) Clinical equipoise in idiopathic normal pressure hydrocephalus: a survey of physicians on the need for randomized controlled trials assessing the efficacy of cerebrospinal fluid diversion. J Neurol Sci 333:13–18.CrossRefGoogle ScholarPubMed
McKhann, GM, Albert, MS, Grossman, M et al. (2001) Clinical and pathological diagnosis of frontotemporal dementia: report of the Work Group on Frontotemporal Dementia and Pick's Disease. Arch Neurol 58:1803–1809.Google Scholar
Meisler, MH, Grant, AE, Jones, JM et al. (2013) C9ORF72 expansion in a family with bipolar disorder. Bipolar Disord 15:326–332.Google Scholar
Mendez, MF, Shapira, JS (2008) The spectrum of recurrent thoughts and behaviors in frontotemporal dementia. CNS Spectr 13:202–208.Google Scholar
Mendez, M, Perryman, K, Miller, B, Swartz, J, Cummings, J (1997) Compulsive behaviors as presenting symptoms of frontotemporal dementia J Geriatr Psychiatry Neurol 10:154–157.Google Scholar
Mendez, MF, Shapira, JS, Miller, BL (2005) Stereotypical movements and frontotemporal dementia. Mov Disord 20:742–745.Google Scholar
Mendez, MF, Shapira, JS, Woods, RJ, Licht, EA, Saul, RE (2008) Psychotic symptoms in frontotemporal dementia: prevalence and review. Dement Geriatr Cogn Disord 25:206–211.Google Scholar
Mesulam, MM (1982) Slowly progressive aphasia without generalized dementia. Ann Neurol 11:592–598.Google Scholar
Midorikawa, A, Kawamura, M (2012) The relationship between subclinical Asperger's syndrome and frontotemporal lobar degeneration. Dement Geriatr Cogn Dis Extra 2:180–186.Google Scholar
Mioshi, E, Hsieh, S, Savage, S, Hornberger, M, Hodges, JR (2010) Clinical staging and disease progression in frontotemporal dementia. Neurology 74:1591–1597.Google Scholar
Modirrousta, M, Price, BH, Dickerson, BC (2013) Neuropsychiatric symptoms in primary progressive aphasia: phenomenology, pathophysiology, and approach to assessment and treatment. Neurodegener Dis Manag 3:133–146.Google Scholar
Nakaaki, S, Murata, Y, Sato, J et al. (2007a) Impairment of decision-making cognition in a case of frontotemporal lobar degeneration (FTLD) presenting with pathologic gambling and hoarding as the initial symptoms. Cogn Behav Neurol 20:121–125.CrossRefGoogle Scholar
Nakaaki, S, Murata, Y, Shinagawa, Y et al. (2007b) A case of late-onset obsessive compulsive disorder developing frontotemporal lobar degeneration. J Neuropsychiatry Clin Neurosci 19(4):487–488.Google Scholar
Neary, D, Snowden, JS, Northen, B, Goulding, P (1988) Dementia of frontal lobe type. J Neurol Neurosurg Psychiatry 51:353–361.Google Scholar
Neary, D, Snowden, JS, Gustafson, L et al. (1998) Frontotemporal lobar degeneration: a consensus on clinical diagnostic criteria. Neurology 51:1546–1554.CrossRefGoogle ScholarPubMed
Onyike, CU, Sloane, KL, Smyth, SF et al. (2011) Estimating severity of illness and disability in frontotemporal dementia: preliminary analysis of the Dementia Disability Rating (DDR). Acta Neuropsychol 9:141–153.Google Scholar
Onyike, CU, Pletnikova, O, Sloane, KL et al. (2013) Hippocampal sclerosis dementia: an amnesic variant of frontotemporal degeneration. Dement Neuropsychol 7:83–87.Google Scholar
Perry, RJ, Miller, BL (2001) Behavior and treatment in frontotemporal dementia. Neurology 56:S46–S51.CrossRefGoogle ScholarPubMed
Pompanin, S, Perini, G, Toffanin, T et al. (2012) Late-onset OCD as presenting manifestation of semantic dementia. Gen Hosp Psychiatry 34:102.e101–102.e104.Google Scholar
Pose, M, Cetkovich, M, Gleichgerrcht, E et al. (2013) The overlap of symptomatic dimensions between frontotemporal dementia and several psychiatric disorders that appear in late adulthood. Int Rev Psychiatry 25:159–167.Google Scholar
Rankin, KP, Santos-Modesitt, W, Kramer, JH et al. (2008) Spontaneous social behaviors discriminate behavioral dementias from psychiatric disorders and other dementias. J Clin Psychiatry 69:60–73.CrossRefGoogle ScholarPubMed
Rascovsky, K, Hodges, JR, Knopman, D et al. (2011) Sensitivity of revised diagnostic criteria for the behavioural variant of frontotemporal dementia. Brain 134:2456–2477.Google Scholar
Robert, P, Onyike, C, Leentjens, A et al. (2009) Proposed diagnostic criteria for apathy in Alzheimer's disease and other neuropsychiatric disorders. Eur Psychiatry 24:98–104.Google Scholar
Rohrer, JD, Geser, F, Zhou, J et al. (2010) TDP-43 subtypes are associated with distinct atrophy patterns in frontotemporal dementia. Neurology 75:2204–2211.CrossRefGoogle ScholarPubMed
Sapolsky, D, Bakkour, A, Negreira, A et al. (2010) Cortical neuroanatomic correlates of symptom severity in primary progressive aphasia. Neurology 75:358–366.Google Scholar
Seeley, WW, Bauer, AM, Miller, BL et al. (2005) The natural history of temporal variant frontotemporal dementia. Neurology 64:1384–1390.Google Scholar
Small, GW, Kepe, V, Ercoli, LM et al. (2006) PET of brain amyloid and tau in mild cognitive impairment. N Engl J Med 355:2652–2663.Google Scholar
Snowden, JS, Goulding, PJ, Neary, D (1989) Semantic dementia: a form of circumscribed cerebral atrophy. Behav Neurol 2:167–182.Google Scholar
Snowden, JS, Bathgate, D, Varma, A et al. (2001) Distinct behavioural profiles in frontotemporal dementia and semantic dementia. J Neurol Neurosurg Psychiatry 70:323–332.Google Scholar
Snowden, JS, Neary, D, Mann, DM (2004) Autopsy proven sporadic frontotemporal dementia due to microvacuolar-type histology, with onset at 21 years of age. J Neurol Neurosurg Psychiatry 75:1337–1339.Google Scholar
Snowden, JS, Rollinson, S, Thompson, JC et al. (2012) Distinct clinical and pathological characteristics of frontotemporal dementia associated with C9ORF72 mutations. Brain 135:693–708.Google Scholar
Torralva, T, Roca, M, Gleichgerrcht, E, Bekinschtein, T, Manes, F (2009) A neuropsychological battery to detect specific executive and social cognitive impairments in early frontotemporal dementia. Brain 132:1299–1309.CrossRefGoogle ScholarPubMed
Velakoulis, D, Walterfang, M, Mocellin, R, Pantelis, C, McLean, C (2009) Frontotemporal dementia presenting as schizophrenia-like psychosis in young people: clinicopathological series and review of cases. Br J Psychiatry 194:298–305.Google Scholar
Warrington, EK (1975) Selective impairment of semantic memory. Q J Exp Psychol 27:635–657.Google Scholar
Whitwell, JL, Jack CR, Jr., Baker, M et al. (2007) Voxel-based morphometry in frontotemporal lobar degeneration with ubiquitin-positive inclusions with and without progranulin mutations. Arch Neurol 64:371–376.Google Scholar
Whitwell, JL, Dickson, DW, Murray, ME et al. (2012a) Neuroimaging correlates of pathologically defined subtypes of Alzheimer's disease: a case-control study. Lancet Neurol 11:868–877.CrossRefGoogle ScholarPubMed
Whitwell, JL, Weigand, SD, Boeve, BF et al. (2012b) Neuroimaging signatures of frontotemporal dementia genetics: C9ORF72, tau, progranulin and sporadics. Brain 135:794–806.Google Scholar
Wicklund, MR, Mokri, B, Drubach, DA et al. (2011) Frontotemporal brain sagging syndrome: an SIH-like presentation mimicking FTD. Neurology 76:1377–1382.Google Scholar
Woolley, J, Wilson, M, Hung, E et al. (2007) Frontotemporal dementia and mania. Am J Psychiatry 164:1811–1816.Google Scholar
Woolley, JD, Khan, BK, Murthy, NK, Miller, BL, Rankin, KP (2011) The diagnostic challenge of psychiatric symptoms in neurodegenerative disease: rates of and risk factors for prior psychiatric diagnosis in patients with early neurodegenerative disease. J Clin Psychiatry 72:126–133.Google Scholar
Xie, SX, Forman, MS, Farmer, J et al. (2008) Factors associated with survival probability in autopsy-proven frontotemporal lobar degeneration. J Neurol Neurosurg Psychiatry 79:126–129.Google Scholar

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