Skip to main content

Movement Disorders in Frontotemporal Dementia

  • Chapter
  • First Online:
Movement Disorders in Dementias

Abstract

Our understanding of frontotemporal dementia (FTD) and its related syndromes has advanced significantly in recent years. One of the most prominent areas of progress is in the overlap syndromes of FTD and movement disorders, at a clinicopathological and genetic level. The aim of this chapter is to discuss the clinical, pathological, and genetic complexities of disorders of movement typically seen in FTD.

This is a preview of subscription content, log in via an institution to check access.

Access this chapter

Chapter
USD 29.95
Price excludes VAT (USA)
  • Available as PDF
  • Read on any device
  • Instant download
  • Own it forever
eBook
USD 89.00
Price excludes VAT (USA)
  • Available as EPUB and PDF
  • Read on any device
  • Instant download
  • Own it forever
Softcover Book
USD 119.99
Price excludes VAT (USA)
  • Compact, lightweight edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info
Hardcover Book
USD 169.99
Price excludes VAT (USA)
  • Durable hardcover edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info

Tax calculation will be finalised at checkout

Purchases are for personal use only

Institutional subscriptions

Abbreviations

ALS:

Amyotrophic lateral sclerosis

bvFTD:

Behavioral variant frontotemporal dementia

CBS:

Corticobasal syndrome

DLB:

Dementia with Lewy bodies

FTD:

Frontotemporal dementia

PNFA:

Progressive nonfluent aphasia

PSP:

Progressive supranuclear palsy

SD:

Semantic dementia

VBM:

Voxel-based morphometry

References

  • Baba Y, Tsuboi Y, Baker MC, Uitti RJ, Hutton ML, Dickson DW, et al. The effect of tau genotype on clinical features in FTDP-17. Parkinsonism Relat Disord. 2005;11(4):205–8.

    Article  PubMed  Google Scholar 

  • Baker M, Mackenzie IR, Pickering-Brown SM, Gass J, Rademakers R, Lindholm C, et al. Mutations in progranulin cause tau-negative frontotemporal dementia linked to chromosome 17. Nature. 2006;442(7105):916–9.

    Article  CAS  PubMed  Google Scholar 

  • Beck J, Rohrer JD, Campbell T, Isaacs A, Morrison KE, Goodall EF, et al. A distinct clinical, neuropsychological and radiological phenotype is associated with progranulin gene mutations in a large UK series. Brain. 2008;131(3):706–20.

    Article  PubMed Central  PubMed  Google Scholar 

  • Béland R, Ska B. Interaction between verbal and gestural language in progressive aphasia: a longitudinal case study. Brain Lang. 1992;43(3):355–85.

    Article  PubMed  Google Scholar 

  • Boeve BF. Links between frontotemporal lobar degeneration, corticobasal degeneration, progressive supranuclear palsy, and amyotrophic lateral sclerosis. Alzheimer Dis Assoc Disord. 2007;21(4):S31–8. Epub 2007/12/20.

    Article  PubMed  Google Scholar 

  • Boeve BF, Hutton M. Refining frontotemporal dementia with parkinsonism linked to chromosome 17: introducing FTDP-17 (MAPT) and FTDP-17 (PGRN). Arch Neurol. 2008;65(4):460–4. Epub 2008/04/17.

    Article  PubMed Central  PubMed  Google Scholar 

  • Boeve BF, Baker M, Dickson DW, Parisi JE, Giannini C, Josephs KA, et al. Frontotemporal dementia and parkinsonism associated with the IVS1+ 1G → A mutation in progranulin: a clinicopathologic study. Brain. 2006;129(11):3103–14.

    Article  PubMed  Google Scholar 

  • Boeve BF, Boylan KB, Graff-Radford NR, DeJesus-Hernandez M, Knopman DS, Pedraza O, et al. Characterization of frontotemporal dementia and/or amyotrophic lateral sclerosis associated with the GGGGCC repeat expansion in C9ORF72. Brain. 2012;135(Pt 3):765–83. Epub 2012/03/01.

    Article  PubMed Central  PubMed  Google Scholar 

  • Caviness JN, Wszolek ZK. Myoclonus in pallido-ponto-nigral degeneration. Adv Neurol. 2002;89:35–9.

    PubMed  Google Scholar 

  • Claassen DO, Parisi JE, Giannini C, Boeve BF, Dickson DW, Josephs KA. Frontotemporal dementia mimicking dementia with Lewy bodies. Cogn Behav Neurol. 2008;21(3):157–63.

    Article  PubMed  Google Scholar 

  • Clinical and neuropathological criteria for frontotemporal dementia. The Lund and Manchester Groups. J Neurol Neurosurg Psychiatry. 1994;57(4):416–8.

    Google Scholar 

  • Cooper-Knock J, Frolov A, Highley JR, Charlesworth G, Kirby J, Milano A, et al. C9ORF72 expansions, parkinsonism, and Parkinson disease: a clinicopathologic study. Neurology. 2013;81(9):808–11. Epub 2013/07/26.

    Article  CAS  PubMed Central  PubMed  Google Scholar 

  • Craenhals A, Raison-Van Ruymbeke A, Rectem D, Seron X, Laterre E. Is slowly progressive aphasia actually a new clinical entity? Aphasiology. 1990;4(5):485–509.

    Article  Google Scholar 

  • Cruts M, Gijselinck I, van der Zee J, Engelborghs S, Wils H, Pirici D, et al. Null mutations in progranulin cause ubiquitin-positive frontotemporal dementia linked to chromosome 17q21. Nature. 2006;442(7105):920–4.

    Article  CAS  PubMed  Google Scholar 

  • Czarnecki K, Kumar N, Josephs K. Parkinsonism and tardive antecollis in frontotemporal dementia–increased sensitivity to newer antipsychotics? Eur J Neurol. 2008;15(2):199–201.

    Article  CAS  PubMed  Google Scholar 

  • Davies RR, Hodges JR, Kril JJ, Patterson K, Halliday GM, Xuereb JH. The pathological basis of semantic dementia. Brain. 2005;128(Pt 9):1984–95. Epub 2005/07/08.

    Article  PubMed  Google Scholar 

  • DeJesus-Hernandez M, Mackenzie IR, Boeve BF, Boxer AL, Baker M, Rutherford NJ, et al. Expanded GGGGCC hexanucleotide repeat in noncoding region of C9ORF72 causes chromosome 9p-linked FTD and ALS. Neuron. 2011;72(2):245–56.

    Article  CAS  PubMed Central  PubMed  Google Scholar 

  • Diehl-Schmid J, Schulte-Overberg J, Hartmann J, Forstl H, Kurz A, Haussermann P. Extrapyramidal signs, primitive reflexes and incontinence in fronto-temporal dementia. Eur J Neurol. 2007;14(8):860–4.

    Article  CAS  PubMed  Google Scholar 

  • Espay AJ, Litvan I. Parkinsonism and frontotemporal dementia: the clinical overlap. J Mol Neurosci. 2011;45(3):343–9. Epub 2011/09/06.

    Article  CAS  PubMed Central  PubMed  Google Scholar 

  • Evans JJ, Heggs AJ, Antoun N, Hodges JR. Progressive prosopagnosia associated with selective right temporal lobe atrophy. A new syndrome? Brain. 1995;118(Pt 1):1–13.

    Article  PubMed  Google Scholar 

  • Forman MS, Farmer J, Johnson JK, Clark CM, Arnold SE, Coslett HB, et al. Frontotemporal dementia: clinicopathological correlations. Ann Neurol. 2006;59(6):952–62.

    Article  PubMed Central  PubMed  Google Scholar 

  • Foster NL, Wilhelmsen K, Sima AA, Jones MZ, D’Amato CJ, Gilman S. Frontotemporal dementia and parkinsonism linked to chromosome 17: a consensus conference. Conference participants. Ann Neurol. 1997;41(6):706–15.

    Article  CAS  PubMed  Google Scholar 

  • Gass J, Cannon A, Mackenzie IR, Boeve B, Baker M, Adamson J, et al. Mutations in progranulin are a major cause of ubiquitin-positive frontotemporal lobar degeneration. Hum Mol Genet. 2006;15(20):2988–3001.

    Article  CAS  PubMed  Google Scholar 

  • Goldman JS, Farmer JM, Wood EM, Johnson JK, Boxer A, Neuhaus J, et al. Comparison of family histories in FTLD subtypes and related tauopathies. Neurology. 2005;65(11):1817–9.

    Article  CAS  PubMed  Google Scholar 

  • Gorno-Tempini ML, Hillis AE, Weintraub S, Kertesz A, Mendez M, Cappa SF, et al. Classification of primary progressive aphasia and its variants. Neurology. 2011;76(11):1006–14. Epub 2011/02/18.

    Article  PubMed Central  PubMed  Google Scholar 

  • Hodges JR, Patterson K, Oxbury S, Funnell E. Semantic dementia. Progressive fluent aphasia with temporal lobe atrophy. Brain. 1992;115(Pt 6):1783–806.

    Article  PubMed  Google Scholar 

  • Hodges JR, Davies RR, Xuereb JH, Casey B, Broe M, Bak TH, et al. Clinicopathological correlates in frontotemporal dementia. Ann Neurol. 2004;56(3):399–406.

    Article  PubMed  Google Scholar 

  • Hornberger M, Piguet O, Graham AJ, Nestor PJ, Hodges JR. How preserved is episodic memory in behavioral variant frontotemporal dementia? Neurology. 2010;74(6):472–9. Epub 2010/02/10.

    Article  CAS  PubMed Central  PubMed  Google Scholar 

  • Houlden H, Baker M, Adamson J, Grover A, Waring S, Dickson D, et al. Frequency of tau mutations in three series of non-Alzheimer’s degenerative dementia. Annals of neurology. 1999;46(2):243–8.

    Article  CAS  PubMed  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(6686):702–5. Epub 1998/06/26.

    Article  CAS  PubMed  Google Scholar 

  • Janssen JC, Warrington EK, Morris HR, Lantos P, Brown J, Revesz T, et al. Clinical features of frontotemporal dementia due to the intronic tau 10(+16) mutation. Neurology. 2002;58(8):1161–8. Epub 2002/04/24.

    Article  CAS  PubMed  Google Scholar 

  • Josephs KA, Duffy JR, Strand EA, Machulda MM, Senjem ML, Master AV, et al. Characterizing a neurodegenerative syndrome: primary progressive apraxia of speech. Brain. 2012;135(5):1522–36.

    Article  PubMed Central  PubMed  Google Scholar 

  • Joshi A, Roy EA, Black SE, Barbour K. Patterns of limb apraxia in primary progressive aphasia. Brain Cogn. 2003;53(2):403–7.

    Article  PubMed  Google Scholar 

  • Karbe H, Kertesz A, Polk M. Profiles of language impairment in primary progressive aphasia. Arch Neurol. 1993;50(2):193.

    Article  CAS  PubMed  Google Scholar 

  • Kertesz A, Hudson L, Mackenzie IR, Munoz DG. The pathology and nosology of primary progressive aphasia. Neurology. 1994;44(11):2065.

    Article  CAS  PubMed  Google Scholar 

  • Kertesz A, McMonagle P, Blair M, Davidson W, Munoz DG. The evolution and pathology of frontotemporal dementia. Brain. 2005;128(Pt 9):1996–2005.

    Article  PubMed  Google Scholar 

  • Kodama K, Okada S, Iseki E, Kowalska A, Tabira T, Hosoi N, et al. Familial frontotemporal dementia with a P301L tau mutation in Japan. J Neurol Sci. 2000;176(1):57–64. Epub 2000/06/24.

    Article  CAS  PubMed  Google Scholar 

  • Le Ber I, Van Der Zee J, Hannequin D, Gijselinck I, Campion D, Puel M, et al. Progranulin null mutations in both sporadic and familial frontotemporal dementia. Hum Mutat. 2007;28(9):846–55.

    Article  PubMed  Google Scholar 

  • Lendon CL, Lynch T, Norton J, McKeel Jr DW, Busfield F, Craddock N, et al. Hereditary dysphasic disinhibition dementia: a frontotemporal dementia linked to 17q21-22. Neurology. 1998;50(6):1546–55.

    Article  CAS  PubMed  Google Scholar 

  • Lillo P, Hodges JR. Frontotemporal dementia and motor neurone disease: overlapping clinic-pathological disorders. J Clin Neurosci. 2009;16(9):1131–5.

    Article  PubMed  Google Scholar 

  • Mann DM, South PW, Snowden JS, Neary D. Dementia of frontal lobe type: neuropathology and immunohistochemistry. J Neurol Neurosurg Psychiatry. 1993;56(6):605–14.

    Article  CAS  PubMed Central  PubMed  Google Scholar 

  • Mathew R, Bak TH, Hodges JR. Diagnostic criteria for corticobasal syndrome: a comparative study. J Neurol Neurosurg Psychiatry. 2012;83(4):405–10.

    Article  PubMed  Google Scholar 

  • Mioshi E, Bristow M, Cook R, Hodges JR. Factors underlying caregiver stress in frontotemporal dementia and Alzheimer's disease. Dement Geriatr Cogn Disord. 2009;27(1):76–81. Epub 2009/01/22.

    Article  PubMed  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(6):1546–54.

    Article  CAS  PubMed  Google Scholar 

  • Newsway V, Fish M, Rohrer JD, Majounie E, Williams N, Hack M, et al. Perry syndrome due to the DCTN1 G71R mutation: a distinctive levodopa responsive disorder with behavioral syndrome, vertical gaze palsy, and respiratory failure. Mov Disord. 2010;25(6):767–70.

    Article  PubMed  Google Scholar 

  • Nuytemans K, Bademci G, Kohli MM, Beecham GW, Wang L, Young JI, et al. C9ORF72 intermediate repeat copies are a significant risk factor for Parkinson disease. Ann Hum Genet. 2013;77(5):351–63.

    Google Scholar 

  • Onari KSH. Anatomishe Beitrage zur Lehre von der Pickschen umschriebenen Grosshirnrinden-Atrophie (‘Picksche Krankheit’). Z Gesamte Neurol Psych. 1926;101:470–511.

    Article  Google Scholar 

  • Padovani A, Agosti C, Premi E, Bellelli G, Borroni B. Extrapyramidal symptoms in frontotemporal dementia: prevalence and clinical correlations. Neurosci Lett. 2007;422(1):39–42.

    Article  CAS  PubMed  Google Scholar 

  • Pickering-Brown SM, Rollinson S, Du Plessis D, Morrison KE, Varma A, Richardson AM, 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(Pt 3):721–31.

    Article  PubMed  Google Scholar 

  • Piggott MA, Perry EK, Marshall EF, McKeith IG, Johnson M, Melrose HL, et al. Nigrostriatal dopaminergic activities in dementia with Lewy bodies in relation to neuroleptic sensitivity: comparisons with Parkinson’s disease. Biol Psychiatry. 1998;44(8):765–74.

    Article  CAS  PubMed  Google Scholar 

  • Piguet O, Brooks WS, Halliday GM, Schofield PR, Stanford PM, Kwok JB, et al. Similar early clinical presentations in familial and non-familial frontotemporal dementia. J Neurol Neurosurg Psychiatry. 2004;75(12):1743–5. Epub 2004/11/19.

    Article  CAS  PubMed Central  PubMed  Google Scholar 

  • Pijnenburg Y, Sampson E, Harvey R, Fox N, Rossor M. Vulnerability to neuroleptic side effects in frontotemporal lobar degeneration. Int J Geriatr Psychiatry. 2003;18(1):67–72.

    Article  CAS  PubMed  Google Scholar 

  • Poorkaj P, Grossman M, Steinbart E, Payami H, Sadovnick A, Nochlin D, et al. Frequency of tau gene mutations in familial and sporadic cases of non-Alzheimer dementia. Arch Neurol. 2001;58(3):383–7.

    Article  CAS  PubMed  Google Scholar 

  • Rascovsky K, Hodges JR, Knopman D, Mendez MF, Kramer JH, Neuhaus J, et al. Sensitivity of revised diagnostic criteria for the behavioural variant of frontotemporal dementia. Brain. 2011;134(Pt 9):2456–77. Epub 2011/08/04.

    Article  PubMed Central  PubMed  Google Scholar 

  • Ratnavalli E, Brayne C, Dawson K, Hodges JR. The prevalence of frontotemporal dementia. Neurology. 2002;58(11):1615–21. Epub 2002/06/12.

    Article  CAS  PubMed  Google Scholar 

  • Reed LA, Wszolek ZK, Hutton M. Phenotypic correlations in FTDP-17. Neurobiol Aging. 2001;22(1):89–107. Epub 2001/02/13.

    Article  CAS  PubMed  Google Scholar 

  • Renton AE, Majounie E, Waite A, Simon-Sanchez J, Rollinson S, Gibbs JR, et al. A hexanucleotide repeat expansion in C9ORF72 is the cause of chromosome 9p21-linked ALS-FTD. Neuron. 2011;72(2):257–68.

    Article  CAS  PubMed Central  PubMed  Google Scholar 

  • Rinne JO, Laine M, Kaasinen V, Norvasuo-Heila MK, Nagren K, Helenius H. Striatal dopamine transporter and extrapyramidal symptoms in frontotemporal dementia. Neurology. 2002;58(10):1489–93.

    Article  CAS  PubMed  Google Scholar 

  • Rohrer JD, Ridgway GR, Modat M, Ourselin S, Mead S, Fox NC, et al. Distinct profiles of brain atrophy in frontotemporal lobar degeneration caused by progranulin and tau mutations. Neuroimage. 2010a;53(3):1070–6.

    Article  CAS  PubMed Central  PubMed  Google Scholar 

  • Rohrer JD, Rossor MN, Warren JD. Apraxia in progressive nonfluent aphasia. J Neurol. 2010b;257(4):569–74.

    Article  PubMed Central  PubMed  Google Scholar 

  • Rosso SM, Donker Kaat L, Baks T, Joosse M, de Koning I, Pijnenburg Y, et al. Frontotemporal dementia in The Netherlands: patient characteristics and prevalence estimates from a population-based study. Brain. 2003;126(Pt 9):2016–22.

    Article  PubMed  Google Scholar 

  • Saito Y, Geyer A, Sasaki R, Kuzuhara S, Nanba E, Miyasaka T, et al. Early-onset, rapidly progressive familial tauopathy with R406W mutation. Neurology. 2002;58(5):811–3.

    Article  CAS  PubMed  Google Scholar 

  • Schroeter ML, Raczka K, Neumann J, von Cramon DY. Neural networks in frontotemporal dementia–a meta-analysis. Neurobiol Aging. 2008;29(3):418–26.

    Article  PubMed  Google Scholar 

  • Seelaar H, Schelhaas HJ, Azmani A, Küsters B, Rosso S, Majoor-Krakauer D, et al. TDP-43 pathology in familial frontotemporal dementia and motor neuron disease without progranulin mutations. Brain. 2007;130(5):1375–85.

    Article  PubMed  Google Scholar 

  • Sha S, Hou C, Viskontas IV, Miller BL. Are frontotemporal lobar degeneration, progressive supranuclear palsy and corticobasal degeneration distinct diseases? Nat Clin Pract Neurol. 2006;2(12):658–65. Epub 2006/11/23.

    Article  PubMed  Google Scholar 

  • Sha SJ, Takada LT, Rankin KP, Yokoyama JS, Rutherford NJ, Fong JC, et al. Frontotemporal dementia due to C9ORF72 mutations: clinical and imaging features. Neurology. 2012;79(10):1002–11. Epub 2012/08/10.

    Article  PubMed Central  PubMed  Google Scholar 

  • Snowden JS, Rollinson S, Thompson JC, Harris JM, Stopford CL, Richardson AM, et al. Distinct clinical and pathological characteristics of frontotemporal dementia associated with C9ORF72 mutations. Brain. 2012;135(Pt 3):693–708. Epub 2012/02/04.

    Article  PubMed Central  PubMed  Google Scholar 

  • Soliveri P, Piacentini S, Girotti F. Limb apraxia in corticobasal degeneration and progressive supranuclear palsy. Neurology. 2005;64(3):448–53.

    Article  PubMed  Google Scholar 

  • Spina S, Murrell JR, Yoshida H, Ghetti B, Bermingham N, Sweeney B, et al. The novel Tau mutation G335S: clinical, neuropathological and molecular characterization. Acta Neuropathol. 2007;113(4):461–70.

    Article  CAS  PubMed  Google Scholar 

  • Stanford PM, Halliday GM, Brooks WS, Kwok JB, Storey CE, Creasey H, et al. Progressive supranuclear palsy pathology caused by a novel silent mutation in exon 10 of the tau gene: expansion of the disease phenotype caused by tau gene mutations. Brain. 2000;123(Pt 5):880–93. Epub 2000/04/25.

    Article  PubMed  Google Scholar 

  • Stanford PM, Brooks WS, Teber ET, Hallupp M, McLean C, Halliday GM, et al. Frequency of tau mutations in familial and sporadic frontotemporal dementia and other tauopathies. J Neurol. 2004;251(9):1098–104.

    Article  CAS  PubMed  Google Scholar 

  • Strong MJ. The syndromes of frontotemporal dysfunction in amyotrophic lateral sclerosis. Amyotroph Lateral Scler. 2008;9(6):323–38.

    Article  CAS  PubMed  Google Scholar 

  • Tsuboi Y. Neuropathology of familial tauopathy. Neuropathology. 2006;26(5):471–4. Epub 2006/11/04.

    Article  PubMed  Google Scholar 

  • Tsuboi Y, Baker M, Hutton ML, Uitti RJ, Rascol O, Delisle MB, et al. Clinical and genetic studies of families with the tau N279K mutation (FTDP-17). Neurology. 2002;59(11):1791–3. Epub 2002/12/11.

    Article  CAS  PubMed  Google Scholar 

  • Van Swieten JC, Heutink P. Mutations in progranulin (<i>GRN</i>) within the spectrum of clinical and pathological phenotypes of frontotemporal dementia. Lancet Neurol. 2008;7(10):965–74.

    Article  PubMed  Google Scholar 

  • Van Swieten J, Stevens M, Rosso S, Rizzu P, Joosse M, De Koning I, et al. Phenotypic variation in hereditary frontotemporal dementia with tau mutations. Ann Neurol. 1999;46(4):617–26.

    Article  PubMed  Google Scholar 

  • Whitwell JL, Weigand SD, Boeve BF, Senjem ML, Gunter JL, DeJesus-Hernandez M, et al. Neuroimaging signatures of frontotemporal dementia genetics: C9ORF72, tau, progranulin and sporadics. Brain. 2012;135(Pt 3):794–806. Epub 2012/03/01.

    Article  PubMed Central  PubMed  Google Scholar 

  • Wszolek ZK, Uitti RJ, Hutton M. A mutation in the microtubule-associated protein tau in pallido-nigro-luysian degeneration. Neurology. 2000;54(10):2028–30. Epub 2000/05/24.

    Article  CAS  PubMed  Google Scholar 

Download references

Author information

Authors and Affiliations

Authors

Corresponding author

Correspondence to John Hodges MBBS, MD, FRCP, FMedSci, FRACP .

Editor information

Editors and Affiliations

Rights and permissions

Reprints and permissions

Copyright information

© 2014 Springer-Verlag London

About this chapter

Cite this chapter

Devenney, E., Hodges, J. (2014). Movement Disorders in Frontotemporal Dementia. In: Merello, M., Starkstein, S. (eds) Movement Disorders in Dementias. Springer, London. https://doi.org/10.1007/978-1-4471-6365-7_9

Download citation

  • DOI: https://doi.org/10.1007/978-1-4471-6365-7_9

  • Published:

  • Publisher Name: Springer, London

  • Print ISBN: 978-1-4471-6364-0

  • Online ISBN: 978-1-4471-6365-7

  • eBook Packages: MedicineMedicine (R0)

Publish with us

Policies and ethics