pubmed.ncbi.nlm.nih.gov

New perspective on parkinsonism in frontotemporal lobar degeneration - PubMed

Review

New perspective on parkinsonism in frontotemporal lobar degeneration

Hee Kyung Park et al. J Mov Disord. 2013 May.

Abstract

Frontotemporal dementia (FTD) is the second most common type of presenile dementia. Three clinical prototypes have been defined; behavioral variant FTD, semantic dementia, and progressive nonfluent aphasia. Progressive supranuclear palsy, corticobasal degeneration, and motor neuron disease may possess clinical and pathological characteristics that overlap with FTD, and it is possible that they may all belong to the same clinicopathological spectrum. Frontotemporal lobar degeneration (FTLD) is a clinicopathological syndrome that encompasses a heterogenous group of neurodegenerative disorders. Owing to the advancement in the field of molecular genetics, diagnostic imaging, and pathology, FTLD has been the focus of great interest. Nevertheless, parkinsonism in FTLD has received relatively less attention. Parkinsonism is found in approximately 20-30% of patients in FTLD. Furthermore, parkinsonism can be seen in all FTLD subtypes, and some patients with familial and sporadic FTLD can present with prominent parkinsonism. Therefore, there is a need to understand parkinsonism in FTLD in order to obtain a better understanding of the disease. With regard to the clinical characteristics, the akinetic rigid type of parkinsonism has predominantly been described. Parkinsonism is frequently observed in familial FTD, more specifically, in FTD with parkinsonism linked to chromosome 17q (FTDP-17). The genes associated with parkinsonism are microtubule associated protein tau (MAPT), progranulin (GRN or PGRN), and chromosome 9 open reading frame 72 (C9ORF72) repeat expansion. The neural substrate of parkinsonism remains to be unveiled. Dopamine transporter (DAT) imaging revealed decreased uptake of DAT, and imaging findings indicated atrophic changes of the basal ganglia. Parkinsonism can be an important feature in FTLD and, therefore, increased attention is needed on the subject.

Keywords: Frontotemporal dementia; Frontotemporal lobar degeneration; Parkinsonism.

PubMed Disclaimer

Conflict of interest statement

The authors have no financial conflicts of interest.

Figures

Figure 1.
Figure 1.

Classification of frontotemporal lobar degeneration based on the clinical manifestations. FTLD: frontotemporal lobar denetation, bvFTD: behavioral variant frontotemporal dementia, PNFA: progressive nonfluent aphasia, SD: semantic dementia, PSP: progressive supranuclear palsy, CBD: corticobasal degeneration, FTDP-17: frontotemporal dementia with parkinsonism linked to chromosome 17, FTD-MND: frontotemporal dementia-motor neuron disease.

Figure 2.
Figure 2.

Brain MRI in frontotemporal dementia patients. A: A patient with behavioral variant frontotemporal dementia who presented with parkinsonism showed frontal and temporal atrophy. B: The presence of symmetric atrophy in bilateral temporal lobes was seen in a semantic dementia case. C: A patient with progressive nonfluent aphasia demonstrated the focal atrophy of the left perisylvian area.

Figure 3.
Figure 3.

Positron emission tomography (PET) images in a frontotemporal dementia (FTD) patient. This FTD patient who presented with parkinsonism demonstrated frontotemporal hypometabolism in fluorodeoxyglucose positron emission tomography (FDG PET) (A). [11C] Pittsburgh compound-B (PIB) PET showed little PIB retention.

Similar articles

Cited by

References

    1. Pick A. Uber die Beziehungen der senilen Hirnatrophie zur Aphasie. Prag Med Wochenschr. 1892:165–167.
    1. Clinical and neuropathological criteria for frontotemporal dementia. The Lund and Manchester Groups. J Neurol Neurosurg Psychiatry. 1994;57:416–418. - PMC - PubMed
    1. 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;51:1546–1554. - PubMed
    1. Hodges JR, Davies RR, Xuereb JH, Casey B, Broe M, Bak TH, et al. Clinicopathological correlates in frontotemporal dementia. Ann Neurol. 2004;56:399–406. - PubMed
    1. Josephs KA, Petersen RC, Knopman DS, Boeve BF, Whitwell JL, Duffy JR, et al. Clinicopathologic analysis of frontotemporal and corticobasal degenerations and PSP. Neurology. 2006;66:41–48. - PubMed

Publication types

LinkOut - more resources