Extrapyramidal symptoms in Frontotemporal Dementia: Prevalence and clinical correlations

doi:10.1016/j.neulet.2007.05.049

Neuroscience Letters

Volume 422, Issue 1, 5 July 2007, Pages 39-42

https://doi.org/10.1016/j.neulet.2007.05.049 Get rights and content

Abstract

In the present study we aimed at evaluating the prevalence and the associated clinical, neuropsychological and behavioral features of extrapyramidal symptoms in Frontotemporal Dementia (FTD) patients. Seventy-five patients fulfilling clinical criteria of FTD were consecutively enrolled. Each patient underwent clinical and extrapyramidal sign examination, and neuropsychological and behavioral disturbance evaluation. Each patient was submitted to both brain MRI and SPECT, documenting frontotemporal atrophy/hypoperfusion pattern. Parkinsonian symptoms in FTD were associated with a specific endophenotype characterized by higher incidence of psychotic symptoms, memory deficits and psychomotor speed ability abnormalities. Careful description of the spectrum of presentation of FTD may be of help for further understanding the underpinnings of the disease.

Section snippets

Acknowledgements

We thank patients and their families for the time and effort they have dedicated to our research. The authors are indebted to Marina Zanetti for valuable help in the neuropsychological assessment. This work was supported by PRIN Ministry of Research 2005 to BB, and by MURST 60% 2005–2006 to BB and AP.

References (13)

J.L. Cummings et al.
The Neuropsychiatric Inventory: comprehensive assessment of psychopathology in dementia
Neurology
(1994)
S. Fahn et al.
(1987)
J.R. Hodges et al.
Clinicopathological correlates in frontotemporal dementia
Ann. Neurol.
(2004)
A. Kertesz et al.
The Frontal Behavioral Inventory in the differential diagnosis of frontotemporal dementia
JINS
(2000)
A. Kertesz
Pick's complex and FTDP-17
Mov. Disord.
(2003)
M.P. Lawton et al.
Assessment of older people: self-maintaining and instrumental activities of daily living
The Gerontologist
(1969)

There are more references available in the full text version of this article.

Cited by (38)

Cognitive Impairment in Older Adults: Epidemiology, Diagnosis, and Treatment
2022, Psychiatric Clinics of North America
Citation Excerpt :
Behavioral symptoms include social disinhibition, apathy, abulia, loss of empathy, emotional blunting, perseverative, stereotyped or compulsive behavior, hyperorality, and dietary changes.33,55 It is important to especially assess for features of motor neuron disease and/or extrapyramidal deficits, as Parkinsonian features are seen in 25%–80% of FTD cases with the most common symptoms being bradykinesia, parkinsonian gait, and rigidity.56–58 International consensus on diagnostic criteria for bvFTD requires imaging results consistent with frontal and/or anterior temporal atrophy on MRI or CT, or frontal and/or anterior temporal hypoperfusion or hypometabolism on PET or single-photon emission computerized tomography (SPECT) for a diagnosis of probable bvFTD.55
Isolated parkinsonism is an atypical presentation of GRN and C9orf72 gene mutations
2020, Parkinsonism and Related Disorders
A phenotype of isolated parkinsonism mimicking Idiopathic Parkinson's Disease (IPD) is a rare clinical presentation of GRN and C9orf72 mutations, the major genetic causes of frontotemporal dementia (FTD). It still remains controversial if this association is fortuitous or not, and which clinical clues could reliably suggest a genetic FTD etiology in IPD patients. This study aims to describe the clinical characteristics of FTD mutation carriers presenting with IPD phenotype, provide neuropathological evidence of the mutation's causality, and specifically address their “red flags” according to current IPD criteria.
Seven GRN and C9orf72 carriers with isolated parkinsonism at onset, and three patients from the literature were included in this study. To allow better delineation of their phenotype, the presence of supportive, exclusion and “red flag” features from MDS criteria were analyzed for each case.
Amongst the ten patients (5 GRN, 5 C9orf72), seven fulfilled probable IPD criteria during all the disease course, while behavioral/language or motoneuron dysfunctions occurred later in three. Disease duration was longer and dopa-responsiveness was more sustained in C9orf72 than in GRN carriers. Subtle motor features, cognitive/behavioral changes, family history of dementia/ALS were suggestive clues for a genetic diagnosis. Importantly, neuropathological examination in one patient revealed typical TDP-43-inclusions without alpha-synucleinopathy, thus demonstrating the causal link between FTD mutations, TDP-43-pathology and PD phenotype.
We showed that, altogether, family history of early-onset dementia/ALS, the presence of cognitive/behavioral dysfunction and subtle motor characteristics are atypical features frequently present in the parkinsonian presentations of GRN and C9orf72 mutations.
Falls in frontotemporal dementia and related syndromes
2018, Handbook of Clinical Neurology
Citation Excerpt :
For example, in one study 71% of 31 bvFTD patents were reported to have a “parkinsonian” gait or posture, as detected by the United Parkinson's Disease Rating Scale (Diehl-Schmid et al., 2007). In contrast, only 22.7% of 75 bvFTD patients in another study had any feature of parkinsonism, with bradykinesia and rigidity being most common (Padovani et al., 2007), and the prevalence of gait disturbance or falls was not specifically reported. A third study of a clinical cohort reported parkinsonism in 22% of FTD patients (Kertesz et al., 2011), but again the precise rate of falls was not reported.
Frontotemporal dementia (FTD) and related diseases are important causes of younger-onset dementia. Falls may be a source of morbidity and mortality in FTD, but remain underreported, and very few high-quality studies have been performed. In this chapter, we briefly review the clinical features of FTD and related syndromes such as motor neuron disease (MND) and atypical parkinsonian syndromes, such as progressive supranuclear palsy (PSP) and corticobasal syndrome (CBS). Falls are frequently encountered in patients who present with FTD syndromes. Although cognitive impairment is associated with falls generally, motor symptoms and signs, as seen in FTD cases that overlap with atypical parkinsonian disorders such as PSP or CBS, or MND, appear to pose the greatest risk. At present, very few systematic studies have been performed to determine the precise frequency, timing, diagnostic implications, and complications of falls in FTD. Further studies are required to understand the scope of this problem, and to develop effective treatments and management strategies.
Clinical characteristics of parkinsonism in frontotemporal dementia according to subtypes
2017, Journal of the Neurological Sciences
Citation Excerpt :
In bvFTD patients, parkinsonism was observed in 22.7%, and cognitive performances were not affected by the presence of parkinsonism [14]. On the other hand, psychotic features were more prominent and instrumental ADL was more impaired in bvFTD patients with parkinsonism than in those without [14]. In PPA patients, nfvPPA patients showed more parkinsonian features than logopenic PPA patients [15].
We investigated the prevalence of parkinsonism in frontotemporal dementia (FTD) subtypes and the cognitive and behavioral differences between FTD with and without parkinsonism in a well-structured, prospective cohort.
One hundred and ninety-one FTD patients were enrolled and all patients underwent comprehensive neurological evaluations, neuropsychological tests, and the Unified Parkinson's Disease Rating Scale.
The prevalence of parkinsonism was 38.7% (74 patients), and included 33 (46.5%) behavioral variant FTD (bvFTD), 16 (24.2%) semantic dementia (SD), 19 (45.2%) progressive nonfluent aphasia (PNFA), and 6 (50%) FTD associated with motor neuron disease (FTD-MND). SD patients with parkinsonism had higher CDR sum of boxes scores (9.7 ± 4.5 vs 6.2 ± 4.5, p = 0.024), frontal behavioral inventory total score (33.7 ± 20.5 vs 24.3 ± 14.5, p = 0.045), and executive function score of frontal executive dysfunction, disinhibition, and apathy (28.9 ± 13.7 vs 19.2 ± 12.9, p = 0.021) than those without parkinsonism. Seoul Instrumental Activities of Daily Living score (bvFTD: 23.5 ± 11.7 vs 17.3 ± 11.3, p = 0.031, SD: 23.1 ± 11.1 vs 11.3 ± 9.3, p = 0.005) was higher for bvFTD and SD with parkinsonism than for those without parkinsonism.
Parkinsonism is found to be more common in patients with bvFTD, PNFA, and FTD-MND patients than those with SD. Behavioral disturbances were more prominent in SD with parkinsonism than without. Additional studies are needed to determine the pathomechanism and optimal treatment of parkinsonism in different FTD subtypes.
Epidemiology and genetics of frontotemporal dementia: A door-to-door survey in Southern Italy
2012, Neurobiology of Aging
The objectives of this study were to estimate frontotemporal dementia (FTD) prevalence, identify FTD-related mutations, and correlate FTD phenotype with mutations in a Southern Italian population. The study population consisted of subjects ≥ 50 years of age residing in the Community of Biv. on January 1, 2004, and a door-to-door 2-phase design was used. Genetic and biochemical analyses were done on samples collected from 32 patients. Prevalence rates were 0.6 for Alzheimer's disease, 0.4 for vascular dementia (VD), 3.5 for FTD, 0.2 for Parkinson dementia, and 1.2 for unspecified dementia. Three GRN (1 known and 2 novel) mutations with reduced plasma protein levels were found associated to 3 distinct phenotypes (behavioral, affective, and delirious type). We report an unusually high FTD prevalence in the investigated population, but a low prevalence of Alzheimer's disease. We confirm the heterogeneity of FTD phenotype associated with different GRN mutations.
Recent Advances in Frontotemporal Dementia
2023, Canadian Journal of Neurological Sciences

View all citing articles on Scopus

View full text

Extrapyramidal symptoms in Frontotemporal Dementia: Prevalence and clinical correlations

Abstract

Section snippets

Acknowledgements

The Neuropsychiatric Inventory: comprehensive assessment of psychopathology in dementia

Neurology

Clinicopathological correlates in frontotemporal dementia

Ann. Neurol.

The Frontal Behavioral Inventory in the differential diagnosis of frontotemporal dementia

JINS

Pick's complex and FTDP-17

Mov. Disord.

Assessment of older people: self-maintaining and instrumental activities of daily living

The Gerontologist