Extrapyramidal Syndromes in Frontotemporal Degeneration

  title={Extrapyramidal Syndromes in Frontotemporal Degeneration},
  author={Andrew Kertesz and Paul Mcmonagle and Sarah Jesso},
  journal={Journal of Molecular Neuroscience},
Descriptions of extrapyramidal (EP) involvement in Pick’s disease (renamed recently as FTD) appeared 80 years ago. CBD pathology was confirmed as a common substrate for primary progressive aphasia (PPA). We suggested that CBD and PPA should be included with frontal lobe dementia as Pick complex. PSP was prototype for “subcortical dementia”, and aphasia and apraxia, considered unusual for PSP, are now seen as a rule. The overlap of PSP and CBD is considerable. We recently reviewed our cohort… 
Neuropsychiatry of corticobasal degeneration and progressive supranuclear palsy
Improved characterization of the clinical syndromes is necessary to better predict underlying pathology and improve education about these diseases would help patients, caregivers and clinicians to anticipate symptom progression and avoid premature nursing home placement.
Longitudinal Clinical Changes of Non-Fluent/Agrammatic Primary Progressive Aphasia as Tau Spectrum Disorder: A Case Report
A patient with clinical course of nfvPPA, CBD, and PSP with initial clinical presentation of a progressive speech disorder is reported, which is likely to represent different points of a single disease spectrum.
New Perspective on Parkinsonism in Frontotemporal Lobar Degeneration
There is a need to understand parkinsonism in FTLD in order to obtain a better understanding of the disease, and increased attention is needed on the subject.
Falls in frontotemporal dementia and related syndromes.
In vivo signatures of nonfluent/agrammatic primary progressive aphasia caused by FTLD pathology
Clinical features in sporadic nfVPPA caused by FTLD subtypes relate to neurodegeneration of GM and WM in frontal motor speech and language networks and it is proposed that early WM atrophy in nfvPPA is suggestive of FTLD-tau pathology while early selective GM loss might be indicative of FT LD-TDP.
Prognostic importance of apathy in syndromes associated with frontotemporal lobar degeneration
The relationship between apathy and survival highlights the need to develop more effective and targeted measurement tools to improve its recognition and facilitate treatment and suggests that neurobehavioral features might be useful to predict survival and stratify patients for interventional trials.
Atypical association of semantic dementia, corticobasal syndrome, and 4R tauopathy
Brain autopsy revealed numerous 4R-tau-positive lesions in the frontal, parietal and temporal lobes, basal ganglia, and brainstem of a 57-year-old male diagnosed with semantic dementia with tauopathy and corticobasal syndrome.
Advances in Treatment of Frontotemporal Dementia.
There are no medications that can slow FTD progression, although new therapies for the genetic forms of FTD are moving into clinical trials, and there is some evidence that antidepressants and second-generation antipsychotics may help individual patients.


Corticobasal degeneration presenting with nonfluent primary progressive aphasia: a clinicopathological study
Progressive supranuclear palsy presenting with dynamic aphasia.
Relatively selective involvement of cognitive processes critical for planning and initiating language output may occur in some patients with PSP, which resembles the phenomenon of "verbal adynamia" or "dynamic aphasia" seen in patients with frontal lobe damage.
Corticobasal degeneration and progressive aphasia
Aphasia is common in the corticobasal degeneration syndrome but there was little correlation with the laterality of clinical deficits and there was no correlation between the side of atrophy or motor impairment and the WAB.
Corticobasal degeneration and its relationship to progressive supranuclear palsy and frontotemporal dementia
The term corticobasal syndrome (CBS) is used to characterize the constellation of clinical features initially considered the defining characteristics of corticOBasal degeneration and reserve use of the term corto-CBD (CBD) for the histopathological disorder.
Are frontotemporal lobar degeneration, progressive supranuclear palsy and corticobasal degeneration distinct diseases?
It is argued that FTLD, CBD and PSP should be considered as pathologically similar but distinct syndromes, whereas recent findings challenge traditional nomenclatures by showing etiological overlap.
The Diagnosis and Course of Frontotemporal Dementia
The clinical associations follow the t Tau-negative and tau-positive pathologic dichotomy to some extent, but there is too much overlap to consider the clinical groups or their associations separate diseases.
The neuropsychological pattern of corticobasal degeneration
Patients with CBD show a specific neuropsychological pattern associating a dysexecutive syndrome, likely due to degeneration of the basal ganglia and prefrontal cortex, and asymmetric praxis disorders, which might be related to premotor and parietal lobe lesions.
Corticobasal Syndrome Associated With the A9D Progranulin Mutation
The peculiar findings observed in this patient indicate that the parietal lobe may represent the most vulnerable anatomical area in some of the PGRN-associated frontotemporal lobar degeneration with ubiquitin/TDP-43-positive inclusion cases.
Dementia as the most common presentation of cortical-basal ganglionic degeneration
Dementia was the most common presentation of CBGD in this study and despite the best efforts to define criteria to enhance the specificity of a diagnosis, it is becoming clear that the clinical syndrome that accompanies this disease is quite varied.
Progressive Supranuclear Palsy: A Review of Co-existing Neurodegeneration
  • J. Keith-Rokosh, L. Ang
  • Medicine, Psychology
    Canadian Journal of Neurological Sciences / Journal Canadien des Sciences Neurologiques
  • 2008
This study has demonstrated the frequent co-existence of pathological changes usually noted in other neurodegenerative diseases in PSP, and whether these coexisting pathological changes contribute to the cognitive impairment in PSP remains uncertain.