Behavioral variant frontotemporal dementia with corticobasal degeneration pathology: phenotypic comparison to bvFTD with Pick's disease.

University of California San Francisco, San Francisco, CA, USA.
Journal of Molecular Neuroscience (Impact Factor: 2.76). 09/2011; 45(3):594-608. DOI: 10.1007/s12031-011-9615-2
Source: PubMed

ABSTRACT Patients with corticobasal degeneration (CBD) pathology present with diverse clinical syndromes also associated with other neuropathologies, including corticobasal syndrome, progressive nonfluent aphasia, and an Alzheimer's-type dementia. Some present with behavioral variant frontotemporal dementia (bvFTD), though this subtype still requires more detailed clinical characterization. All patients with CBD pathology and clinical assessment were reviewed (N = 17) and selected if they initially met criteria for bvFTD [bvFTD(CBD), N = 5]. Available bvFTD patients with Pick's [bvFTD(Pick's), N = 5] were selected as controls. Patients were also compared to healthy older controls [N = 53] on neuropsychological and neuroimaging measures. At initial presentation, bvFTD(CBD) showed few neuropsychological or motor differences from bvFTD(Pick's). Neuropsychiatrically, they were predominantly apathetic with less florid social disinhibition and eating disturbances, and were more anxious than bvFTD(Pick's) patients. Voxel-based morphometry revealed similar patterns of predominantly frontal atrophy between bvFTD groups, though overall degree of atrophy was less severe in bvFTD(CBD), who also showed comparative preservation of the frontoinsular rim, with dorsal > ventral frontal atrophy, and sparing of temporal and parietal structures relative to bvFTD(Pick's) patients. Despite a remarkable overlap between the two patient types, bvFTD patients with underlying CBD pathology show subtle clinical features that may distinguish them from patients with Pick's disease neuropathology.

  • [Show abstract] [Hide abstract]
    ABSTRACT: Frontotemporal dementia was documented over a century ago. The last decade, however, has seen substantial changes in our conceptions of this increasingly recognized disorder. Different clinical variants have been delineated, the most common of which is the behavioral variant (bvFTD). Updated diagnostic criteria have been established. New histopathological findings and genetic etiologies have been discovered. Research continues to uncover molecular mechanisms by which abnormal proteins accumulate in degenerating brain tissue. Novel neuroimaging techniques suggest that functional networks are diminished in bvFTD that might be relevant to empathy and social behavior. Despite rapid advances in our understanding of bvFTD, the disease is still under-recognized and commonly misdiagnosed. The result is inappropriate patient care. Recognizing the various presentations of bvFTD and its histological and genetic subtypes might further diagnosis, treatment, and research.
    Biological psychiatry 11/2013; · 8.93 Impact Factor
  • [Show abstract] [Hide abstract]
    ABSTRACT: Abstract Corticobasal syndrome (CBS) and progressive supranuclear palsy syndrome (PSPS) are two of the atypical Parkinsonism syndromes, in that patients exhibit rigidity, occasional tremor and postural instability, but do not symptomatically respond to dopamine replacement. CBS and PSPS can often present with complex cognitive difficulties and neuropsychiatric disturbances. Symptoms of depression, apathy, or agitation can be subtle and are often overlooked as reactions to learning a new diagnosis of Parkinsonism. These symptoms may be the earliest presenting evidence of CBS or PSPS, and these syndromes can be misdiagnosed with a primary psychiatric disorder rather than a neurodegenerative condition. Patients may be inappropriately treated with antipsychotic medications that exacerbate the extra-pyramidal motor features of the syndromes. When symptoms are considered to comprise a neurodegenerative syndrome, it may be an inaccurate diagnosis as many features of CBS and PSPS not only overlap with each other, but also with other dementia syndromes. This review discusses similarities and differences between the syndromes of CBS and PSPS in terms of neuropsychiatric features. Improved characterization of the clinical syndromes is necessary to better predict underlying pathology. Improved education about these diseases would help patients, caregivers and clinicians to anticipate symptom progression and avoid premature nursing home placement.
    International Review of Psychiatry 04/2013; 25(2):197-209. · 1.80 Impact Factor
  • [Show abstract] [Hide abstract]
    ABSTRACT: Neuroimaging has played an important role in the characterization of the frontotemporal dementia (FTD) syndromes, demonstrating neurodegenerative signatures that can aid in the differentiation of FTD from other neurodegenerative disorders. Recent advances have been driven largely by the refinement of the clinical syndromes that underlie FTD, and by the discovery of new genetic and pathological features associated with FTD. Many new imaging techniques and modalities are also now available that allow the assessment of other aspects of brain structure and function, such as diffusion tensor imaging and resting-state functional MRI. Studies have used these recent techniques, as well as traditional volumetric MRI, to provide further insight into disease progression across the many clinical, genetic, and pathological variants of FTD. Importantly, neuroimaging signatures have been identified that will improve the clinician's ability to predict underlying genetic and pathological features, and hence ultimately improve patient diagnosis.
    Current Neurology and Neuroscience Reports 09/2012; · 3.78 Impact Factor


Available from

Suzee E Lee