Current criteria for the clinical diagnosis of pathologically confirmed corticobasal degeneration (CBD) no longer reflect the expanding understanding of this disease and its clinicopathologic ...correlations. An international consortium of behavioral neurology, neuropsychology, and movement disorders specialists developed new criteria based on consensus and a systematic literature review. Clinical diagnoses (early or late) were identified for 267 nonoverlapping pathologically confirmed CBD cases from published reports and brain banks. Combined with consensus, 4 CBD phenotypes emerged: corticobasal syndrome (CBS), frontal behavioral-spatial syndrome (FBS), nonfluent/agrammatic variant of primary progressive aphasia (naPPA), and progressive supranuclear palsy syndrome (PSPS). Clinical features of CBD cases were extracted from descriptions of 209 brain bank and published patients, providing a comprehensive description of CBD and correcting common misconceptions. Clinical CBD phenotypes and features were combined to create 2 sets of criteria: more specific clinical research criteria for probable CBD and broader criteria for possible CBD that are more inclusive but have a higher chance to detect other tau-based pathologies. Probable CBD criteria require insidious onset and gradual progression for at least 1 year, age at onset ≥ 50 years, no similar family history or known tau mutations, and a clinical phenotype of probable CBS or either FBS or naPPA with at least 1 CBS feature. The possible CBD category uses similar criteria but has no restrictions on age or family history, allows tau mutations, permits less rigorous phenotype fulfillment, and includes a PSPS phenotype. Future validation and refinement of the proposed criteria are needed.
Background: Since design and publication of the Western Aphasia Battery (WAB), increasing use to assess patients with aphasia in a clinical and research setting in stroke and in degenerative disease ...of the brain became evident. It has proven to be useful in determining the severity of and nature of the language impairment and providing clues for the location and function of the brain structures affected.
Methods: Articles of WAB use were reviewed from the National Library of Medicine, Cochrane database under several headings of aphasia testing, stroke aphasia, primary progressive aphasia and others.
Results: Available statistic indicated that the WAB is the most used comprehensive aphasia test. The overall severity score and quantitation of the components of the language impairment allows to define and classify aphasia, measure outcome in treatment modalities e.g., standard or constrained therapies, melodic intonation therapy, medications and transcranial stimulation and to study the linguistic features of aphasia and related cognition. Technological and scientific advances in neuroimaging from isotope scans to voxel-based morphometry, functional magnetic resonance and tractography uses the WAB for functional, anatomical and biological correlations of language.
Conclusion: The acceptance of the WAB by researchers as well as clinicians appears to be related to the comprehensive measuring of essential and distinct language functions and practical length allowing it to be administered to a large variety of patients in diverse clinical and research conditions.
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BFBNIB, NUK, PILJ, SAZU, UL, UM, UPUK, VSZLJ
This is a clinicopathologic study of a prospective, clinic-based cohort of patients with frontotemporal dementia (FTD)/Pick complex, who were followed to autopsy. A total of 60 patients with the ...clinical syndromes of the behavioural variant of FTD (FTD-bv) (n = 32), primary progressive aphasia (PPA) (n = 22), corticobasal degeneration syndrome (CBDS) (n = 4) and progressive supranuclear palsy (PSP) (n = 2) at onset, referred to a cognitive neurology clinic who had subsequent post-mortem examination were included. The most common histological variety was motor neurone disease type inclusion (MNDI) (n = 18), followed by corticobasal degeneration (CBD) (n = 12), then Pick's disease (n = 6), dementia lacking distinctive histology (DLDH) (n = 6) and PSP (n = 3). Others fulfilled the histological criteria for Alzheimer's disease combined with glial pathology (n = 6), Alzheimer's disease only (n = 4), Lewy body variant (n = 2), prion disease (n = 1), vascular dementia (n = 1) and undetermined (n = 1). The most common first syndrome among the MNDI and DLDH (tau negative) pathologies was FTD-bv, but subsequently progressive aphasia (PA), occasionally CBDS and semantic dementia also developed. Tau positive histologies of CBD, PSP and Pick bodies were most frequently associated with PPA onset or CBDS/PSP, but behavioural symptoms were also common. Age of onset was earlier in tau negative cases, but the duration of illness and gender distribution were about the same in all histological variants. Although the tau negative and positive histologies are predicted to some extent by the clinical onset, the extent of the overlap and the convergence of the syndromes in the course of the disease argue in favour of maintaining the clinical and pathological varieties under a single umbrella.
Posterior cortical atrophy (PCA) is a progressive dementia characterized by prominent disorders of higher visual processing, affecting both dorsal and ventral streams to cause Balint's syndrome, ...alexia, and visual agnosia.
To define the cognitive profile of PCA and compare to the typical, primary amnestic dementia of the Alzheimer's type (DAT).
The authors used standard cognitive tests and a novel battery designed to reflect dysfunction in both ventral (Object, Face & Color Agnosia Screen OFCAS) and dorsal (complex pictures and compound stimuli) visual streams. The authors identified 19 patients with PCA and compared their performance to a matched group of patients with DAT and normal controls.
Patients with PCA were younger with marked impairment in visuospatial tasks, reading, and writing but relative preservation of memory compared to DAT using standard tests. Dorsal stream signs were most prevalent among the patients with PCA with no pure ventral stream syndromes found. All novel tests distinguished reliably between subjects with complex picture descriptions and processing of compound stimuli showing the most significant differences compared to DAT.
PCA is predominantly a dorsal stream syndrome, distinct from typical DAT, which involves occipitotemporal regions over time.
Cerebral visual impairments have been of great interest to neurologists, ophthalmologists, and neuroscientists. Complicated or partial varieties related to cortical blindness are discussed in this ...review. They are a fascinating alphabet of eponymic clinical syndromes, bordering neurology, ophthalmology, and even psychiatry. Recent functional imaging and experimental studies have contributed further knowledge of cognitive visual organization in addition to the classical lesion evidence.
Although misidentification syndromes (MISs) have been often described in Alzheimer disease (AD), the prevalence of these phenomena in different neurodegenerative diseases has not been systematically ...studied. Three hundred ninety-two individuals with probable AD, 119 patients with the behavioral variety of frontotemporal dementia (FTD-bv), 101 patients with primary progressive aphasia, 24 subjects with semantic dementia, 18 subjects with corticobasal degeneration, 8 patients with progressive supranuclear palsy, 36 individuals with probable Lewy body dementia (DLB), and 26 subjects with Parkinson disease (PD) were the participants of this study. On the basis of a semistructured interview with both patients and their reliable caregivers, MIS was identified in 15.8% of cases with AD, 16.6% of patients with DLB, and in 8.3% of individuals with semantic dementia. The most frequent form of MIS was Capgras delusions, often accompanied by reduplication of place, phantom border phenomenon, or both. Although MIS typically appears in later stages of the disease, it can also occur surprisingly early in patients with AD. None of the patients with FTD-bv, primary progressive aphasia, corticobasal degeneration/supranuclear palsy, or PD developed MIS. Thus, our findings suggest that MISs are characteristic of AD and DLB, and tend to exclude FTD/Pick complex and PD.
Diagnosis and treatment of dementia: 2. Diagnosis Feldman, Howard H., MD; Jacova, Claudia, PhD; Robillard, Alain, MD ...
Canadian Medical Association journal,
03/2008, Volume:
178, Issue:
7
Journal Article
Peer reviewed
Open access
Dementia can now be accurately diagnosed through clinical evaluation, cognitive screening, basic laboratory evaluation and structural imaging. A large number of ancillary techniques are also ...available to aid in diagnosis, but their role in the armamentarium of family physicians remains controversial. In this article, we provide physicians with practical guidance on the diagnosis of dementia based on recommendations from the Third Canadian Consensus Conference on the Diagnosis and Treatment of Dementia, held in March 2006.
We developed evidence-based guidelines using systematic literature searches, with specific criteria for study selection and quality assessment, and a clear and transparent decision-making process. We selected studies published from January 1996 to December 2005 that pertained to key diagnostic issues in dementia. We graded the strength of evidence using the criteria of the Canadian Task Force on Preventive Health Care.
Of the 1591 articles we identified on all aspects of dementia diagnosis, 1095 met our inclusion criteria; 620 were deemed to be of good or fair quality. From a synthesis of the evidence in these studies, we made 32 recommendations related to the diagnosis of dementia. There are clinical criteria for diagnosing most forms of dementia. A standard diagnostic evaluation can be performed by family physicians over multiple visits. It involves a clinical history (from patient and caregiver), a physical examination and brief cognitive testing. A list of core laboratory tests is recommended. Structural imaging with computed tomography or magnetic resonance imaging is recommended in selected cases to rule out treatable causes of dementia or to rule in cerebrovascular disease. There is insufficient evidence to recommend routine functional imaging, measurement of biomarkers or neuropsychologic testing.
The diagnosis of dementia remains clinically integrative based on history, physical examination and brief cognitive testing. A number of core laboratory tests are also recommended. Structural neuroimaging is advised in selected cases. Other diagnostic approaches, including functional neuroimaging, neuropsychological testing and measurement of biomarkers, have shown promise but are not yet recommended for routine use by family physicians.
Language decline is usually the fastest and predominant change in primary progressive aphasia (PPA). In Alzheimer's disease (AD), it is usually associated with global cognitive deficits. Decreased ...speech output, reduced conversational initiation, echolalia, and changes in the pragmatics of conversation are seen in the behavioral variant of frontotemporal dementia (FTD-bv), however, the evolution of language disturbance in FTD-bv patients is rarely examined systematically with a standardized language battery. We aimed to longitudinally track the nature of language change in FTD-bv, PPA, and AD using a standardized measure of language functioning. We also explored the nature of language deficits between semantic dementia (SD) patients and the fluent subgroup of PPA patients. The Western Aphasia Battery was administered to 105 AD, 20 FTD-bv, 54 PPA, and 10 SD patients on 2 occasions with approximately 1 year between assessments. Ninety-nine of these patients were examined an additional year. FTD-bv and PPA patients showed a faster language decline than AD patients. The eventual overlap in language functioning in FTD-bv and PPA suggests that these syndromes belong to the same spectrum of disorders. In conclusion, longitudinal language assessment provides us with a unique understanding of the evolution and progression of language deterioration in various dementias.
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