Neuroscience
Godbolt AK, Josephs KA, Revesz T, Warrington EK, Lantos P, King A, Fox NC, Al Sarraj S, Holton J, Cipolotti L, Khan MN, & Rossor MN. Sporadic and familial dementia with ubiquitin-positive tau-negative inclusions: Clinical features of one histopathological abnormality underlying frontotemporal lobar degeneration. Archives of Neurology. 2005 Jul; 62(7): 1097-1101.
Author Affiliations: Dementia Research Centre.
BACKGROUND: Frontotemporal lobar degeneration comprises a group of diseases with clinical presentations and underlying histopathologies that overlap. Familial disease occurs in up to 50% of frontotemporal lobar degeneration cases. One of several underlying histopathological abnormalities is of ubiquitin-positive tau-negative inclusions, similar to those in motor neuron disease. OBJECTIVE: To compare clinical features of familial and sporadic cases in this pathological subgroup.Design and Patients Case note review of dementia patients with ubiquitin-positive tau-negative inclusion pathological abnormalities proven by autopsy. SETTING: United Kingdom tertiary referral center. MAIN OUTCOME MEASURES: Analysis of clinical features. RESULTS: Eleven familial cases (autosomal dominant) and 18 sporadic cases were identified. Most familial case patients presented with behavioral disturbances similar to those seen in sporadic behavioral cases. Semantic dementia was only seen in sporadic cases. Atypical features occurred in a minority. Sporadic and familial behavioral cases showed no differences in age at onset or disease duration. Neuropsychological test results revealed frontal or temporal deficits in most, but unexpected early parietal deficits in 1. CONCLUSIONS: Behavioral features in familial and sporadic cases were similar, but semantic dementia only occurred in sporadic cases. Diagnostic confusion with Alzheimer disease and corticobasal degeneration occurred in some cases.
PMID: 16009765 [PubMed - in process]
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Anthony H. Risser | neuroscience | neuropsychology | brain
- Polka Music And Semantic Dementia: Not Such A Barrel Of Fun
Semantic dementia is a neurodegenerative disorder in the general class of frontotemporal lobar degeneration. Atrophy occurs bilaterally in the anterior temporal lobes, with the left hemisphere affected to a greater extent (Lambon Ralph & Patterson,...
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Suicidal Behavior in Dementia: A Special Risk in Semantic Dementia. American Journal of Alzheimer's Disease and Other Dementias. 2013 Jul 1; Sabodash V, Mendez MF, Fong S, & Hsiao JJAbstractBackground: Some studies report a low suicide risk in general...
- Neuropsychology Abstract Of The Day: Amnestic Mild Cognitive Impairment (amci)
Outcome of Mild Cognitive Impairment Comparing Early Memory Profiles. American Journal of Geriatric Psychiatry. 2011 Sep 19; Authors: Gómez-Tortosa E, Mahillo-Fernández I, Guerrero R, Montoya J, Alonso A, Sainz MJ Abstract BACKGROUND:: Finding variables...
- Neuropsychology Abstract Of The Day: Frontotemporal Dementia
Wittenberg D, Possin KL, Rascovsky K, Rankin KP, Miller BL, & Kramer JH. The Early Neuropsychological and Behavioral Characteristics of Frontotemporal Dementia. Neuropsychological Review. 2008 Feb 29 [Epub ahead of print] Memory and Aging Center, University...
Neuroscience
Abstract of the Day: Dementia
Godbolt AK, Josephs KA, Revesz T, Warrington EK, Lantos P, King A, Fox NC, Al Sarraj S, Holton J, Cipolotti L, Khan MN, & Rossor MN. Sporadic and familial dementia with ubiquitin-positive tau-negative inclusions: Clinical features of one histopathological abnormality underlying frontotemporal lobar degeneration. Archives of Neurology. 2005 Jul; 62(7): 1097-1101.
Author Affiliations: Dementia Research Centre.
BACKGROUND: Frontotemporal lobar degeneration comprises a group of diseases with clinical presentations and underlying histopathologies that overlap. Familial disease occurs in up to 50% of frontotemporal lobar degeneration cases. One of several underlying histopathological abnormalities is of ubiquitin-positive tau-negative inclusions, similar to those in motor neuron disease. OBJECTIVE: To compare clinical features of familial and sporadic cases in this pathological subgroup.Design and Patients Case note review of dementia patients with ubiquitin-positive tau-negative inclusion pathological abnormalities proven by autopsy. SETTING: United Kingdom tertiary referral center. MAIN OUTCOME MEASURES: Analysis of clinical features. RESULTS: Eleven familial cases (autosomal dominant) and 18 sporadic cases were identified. Most familial case patients presented with behavioral disturbances similar to those seen in sporadic behavioral cases. Semantic dementia was only seen in sporadic cases. Atypical features occurred in a minority. Sporadic and familial behavioral cases showed no differences in age at onset or disease duration. Neuropsychological test results revealed frontal or temporal deficits in most, but unexpected early parietal deficits in 1. CONCLUSIONS: Behavioral features in familial and sporadic cases were similar, but semantic dementia only occurred in sporadic cases. Diagnostic confusion with Alzheimer disease and corticobasal degeneration occurred in some cases.
PMID: 16009765 [PubMed - in process]
-
Anthony H. Risser | neuroscience | neuropsychology | brain
- Polka Music And Semantic Dementia: Not Such A Barrel Of Fun
Semantic dementia is a neurodegenerative disorder in the general class of frontotemporal lobar degeneration. Atrophy occurs bilaterally in the anterior temporal lobes, with the left hemisphere affected to a greater extent (Lambon Ralph & Patterson,...
- Mooc: "the Many Faces Of Dementia"
UCL (University College London) is presenting a four-week MOOC on the FutureLearn platform (no cost) titled "The Many Faces of Dementia". The course begins on 14th March. Course Description Four domains are explored: Familial Alzheimer's Disease,...
- Neuropsychology Abstract Of The Day: Suicidal Behavior And Semantic Dementia
Suicidal Behavior in Dementia: A Special Risk in Semantic Dementia. American Journal of Alzheimer's Disease and Other Dementias. 2013 Jul 1; Sabodash V, Mendez MF, Fong S, & Hsiao JJAbstractBackground: Some studies report a low suicide risk in general...
- Neuropsychology Abstract Of The Day: Amnestic Mild Cognitive Impairment (amci)
Outcome of Mild Cognitive Impairment Comparing Early Memory Profiles. American Journal of Geriatric Psychiatry. 2011 Sep 19; Authors: Gómez-Tortosa E, Mahillo-Fernández I, Guerrero R, Montoya J, Alonso A, Sainz MJ Abstract BACKGROUND:: Finding variables...
- Neuropsychology Abstract Of The Day: Frontotemporal Dementia
Wittenberg D, Possin KL, Rascovsky K, Rankin KP, Miller BL, & Kramer JH. The Early Neuropsychological and Behavioral Characteristics of Frontotemporal Dementia. Neuropsychological Review. 2008 Feb 29 [Epub ahead of print] Memory and Aging Center, University...